Diagnostic Evaluation for Autism Spectrum Disorder: a Survey of Health Professionals in Australia

Downloaded from http://bmjopen.bmj.com/ on March 13, 2018 - Published by group.bmj.com

Open Access Research Diagnostic evaluation for autism spectrum disorder: a survey of health professionals in Australia

Lauren J Taylor,1,2 Valsamma Eapen,2,3 Murray T Maybery,1,2 Sue Midford,4 Jessica Paynter,2,5,6 Lyndsay Quarmby,7 Timothy Smith,2,8 Katrina Williams,2,9,10,11 Andrew J O Whitehouse2,12

To cite: Taylor LJ, Eapen V, ABSTRACT et al Strengths and limitations of this study Maybery MT, . Diagnostic Objectives: There is currently no agreed Australian evaluation for autism standard for the diagnosis of autism spectrum disorder ▪ spectrum disorder: a survey This is the first national study to investigate the (ASD) even though there are specific diagnostic of health professionals in diagnostic practices of health professionals from Australia. BMJ Open 2016;6: services available. We suspected inconsistency in the different disciplines across Australia. e012517. doi:10.1136/ diagnostic practices of health professionals in Australia ▪ While the study included clinicians from a range bmjopen-2016-012517 and aimed to assess these practices across the nation of professional backgrounds, there were an by surveying all relevant professional groups. unequal number of respondents from each pro- ▸ Prepublication history and Design: In this study, we completed a survey of 173 fessional group. additional material is health professionals whose clinical practice includes ▪ Respondents were self-selected and may be available. To view please visit participating in the diagnostic process for ASD in committed to high standards of clinical practice. the journal (http://dx.doi.org/ Australia. Participants completed an online 10.1136/bmjopen-2016- questionnaire which included questions about their ‘ ’ 012517). diagnostic setting, diagnostic practice and diagnostic individual. The gold standard in ASD diag- outcomes in 2014–2015. nosis is a best estimate clinical diagnosis Participants: Participants covered a range of which is determined in accordance with Received 10 May 2016 disciplines including paediatrics, psychiatry, current diagnostic classiﬁcation systems and Revised 11 August 2016 psychology, speech pathology and occupational following rigorous assessment practices. Accepted 16 August 2016 therapy. All states and territories of Australia were There is general consensus that a rigorous represented. diagnostic assessment for ASD would consist Setting: Participants came from a range of service of a physical examination, hearing test, child settings which included hospitals, non-governmental observation and parent interview which organisations, publicly funded diagnostic services and includes a full developmental history (NICE, private practice. 2011). ‘Best-practice’ ASD assessments are Results: There was variability in diagnostic practices more comprehensive, and also comprise for ASD in Australia. While some clinicians work within standardised developmental or cognitive a multidisciplinary assessment team, others practice independently and rarely collaborate with other testing, language assessment and information clinicians to make a diagnostic decision. Only half of from more than one setting, ideally from a the respondents reported that they include a source other than the parent or carer who 1–5 standardised objective assessment tool such as the has been interviewed. Rigorous assess- Autism Diagnostic Observation Schedule in ASD ments enhance the accuracy of diagnoses assessments, and one-third indicated that they do not and provide information about an indivi- include measures of development, cognition and dual’s strengths and difﬁculties which is language in assessments where ASD is suspected. important for intervention planning. Conclusions: Reported practice of some professionals Despite the internationally recognised best in Australia may not be consistent with international practice guidelines for ASD diagnosis,3 and best practice guidelines for ASD diagnosis. These position statements from professional findings highlight the need for a minimum national – bodies6 8 Australia does not have a national standard for ASD diagnosis throughout Australia that ensures best practice regardless of the type of setting in standard for ASD diagnosis. In addition, For numbered affiliations see which the service is provided. health services are governed on a state or ter- end of article. ritory basis, rather than on a national level. While in some jurisdictions, eligibility for Correspondence to In the absence of biomarkers, diagnosis of publicly funded intervention for children Dr Lauren Taylor; autism spectrum disorder (ASD) is based on with ASD requires a diagnosis made by a [email protected] the behavioural presentation of an multidisciplinary team comprising a medical

Taylor LJ, et al. BMJ Open 2016;6:e012517. doi:10.1136/bmjopen-2016-012517 1 Downloaded from http://bmjopen.bmj.com/ on March 13, 2018 - Published by group.bmj.com

Open Access professional, psychologist and speech pathologist, other METHOD states have less stringent criteria, requiring only a paedia- Participants trician or a psychiatrist. Furthermore, eligibility for We aimed to include responses from a range of profes- funding from the Helping Children with Autism sional groups, working across different clinical and geo- funding package is determined following a conclusive graphical locations, to reflect the diversity of diagnostic ASD diagnosis which can be provided by a single paedia- assessment practice in Australia. Recruitment took a trician or a psychiatrist.9 multifaceted approach: (1) The Cooperative Research Identifying ASD accurately and early is essential given Centre for Living with Autism (Autism CRC) website that state and federal funding for early intervention is and social media platforms were used to advertise the based on a formal diagnosis made before a child turns research. The Autism CRC is a national cooperative 7 years.9 An inaccurate diagnosis may mean that chil- research effort focused on ASD across the lifespan which dren are not eligible for early intervention which con- includes universities, government agencies, service provi- tains elements that specifically focus on areas of ders, Autism awareness groups and professional bodies; difficulty in ASD.10 Alternatively, children who are mis- (2) relevant member bodies and professional organisa- diagnosed with ASD may access services that are not tions, including Speech Pathology Australia, the relevant or effective for their areas of difficulty thereby National Rural Health Alliance, the Western Australian wasting resources. In addition, it is well established that Autism Diagnostician’s Forum, the Neurodevelopmental early intervention improves outcomes for children with and Behavioural Paediatric Society of Australasia and ASD11 12 and that children who start intervention at a the Autism South Australia diagnostician’s membership young age make more improvements than children who list, distributed information about the survey via mailing – start at an older age.13 15 While ASD can be reliably lists, newsletters and social media platforms; (3) psychol- – diagnosed at two years,16 18 the average age of diagnosis ogists with contact details listed on the Australian in Australia is over 4 years (49 months), with ASD most Psychological Society ASD Provider list were individually frequently diagnosed at 71 months19 Variability in the emailed and invited to participate in the research; (4) age of diagnosis has been observed between Australian government-funded diagnostic services in each state states, with significantly earlier age of diagnosis in were identified and the survey was either emailed or Western Australia and New South Wales relative to all mailed to each service, along with a personalised letter other states and territories. The variation in age at diag- inviting the service to complete the survey and (5) the nosis may be associated with inconsistent diagnostic researchers used individual networks to distribute the practices across the nation. survey to colleagues. All states and territories,i as well as While three previous studies have investigated diagnos- all disciplines included in multidisciplinary assessments tic practices for ASD in Australia, this research has for ASD were represented (see table 1). – included only paediatricians,20 24 psychiatrists20 23 24 and There was a similar number of responses from the psychologists.24 Skellern et al22 examined the assessment public/non-government (n=66, 38%) and private (n=76, practices of paediatricians (N=79) and child psychiatrists 43%) sectors, with 31 (19%) respondents working in the (N=26) in Queensland, finding considerable variability in public and private sectors.ii Public services and non- the diagnostic practices of these clinicians.2 The results government organisations (NGOs) were grouped when of a more recent survey has shown similar findings. In a participants provided background information. study of 124 paediatricians across Australia, Randall et al20 Respondents who indicated that they worked in the found that only a minority of participating clinicians public sector or NGO (n=38) were asked to specify usually included information from cognitive/develop- whether this was a hospital (n=27), government (n=21) mental assessments, or involved other disciplines in the or NGO (n=17). There were only eight completed diagnostic process. These practices are inconsistent with responses obtained for health professionals working in current clinical guidelines for ASD diagnosis.47The lack NGOs, so the public/NGO grouping was retained of consistent standards in Australia likely results in differ- throughout the data analysis. ent diagnostic protocols and variability in the quality and A similar proportion of the respondents worked as accuracy of ASD diagnoses. sole practitioners (n=49; 37%) and within a multidiscip- To date, no national research has been conducted to linary team (n=52; 39%), with 31 (23%) respondents fol- investigate diagnostic practices for ASD across all health lowing ‘other’ models of practice, working as a sole professions and throughout Australia. We aimed to include a sample of clinicians that covered a range of disciplines, states, geographic locations and service settings. i In this study, we compared diagnostic practices for ASD There are six Australian states, New South Wales (NSW), South Australia (SA), Victoria (Vic), Queensland (Qld), Tasmania (Tas) and across the Australian states and between clinicians who Western Australia (WA), and two territories, the Australian Capital are experienced in the assessment and diagnosis of ASD. Territory (ACT) and the Northern Territory (NT). ii Given that there is no national standard for ASD diagno- Participants who reported that they had part-time employment in the public and the private sectors were asked to indicate which setting was sis, it was hypothesised that there would be variability in their primary setting, that is, where they spend more than 50% of their diagnostic practices across states and between clinicians. time. All responses were then based on the primary practice setting.

2 Taylor LJ, et al. BMJ Open 2016;6:e012517. doi:10.1136/bmjopen-2016-012517 Downloaded from http://bmjopen.bmj.com/ on March 13, 2018 - Published by group.bmj.com

Open Access

Table 1 Number of respondents from each state or territory of Australia, categorised by profession Australian state/territory ACT NSW NT Qld SA Tas Vic WA Total General practice ––––––1 – 1 Paediatrics – 714835432 Psychiatry – 21––––14 Psychology 2 13 – 9 14 6 14 17 75 Speech pathology – 3 3 2 15 4 11 8 46 Occupational therapy – 1 – 22– 4 – 9 Other* – 2 – 1111– 4 Total 2 28 5 18 40 14 36 30 173 *Comprising one manager (TAS), researcher (QLD), dual paediatrics/psychiatry (VIC), autism consultant/researcher (SA) and not specified (NSW). ACT, Austrlian Captial Territory; NT, Northern Territory; NSW, New South Wales; QLD, Queensland; SA, South Australia; Tas, Tasmania; Vic, Victoria; WA, Western Australia. practitioner and within a multidisciplinary team (MDT). the percentage of assessments in which they collaborate While there was no significant difference in the propor- with other professionals. A copy of the questionnaire is tion of practitioners working in MDTs or as sole practi- available from the Autism CRC on request. tioners across states, χ2 (5, N=101)=10.16, p=0.07, Φ=0.32, there was a higher proportion of MDT practi- Statistical analysis tioners in the public (69%), relative to the private The survey was designed so that participants were only (35%) sector, χ2 (1, N=101)=12.21, p<0.001, Φ=0.35. required to respond questions that were directly relevant Conversely, a higher proportion of sole practitioners to their practice. This resulted in different numbers of came from the private (69%) relative to the public responses for different questions. Data were analysed (31%) sector. based on the number of responses recorded for each Participants had been involved in ASD diagnosis for a question. All data were screened for normality prior to median of 9.5 years (SD=7.50 years, range 1–30 years). analysis. Non-parametric tests (Kruskal-Wallis and Seventy-six participants (85%) had completed training Mann-Whitney U) were used where the data were not in ASD assessment. Training consisted of either under- normally distributed. Otherwise, continuous data were graduate or postgraduate education, supervision, case analysed using analysis of variance (ANOVA), with post discussions or participation in courses for the adminis- hoc (Scheffe’ test) comparisons used to follow-up signifi- tration of the Autism Diagnostic Observation cant omnibus ANOVA results. Categorical data were ana- Schedule,25 Autism Diagnostic Interview-Revised26 or lysed using the chi-square test. Developmental, Dimensional and Diagnostic Interview.27 RESULTS Procedure Response and completion rates Participants completed an anonymous 141-item ques- Two-hundred and forty-five potential participants tionnaire that was presented via Qualtrics (Qualtrics, opened the survey, with 173 of these individuals pro- Utah, USA), an online survey platform. The survey was ceeding beyond the first question. Complete responses initially piloted by eight clinicians and researchers with were obtained for 99 of the respondents who started the expertise in ASD and revised based on feedback from questionnaire, resulting in a completion rate of 58%. this group prior to administration. The survey contained questions that fell into three categories: (1) diagnostic Diagnostic processes service, (2) diagnostic practice and (3) diagnostic out- Wait-list periods: The wait-list period for an ASD assess- comes (see online supplementary appendix A for more ment was significantly longer in public/NGO relative to information on the types of questions included in the private settings (see table 2). While most of the private survey). diagnostic services (n=52, 88%) start ASD assessments Background information, such as discipline, years of within 3 months of referral, wait times in the public professional experience and type of training the sector were variable, with only 23 of the 50 respondents respondent had participated in was also gathered. (46%) reporting wait-list times of 3 months or less (see Questions were presented in a categorical format (Yes/ figure 1). The wait for an MDT assessment (median=12 No), or on a seven-point Likert scale which ranged from weeks, SD=25.75, range=1.5–108 weeks) was also longer 1 (Never) to 7 (Always). Some questions, such as the than for a sole practitioner (median=4 weeks, SD=9.84, number of assessments completed in the past range=1–52), Mann–Whitney U=511, p=0.005, perhaps 12 months, required a numerical response. Respondents due to the higher number of MDTs in the public sector also provided some percentage responses, for example, relative to the private sector.

Taylor LJ, et al. BMJ Open 2016;6:e012517. doi:10.1136/bmjopen-2016-012517 3 Downloaded from http://bmjopen.bmj.com/ on March 13, 2018 - Published by group.bmj.com

Open Access

Table 2 Median (SD) and range for the wait-list period, number of assessment sessions and length of assessment sessions for diagnosticians in the private and public sectors Private Public U p Value Wait-list period (in weeks) Median (SD) 4 (15.5) 16 (20) 581 <0.001 Range 1–52 2–108 Assessment sessions Median (SD) 2 (1.3) 2 (1.3) 1072 0.056 Range 0–6.5 1–6.5 Assessment length (in minutes) Median (SD) 90 (88.6) 120 (108.8) 1012 0.03 – – Range 30 420 40 600 Figure 2 Proportion of sole practitioners and MDTs who conduct ASD assessments in isolation, together, partially together, in series or in collaboration with professionals from Assessment sessions and length: Respondents (n=106) another discipline. ASD, autism spectrum disorder; MDT, completed a median of 2 (range=1–6.5) sessions for multidisciplinary team. ASD assessments with a median assessment length of – 90 min (SD=98.15 min, range 30 600 min). There was Multisetting assessment: The majority of the 108 no difference in the number of assessment sessions com- respondents (95%) observed the individual in the clinic pleted in the private relative to the public sector (see in all assessments (median frequency=100% of assess- table 2). However, assessment sessions in the public ments, SD=31%, range=0–100%). Assessments in the ﬁ sector were signi cantly longer than those in the private home or school/daycare settings were less frequent, with sector. 47% of respondents including home observations Multidisciplinary assessment: MDTs (n=52) most com- (median frequency=0% of assessments, SD=22%, monly consisted of one medical and two allied health range=0–100%) and 77% of respondents including professionals, usually a psychologist and a speech path- observations in the school or daycare (median fre- ologist. All of the participating occupational therapists quency=20% of assessments, SD=31%, range=0–100%). were part of an MDT. Most MDTs conducted assessments Only two clinicians (2%), both from the public sector, either in series, together, partially together or in collab- include clinic and school/daycare observations in more oration, that is, each clinician completes an independ- than 75% of the assessments, with 12 clinicians (11%), 4 ent assessment, but all assessors meet to make a private practitioners and 8 from the public sector, com- ﬁ consensus diagnostic decision (see gure 2). In contrast, pleting observations in the clinic and home settings. sole practitioners (n=49) tended to complete assess- Three respondents (3%), 2 from the private sector and ments in isolation or in series, that is, assess an individ- one from the public sector, observed a child in the ual independently one after the other. Only small home and school/daycare settings in more than 75% of numbers of the sole practitioners reported collaborating the assessments. with other clinicians, with 1 of the 15 (7%) sole practitioners working in isolation and 4 of those working in Diagnostic practices series (17%) collaborating with external agencies. Hearing test: In the Australian clinical pathway, it would be expected that the paediatrician conducts a hearing assessment before referring a child to an allied health team for an ASD assessment.7 Of the paediatricians (n=20) surveyed, only 2 (10%) reported that a hearing test is included in all ASD assessments. Another 2 paediatricians (10%) indicated that they rarely include a hearing test in ASD assessments, 5 reported occasionally or usually (ie, in 30–50% of assessments) and 11 paediatricians (55%) reported to include a hearing test frequently or usually (ie, 70–90% of assessments) where ASD is suspected. Medical investigations: All of the paediatricians who include medical investigations in ASD assessments reported completing a genetic screen, Fragile X test and Figure 1 Total number of public and private services which neurological and physical examinations. Of the 21 pae- have wait-list periods of <3 months to more than 12 months. diatricians, 15 respondents (71%), reported that they

4 Taylor LJ, et al. BMJ Open 2016;6:e012517. doi:10.1136/bmjopen-2016-012517 Downloaded from http://bmjopen.bmj.com/ on March 13, 2018 - Published by group.bmj.com

Open Access

Table 3 Proportion of respondents (N=105) from each discipline who administer developmental, cognitive, language, adaptive, ASD or psychometric assessments Developmental (%) Cognitive (%) Language (%) Adaptive (%) ASD (%) Other (%) Paediatrics (N=21) 23.81 28.57 28.57 19.05 57.14 0 Psychiatry (N=3) 0 0 0 33.30 66.67 0 Psychology (N=51) 35.29 70.59 29.41 72.55 90.20 39 Speech pathology (N=25) 20.00 20.00 92.00 24.00 64.00 12 Occupational Therapy (N=3) 33.33 33.33 33.33 66.67 66.67 0 Other (N=2) 50 0 0 50 100 0 Respondents were given a list of assessments that fitted within each category, but were also able to specify other measures that they administer. Example assessments within each category are described at the foot of the table. Developmental assessments: Griffiths, Bayley, Mullen. Cognitive assessments: WPPSI, WISC, WAIS, Leiter, UNIT, WNV. Language assessments: CELF, CASL, CCC-2, CC-A, PLS. Adaptive assessments: VABS, ABAS, ABS, Scales of Independent Behaviour. ASD assessments: ADOS, ADI, 3Di, DISCO, CARS, M-CHAT, SCQ, ASSQ, ASRS, CAST, SRS. Other assessments: Connor’s, CBCL, SDQ, BRIEF, Depression, Anxiety. ASD, autism spectrum disorder.

include medical investigations frequently or usually adaptive skills and ASD symptoms, and 14 (13%) com- (more than 70% of their assessments), with only 4 pedia- pleting a cognitive assessment in addition to these other tricians (19%) including medical investigations in all measures. Taking the profession-speciﬁc assessments sep- assessments where ASD is suspected. arately showed that 27 psychologists (53%) administered Assessment measures: A total of 107 participants cognitive and adaptive assessments in addition to mea- responded to questions regarding the administration of sures of ASD symptomatology, and 14 speech pathologists assessment tools. A developmental history was reported (56%) administered language assessments in addition to to have been undertaken by 89% of these respondents. measures of ASD symptomatology. Of these, 66 (62%) reported always administering stan- To account for clinicians who may review the results of dardised assessments in diagnostic evaluations for ASD, assessments that are administered by other disciplines, and 21 (20%) reported doing so frequently or usually we also asked participants to indicate whether they (in 70–90% of ASD assessments). There was no differ- review assessment results. Sixty-seven (68%) respondents ence in the proportion of respondents who frequently reported that they review assessment results. There were administer assessments in private (n=51, 88%) relative to no differences in the proportion of respondents from public (n=36, 77%) service settings, χ2 (1, N=87)=2.35, each profession, χ2 (4, N=98)=6.29, p=0.18, Φ=0.18, or p=0.13, Φ=0.15, nor were there differences in the pro- state; χ2 (5, N=98)=2.31, p=0.80, Φ=0.15 who review the portion of respondents who administer assessments by results of assessments. Finally, we investigated whether state, χ2 (5, N=105)=2.95, p=0.71, Φ=.17. respondents who do not regularly administer assess- Participants who reported administering assessments ments (ie, in <30% of assessments), review assessment (n=105) were asked to indicate which types of assess- results instead. Six of the 17 respondents (35%) who do ments are included in diagnostic evaluations for ASD. not regularly administer assessments always review assess- Since some measures are restricted to particular disci- ment results. A further 3 respondents (18%) who do not plines, it was unsurprising that there was variability in regularly administer standardised assessments often the proportions of clinicians administering cognitive, review these results of assessments that have been admi- language and adaptive assessments and measures of nistered in other settings. ASD symptomatology (see table 3). In addition, only 50 Two respondents, both sole practitioners, reported respondents (47%) administer the ADOS and 41 (39%) that they do not administer standardised assessments in the ADI-R. Thirty-two respondents (30%) use the ADOS diagnostic evaluations for ASD. These respondents and the ADI-R together in diagnostic evaluations for reported that they do not administer standardised mea- ASD. sures because they are not part of everyday practice, or Of the 105 respondents who indicated that they admin- because they have already been administered at another ister assessments as part of diagnostic evaluations for service. In addition, one respondent reported that the ASD, only 8 (8%) reported that they complete an assess- standardised measures are not required because diag- ment battery comprising measures of developmental, nostic decision-making is outlined in the Diagnostic and cognitive, language, adaptive skills and ASD symptomatol- Statistical Manual Fifth Edition. ogy. The numbers increased only marginally when we Assessment of co-occurring neurodevelopmental or examined developmental and cognitive assessments sep- mental health conditions: Only 23 of the 105 respon- arately, with 11 respondents (10%) completing a develop- dents (22%) who administer standardised assessments mental assessment in addition to measures of language, include additional measures of behaviour or

Taylor LJ, et al. BMJ Open 2016;6:e012517. doi:10.1136/bmjopen-2016-012517 5 Downloaded from http://bmjopen.bmj.com/ on March 13, 2018 - Published by group.bmj.com

Open Access psychopathology. The majority of these clinicians were observations of the individual across settings is prohibi- psychologists (see table 3). tive for practitioners. Facing uncertainty in diagnosis: Forty-seven of the 97 A small proportion of respondents (17%) reported (48%) participants who responded to this question that they have diagnosed ASD when a person does not reported that they make provisional ASD diagnoses when meet the criteria for the disorder. This was more likely to faced with diagnostic uncertainty. There was no differ- happen in the private sector than in the public sector. ence in the proportion of provisional diagnoses between The reasons for making this diagnosis included the the private and public settings, χ2 (1, N=97)=2.53, p=0.11, assessment did not adequately show the actual diagnostic Φ=0.16, or across the states, χ2 (7, N=97)=12.8, p=0.08, status or to ensure that the person could access early Φ=0.36. Of the respondents who make provisional diag- intervention, support at school or access other disability noses, 34 (72%) reported that this label is rarely used, services. These findings are consistent with previous with the remaining 5 (11%) and 2 (4%) reporting that research that has investigated the diagnostic practices of provisional diagnoses are given occasionally and some- clinicians in Queensland, the USA and the UK.21 23 28 times respectively. Provisional diagnoses were reportedly The results of these previous studies have indicated that given when individuals demonstrated subthreshold symp- up to 55% of participating health professionals had toms, were very young or would benefit from interven- increased the level of diagnosis or severity, most com- tion. Thirty-nine respondents (83%) reported that they monly because of a lack of diagnostic certainty, but also reassess individuals with provisional diagnoses within a to ensure that the individual could access funding, educa- mean of 13 months (SD=7.96 months). tional support or other services. The intentional ascrip- From 94 respondents, 16 (17%) reported that they tion of an inaccurate diagnosis to facilitate access to have diagnosed ASD when the person did not meet full services may contribute to the increased diagnostic rates criteria for the condition. Fifteen of these respondents over time. Indeed, Nassar et al29 found that the increased (88%) reported that this has rarely occurred (<10% of incidence of ASDs in WA was related to changes in diag- their assessments), with the remaining respondents indi- nostic practices and service provision in this state. These cating that it has been occasional (about 30% of assess- findings highlight the complexity of ASD diagnosis and ments). While there was no significant difference in the the challenges associated with funding being designated proportion of overdiagnosis coming from each state, χ2 according to diagnosis rather than need. (7, N=94)=2.41, p=0.79, Φ=0.16, a significantly higher While the sample size in this study was small, the proportion of respondents who made a diagnosis when response rate is consistent with previous research indicat- the individual did not meet criteria came from the ing that the average response rate for surveys is 55.6.30 private (81%) relative to the public (19%) sector, χ2 (1, The number of completed responses in our survey is N=94)=4.50, p=0.035, Φ=0.22. When asked about the also consistent with similar surveys conducted in the UK reasons for making the ASD diagnosis, 14 (88%) clini- and Australia.24 28 In particular, Ward et al24 included cians reported that they thought that the individual did 104 psychologists, psychiatrists and paediatricians. While have ASD, but that the assessment did not reveal the the number of responses in this study was similar to individual’s actual diagnostic status. Other commonly Ward et al, our survey includes a wider range of disci- reported reasons for the diagnosis were to ensure that plines and covers a greater number of Australian states the child could access early intervention (n=6), school and territories. Furthermore, our results are consistent support (n=5) or disability services (n=3). with several smaller surveys of Australian paediatricians and psychiatrists, which reported variations in assessment and diagnostic practices between clinicians and DISCUSSION across professional groups.22 23 This study investigated the diagnostic practices for ASD Although our sample had representatives from all across Australia. The results show inconsistencies in ASD states and relevant professions, the numbers were not assessment practices across the states, and between the consistent across categories. In addition, respondents in private and public service settings. In addition, some this study were self-selected, perhaps because of a strong clinicians in Australia may not be practising in a manner interest in good diagnostic standards, so the results may that is consistent with international best practice guide- not represent the broader population of diagnosticians lines4 or statements from Australian professional bodies7 in Australia. Notwithstanding this, the findings suggest for ASD assessment and diagnosis. the need for the development of national standards in The current findings are consistent with the results ASD diagnostic practices and the subsequent evaluation reported by Randall et al,20 who found that only a minor- of these standards. ity of Australian paediatricians follow current recommendations for diagnosing ASD. Comprehensive diagnostic assessments that comprise all of the recommended ele- CONCLUSION ments are lengthy and expensive, so it is possible that This research is the first study to investigate diagnostic prac- the time required to administer standardised assess- tices for a range of professionals across Australia. We found ments of ASD symptomatology in addition to conducting that a number of health professionals are conducting

6 Taylor LJ, et al. BMJ Open 2016;6:e012517. doi:10.1136/bmjopen-2016-012517 Downloaded from http://bmjopen.bmj.com/ on March 13, 2018 - Published by group.bmj.com

Open Access assessments that are inconsistent with best practice guide- commercially, and license their derivative works on different terms, provided lines. This may contribute to poor quality assessments and the original work is properly cited and the use is non-commercial. See: http:// creativecommons.org/licenses/by-nc/4.0/ impact estimates of ASD prevalence in Australia. Assessments that do not comply with best practice guidelines may also lead to delayed diagnosis and access to intervention services. Conversely, assessments that more closely REFERENCES 1. Anagnostou E, Zwaigenbaum L, Szatmari P, et al. Autism spectrum resemble current clinical guidelines for ASD diagnosis may disorder: advances in evidence-based practice. Can Med J be costly and associated with longer wait-list periods. 2014;186:509–19. 2. Baird G, Cass H, Slonims V. Diagnosis of autism. BMJ Therefore, there is a need to balance the quality and cost 2003;327:488–93. of ASD assessments to ensure that ASD diagnoses are con- 3. Filipek PA, Accardo PJ, Baranek GT, et al. The screening and ducted in a thorough but efficient manner. In addition, diagnosis of autism spectrum disorders. J Autism Dev Disord 2009;29:439–84. findings regarding the possible overdiagnosis of ASD have 4. NICE. Autism in under 19s: recognition, referral and diagnosis. 2011. implications for service delivery, particularly when funding https://www.nice.org.uk/guidance/cg128 (accessed 28 Nov 2015). 5. Ozonoff S, Goodlin-Jones BL, Solomon M. Evidence-based and service provision is determined based on diagnosis. assessment of autism spectrum disorders in children and These findings have implications for future policy, particu- adolescents. J Clin Child Adolesc 2005;34:523–40. larly with regard to establishing a national standard in ASD 6. Australian Advisory Board on Autism Spectrum Disorders. Position paper on the diagnosis and assessment of Autism Spectrum diagnosis, to ensure an equitable process for families and Disorders in Australia. http://www.autismadvisoryboard.org.au/ individuals with ASD. uploads/Position%20Paper%20on%20Diagnosis%20July%202011% 20Final.pdf (accessed 28 Jan 2016). 7. Silove N, Blackmore R, Warren A, et al. A consensus approach for Author affiliations the paediatrician’s role in the diagnosis and assessment of autism 1School of Psychology, The University of Western Australia, Crawley, Western spectrum disorders in Australia. https://www.racp.edu.au/docs/ Australia, Australia default-source/advocacy-library/pa-pol-a-consensus-approach-for- 2 the-paediatricians-role-in-the-diagnosis-and-assessment-of-autism. Cooperative Research Centre for Living with Autism (Autism CRC), Brisbane, pdf (accessed 28 Jan 2016). Queensland, Australia 8. Glasson EJ, MacDermott S, Dixon G, et al. Management of 3University of New South Wales, New South Wales, Australia assessments and diagnoses for children with autism spectrum 4Western Australian Autism Diagnostician’s Forum, Western Australia, disorders: the Western Australian model. Med J Aust 2008;188: Australia 288–91. 5Research Department, AEIOU Foundation, Queensland, Australia 9. Australian Government. Disability and Carers: Helping Children with 6 Autism (HCWA) Eligibility. https://www.dss.gov.au/our- School of Applied Psychology, Griffith University, Gold Coast, Queensland, responsibilities/disability-and-carers/program-services/for-people- Australia with-disability/is-my-child-eligible (accessed 12 Nov 2015). 7Centre for Rural Health, University of Tasmania, Australia 10. Prior M, Roberts JMA, Rodger S, et al. A review of the research to 8Disability Services Commission, Western Australia, Australia identify the most effective models of practice in early intervention for 9Department of Paediatrics, University of Melbourne, Melbourne, Victoria, children with autism spectrum disorders. Australian Government Department of Families, Housing, Community Services and Australia Indigenous Affairs, Australia, 2011. https://www.dss.gov.au/our- 10 ’ Developmental Medicine, Royal Children s Hospital, Melbourne, Victoria, responsibilities/disability-and-carers/program-services/for-people- Australia with-disability/early-intervention-for-children-with-autism-spectrum- 11Murdoch Children’s Research Institute, Melbourne, Victoria, Australia disorders-guidelines-for-good-practice-2012 (accessed 28 Jan 2016). 12Telethon Kids’ Institute, The University of Western Australia, Australia 11. Magiati I, Tay XW, Howlin P. Early comprehensive behaviourally based interventions for children with autism spectrum disorders: a summary of findings from recent reviews and meta-analyses. Acknowledgements The authors also acknowledge the health professionals Neuropsychiatry 2012;2:543–70. who dedicated their time to participate in this research. 12. Reichow B. Overview of meta-analyses on early intensive behavioral intervention for young children with autism spectrum disorders. Contributors LJT designed and administered the survey for health J Autism Dev Disord 2012;42:512–20. professionals, was involved in data analysis and interpretation and wrote and 13. Fenske EC, Zalenski S, Krantz PJ, et al. Age at intervention reviewed the manuscript. VE, MTM, SM, JP, LQ, TS, KW and AJOW were and treatment outcome for autistic children in a comprehensive involved in the study design, including survey design and these authors also intervention program. Anal Intervent Dev Disabil 1985;5:49–58. contributed to the preparation of the manuscript. All authors contributed to 14. Granpeesheh D, Dixon DR, Tarbox J, et al. The effects of age and treatment intensity on behavioural intervention outcomes for children participant recruitment. with autism spectrum disorders. Res Autism Spectr Disord Funding The authors acknowledge the financial support of the Cooperative 2009;3:1014–22. Research Centre for Living with Autism (Autism CRC), established and 15. Rogers SJ, Estes A, Lord C, et al. Effects of a brief Early Start ’ Denver Model (ESDM)-based parent intervention on toddlers at risk supported under the Australian Government s Cooperative Research Centres for autism spectrum disorders: a randomized controlled trial. JAm Programme and the Department of Social Services. Financial support was Acad Child Adolesc Psychiatry 2012;51:1052–65. also obtained from the Commonwealth Department of Social Services. 16. Cox A, Klein K, Charman T, et al. Autism spectrum disorders at 20 and 42 months of age: stability of clinical and ADI-R diagnosis. Competing interests None declared. J Child Psychol Psyc 1999;40:719–32. 17. Moore V, Goodson S. How well does early diagnosis of autism stand Ethics approval This study was approved by the Ethics Committee of the the test of time? Autism 2003;7:47–63. University of Western Australia (Ref RA/4/1/6997). 18. Eaves LC, Ho HH. The very early identification of autism: Provenance and peer review Not commissioned; externally peer reviewed. outcome to age 4½ -5. J Autism Dev Disord 2004;34: 367–78. Data sharing statement No additional data are available. 19. Bent CA, Dissanayake C, Barbaro J. Mapping the diagnosis of autism spectrum disorders in children aged under 7 years in Australia, 2010-2012. Med J Aust 2015;202:317–20. Open Access This is an Open Access article distributed in accordance with 20. Randall M, Albien-Urious N, Brignell A, et al. Diagnosing autism: the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, an Australian paediatric research network survey. J Paediatr Child which permits others to distribute, remix, adapt, build upon this work non- Health 2016;52:11–17.

Taylor LJ, et al. BMJ Open 2016;6:e012517. doi:10.1136/bmjopen-2016-012517 7 Downloaded from http://bmjopen.bmj.com/ on March 13, 2018 - Published by group.bmj.com

Open Access

21. Rushton JL, Felt BT, Roberts MW. Coding of pediatric 26. Rutter M, LeCouter A, Lord C. Autism diagnostic interview, behavioral and mental disorders. Pediatrics 2002; revised (ADI-R). Torrance, CA: Western Psychological Services, 2003. 110:1–6. 27. Skuse D, Warrington R, Bishop D, et al. The developmental, 22. Skellern C, McDowell M, Schluter P. Diagnosis of autistic spectrum dimensional and diagnostic interview (3di): a novel computerised disorders in Queensland: variations in practice. J Paediatr Child H assessment for autism spectrum disorders. J Am Acad Child 2005;41:413–18. Adolesc Psychiatry 2004;43: 548–58. 23. Skellern C, Schluter P, McDowell M. From complexity to category: 28. Rogers CL, Goddard L, Hill EL, et al. Experiences of diagnosing responding to diagnostic uncertainties of autistic spectrum disorders. autism spectrum disorder: a survey of professionals in the United J Paediatr Child Health 2005;41:407–12. Kingdom. Autism. Published Online First: 16 Dec 2015. doi:10.1177/ 24. Ward SL, Sullivan KA, Gilmore L. Practitioner perceptions of the 1362361315611109 assessment and diagnosis of Autism in Australia. Aust Psychol 29. Nassar N, Dixon G, Bourke J, et al. Autism spectrum disorders in 2016;51:272–9. young children: effect of changes in diagnostic practices. Int 25. Lord C, Rutter M, DiLavore P, et al. Autism diagnostic observation J Epidemiol 2009;38:1245–54. schedule, second edition (ADOS-2). Torrance, CA: Western 30. Baruch Y. Response rate in academic studies—a comparative Psychological Services, 2012. analysis. Hum Relat 1999;52:421–38.

8 Taylor LJ, et al. BMJ Open 2016;6:e012517. doi:10.1136/bmjopen-2016-012517 Downloaded from http://bmjopen.bmj.com/ on March 13, 2018 - Published by group.bmj.com

Diagnostic evaluation for autism spectrum disorder: a survey of health professionals in Australia Lauren J Taylor, Valsamma Eapen, Murray T Maybery, Sue Midford, Jessica Paynter, Lyndsay Quarmby, Timothy Smith, Katrina Williams and Andrew J O Whitehouse

BMJ Open2016 6: doi: 10.1136/bmjopen-2016-012517

Updated information and services can be found at: http://bmjopen.bmj.com/content/6/9/e012517

These include: References This article cites 22 articles, 3 of which you can access for free at: http://bmjopen.bmj.com/content/6/9/e012517#ref-list-1 Open Access This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ Email alerting Receive free email alerts when new articles cite this article. Sign up in the service box at the top right corner of the online article.

Topic Articles on similar topics can be found in the following collections Collections Health policy (694) Paediatrics (684)

Notes

To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions

To order reprints go to: http://journals.bmj.com/cgi/reprintform

To subscribe to BMJ go to: http://group.bmj.com/subscribe/ Braz J Otorhinolaryngol. 2011;77(5):670-4. REVIEW ARTICLE BJORL.org Plasticity of the auditory system: theoretical considerations

Vanessa Kappel1, Ana Clara de Paula Moreno2, Ceres Helena Buss3

Keywords: Abstract hearing, hearing loss, uditory plasticity refers to the possibility of anatomical and/or functional changes in the system neuronal plasticity. A where transmission of auditory information takes place. The auditory system is often required in communication; it is important to learn how the auditory system reacts to stimuli in order to improve performance in individual communication of subjects with impaired hearing.

Aim: To review the literature on auditory plasticity and the possibility and ability of plastic responses in the auditory system; also to review the evidence of auditory plasticity.

Methodology: A review of the Brazilian and international literature (journals, books, and graduate studies) was carried out. The MEDLINE, SCIELO, BIREME, PUBMED, and LILACS data bases were consulted, as well as 24 papers from the 1990s to the present date; each paper was assessed for relevance to the topic.

Conclusion: The findings showed that the auditory system is able to reorganize itself if there is variation, whether by by reducing, increasing, or conditioning of sound stimuli. This is evidence of plasticity in the auditory system.

1 Speech therapist. 2 Speech therapist. 3 Doctoral degree, speech therapist, adjunct professor in the Speech Therapy Department, Santa Maria Federal University (Universidade Federal de Santa Maria). Paper submitted to the BJORL-SGP (Publishing Management System – Brazilian Journal of Otorhinolaryngology) on December 10, 2009; and accepted on January 31, 2010. cod. 6833

Brazilian Journal of Otorhinolaryngology 77 (5) September/October 2011 http://www.bjorl.org / e-mail: [email protected] 670

77(5)-Ingles.indb 670 03/10/2011 09:18:39 INTRODUCTION to change. James wrote: “Plasticity [...] means the possession of a structure weak enough to yield to an influence, Neuroplasticity is the ability of the central nervous but strong enough not to yield all at once. Each relatively system to adapt to different stimuli - it is a current object stable phase of equilibrium in such a structure is marked of study. The ability that the auditory system has to change by what we may call a new set of habits. Organic matter, 1 anatomically or functionally is named auditory plasticity . especially nervous tissue, seems endowed with a very Hearing loss decreases the ability to perceive extraordinary degree of plasticity of this sort; so that we sounds, which causes several limitations in individual, may without hesitation lay down as our first proposition 2,3 social, and emotional development . the following, that the phenomena of habit in living beings Several studies have investigated the ability of the are due to the plasticity of the organic materials of which auditory sensory system to respond with plasticity to dif- their bodies are composed” (p. 68)1. 4 ferent types of injury . According to Pascual-Leone et al.1, plasticity in the Scientific data on the evidences of plasticity in the brain may be defined as a property of the nervous system auditory system were reviewed because of the importance to adapt, to change its structural and functional organiza- of hearing, the number of individuals in all age groups tion. These authors also stated that brain plasticity is an being diagnosed with hearing loss, and the relevance of intrinsic property of the nervous system that makes it this topic. possible for structure to change in response to experience A review of the Brazilian and international literature and changes in the environment. (journals, books, and theses) was made. The following Behavior and experience equate with the activity databases were consulted: MEDLINE, SCIELO, BIREME, of all neurons in the brain. These consist of neuronal PUBMED, and LILACS. The key words were: “audition, networks that are an efficient, spatially compact, and pre- hearing loss, neuronal plasticity”. As the review progresses, cise medium for processing input signals and generating important references cited in the databases above were responses8. also consulted. After reading and analysis, this review Kaas9 has stated that there are ordered neuronal comprised 24 published papers from the 1990s to the connections in sensorial systems in several levels of the current date, taking into account the relevance of each brain that link with peripheral neural receptors and form document for the study topic. sensory maps. Neuronal activity in a specific site of the somatosensory cortex is evoked by, for instance, stimulat- Hearing ing a finger. These maps are altered when the surface of a The hearing system consists of auditory sensory peripheral receptor changes. If input is removed - such as organs, nervous system auditory pathways, and brain when a limb is amputated - its somatotropic representation structures that receive, analyze, and interpret sound area in the sensory or motor cortex is not silenced; other 5 information - it is used in communication contexts . It sensorial receptors will stimulate central neurons that previ- is through language that Man is able to understand the ously responded to the damaged receptors. Thus, plasticity world, organize his universe, make abstractions and con- or reorganization of the sensory map has taken place. vey thoughts and feelings, understand others, interact with Some authors1 have argued that plasticity is always the milieu, and acquire knowledge. Thus, the more sound active. Therefore, it is a mistake to see plasticity as a stimuli we receive, the more prepared are we to interact property of the brain that is only active following injury 2,3 with other individuals . to promote functional recovery or compensate functional However, the auditory system is not always whole. loss. So, plasticity occurs whenever we undergo new The impact of hearing loss is significant; it affects not only experiences. After brain injury, behavior remains a con- the ability to understand information contained in sound sequence of whole brain, and thus a consequence of a but also the way in which an individual may related with plastic nervous system. the environment and culture. Additionally, hearing loss In the past it was thought that plasticity did not take 2,6,7 may have biological, psychological, and social effects . place in older individuals, and that it was limited in adult nervous systems. However, adult brains have been shown Neuroplasticity to be able to change and adapt to circumstances. Plasticity “Plastic” derives from the Greek “plastikós”, which is expected to be less efficient with age; however, plastic means molded. According to the Oxford English Diction- mechanisms do not end at this or that age, and also take 1 ary, plasticity is the ability to undergo a change in shape . place in the elderly. Thus, it is incorrect to set limits to The first author to introduce the term plasticity in neurosci- age groups before beginning treatment10-12. ence was William James (in The Principles of Psychology, During the 1950s, it was stated that language learn- 1890), when referring to the human behavior’s inclination ing was only possible until the age 10 to 12 years; in other

Brazilian Journal of Otorhinolaryngology 77 (5) September/October 2011 http://www.bjorl.org / e-mail: [email protected] 671

77(5)-Ingles.indb 671 03/10/2011 09:18:39 words, plasticity was generally associated with critical win- inadequate, as an abnormal number of neurons are ex- dows. This was based on the idea that the nervous system cited by specific stimuli, which change the natural neural had a limited time to develop, and that certain abilities did coding. The sound coding ability is also altered by two not develop beyond a specific growth period in children. other conditions: the system does not realize that several These ideas are currently being questioned10,13. neurons are now responding to the wrong frequencies; and it makes appropriate new associations between stimuli Plasticity of the Auditory System and neural responses. Musiek et al.14 have defined plasticity of the audi- Interaction plasticity (excitatory or inhibitory) that is tory system as change by nervous cell improvement due evoked by binaural stimuli should also impact perception. to environmental influences. This is particularly relevant for deprivation associated with The hearing system is not fully developed at birth; monaural use of hearing aids. An example is the weakened it matures slowly after the inner ear starts to function. ability of unaided ears - and the strengthened ability of Sensory neuron plasticity is fundamental at the beginning stimulated ears - to evoke central responses. This would of intrauterine and neonatal life, to consolidate hearing4,15. lead to unbalanced neural responses where the aided Sensory system plasticity occurs in both peripheral ear would be supported by binaural neurons at the cost receptor neurosensory tissues and central pathways4. of the less stimulated ear. With time, this could result in Plasticity of the auditory system changes the physiologi- deprivation. Thus, the term deprivation would not be the cal, biochemical, and/or anatomical properties of central most correct word; an association between plasticity and neurons in response to demands for transmitting acoustic deprivation would cause hearing loss in unaided ears19,20. information - it is also a biodynamic phenomenon. The Some authors have given much value to early fit- auditory system reorganizes itself after any variation in ting of hearing aids and balanced stimulation of ears, and auditory input because stimulus input may be decreased have suggested that symmetrical bilateral hearing loss with (cochlear injury) or new (postnatal development and when monaural hearing aid adaptation may become asymmetric cochlear implants or hearing aids are placed)16. because of unequal stimulation2,21. Mecklenburg & Babighian13 assessed the results of a longitudinal study at the Medical School of Hannover of Secondary Plasticity adult patients with deafness lasting over 20 years in whom Studies on the effects of deprivation and auditory cochlear implants were placed. With regards to auditory stimulation on hearing perception have shown conclu- plasticity, the authors concluded that age-related neural sively that the development and function of the auditory stability may change, and that plasticity in the auditory system is related with the quantity and quality of auditory nervous center appears to become slower in mature ner- input2. Rodrigues & Miranda11 have shown that adequate vous systems, although it persists throughout life. stimuli need to be offered to the nervous system so that Musiek & Berge17 argue that one of the types of plasticity mechanisms may be set in motion. plasticity is developmental. It is present when immature If the central auditory system is able to reorganize brains start to process sensory information, and remains itself following injury, the question is whether reintroduc- until adult life. A recent study18 has suggested that the tion of auditory stimuli by hearing aids or cochlear implants human auditory system also has developmental plasticity has the potential of provoking auditory plasticity. in the brainstem, and not only the cortex. This discovery Studies on plasticity have suggested that increased has been interpreted in different stimuli and experience auditory stimulation because of amplification may induce contexts. secondary plasticity, which facilitates acclimation19-21. Changes in the anatomical or physiological prop- Munro & Lutman22 have suggested that acclimation erties of the central auditory system may be induced by is the period that follows adaptation to hearing aids; au- sensorineural hearing loss (primary plasticity), by rein- ditory abilities and speech recognition gradually improve troducing auditory stimuli (secondary plasticity), or by because of new cues that become available to users. conditioning2,19. If beneficial primary plasticity occurs in an individual, sound amplification may have a negative effect, at Primary Plasticity least initially, due to distortion between acoustic stimuli According to Willot19, hearing loss induces primary and auditory responses that were initially used advanta- plasticity; however, it is not clear how it affects human geously by the hearing system. Frequencies that used to hearing. More neurons focus on the frequencies that are go unheard start to be heard again because of hearing still heard when frequency maps are reorganized. This al- aids, and the result is a competition for the old neurons. lows the auditory system to continue responding to sound, Thus, neural coding of sounds may raise new problems19. which benefits hearing. Nevertheless, the responses remain Changes may occur in the organization of the

Brazilian Journal of Otorhinolaryngology 77 (5) September/October 2011 http://www.bjorl.org / e-mail: [email protected] 672

77(5)-Ingles.indb 672 03/10/2011 09:18:39 central auditory system between the onset of hearing before fitting hearing aids, 4 weeks after, and 16/18 weeks loss and the moment when hearing aids are used. These after fitting hearing aids. The authors found that speech should be fitted before the auditory system reorganizes recognition values increased in line with the duration of and the opportunity for improving hearing passes. Sound hearing aid use; this improvement may have been due to amplification increases auditory stimuli, which may induce brain plasticity, but speech recognition score differences secondary plasticity, thereby contributing to aclimation3. were not statistically significant. These authors also related The true purpose of hearing aids should be to use and compared the speech recognition scores among 8 stimulus reintroduction to induce secondary plasticity, subjects who chose monaural fitting, and compared their returning representation maps to the due places. Hearing mean values with those of hearing aid fitted and non-fitted aids could have two effects if plasticity were used as a ears at the three points in the study. The results were not clinical tool: to restore sensitivity, and to change the central statistically significant, but the findings suggested that with nervous system19. time speech recognition performance improved in aided Mecklenburg & Babighian13 have suggested that ears and worsened in non-aided ears. This suggested the auditory plasticity is important for the treatment of deaf existence of acclimation of aided ears and sensory depri- individuals of all ages in which cochlear implants are vation in non-aided ears. The mean speech recognition placed, as plasticity is related with the ability of the ner- scores in right and left ears at the three points of the study vous system to adapt to artificial stimuli. These findings were compared in 8 subjects that chose binaural fitting. demonstrate the capacity of the central nervous system The results were not statistically significant, but the mean to adapt to new auditory sensations following variable speech recognition scores in both ears increased with periods of deprivation. amplification, except for the mean speech recognition Boéchat2 demonstrated plasticity in the auditory score of the right ear, in which a decrease was seen after nervous system with physiologic evidence in a study that 4 weeks of amplification. The authors21 pointed out that evaluated the effect of deprivation time and stimulation although the results were not statistically significant, they time on auditory sensitivity variations for pure tones and concur with the findings of Boéchat2. the speech recognition score. The study sample comprised 72 subjects aged from 10 to 86 years and diagnosed with Conditioning unilateral or bilateral asymmetric sensorineural hearing Some authors19,23 have noted that conditioning is loss. Forty-three of these individuals were fitted with hear- also a form of neuroplasticity, and that is fundamental for ing aids in the worse ear, and 29 did not have hearing training auditory abilities. Conditioning is able to change aids in the worse ear. Audiologic testing was done (initial, the frequency maps and to generate new connections intermediary and final). Variations in sensitivity to pure in response to demands. When correlated with learning, tones and speech recognition scores were studied among repetition fosters an increase in the number of synapses the groups for up to 6 years relative to the stimulation in related neuronal circuits. time, duration of deprivation, asymmetry between ears, Studies have shown that hearing improved after and level of hearing loss. The author found that hearing aid auditory training. Ambient influences - such as auditory users had less tone threshold variations and better speech training - stimulated neural structures involved with the recognition scores, and concluded that there was plasticity performance of trained hearing abilities, thereby benefit- due to auditory deprivation in non-users of hearing aids in ing individuals. Neural plasticity, therefore, is essential for the worse ear, and secondary plasticity following auditory improvements gained in auditory training17,24,25. stimulation in hearing aid users. Furthermore, hearing aid users appeared to gain more within the first two years of CONCLUSION introduction of auditory stimuli, while the negative effects of deprivation presented gradually and homogeneously Professionals involved in habilitation and/or re- with time. The author concluded that the quantity and habilitation of patients with hearing loss need to under- quality of stimuli developed and maintained the central stand plasticity in the auditory system, and consider it an auditory system, and also underlined the importance of important clinical tool. Thus, the possibility of primary, binaural auditory stimulation. secondary, and/or conditioned plasticity should be taken Amorim & Almeida21, on auditory plasticity, stud- into account in clinical practice. Future studies will be very ied 16 subjects aged from 17 to 89 years with bilateral helpful to add knowledge about plasticity in the auditory symmetrical sensorineural or mixed moderate to severe system. Our review of the literature showed that the audi- hearing loss. In one of the steps of this study, 24 ears tory system is able to reorganize itself when the input of were analyzed by comparing the mean values of speech auditory stimuli increases or decreases. So, subjects with recognition scores in each ear at three points in the study: hearing loss who require hearing aids or cochlear implants

Brazilian Journal of Otorhinolaryngology 77 (5) September/October 2011 http://www.bjorl.org / e-mail: [email protected] 673

77(5)-Ingles.indb 673 03/10/2011 09:18:39 may benefit in their social, emotional, and intellectual 13. Mecklenburg D, Babighian G. Cochlear performance as an indicator aspects, and enjoy an improved quality of life. of auditory plasticity in humans. In: Salvi RJ, Henderson D, Fiorino F, Colletti V, editors. Auditory system plasticity and regeneration. New York: Thieme Medical; 1996. p. 395-404. REFERENCES 14. Musiek F, Shinn J, Hare C. Plasticity, auditory training, and auditory processing disorders. Seminars in hearing. 2002;23(4):263-75. 1. Pascual-Leone A, Amedi A, Fregni F, Merabet LB. The plastic human 15. Hernández-Muela S, Mulas F, Mattos L. Plasticidad neuronal functio- brain cortex. Annu Rev Neurosci. 2005;28:377-401. nal. Rev Neurol. 2004;38(1):58-68. 2. Boéchat EM. Plasticidade do sistema auditivo quanto à sensibilidade 16. Knobel KAB, Sanchez TG. Privação Auditiva, Circuitos Inibitórios auditiva para tons puros e respostas para fala na deficiência auditiva e Plasticidade: Implicações na Compreensão do Zumbido e da neurossensorial [tese]. São Paulo: Faculdade de Medicina da Univer- Hiperacusia, Estado de São Paulo. Arquivos Internacionais de Otor- sidade de São Paulo; 2003. rinolaringologia [Internet]. 2005 Out-Dez [citado 2008 Jun 17];9(4) 3. Silva LPA, Queiroz F, Lima I. Fatores etiológicos da deficiência auditiva Disponível em: http://www.arquivosdeorl.org.br/conteudo/acer- em crianças e adolescentes de um centro de referência APADA em vo_port.asp?id=341. Salvador-BA. Braz J Otorhinolaryngol. 2006;72(1):33-6. 17. Musiek FE, Berge B. A neuroscience view of auditory training/ 4. Féres MCLC, Cairasco NG. Plasticidade do sistema auditivo. Rev Bras stimulation and central auditory processing disorders. In: Masters Otorrinolaringol. 2001;65(5):716-20. M, Stecker N, Katz J. Central auditory processing disorders: mostly 5. Momensohn-Santos TM, Dias AMN, Valente CHB, Assayag FM. Anato- management. Boston: Allyn & Bacon; 1998. p. 15-32. mia e fisiologia do órgão da audição e do equilíbrio. In: Momensohn- 18. Johnson KL, Nicol T, Zecker SG, Kraus N. Developmental plasticity Santos TM, Russo ICP, orgs. Prática da Audiologia Clínica. 6ª ed. São in the human auditory brainstem. J Neurosci. 2008;28(15):4000-7. Paulo: Cortez; 2007. p. 12-44. 19. Willot JF. Physiological plasticity in the auditory system and its 6. Silman S, Iório MCM, Mizhahi MM, Parra VM. Próteses auditivas: possible relevance to hearing aid use, deprivation effects and accli- um estudo sobre seu benefício na qualidade de vida de indivídu- matization. Ear hear. 1996;7(3 Suppl):66S-77S. os portadores de perda auditiva neurossensorial. Disturb Comun. 20. Tremblay K. Central auditory plasticity: implications for auditory 2004;16(2):153-65. rehabilitation. J Speech Hear Disord. 2003;56(1):10-5. 7. Veiga LR, Merlo ARC, Mengue SS. Satisfação com a prótese auditiva 21. Amorim RMC, Almeida K. Estudo do benefício e da aclimatização em na vida diária em usuários do sistema de saúde do Exército. Braz J novos usuários de próteses auditivas. Pro Fono. 2007;19(1):39-48. Otorhinolaryngol. 2005;71(1):67-73. 22. Munro KJ, Lutman ME. The effect of speech presentation level on 8. Laughlin SB, Sejnowski TJ. Communication in neuronal networks. measurement of auditory acclimatization to amplified speech. J Acoust Science. 2003;301:1870-3. Soc Am. 2003;114(1):484-95. 9. Kaas JH. Plasticity of sensory and motor maps in adult mammals. 23. Gielow I. Escutação: treinamento auditivo para vida. São Paulo: Thot Annu Rev Neurosci. 1991;14:137-67. cognição e linguagem; 2008. Habilidades Auditivas; p. 22-5. 10. Irvine DR, Fallon JB, Kamke MR. Plasticity in the adult central auditory 24. Santos JN, Couto ICP, Amorim RMC. Treinamento auditivo em ofici- system. Acoust Aust. 2006;34(1):13-7. nas: opção terapêutica grupal. Pro Fono. 2006;18(3):293-302. 11. Rodrigues MFA, Miranda SM. A estimulação da criança especial em 25. Zalcman TE, Schochat E. A eficácia do treinamento auditivo formal casa: entenda o que acontece no sistema nervoso da criança deficiente em indivíduos com transtorno de processamento auditivo. Rev Soc e como você pode atuar sobre ele. O que é plasticidade neuronal. Bras Fonoaudiol. 2007;12(4):310-4. São Paulo: Atheneu; 2005. p. 53-60. 12. Brasil Neto J. Neurofisiologia e plasticidade no córtex cerebral pela estimulação magnética transcraniana repetitiva. Rev Psiquiatr Clín. 2004;31(5):216-20.

Brazilian Journal of Otorhinolaryngology 77 (5) September/October 2011 http://www.bjorl.org / e-mail: [email protected] 674

77(5)-Ingles.indb 674 03/10/2011 09:18:39 International Journal of Audiology 2015; Early Online: 1–9

Original paper

Autonomous motivation is associated with hearing aid adoption

Jason Ridgway* , Louise Hickson* & Christopher Lind* *

* School of Health and Rehabilitation Sciences, The University of Queensland, Brisbane, Australia * * Speech Pathology and Audiology, Flinders University, Adelaide, Australia

Abstract Objective: To use the self-determination theory of motivation to investigate whether different forms of motivation were associated with adults ’ decisions whether or not to adopt hearing aids. Design: A quantitative approach was used in this cohort study. Participants completed the treatment self-regulation questionnaire (TSRQ), which measured autonomous and controlled motivation for hearing aid adoption. Sociodemographic data and audiometric information were also obtained. Study sample: Participants were 253 adults who had sought information about their hearing but had not consulted with a hearing professional. Participants were categorized as hearing aid adopters if they had been fi tted with hearing aids 4– 6 months after completing the TSRQ, and as non-adopters if they had not. Results: Multivariate logistic regression was used to examine associations between autonomous and controlled motivation, sociodemographic and audiometric variables, and hearing aid adoption (n ϭ 160). Three factors were signifi cantly associated with increased hearing aid adoption when the infl uence of other variables was accounted for: autonomous motivation, perceived hearing diffi culty, and poorer hearing. Con- trolled motivation was not found to infl uence hearing aid adoption. Conclusion: These empirical fi ndings that link autonomous motivation to decisions of hearing help-seekers have implications for the ways practitioners may evaluate motivation and could inform discussions with clients about hearing aid adoption. For personal use only. Key Words: Hearing impairment; motivation; hearing aids; hearing aid adoption; autonomy; self-determination theory

The majority of adults with measurable hearing impairment do not from people’ s active and ongoing interpretation of their immediate seek help for their hearing (Hartley et al, 2010). Among people who environmental and sociocultural experiences (Deci & Ryan, 2000). do seek help, a choice often faced is whether or not to adopt hearing As these experiences vary considerably, most cognitive theories aids, and a variety of audiological and non-audiological factors of motivation emphasize the effects experiences may have on the contribute to the choice made (see reviews by Knudsen et al, 2010; initiation and maintenance of purposeful behaviour (Ryan & Deci, Meyer & Hickson, 2012). Amongst non-audiological factors, client 2008). This is especially important to researchers seeking to focus Int J Audiol Downloaded from informahealthcare.com by 110.33.191.149 on 03/13/15 motivation has emerged as an important factor associated with deci- on the practical application of these theories in health, educational, sions about hearing aid adoption (Laplante-L é vesque et al, 2013). or vocational settings (Deci & Ryan, 2000). Motivation is an internal process that initiates, controls, and In health care, motivation is regarded as central to client engage- sustains activity. With this broad defi nition in mind, motivation ment in treatment, and is a signifi cant contributor to the success researchers have sought to understand the circumstances and char- or failure of treatment across many health domains (Ryan et al, acteristics that affect a person ’ s motivation. Historical studies of 2008). To increase engagement by clients identifi ed as unmotivated motivation have explored instincts (James, 1890), drives and needs in clinical settings, models of health care such as the health belief (Maslow, 1943), external reinforcement (Skinner, 1953), cognition model (Rosenstock, 1966), the transtheoretical model (Prochaska & (Bandura, 1977), and neuropsychology (Berridge, 2004). This range Di Clemente, 1992), or social cognitive theory (Bandura, 1977) of perspectives highlights the importance of both internal mecha- have often been used (Nieuwenhuijsen et al, 2006). Such models nisms and environmental factors to motivation. Most theories of commonly characterize behaviour change as a process arising from motivation concerned with human behaviour take the cognitive per- internalization, whereby client attitude is transformed from a lack spective, which is underpinned by the notion that motivation stems of awareness of, or resistance to, the need for treatment, through to

Correspondence: Jason Ridgway, School of Health and Rehabilitation Sciences, The University of Queensland, St. Lucia, Brisbane, Queensland, 4072, Australia. E-mail: jason. [email protected]

(Received April 5, 2014; accepted January 9, 2015 ) ISSN 1499-2027 print/ISSN 1708-8186 online © 2015 British Society of Audiology, International Society of Audiology, and Nordic Audiological Society DOI: 10.3109/14992027.2015.1007213 2 J. Ridgway et al.

hearing aid adoption decisions. An examination of various features Abbreviations of motivation in a sample of hearing help-seekers would therefore be 4FAHL 4-frequency average hearing loss of considerable value, to better understand (1) the characteristics of AUC Area under curve motivation in this population, and (2) the relationship of motivation KMO Kaiser-Meyer-Olkin to clinical engagement via hearing aid adoption. OR Odds ratio Health research that has applied SDT to investigate relationships PCA Principal components analysis between SDT constructs and specifi c health variables has typically ROC Receiver operating characteristic taken a quantitative approach using self-report measures (Ng et al, SDT Self-determination theory 2012). One self-report measure that assesses different forms of moti- TSRQ Treatment self-regulation questionnaire vation and the role of autonomy in decision-making is the treatment VIF Variance infl ation factor self-regulation questionnaire (TSRQ; Williams et al, 1996). The WANT Wishes and needs tool self-regulation questionnaire was originally designed for use with children in education research (Ryan & Connell, 1989), and was modifi ed for health research in a study that explored autonomous an adoption of need and a subsequent action to address this need and controlled motivational predictors for participation in a weight (Prochaska & Di Clemente, 1992). Although the majority of health loss management program (Williams et al, 1996). The TSRQ has behaviour change models structure the steps involved to achieve since been adapted for studies that investigated medication adher- internalization, and focus on the clinical approaches used to achieve ence (Williams et al, 1998b), tobacco abstinence (Williams et al, this (Nigg et al, 2002), they do not routinely distinguish between 2011), dental brushing and fl ossing (Mü nster Halvari et al, 2012), internal and external motivating factors, nor do they identify ante- and a range of other health disciplines (Ng et al, 2012). Therefore, cedents of motivational characteristics (Nieuwenhuijsen et al, 2006). evidence from other areas of health care suggests that an adapted The self-determination theory of motivation (SDT; Deci & Ryan, TSRQ may help to identify patterns of motivation among hearing 1985) is a theory that classifi es different dimensions of motivation help-seekers. This may then enable relationships between different (i.e. internal and external forms of motivation) along a continuum forms of motivation and decisions such as hearing aid adoption to of internalization (Ryan & Connell, 1989), and emphasizes the probe explored. If motivation is found to predict hearing aid adoption, cesses through which a person internalizes health behaviours (Ryan differentiating autonomous and controlled forms of motivation may et al, 2008). be useful in a clinical context to help guide discussions about pos- The internalization continuum referred to in SDT is a systematic sible hearing aid adoption with clients. representation of the relative autonomy felt towards a behaviour With this in mind, the aim of this study was to investigate the change action. At one end of the continuum is more internalized, or association between motivational factors and the decision to adopt autonomous, motivation. These forms of motivation are also referred hearing aids or not. The TSRQ was used to analyse patterns of moti- to as ‘ integrated motivation’ , a motivation that stems from integra- vation in a sample of adults seeking help for their hearing for the For personal use only. tion of action with personal beliefs, or ‘ identifi ed motivation ’ , which fi rst time. Associations between sociodemographic and audiometric relates to identifying the value of an action (Deci & Ryan, 2000). factors and hearing aid adoption were also explored. The other end of the continuum represents less internalized, or controlled, motivation. These forms of motivation are also referred to as Method ‘ introjected motivation ’ , which is motivation that originates from an internal sense of guilt or obligation, or ‘ external motivation ’ , which Participants stems from external pressure (Deci & Ryan, 2000). ‘ Amotivation ’ , Participants were recruited from amongst people who had sought which is characterized by lack of willingness to participate, is the information about their hearing but had not yet attended for a fi rst least internalized form of motivation. Self-determination theory also consultation with a hearing professional. Potential participants were

Int J Audiol Downloaded from informahealthcare.com by 110.33.191.149 on 03/13/15 argues that successful internalization of new health-related attitudes approached if: (1) they attended promotional activities such as a requires conditions that encourage autonomy (afﬁ rmation of per- hearing screening or information session about hearing services run sonal decisions and beliefs), competence (conﬁ dence and ability to by a hearing service provider; (2) they made contact with a hearing master an activity), and relatedness (a sense of connectedness and service provider for an appointment directly; and/or (3) they made trust). Further discussion about SDT, the internalization continuum contact with a hearing service provider for an appointment, after and its potential applicability to hearing rehabilitation is found in submitting an application to the Australian Government Hearing Ser- Ridgway et al (2013). vices Program1 via their doctor. A total of 3347 people were mailed, The past decade has seen audiological literature shift from describ- emailed, or given the study questionnaires, a consent form for paring motivation as reason for referral (self-motivated to attend, or ticipation, and an audiogram release authorization by administration motivated by others; Wilson & Stevens, 2003) or problem awareness staff of the hearing services provider. A total of 291 responses were (recognition there is a problem and seeking a solution; Kochkin, received, for an 8.7% response rate (see Figure 1 for an overview 2007), to describing motivation as an internalization process within of the study procedure). Thirty-eight responses from people with the behaviour change construct (Saunders et al, 2012; Laplante- previous hearing aid experience were excluded from the study, leav- Lé vesque et al, 2013). Using the transtheoretical model, Laplante- ing a sample size of 253. Ninety-one percent of participants were L é vesque et al (2013) proposed that adults ’ orientation to the action recruited from one large hearing service provider with clinics across stage of change was a predictor of hearing aid adoption or commu- all states and territories of Australia, and the remaining 9% of par- nication program uptake. Although this latter research supports the ticipants were recruited from a number of smaller service providers clinical application of behaviour change models to understand inter- located in various states and in rural and metropolitan areas. The nalization, little is yet known about different aspects of motivation sociodemographic and audiometric characteristics of participants are in a population of hearing help-seekers, or the role of autonomy in shown in Table 1. Autonomous motivation and hearing aid adoption 3

Figure 1. Flow diagram showing an overview of the study procedure. Numbers in parentheses represent the numbers of potential or actual participants at the various stages of the study.

Measures Australian Government Hearing Services Program to assist prac- T REATMENT SELF-REGULATION QUESTIONNAIRE titioners assess motivation for hearing aid adoption in clients with The TSRQ was used to assess motivation for hearing aid adoption. In minimal or mild hearing losses. The two questions were originally this study, we adapted the TSRQ from the Health Care SDT package (http://www.selfdeterminationtheory.org/questionnaires) by replac- Table 1. Sociodemographic and audiometric characteristics of For personal use only. ing words associated with medical treatment such as medication , participants. diabetes, or glucose with the words hearing , hearing aids, or com- Characteristic Full sample (N ϭ 253) munication where appropriate. The scale consisted of 19 items that represented possible reasons for considering hearing aid adoption. Age in years, mean (SD) 69.9 (10.5) Participants were presented with the item ‘ I am thinking about get- Source of referral, n (%) ting hearing aids because …’ and were then asked to rate how true Self 148 (60.2) each item was to them on a 7-point Likert scale that ranged from 1 Spouse or family member 56 (22.8) ( not at all true) to 7 (very true). Items varied in the extent to which General practitioner 20 (8.1) they reﬂ ected autonomous and controlled motivation. Examples of Other 22 (8.9)

Int J Audiol Downloaded from informahealthcare.com by 110.33.191.149 on 03/13/15 more autonomous items were ‘ I ’ ve carefully thought about hearing Four-frequency average hearing loss aids and believe that getting them is the right thing to do’ , and ‘ I per- in better ear measured at 500, 1000, 2000 and 4000 Hz (dB), sonally believe that doing something about my hearing will improve mean (SD) 31.2 (12.7) my quality of life.’ Examples of more controlled items were ‘ I would Յ 25 dBHL, n (%) 58 (31.7) ʽ be ashamed of myself if I didn ’ t ’ and I think other people would be 26 – 40 dBHL, n (%) 86 (47.0) upset with me if I didn ’ t.ʼ Ն 41 dBHL, n (%) 39 (21.3) Desire for hearing aids* , n (%) W ISHES AND NEEDS TOOL Don ’ t want them 48 (21.4) The wishes and needs tool (WANT; Dillon, 2012) was designed Slightly want them 41 (17.0) as a measure of desire for hearing aids and perceived hearing Want moderately 89 (37.0) diffi culty. The WANT uses a 5-point Likert scale, and consists Want them quite a lot 45 (15.2) of the following two items: ‘ How strongly do you want to get Want them very much 36 (9.4) hearing aids ’ ( 1 Don t want them ; 2 Slightly want them ; 3 Want Perceived hearing diffi culty without * moderately ; 4 Want them quite a lot ; 5 Want them very much), hearing aids , n (%) No diffi culty 20 (8.0) and ‘ Overall, how much diffi culty do you have hearing (without Slight diffi culty 85 (34.8) hearing aids)? ’ ( 1 No diffi culty ; 2 Slight diffi culty ; 3 Moderate Moderate diffi culty 94 (34.8) diffi culty; 4 Quite a lot of diffi culty; 5 Very much diffi culty ). Thus, Quite a lot of diffi culty 58 (17.9) higher scores on the two WANT items are suggested to be associ- Very much diffi culty 18 (4.5) ated with increased desire for hearing aids and more perceived hearing diffi culty respectively. The WANT was developed for the * Questions are from the wishes and needs tool (WANT; Dillon, 2012). 4 J. Ridgway et al.

included as part of a fi tting outcomes survey conducted for the subjected to principal component analysis (PCA) and multivariate program in 2005 (Dillon, 2012). logistic regression performed using Stata version 11.1 (StataCorp, Participants were also asked to answer the following questions: College Station, USA), to investigate the extent to which scores ‘ Whose idea was it to seek services? ‘ (mine / my spouse or family on the TSRQ describe motivation for hearing help-seekers, and to member / my doctor / someone else ); and ‘ Have you worn a hearing examine associations between motivation and hearing aid adoption, aid before? ’ (yes / no ). respectively. Alongside motivation, age, 4-frequency average hearing loss (4FAHL; the average of 500, 1000, 2000, and 4000 Hz in the better ear), gender, referral source, desire for hearing aids, Procedure and perceived hearing diffi culty were also included as independent The study questionnaires were provided before the fi rst consulta- variables. tion with a hearing practitioner. On receipt of completed questionnaires, responses were collated, and then contact was made with Results participants ’ hearing service providers 4 – 6 months later to obtain the audiogram and to identify whether or not participants had agreed to Data from the 253 participants were analysed to obtain statistics get hearing aids (i.e. whether hearing aids were adopted). The delay for the motivation, sociodemographic and audiometric variables. As of 4 – 6 months was to allow suffi cient time for participants to attend the TSRQ had not been used with a sample of hearing help-seekers for consultation with a hearing professional and to make a decision previously, its psychometric properties were tested using data from about hearing aid adoption. this study to establish motivation scores. Following this analysis, par- If the hearing aid fi tting had taken place by this time, a participant ticipant data were grouped as adopters and non-adopters to identify was considered to have adopted hearing aids ( ‘ adopter ’ ). Participants associations of independent variables with hearing aid adoption. not fi tted with hearing aids at this time are referred to as ‘ non-adopters ’ . A total of 230 of the 253 participants consulted with a hearing professional, at which time clinical history, audiometric and speech TSRQ factor structure and subscale derivation assessment, and discussion about rehabilitation options including In order to assess patterns of motivation in the sample of fi rst time hearing aids were completed. Audiograms were obtained for 183 hearing help-seekers, the factor structure and internal consistency of the 253 participants (34 participants did not authorize release of of the TSRQ was assessed initially. To establish the factor struc- their audiogram, 23 participants did not attend for a hearing test, ture of the TSRQ, the inter-item correlation matrix was reviewed to and 13 audiograms were not available for various reasons, such as identify correlation coeffi cients Ն 0.3, correlations were tested for participant relocation to another hearing service provider). signifi cance at the .05 level with the Bartlett test of sphericity, and All participants except one were eligible to receive subsidized the Kaiser-Meyer-Olkin (KMO) measure was used to examine sam- hearing services through the Australian Government Hearing Ser- pling adequacy. A KMO value of Ն 0.6 was considered to be accept- vices Program, which included the option of receiving free digital able (Kaiser, 1974). Inspection of the inter-item correlation matrix For personal use only. hearing aid/s of a variety of styles with the following minimum identifi ed numerous coeffi cients Ն 0.3, which according to Bartlett ’ s specifi cations: automatic directional microphone (if fi tted with test of sphericity were not intercorrelated (χ 2 ϭ 1612.63, d.f. ϭ 171, behind-the-ear hearing aids), feedback cancellation, adaptive noise p Ͻ 0.001), and the KMO value was 0.89; therefore, the data were reduction, multi-channel compression, multi-memory, and telecoil. considered suitable for PCA. The PCA with varimax rotation was The ineligible participant did not adopt hearing aids. The option of then applied to the TSRQ data to examine the factor structure. The co-payment for hearing aids with features additional to these mini- fi rst iteration of the PCA produced three factors with eigenvalues mum specifi cations was also available to participants who adopted greater than 1, which explained 41.7%, 15.6%, and 6.4% of the total hearing aids. It was not known which participants (if any) opted to variance in scores respectively. A scree plot mapping the eigenval- co-pay for hearing aids. Approximately 85% of adopters received ues for each factor in decreasing order of size confi rmed the presence

Int J Audiol Downloaded from informahealthcare.com by 110.33.191.149 on 03/13/15 two hearing aids. Information about the model or style of hearing of three factors before the plot line elbow. The three-factor rotated aid/s adopted was not sought for this study. For participants with a analysis explained 63.8% of the variance, and all factors included at 3-frequency average hearing loss Յ 23 dB (measured at 500, 1000, least three of the initial variables with factor loadings of 0.4 or above. and 2000 Hz), the Australian Government Hearing Services Program Items were examined for suitability to their expected construct and allows hearing aids to be fi tted if the following criteria are met: eliminated if items loaded uniformly on more than one factor, or if (1) the average hearing loss at higher frequencies (i.e. 2000, 3000, and their unique variance (communality) was below 0.4. Three items that 4000 Hz) is Ն 40 dBHL, or tinnitus can be addressed by hearing aid did not load clearly onto any one factor (‘ It ’ s exciting to try to do fi tting, or signifi cant vision impairment is present, and (2) responses something about my hearing ’ , ‘I want others to see that I can wear to each WANT question are Ն 2 with a combined score of Ն 5. The hearing aids and communicate well ’ , and ‘ Wearing hearing aids is number of participants meeting these criteria was not known. something I really want to do’ ) and one item with a communality Ethical clearance for this research was granted by the University score of 0.39 ( ‘I just do it because my doctor said to ’ ) were not of Queensland ’ s Behavioural and Social Sciences Ethical Review included in the analysis based on the PCA fi ndings. Committee, which adheres to the Australian Government National The second iteration of the rotated PCA derived three factors Health and Medical Research Council, National Statement on Ethi- (eigenvalues were 6.3, 2.8, and 1.1), which explained 41.8%, 18.7%, cal Conduct in Human Research (2007). and 7.2% of variance respectively. Nine items clearly loaded onto Factor 1 and four items loaded onto Factor 2. Because the third factor only contained one clearly-loaded item in the second iteration ( ‘I like Data analysis the challenge of pursuing something new and interesting ’ ) the deci- Data consisted of participant responses to the TSRQ, the WANT, sion was made to eliminate this item from the analysis, leaving two sociodemographic questions, and the audiograms. These data were distinct factors. One further item that loaded onto all three factors Autonomous motivation and hearing aid adoption 5

and whose communality was below 0.4 ( ‘It ’ s a challenge to learn chi-squared for categorical variables. Results were obtained for a how to live with hearing aids ’ ) was also removed. total of 237 participants because hearing aid adoption data was miss- Each of the 13 items that loaded onto Factors 1 and 2 was coming for 16 participants. Table 3 presents a comparison of the means pared to its respective item from the original TSRQ and was found and standard deviations (or counts and percentages for categorical to fall into the same subscale as described in the TSRQ Health Care variables) of each independent variable for adopters and non-adopters SDT package. Autonomous and controlled motivation emerged as groups. Sample sizes for independent variables varied due to pairwise distinct factors that described the content of items within the two deletion of missing values. Variables were included in the multivariate subscales. This two-factor construct was largely consistent with pre- regression model if they were associated with the outcome with a p vious research that used the TSRQ (see review of Ng et al, 2012), value below 0.1. Student ’ s t-test and chi-squared analyses showed although four-factor (Levesque et al, 2007) and six-factor (Pellitier higher autonomous motivation scores were signifi cantly associated et al, 2013) solutions have also been reported. Table 2 shows the with hearing aid adoption (p Ͻ 0.001), whereas controlled motiva- two-factor solution with factor loading, mean, and standard deviation scores were not (p ϭ 0.50). Relative to non-adopters, hearing tion for each of the retained TSRQ items. Internal consistency of the aid adopters reported greater desire for hearing aids (p Ͻ 0.001), TSRQ was evaluated with Cronbach ’ s α of 0.7 or above considered perceived greater hearing diffi culty (p Ͻ 0.001), had greater 4FAHL to show good internal consistency of the scale (Kline, 1999). Cron- (p Ͻ 0.001), and were male (p ϭ 0.03). Age (p ϭ 0.91) and source of bach ’ s α for the revised TSRQ was 0.89, and was 0.91 and 0.83 for referral (p ϭ 0.72) were not associated with hearing aid adoption. the two subscales respectively. Thus, each subscale demonstrated The fi ve signifi cant variables (autonomous motivation, gender, good internal consistency. The PCA results therefore suggest the desire for hearing aids, perceived diffi culty, and 4FAHL) were adapted TSRQ to be a potentially useful measure of autonomous entered in the regression model and tested for multicollinearity using and controlled motivation for hearing help-seekers. variance infl ation factors (VIF). Variance infl ation factors were all Responses to items in each TSRQ subscale were then averaged to less than 2, which indicated low correlation among variables. Thus, obtain autonomous and controlled motivation scores for each par- all fi ve variables were retained in the model. Outlying participant ticipant, and were included as the independent motivation variables responses were eliminated from the model if the studentized residual (autonomous motivation and controlled motivation). had an absolute value Ն 2.58. Four outliers were identifi ed in the fi nal model and were removed from the analysis. The fi nal sample size for the regression model was 160 as only those participants with Motivation and hearing aid adoption complete data for each of the fi ve signifi cant variables were included To address whether motivation was associated with hearing aid in the regression analysis. adoption, participants were categorized as adopters or non-adopters Multivariate logistic regression reporting odds ratios was then (as described in the Method section) for the analyses. Prior to multi- used to explore associations between independent variables and variate regression analysis, adopters were compared to non-adopters the outcome variable (hearing aid adoption or non-adoption), by testing each independent variable for association with hearing aid accounting for the effects of other variables. The odds ratio (OR) For personal use only. adoption using Student’ s t-tests for continuous variables and describes the strength of association between motivation and hear-

Table 2. Treatment self-regulation questionnaire principal component analysis with factor loading, mean and standard deviation for retained items (n ϭ 253). Range of scores for each item ϭ 1 – 7.

TSRQ item ‘ I am thinking about getting hearing aids Factor because … ’ loading Mean (SD)

Int J Audiol Downloaded from informahealthcare.com by 110.33.191.149 on 03/13/15 Combined scale 3.48 (1.92) Autonomous motivation 5.52 (1.48) 3. I personally believe that doing something about my 0.76 5.77 (1.66) hearing will improve my quality of life 10. I personally believe that hearing aids are important for 0.69 6.04 (1.47) effective communication 13. I ’ ve carefully thought about hearing aids and believe 0.84 5.34 (1.94) getting them is the right thing to do 16. I feel personally that wearing hearing aids is the best 0.83 4.84 (2.05) thing for me Controlled motivation 2.74 (1.71) 1. Other people would be angry with me if I didn ’ t 0.76 2.32 (1.91) 4. I would feel guilty if I didn ’ t do what my doctor said 0.74 2.72 (2.27) 5. I want my doctor to think I ’ m a good patient 0.74 2.51 (2.34) 6. I would feel bad about myself if I didn ’ t 0.61 2.87 (2.13) 8. I don ’ t want other people to be disappointed with me 0.81 2.44 (2.11) 9. I think other people would be upset with me if I didn ’ t 0.82 2.40 (1.92) 11. I would be ashamed of myself if I didn ’ t 0.74 2.48 (2.05) 12. It ’ s easier to do what I ’ m told than to think about it 0.77 1.88 (1.68) 17. I ’ d feel guilty if I didn ’ t do something about my hearing 0.54 3.77 (2.30)

S D ϭ Standard deviation. 6 J. Ridgway et al.

Table 3. Means and standard deviations (or counts and percentages for categorical variables) of independent variables for hearing aid adopters and non-adopters, along with their signiﬁ cance.

p value Adopters Non-adopters (adoption vs. (n ϭ 1 2 5 c ) (n ϭ 1 1 2 c ) n t / c 2 value non-adoption)

Independent variable Autonomous motivation 5.94 (1.23) 5.06 (1.64) 227 4.61a Ͻ 0.001 Controlled motivation 2.73 (1.77) 2.57 (1.56) 215 0.68a 0.50 Age in Years 69.58 (10.59) 69.74 (10.13) 230 0.12a 0.91 4FAHL better ear 35.03 (10.97) 25.37 (12.92) 182 5.42a Ͻ 0.001 Desire for hearing aids 3.10 (1.21) 2.41 (1.14) 213 4.24a Ͻ 0.001 Perceived difﬁ culty 3.11 (0.90) 2.36 (0.86) 225 6.36a Ͻ 0.001 Gender 237 4.72b 0.03 Female 56 (23.63) 66 (27.85) Male 69 (29.11) 46 (19.41) Source of referral 231 1.34b 0.72 Self 74 (30.74) 64 (27.71) Spouse or family member 28 (12.12) 24 (10.86) General practitioner 8 (3.46) 11 (4.76) Other 10 (4.33) 12 (5.19)

a t values shown (continuous variables). b Chi-squared values shown (categorical variables). c Data for 16 of the 253 participants were removed from the analysis, as their hearing aid adoption decision could not be determined. The n values for independent variables vary due to missing data.

ing aid adoption by measuring the odds that hearing aid adoption classifi ed). The receiver operating characteristic (ROC) curve por- would take place, given the presence or absence of the independent trayed excellent predictive power of the model (AUC ϭ 0.87). variables. The regression analysis showed autonomous motivation, These results show that the multivariate regression model was perceived hearing diffi culty, and 4FAHL to be positively and sig- a very good predictor of the decision to adopt or not adopt hear- nifi cantly associated with hearing aid adoption after other variables ing aids. Participants with higher autonomous motivation, greater were accounted for (Table 4). The analysis indicated that for each perceived hearing diffi culty, and greater 4FAHL were signifi cantly For personal use only. one-point increase in autonomous motivation score, participants more likely to adopt hearing aids than those with lower scores for were 55% more likely to adopt hearing aids (OR 1.55, p ϭ 0.012). these variables. Likewise, for each one-point increase in perceived hearing diffi culty score, participants had 2.3 times greater odds of adopting hear- Discussion ing aids (OR 2.32, p ϭ 0.013). Participants were 16% more likely to adopt hearing aids for each 1dB increase in 4FAHL (OR 1.16, The present study set out to determine how autonomous and controlled p Ͻ 0.001). motivation were related to hearing aid adoption of a sample of fi rst Post-estimation tests were used to evaluate the goodness-of-fi t time hearing help-seekers using the TSRQ as the measure of moti- and the predictive accuracy of the logistic regression model. The vation. Our fi nding that autonomous motivation was associated with Int J Audiol Downloaded from informahealthcare.com by 110.33.191.149 on 03/13/15 Hosmer-Lemeshow goodness-of-fi t results confi rmed the fi nal hearing aid adoption represents the fi rst empirical evidence linking model fi tted the data well (χ 2 ϭ 5.17, d.f. ϭ 8, p ϭ 0.74). The autonomy to the health decisions of fi rst time hearing help-seekers. classifi cation matrix correctly classifi ed 78.12% of cases using the Although there was no literature to compare the results of this study model (68.33% of adopters and 84% of non-adopters were correctly with other samples of hearing help-seekers, studies that applied the TSRQ in the broader health literature also found positive associations between autonomous motivation and the adoption of other health- related behaviours. Shigaki et al (2010), for example, administered Table 4. Multivariate logistic regression analyses of associations the TSRQ to 77 patients with Type 2 diabetes and found autonomous between predictor variables and hearing aid adoption (n ϭ 160). motivation was associated with maintaining diet and increased test- Hearing aid adoption vs. Non-adoption ing of blood glucose. Likewise, in a large-scale cross-sectional study (outcome variable) a of elite athletes, Chan and Hagger (2012) reported that the more an athlete was autonomously motivated to engage in sports injury pre- Predictor variable OR 95% CI p vention and rehabilitation, the more likely she/he would regard the Autonomous motivation 1.55 [1.10, 2.18] 0.012 behaviour as positive and commit to engaging in future treatment. Gender 1.29 [0.55, 3.02] 0.555 Autonomous motivation is therefore an important consideration for Desire for hearing aids 1.04 [0.63, 1.71] 0.874 the adoption of health-related behaviours across a range of health set- Perceived diffi culty 2.32 [1.19, 4.52] 0.013 tings, including for adults contemplating hearing aid adoption. 4FAHL better ear 1.16 [1.10, 1.22]Ͻ 0.001 The lack of association between controlled motivation and a Hosmer-Lemeshow goodness-of-fi t (p ϭ 0.74); Area under receiver operating hearing aid adoption found in this study was consistent with previous characteristic (ROC) curve (0.87). OR ϭ Odds ratio; CI ϭ Confi dence interval. research, which has generally reported nil or negative associations Autonomous motivation and hearing aid adoption 7

between controlled forms of motivation and the adoption of health the multivariate analysis. Although Dillon (2012) reported increased behaviours (Ng et al, 2012). In a study of 128 patients who took med- desire for hearing aids to be associated with hearing aid use, benefi t ication for diabetes, Williams et al (1998a) reported that controlled and quality of life 6– 12 months post-fi tting, its lack of multivari- motivation was not signifi cantly correlated with regulation of glucose ate association with hearing aid adoption in this study suggests that levels. Furthermore, Levesque et al (2007), who obtained TSRQ data interrelationships among additional factors that infl uence desire for from 2731 participants across three different health domains (smok- hearing aids at or following hearing aid adoption warrant further ing cessation, healthy eating, and exercise management), found that, investigation. As the psychometric properties of the WANT have in addition to positive associations between autonomous motiva- not been determined, research that explores associations between the tion and engagement with health behaviours, controlled motivation WANT and hearing aid adoption might help clarify the sensitivity was signifi cantly associated with adverse health outcomes such as and validity of this measure. depression, or negatively associated with healthy eating. Interest- The use of multivariate regression can reduce the confounding ingly, Levesque et al (2007) also found that controlled motivation effects of interrelations between factors, which strengthens analyses was signifi cantly associated with greater levels of physical activity. (Meyer & Hickson, 2012). Nonetheless, the list of variables included This positive association differs from the fi ndings of the present in this study was not exhaustive (see Meyer et al, 2014, for a com- study, and suggests that the effects of autonomous and controlled prehensive list of factors). While the logistic regression model in motivation may vary among different health behaviours and treat- this study predicted 87% of hearing aid adoption decisions with the ment environments. ROC curve, its predictive power might have further increased with As autonomous motivation, but not controlled motivation, was the inclusion of additional factors. For example, an exploration of the associated with hearing aid adoption, the link between autonomy attitudes of signifi cant others to hearing aid adoption might provide and positive health behaviours, previously reported as fundamen- further insight into the decision to adopt or not adopt hearing aids tal to SDT (Ryan et al, 2008; Ng et al, 2012), was supported by (Meyer & Hickson, 2012). the fi ndings of this study. While differences in TSRQ autonomous This study found hearing aid adopters to have, on average, motivation scores between adopters and non-adopters imply sup- 9.7 dB greater 4FAHL than non-adopters, and for the proportion of port for an internalization continuum, the different behaviours of the sample whose audiograms were available, the strong multivariate autonomous and controlled motivation subscales could also sug- association between greater 4FAHL and hearing aid adoption was gest these variables are independently scaled. Assuming the evi- consistent with previous literature (Knudsen et al, 2010; Meyer & dence for an internalization continuum within the SDT model of Hickson, 2012). Such a relationship is typically not evident for those health behaviour change (Ryan & Connell, 1989; Ng et al, 2012) is with mild to moderate hearing impairment (Laplante-L é vesque et al, accepted, a two-factor TSRQ, such as that obtained in the present 2012), but in this study 31.0% of those with 4FAHL Յ 25 dBHL and study, might not adequately represent the various forms of motiva- 69.8% of those with 4FAHL 26– 41 dBHL adopted hearing aids. tion along the continuum (see Ridgway et al, 2013). Illustrating the A possible explanation for this fi nding is the ease with which people value of more precisely classifying motivation, Pelletier et al (2001) with mild to moderate hearing impairment can adopt hearing aids For personal use only. showed motivation for the practice of competitive sport varied with under the Australian Government Hearing Services Program. As different forms of controlled motivation (introjected and external stated in the Method section, participants with hearing loss Յ 2 3 motivation). Moreover, in their large, multi-site study, Levesque et al dBHL (pure-tone average of 500, 1000, and 2000 Hz) could be fi tted (2007) derived a four-factor TSRQ structure (autonomous motiva- with hearing aids if specifi c additional criteria were met. Neverthe- tion, introjected motivation, external motivation, and amotivation) less, the proportion of participants with mild to moderate hearing that more closely represented the internalization continuum than the impairment who adopted hearing aids, irrespective of these criteria, fi ndings of this study. Differences in participant numbers, demo- suggests it was not a signifi cant factor that infl uenced participant graphics, and the wording of some items might have contributed decisions. to the different TSRQ subscale solutions of Levesque et al (2007) Information about whether or not hearing aids were recommended

Int J Audiol Downloaded from informahealthcare.com by 110.33.191.149 on 03/13/15 and the present study. Pelletier et al (2013), in two studies that used to non-adopters in this study was not available, but may have been a version of the TSRQ (the sport motivation scale) to investigate benefi cial for the analysis, because removal of non-adopters for motivation in athletes, acknowledged criticism that the scale may not whom hearing aids were not recommended might have strengthened completely represent all SDT motivation constructs, and presented the regression model. the psychometric properties of a redesigned sport motivation scale Overall, the empirical fi ndings of this study add to the growing that more closely aligned its item content with SDT. On balance, the body of literature that recognizes motivation as an important con- two-factor TSRQ used in this study provided evidence that supported tributor to decision-making in hearing rehabilitation. The use of the the principles of SDT, and was consistent with other health research TSRQ to classify autonomous and controlled motivation offers a that demonstrated the importance of autonomy to health decisions. new approach to understanding the relationships between motivation However, it is acknowledged that further review and refi nement of and hearing aid adoption. Our results associate autonomous moti- the TSRQ for hearing help-seekers could more precisely classify vation with hearing aid adoption and are similar to fi ndings from motivation along the internalization continuum so that motivations other health disciplines such as tobacco abstinence, diabetes man- of people seeking to adopt hearing aids are further understood. agement, dental self-care, and medication adherence, and reinforce Our fi nding that perceived hearing diffi culty was positively asso- the importance of autonomy to health decisions. According to SDT, ciated with hearing aid adoption was not surprising and has been development of a sense of autonomy is central to internalization consistently reported elsewhere (Knudsen et al, 2010; Meyer & of health-related behaviours (Ryan et al, 2011). That is, health-care Hickson, 2012). While awareness of hearing diffi culty was evidently decisions that come to be personally valued and are congruent with an important factor for people considering hearing aid adoption, it personal beliefs result in greater engagement with treatment. Our was interesting that no association was found between desire for fi ndings could therefore have implications for the ways practitioners hearing aids, as measured by the WANT, and hearing aid adoption in evaluate motivation and the ways this information is used to guide 8 J. Ridgway et al.

discussions with clients about hearing aid adoption. Practitioners Kaiser H.F . 1974 . An index of factorial simplicity . Psychometrika , might, for example, consider how autonomous motivation can be 39 , 31 – 36 . encouraged in clients, so that clients who are not autonomously Kline P . 1999 . The Handbook of Psychological Testing (2nd edn.) . London: motivated can become more engaged with clinical decisions. Candi- Routledge . dates for hearing aid adoption might also be predicted by identifying Knudsen L.V. , Ö berg M. , Nielsen C. , Naylor G. & Kramer S.E . 2010 . Fac- tors infl uencing help seeking, hearing-aid uptake, hearing-aid use, and autonomous motivation in clients. satisfaction with hearing aids: A review of the literature. Trends Amplif , 14 , 127 – 154 . Conclusion Kochkin S . 2007 . MarkeTrack VII: Obstacles to adult non-user adoption of hearing aids . Hear J , 60(4) , 24 – 51 . This study applied the TSRQ to explore relationships between auton- Laplante-L é vesque A. , Hickson L. & Worrall L . 2012 . What makes adults omous and controlled motivation and the decision to adopt or not with hearing impairment take up hearing aids or communication pro- adopt hearing aids. While positive associations between autonomous grams and achieve successful outcomes? Ear Hear , 33(1) , 79 – 93 . motivation and hearing aid adoption highlight the role of autonomy Laplante-L é vesque A. , Hickson L. & Worrall L . 2013 . Stages of change in in the initiation of new health behaviours, further research to inves- adults with acquired hearing impairment seeking help for the fi rst time: tigate relationships of different dimensions of motivation to client Application of the transtheoretical model in audiologic rehabilitation. outcomes beyond hearing aid adoption, such as hearing aid use or Ear Hear , 34(4) , 447 – 457 . satisfaction, would be of benefi t. Levesque C. , Williams G.C. , Elliot D. , Pickering T. , Bodenhammer B. et al . 2007 . Validating the theoretical structure of the Treatment Self-Regulation Questionnaire (TSRQ) across three different health be- Notes haviors . Health Educ Res , 22(5) , 691 – 702 . Maslow A.H . 1943 . A theory of human motivation . Psychol Rev , 50(4) , 1. The Australian Government Hearing Services Program subsi- 370 – 396 . dizes the provision of hearing services to eligible Australians. Meyer C. & Hickson L . 2012 . What factors infl uence help-seeking and More information: http://www.hearingservices.gov.au hearing-aid adoption in older adults? Int J Audiol , 51(2) , 66 – 74 . Meyer C. , Hickson L. , Khan A. , Hartley D. , Dillon H. et al . 2011 . Investiga- Acknowledgements tion of the actions taken by adults who failed a telephone-based hearing screen . Ear Hear , 32 , 720 – 731 . This research is part of the principal author’ s PhD, and was sup- Meyer C. , Hickson L. , Lovelock K. , Lampert M. & Khan A . 2014 . An inves- ported through the University of Queensland School of Health tigation of factors that infl uence help-seeking for hearing impairment in and Rehabilitation Sciences. The authors would like to thank Dr older adults . Int J Audiol , 53(Suppl. 1) , S3 – S17 . Asad Khan from the University of Queensland School of Health M ü nster Halvari A.E. , Halvari H. , Bj ö rnebekk G. & Deci E.L . 2012 . Self- and Rehabilitation Sciences who provided statistical guidance. The determined motivational predictors of increases in dental behaviours, de- authors also wish to acknowledge the support of Australian Hear- creases in dental plaque, and improvement in oral health: A randomized ing, National Hearing Care, and John Pearcy Audiology for their clinical trial . Health Psychol , 31(6) , 777 – 788 . For personal use only. Ng J.Y.Y. , Ntoumanis N. , Th ø gersen-Ntoumani C. , Deci E.L. , Ryan R.M. assistance with participant recruitment, and thank the participants et al . 2012 . Self-determination theory applied to health contexts: A meta- for their individual contributions to the study. analysis . Perspect Psychol Sci , 7(4) , 325 – 340 . Nieuwenhuijsen E.R. , Zemper E. , Miner K.R. & Epstein M . 2006 . Health behavior change models and theories: Contributions to rehabilitation. Declaration of interest: The authors declare no confl icts of inter- Disabil Rehabil , 28(5) , 245 – 256 . est. No external fi nancial assistance was received for this study. Nigg C.R. , Allegrante J.P. & Ory M . 2002 . Theory-comparison and multiple- The authors alone are responsible for the content and writing of behavior research: Common themes advancing health behavior research . this paper. Health Educ Res , 17(5) , 670 – 679 . Pelletier L.G. , Fortier M.S. , Vallerand R.J. & Bri è re N.M . 2001 . Associations Int J Audiol Downloaded from informahealthcare.com by 110.33.191.149 on 03/13/15 References among perceived autonomy support, forms of self-regulation and persistence: A prospective study . Motiv Emot , 25(4) , 279 – 306 . Bandura A . 1977 . Self-effi cacy: Toward a unifying theory of behavioral Pelletier L.G. , Rocchi M.A. , Vallerand R.J. , Deci E.L. & Ryan R.M . 2013 . change . Psychol Rev , 84(2) , 191 – 215 . Validation of the revised sport motivation scale (SMS-II). Psychol Sport Berridge K.C . 2004 . Motivation concepts in behavioral neuroscience . Physiol Exerc , 14 , 329 – 341 . Behav , 81 , 179 – 209 . Prochaska J.O. , DiClemente C.C. & Norcross J.C . 1992 . In search of how Chan D.K.C. & Hagger M.S . 2012 . Self-determined forms of motivation people change: Applications to the addictive behaviors. Am Psychol , 47 , predict sports injury prevention and rehabilitation intentions . J Sci Med 1102 – 1114 . Sport , 15 , 398 – 406 . Ridgway J. , Hickson L. & Lind C . 2013 . Self-determination theory: Motiva- Deci E.L. & Ryan R.M . 1985 . Intrinsic Motivation and Self-determination in tion and hearing-aid adoption . J Acad Rehabil Audiol , 46 , 11 – 37 . Human Behavior . New York: Plenum Publishing Co . Rosenstock I . 1966 . Why people use health services . Milbank Memorial Fund Deci E.L. & Ryan R.M . 2000 . The what and why of goal pursuits: Human Quarterly , 44 (Suppl. 3) , 94 – 127 . needs and self-determination of behavior . Psychol Inq , 11 , 227 – 268 . Ryan R.M. & Connell J.P . 1989 . Perceived locus of causality and internaliza- Dillon H . 2012 . Hearing Aids, Second edition . Sydney, Australia: Boomer- tion: Examining reasons for acting in two domains . J Pers Soc Psychol , ang Press . 57 , 749 – 761 . Hartley D. , Rochtchina E. , Newall P. , Golding M. & Mitchell P . 2010 . Use of Ryan R.M. & Deci E.L . 2008 . A self-determination theory approach to psy- hearing aids and assisting listening devices in an older Australian popu- chotherapy: The motivational basis for effective change . Can Psychol , lation . J Am Acad Audiol , 21 , 642 – 653 . 49(3) , 186 – 193 . James W . 1890 . The Principles of Psychology (Vol II). Retrieved 3 May 2012, Ryan R.M. , Lynch M.F. , Vansteenkiste M. & Deci E.L . 2011 . Motivation and from http://ia600402.us.archive.org/15/items/theprinciplesofp00jameuoft/ autonomy in counseling, psychotherapy, and behavior change: A look at theprinciplesofp00jameuoft.pdf theory and practice . Couns Psychol , 39(2) , 193 – 260 . Autonomous motivation and hearing aid adoption 9

Ryan R.M. , Patrick H. , Deci E.L. & Williams G.C . 2008 . Facilitating Williams G.C. , Grow V.M. , Freedman Z.R. , Ryan R.M. & Deci E.L . 1996 . health behavior change and its maintenance: Interventions based on Motivational predictors of weight loss and weight-loss maintenance. J self-determination theory . The European Health Psychologist , 10 , 2 – 5 . Pers Soc Psychol , 70 , 115 – 126 . Saunders G.H. , Chisolm T.H. & Wallhagen M.I . 2012 . Older adults and hear- Williams G.C. , Patrick H. , Niemiec C.P. , Ryan R.M. , Deci E.L. et al . 2011 . ing help-seeking behaviors . Am J Audiol , 21 , 331 – 337 . The smoker’ s health project: A self-determination theory intervention Shigaki C. , Kruse R.L. , Mehr D. , Sheldon K.M. , Ge B. et al . 2010 . Chronic to facilitate maintenance of tobacco abstinence . Contemp Clin Trials , Illn , 6 , 202 – 214 . 32 , 535 – 543 . Skinner B . 1953 . Science and Human Behavior . Retrieved 23 February 2011 Williams G.C. , Rodin G.C. , Ryan R.M. , Grolnick W.S & Deci E.L . 1998b . from http://www.bfskinner.org/bfskinner/Society_ﬁ les/Science_and_ Autonomous regulation and long-term medication adherence in adult Human_Behavior.pdf outpatients . Health Psychol , 17(3) , 269 – 276 . Williams G.C. , Freedman Z.R. & Deci E.L . 1998a . Supporting autonomy Wilson C. & Stephens D . 2003 . Reasons for referral and attitudes to- to motivate patients with diabetes for glucose control. Diabetes Care , ward hearing aids: Do they affect outcome? Clin Otolaryngol , 21(10) , 1644 – 1651 . 28 , 81 – 84 . For personal use only. Int J Audiol Downloaded from informahealthcare.com by 110.33.191.149 on 03/13/15

International Journal of Pediatric Otorhinolaryngology 78 (2014) 96–101

Contents lists available at ScienceDirect

International Journal of Pediatric Otorhinolaryngology

jo urnal homepage: www.elsevier.com/locate/ijporl

Autism and peripheral hearing loss: A systematic review

a b b

Alison N. Beers , Melanie McBoyle , Emily Kakande ,

b b,

Rachelle C. Dar Santos , Frederick K. Kozak *

Department of Audiology, BC Children’s & Women’s Hospital, Vancouver, BC, Canada

Division of Pediatric Otolaryngology, Department of Surgery, BC Children’s & Women’s Hospital, Vancouver, BC, Canada

A R T I C L E I N F O A B S T R A C T

Article history: Objective: To systematically review the literature describing the relationship between autism spectrum

Received 25 June 2013

disorder (ASD) and peripheral hearing loss including literature recommendations for audiological

Received in revised form 24 October 2013

assessment and auditory habilitation in cases where peripheral hearing loss and ASD coexist.

Accepted 29 October 2013

Data sources: Published studies indexed in MEDLINE (1948–2011).

Available online 15 November 2013

Review methods: The search strategy identiﬁed 595 potential studies. After a review of the titles, 115

abstracts were reviewed and 39 articles were retrieved and assessed independently by at least two

Keywords:

authors for possible inclusion. 22 articles pertained to children with ASD and peripheral hearing loss,

Autism

hearing assessment in children with ASD, audiological habilitation for children with ASD or hyper-

Hearing loss

responsiveness in children with ASD. 17 further studies were garnered from the reference section of the

Hearing aids

Cochlear implant 22 papers.

Hyper-responsiveness Results: Controversy exists in the literature regarding prevalence of hearing impairment among

Hyperacusis individuals with ASD. In cases where ASD and hearing impairment co-exist, diagnosis of one condition

often leads to a delay in diagnosing the other. Audiological assessment can be difﬁcult in children with

ASD and test–retest reliability of behavioural thresholds can be poor. In cases where hearing impairment

exists and hearing aids or cochlear implantation are recommended, devices are often ﬁt with special

considerations for the child with ASD. Hyper-responsiveness to auditory stimuli may be displayed by

individuals with ASD. Evidence or the suspicion of hyper-responsiveness may be taken into

consideration when ﬁtting ampliﬁcation and planning behavioural intervention.

Conclusions: Prevalence rates of hearing impairment among individuals with ASD continue to be

debated. At present there is no conclusive evidence that children with ASD are at increased risk of

peripheral hearing loss. A complete audiological assessment is recommended in all cases where ASD is

suspected so as not to delay the diagnosis of hearing impairment in the event that hearing loss and ASD

co-exist. Objective assessment measures should be used to conﬁrm behavioural testing in order to

ensure reliability of audiological test results. Fitting of hearing aids or cochlear implantation are not

contraindicated when hearing loss is present in children with ASD; however, success with these devices

can be variable.

1. Introduction and language delay, regression of developmental milestones at 18–

24 months of age, avoidance of eye contact, tactile defensiveness

Autism is a pervasive and complex developmental disorder and engagement in repetitive and self-stimulating behaviours. See

characterized by impaired social interaction, atypical communi- Table 1 in Egelhoff et al. [2] for a more comprehensive list.

cation and restricted, repetitive and stereotypical patterns of Approximately 80% of children with autism have some degree of

behaviours, activities and interests [1]. Individuals with autism cognitive impairment [3].

differ markedly in the number and severity of symptoms displayed Autism is currently classiﬁed as a spectrum disorder, which

[2]. Typical signs of autism include, but are not limited to; speech includes autistic disorder, Asperger’s disorder, Rett’s disorder,

childhood disintegrative disorder and pervasive developmental

disorder not otherwise speciﬁed [4]. Prevalence estimates of

autism spectrum disorder (ASD) are controversial. A review by

* Corresponding author at: Division of Pediatric Otolaryngology, B.C. Children’s

Frombonne [3] reported ASD prevalence rates ranging from 0.7/10,

Hospital, K2-184, ACB, 4480 Oak Street, Vancouver, BC, Canada V6H 3V4.

000 to 21.1/10,000, with a median value of 5.2/10,000 (equivalent

Tel.: +1 604 875 2113; fax: +1 604 875 2498.

E-mail address: [email protected] (F.K. Kozak). to: 1/1925) and that prevalence rate increased with study

A.N. Beers et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 96–101 97

Table 1

Summary of studies that evaluate the prevalence of hearing loss in children with ASD.

Study and year Subject group Cognitive function Audiological measures Audiological results

Rosenhall et al. [8] 199 children and adolescents Range Behavioural audiometry, ABR Hearing loss at a prevalence rate of

with ASD 3.5% among ASD population, higher

than within general population

Tas et al. [9] 30 children with ASD Not speciﬁed OAE, ABR Possible increased hearing loss

prevalence among a population with ASD

Jure et al. [10] 61 cases of ASD and hearing Range Not available: retrospective Prevalence estimates not possible;

loss from 1150 cases of review of children with ASD co-occurrence ASD and hearing loss

hearing loss and known hearing loss may be increased

Gravel et al. [11] 37 children with ASD High functioning Behavioural audiometry and All had normal peripheral hearing and

computer-assisted threshold normal peripheral auditory system function.

measurement, tympanometry,

OAE, MEMR

Tharpe et al. [12] 22 children with ASD Range Behavioural audiometry, ABR, All had normal peripheral hearing

OAE, MEMR, tympanometry

ASD: autism spectrum disorder; OAE: otoacoustic emissions; ABR: auditory brainstem response; MEMR: middle ear muscle reﬂex.

publication year. A review of published ASD prevalence rates in peripheral hearing loss, audiological assessment or audiological

studies since 2000, and primarily since 2006 revealed a median management (deafness, cochlear implantation, hearing aids, or

value of 62/10,000 (equivalent to: 1/161) [5]. As ASD prevalence hyper-responsiveness to auditory stimuli). The included studies

rates over the past decade have shown a rapid increase [4], many comprised; 9 Expert opinion, 8 Case series, 16 Individual Case-

argue that the recent increase is not directly due to an increased control series, 3 Systematic Review of Case-control series, 2

prevalence of the disorder, but is due to an increased awareness of Individual Cohort studies and 1 Systematic Review of Cohort

the disorder and broadening of its diagnostic criteria [6]. studies. The main purpose of this study was to review and collate

This review sought to identify whether a relationship exists the English literature relating ASD and peripheral hearing loss. Due

between peripheral hearing loss and ASD. Due to the extent and to the variability between studies, subject inclusion, test method-

variability of the literature reporting on central issues (primarily ologies and outcomes measured, it was not possible to perform

central auditory processing) in individuals with ASD and the meta-analysis of the data. At least two authors independently

degree to which input from other modalities integrates with the assessed the titles and abstracts of all studies identiﬁed by the

auditory system at the central level, only the peripheral auditory searches. The full articles were obtained where they appeared to

system was considered. meet the inclusion criteria (i.e. an association between ASD and

2. Methods

595 potentially relevant citations

2.1. Search strategy identified and screened for retrieval.

480 citations excluded as not

Original published studies on autism and hearing loss were

relevant to the review.

identiﬁed with a comprehensive search strategy in MEDLINE (from

1948 to 2011). The search terms used for autism were ‘‘autistic 115 abstracts retrieved for more

detailed evaluation.

disorder [MeSH]’’, ‘‘autism’’ and ‘‘schizophrenia, childhood

[MeSH]’’. The search terms used for hearing loss were ‘‘hearing 76 articles excluded as not

loss [MeSH]’’, ‘‘deafness’’, ‘‘hearing loss, bilateral [MeSH]’’, ‘‘hearing relevant to the review.

loss, high-frequency [MeSH]’’, ‘‘hearing loss, sensorineural

[MeSH]’’, ‘‘hearing loss, central [MeSH]’’, ‘‘hearing loss, unilateral

39 articles retrieved for more

[MeSH]’’, ‘‘hearing impairment’’, ‘‘deaf’’, ‘‘auditory perception detailed evaluation.

[MeSH]’’, ‘‘cochlear nerve [MeSH]’’, ‘‘cochlea [MeSH]’’, ‘‘tinnitus

[MeSH]’’, ‘‘vestibulocochlear nerve [MeSH]’’, ‘‘auditory cortex 17 articles excluded from the

study.

[MeSH]’’, ‘‘auditory pathways [MeSH]’’, ‘‘auditory system’’, ‘‘audi-

ABR wave features = 7

tory processing’’ ‘‘hyperacusis’’, ‘‘evoked potentials, auditory brain Central auditory = 10

stem [MeSH]’’, ‘‘hearing [MeSH]’’, ‘‘cochlear diseases [MeSH]’’,

22 articles included in the

‘‘cochlear implants [MeSH]’’, ‘‘cochlear implantation [MeSH]’’, systematic review (SR).

‘‘auditory threshold [MeSH]’’, ‘‘otoacoustic emissions, spontaneous

[MeSH]’’ and ‘‘hearing aids [MeSH]’’. These terms were combined 11 further articles were identified

from the 22 unique articles from

with Boolean operators. Reference lists from relevant articles,

the original SR.

including other reviews, were searched. Search was limited to the

English language and humans (see Fig. 1). 33 articles included in the systematic

review (SR).

- 1 SR of Cohort studies

2.2. Purpose of study - 2 Individual Cohort studies

- 3 SR of Case-control studies

- 13 Individual Case-control studies

To determine the relationship between ASD and peripheral

- 8 Case studies

hearing loss, hearing assessment in children with ASD, audiological - 6 Expert opinion

habilitation for children with ASD, or hyper-responsiveness in

children with ASD.

Fig. 1. Flow chart of systematic review.

The identiﬁed studies were assessed for eligibility and were

included only if they explored an association between autism and

98 A.N. Beers et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 96–101

peripheral hearing loss). References found in the articles identiﬁed was reported between these children with ASD and their typically

were also pursued where appropriate. Two other papers identiﬁed developing peers.

were also included. Studies not available in English were excluded. Tharpe et al. [12] prospectively studied 22 children with autism

who ranged in level of cognitive function. Children with abnormal

2.3. Assessment of quality tympanometry were excluded from the study. Audiological

function was assessed using a test battery of ABR, behavioural

The evidence presented in the selected studies was categorized audiometry, otoacoustic emissions and middle ear muscle reﬂex

by level of evidence as deﬁned by the Oxford Centre for Evidence- measurements and tympanometry. All study participants with

Based Medicine [7]. autism had normal peripheral hearing.

Many studies have investigated auditory brainstem response

2.4. Data extraction and analysis (ABR) in children with ASD, speciﬁcally waveform peak and inter-

peak latencies [8,9,13–17], though subject inclusion criteria, as

Information was extracted from the included articles indepen- well as study methodology and results have been highly variable.

dently by two authors and summarized in a narrative format. Elevated thresholds to click stimuli have been identiﬁed in some

cases [13,18], but in most of the ABR studies, methodology was not

3. Results sensitive to detect mild to moderate or frequency-speciﬁc hearing

loss. Prolonged brainstem transmission of auditory stimuli, while it

3.1. Prevalence of hearing impairment among children with autism may differ signiﬁcantly between ASD and control groups, does not

spectrum disorder consistently correlate with clinically deﬁned hearing impairment.

It is for this reason that studies involving prolonged ABR wave

There is currently no consensus in the literature regarding latencies and inter-peak intervals were not reviewed in detail for

prevalence of hearing impairment among children with autism this paper.

spectrum disorder (ASD). The studies described in this section The variability in prevalence estimates of peripheral hearing

evaluating the prevalence of hearing loss among a population with impairment among individuals with ASD may be attributed to

ASD are summarized in Table 1. differences between study methodologies and sample sizes as

Rosenhall et al. [8] prospectively studied 199 children and well as inconsistencies in subject inclusion criteria. However,

adolescents with autism who ranged in level of intellectual among children with ASD there is no conclusive evidence that the

functioning. Audiometry was conducted to the developmental prevalence of peripheral hearing impairment is elevated

level of the child and auditory brainstem response (ABR) testing compared to the prevalence of hearing impairment within a

was performed. Authors reported pronounced to profound general population. In cases where ASD and hearing impairment

bilateral hearing loss at a prevalence rate of 3.5% among a co-occur, diagnosis of one condition often leads to a delay in

population with autism, ten times higher than the prevalence of diagnosing the other [10,19]. Jure et al. [10] reported that in 11 of

hearing loss within a general population. They reported no 46 children, diagnosis of ASD was delayed on average 4 years

association between degree of intellectual functioning and hearing beyond the diagnosis of hearing loss, and in 10 of 46 children,

loss. diagnosis of hearing loss was delayed several years following the

Tas et al. [9] objectively studied auditory function in a group of diagnosis of autism. Roper et al. [19] studied autistic behaviours

30 children with autism using a combination of otoacoustic in a group of 13 students with ASD coexisting with hearing

emission and ABR assessment. All participants had normal type A impairment compared to 12 students with ASD and normal

tympanograms on the day of testing, consistent with normal hearing. No differences in autistic symptomology between the

middle ear status. Severe bilateral sensorineural hearing loss was two groups was found, but it was suggested that the diagnosis of

reported in 3 subjects (10%) with autism based on absent hearing loss may have obscured recognition of autistic beha-

otoacoustic emissions and no ABR response to a high level (80 viours as ASD was diagnosed up to 5 years following the hearing

dBnHL) click stimulus bilaterally, and mild unilateral sensorineural loss diagnosis [19]. It is recommended that in all cases where ASD

hearing loss in 2 subjects (6.6%) with autism based on absent is suspected, children receive a complete audiological assess-

otoacoustic emissions and a moderately elevated ABR response. It ment so that any peripheral hearing loss can be diagnosed early

was concluded that hearing loss might be more prevalent among a and managed as part of the child’s habilitation and education

population with autism compared to the general population. programme [9,19].

Jure et al. [10] retrospectively reviewed and characterized the Increased prevalence of serous otitis media (OM) (23.5%) and

clinical features of children with hearing impairment and autism. associated temporary conductive hearing impairment (18.3%) have

Sixty-one cases of autism co-existing with hearing loss were been reported among children with ASD [8]. However, studies

identiﬁed from a sample of 1150 cases of hearing loss (5.3%) involving reports of OM may be confounded as parents are often

suggesting that co-occurrence may be more likely than due to unable to recognize the presence of acute OM, OM with effusion

chance alone. Prevalence estimates, however, cannot be made and associated conductive hearing loss [20] and retrospective

from this study as samples were drawn from a biased population. prevalence rates of OM may be underrepresented among typically

No clear relationship was found between severity of autistic developing children that often serve as controls. For this reason,

behaviours and degree of hearing loss. Gravel et al. [11] did not consider the role of OM in their evaluation

Gravel et al. [11] prospectively studied peripheral auditory of the peripheral auditory mechanism among children with ASD.

function in a group of 37 children with autism using a test battery As reports of OM can be inaccurate and symptoms are transient,

of conventional behavioural audiometry and computer-assisted the authors of this paper similarly did not review the literature on

threshold measurement, tympanometry and middle ear muscle OM among individuals with ASD.

reﬂex and otoacoustic emission measurement. All children

included in this study had normal behavioural hearing thresholds 3.2. Audiological assessment in children with autism spectrum

and middle ear status. Children with ASD were high functioning disorder

and did not present with signiﬁcant cognitive or neurological

deﬁcits. All children wore earphones and completed the entire Audiological test settings and procedures are often modiﬁed

assessment. No evidence of peripheral auditory system differences when assessing children with ASD, as this population can be

A.N. Beers et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 96–101 99

unresponsive to audible stimuli and may become upset in the 3.3. Hearing aids and cochlear implant considerations for children

soundbooth. The audiologist may send materials home prior to with autism spectrum disorder

the appointment; picture books, social stories or online videos

may help prepare the child for an audiological assessment Accurate audiological assessment results provide the founda-

[21,22]. During the assessment the child may take comfort in tion for appropriate intervention and communication planning. In

having a picture schedule and a means to take a break [22]. the event that hearing loss is diagnosed and ampliﬁcation is

Audiological testing may need to occur over several sessions. recommended, hearing aids are selected and set using a prescrip-

Children with ASD may display resistance to wear headphones or tive procedure that incorporates ear canal acoustics and audiolog-

a bone oscillator, or to having a probe inserted into the ear canal ical thresholds [28]. In cases where behavioural audiological

for the purpose of measuring middle ear impedance and thresholds are unreliable, hearing aids are set based on

otoacoustic emissions. Parents may want to introduce head- electrophysiological ABR results and settings are re-evaluated as

phones or earphones at home in preparation for the audiology behavioural threshold information becomes available [28]. The

appointment [22]. goal of any paediatric hearing aid ﬁtting is to provide the child

Behavioural hearing assessment results in children with ASD access to the acoustic features of speech while ensuring that

can be of questionable reliability and test–retest reliability of maximum hearing aid output does not exceed recommended

audiological thresholds can be poor. Tharpe et al. [12] found that levels [28]; hearing aid ﬁttings for children with ASD are no

the majority of children with autism demonstrated elevated exception. Some children with ASD experience increased percep-

behavioural audiological thresholds despite normal objective tion of loudness [30] and display hyper-responsiveness behaviours

measures of auditory function, as well as greater than normal to auditory stimuli [8,12,31,32]; this may be reason to initially

test–retest variability of pure tone thresholds and poor agreement limit maximum acoustic output levels of hearing aids in order to

between pure tone and speech reception thresholds. There is a risk facilitate acceptance of new equipment.

of over-estimating hearing thresholds due to difﬁculty in Individual ear canal acoustics are incorporated into the

measuring reliable responses at low stimulus levels, while at audiological assessment and hearing aid ﬁtting and veriﬁcation

the same time mild or notched hearing loss may go undetected procedures by way of measuring real-ear to coupler differences

[8,10]. (RECD) [28]. No studies were found regarding RECD measurements

It is standard to use a test battery approach for audiological among a population with ASD; however, the recommendation to

assessment with objective tests to verify behavioural audiometry measure RECD whenever possible [28] is considered best practice

results. Evoked otoacoustic emissions measure the cochlea’s for all children. Due to the increased likelihood of tactile

active response to an auditory stimulus [23] and can be recorded defensiveness among individuals with ASD [2], reliable RECD

at the level of the external auditory canal in almost all ears with measurements, which requires placement of a probe tube into the

normal to near-normal hearing. Otoacoustic emission testing is ear canal, may not be possible. No studies were found pertaining to

an objective, quick and non-invasive way to assess hearing status taking ear impressions on children with ASD, but similar

through to the level of the cochlea and is sensitive to frequency- challenges exist as with RECD measurements; this population is

speciﬁc pathology [24]. Otoacoustic emission testing has been more likely to demonstrate tactile defensiveness [2]. Caregivers

found to be a valuable component of the audiological test battery may be required to restrain or sedate children for ear impressions.

for children with autism [25]. Also of beneﬁt in the audiological The audiologist must ensure that hearing aids are physically set to

evaluation of children with ASD is tone-burst auditory brainstem the developmental level of the child. Equipment can be provided

response (ABR) testing, which is an objective assessment method with a locking battery door and deactivated volume control and

that enables detection of frequency-speciﬁc hearing loss [26]. programming buttons. Retention devices should be used for safety,

Surface electrodes record neurological activity at the level of the especially if the child mouths objects [22].

brainstem in response to auditory stimuli [27]. Tone-burst ABR is No outcomes measure studies could be found on the effective-

the best tool available to approximate the behavioural audio- ness of ampliﬁcation in children with ASD. It is important to obtain

gram in cases where it is not possible to obtain reliable reliable audiological data so that hearing aids can be optimally set;

behavioural assessment data [28]. Sloping and notched hearing hearing aid validation measures are confounded and aural

losses can be identiﬁed using tone-burst ABR. The predictive habilitation outcomes measured are incorrect if ampliﬁcation is

accuracy of tone-burst ABR to behavioural audiological thresh- inappropriately ﬁt.

olds was determined by Stapells et al. [26,29] to be within 20 dB Positive beneﬁts have been reported following the cochlear

for 91–93% of subjects and within 15 dB for 80% of subjects implantation in children with ASD, which include improvements in

[26,29]. social communication, behaviour and environmental awareness,

It is important to note that a peripheral audiological assessment as well as increased vocalizations, eye contact and reaction to

which measures the function of the ear and primary ascending music [33]. Daneshi et al. [34] found that children with ASD

auditory pathways, is limited to testing the integrity of the showed limited development in auditory perception following

peripheral auditory system and does not conﬁrm normal central implantation compared to deaf patients with other secondary

auditory system function [21]. There is a tendency among disabilities. Due to the limited number of reports published on

audiologists to report that hearing is adequate for speech and cochlear implantation in children with ASD, post-implant success

language development once peripheral hearing loss has been ruled of children with ASD cannot be predicted. Pre-operative counsel-

out. Davis cautions against making this claim, as central auditory ling with families is recommended in order to establish realistic

processing deﬁcits can exist in the presence of a normal peripheral expectations [33]. While cochlear implantation is not contra-

auditory system which can impede the acquisition of speech and indicated in children with ASD, Daneshi et al. [34] highlight the

language. Individuals with ASD have reported difﬁculty listening in need for a very unique and team-based habilitation programme in

the presence of background noise and maintaining focus on order to enable auditory skill development.

auditory stimuli [2]. Adults with ASD and normal peripheral

hearing thresholds have reported that though they are able to hear 3.4. Hyper-responsiveness in children with autism spectrum disorder

well, the way in which they process auditory stimuli is inconsistent

and the ability to utilize auditory information declines under Hyper-responsiveness is the pattern of exaggerated beha-

conditions of sensory stress [22]. vioural reactions to sensory stimuli often displayed by children

100 A.N. Beers et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 96–101

with ASD and is an umbrella term that includes hyperacusis, audiological test results. Fitting of hearing aids or cochlear

hypersensitivity, sensory defensiveness, sensory modulation implantation are not contraindicated when hearing loss is present

dysfunction, aversion, avoidance, hyperarousal and lack of in children with ASD. Audiological programming considerations

habituation to sensory stimuli. [35]. While hyper-responsiveness may facilitate acceptance of ampliﬁcation equipment; however,

is not universal among individuals with ASD, it is common and success with these devices can be variable. Children with ASD may

evidence of hyper-responsive behaviours is evaluated in making a display increased perception of loudness and hyper-responsive

differential diagnosis of ASD [36]. An individual’s daily routines, behaviours to auditory stimuli.

quality of peer interactions, and curiosity about the environment

depend on the extent to which stimuli trigger hyper-responsive References

reactions; individuals who demonstrate avoidance of sensory

stimuli engage less in novel play environments and show reduced [1] American Psychiatric Association, Diagnostic and Statistical Manual of Mental

independence in self-care at home [37]. Disorders, 4th ed., Text Revision, American Psychiatric Association, Washington,

DC, 2000.

Khalfa et al. [30] used psychoacoustic tests to measure the

[2] K. Egelhoff, G. Whitelaw, P. Rabidoux, What audiologists need to know about

perception of loudness in children with autism and reported

autism spectrum disorders, Semin. Hear. 26 (November (4)) (2005) 202.

smaller auditory dynamic range and reduced tolerance of loudness, [3] E. Fombonne, The epidemiology of autism: a review, Psychol. Med. 29 (July (4))

(1999) 769–786.

consistent with hyperacusis in this group [30]. Reported preva-

[4] Autism Developmental Disabilities Monitoring Network Surveillance Year 2002

lence rates of hyper-responsiveness among children with ASD vary

Principal Investigators, Centers for Disease Control and Prevention, Prevalence of

from 18% to 53% [8,31,32] and the presence of hyper-responsive- autism spectrum disorders-autism and developmental disabilities monitoring

network 14 sites, United States, 2002, MMWR Surveill. Summ. 56 (2007) 12–28.

ness is often determined based on the inability to tolerate a

[5] M. Elsabbagh, G. Divan, Y.J. Koh, Y.S. Kim, S. Kauchali, C. Marcı´n, et al., Global

component of the audiological test battery [8] or from caregiver

prevalence of autism and other pervasive developmental disorders, Autism Res. 5

report [12,31,32]. Typically developing children do not show (June (3)) (2012) 160–179.

[6] C.P. Johnson, S.M. Myers, From the American Academy of Pediatrics Council on

evidence of hyperacusis [8]. The presence of hyper-responsive

Children with Disabilities. Identiﬁcation and evaluation of children with autism

behaviour can be affected by subject characteristics such as mental

spectrum disorders, Pediatrics 120 (2007) 1183–1215.

age [38] and was found by Baranek et al. to diminish with [7] J.I.C. Howick, P. Glasziou, T. Greenhalgh, C. Heneghan, A. Liberati, I. Moschetti,

et al., The Oxford 2011 Levels of Evidence. Oxford Centre for Evidence-Based

increasing mental age [35]. Baranek et al. [35] concluded that

Medicine, 2011 http://www.cebm.net/index.aspx?o=5653.

hyper-responsiveness may be a more general deﬁcit associated

[8] U. Rosenhall, V. Nordin, M. Sandstro¨ m, G. Ahlse´n, C. Gillberg, Autism and hearing

with developmental disabilities and not speciﬁc to children with loss, J. Autism Dev. Disord. 29 (October (5)) (1999) 349–357.

ASD. [9] A. Tas, R. Yagiz, M. Tas, M. Esme, C. Uzun, A. Karasalihoglu, Evaluation of hearing in

children with autism by using TEOAE A.B.R., Autism: Int. J. Res. Practice 11 (1)

A study by Gomes et al. [39] aimed to verify parent and

(2007) 73–79.

caregiver report of auditory hyper-responsiveness displayed by

[10] R. Jure, I. Rapin, R. Tuchman, Hearing-impaired autistic children, Dev. Med. Child

children with ASD using audiological measures of acoustic Neurol. 33 (December (12)) (1991) 1062–1072.

[11] J. Gravel, M. Dunn, W. Lee, M. Ellis, Peripheral audition of children on the autistic

stapedial reﬂex and behavioural response to high level tonal

spectrum, Ear Hear. 27 (June (3)) (2006) 299–312.

stimuli presented in soundﬁeld. Eleven of 46 children with ASD

[12] A. Tharpe, F. Bess, D. Sladen, H. Schissel, S. Couch, T. Schery, Auditory character-

(24%) were previously diagnosed as clinically hypersensitive to istics of children with autism, Ear Hear. 27 (August (4)) (2006) 430–441.

[13] M.J. Taylor, B. Rosenblatt, L. Linschoten, Auditory brainstem response abnormali-

auditory stimuli; however, only 2 of these 11 children (18.2%)

ties in autistic children, Can. J. Neurol. Sci. 9 (1982) 429–433.

demonstrated discomfort to high level stimuli in soundﬁeld.

[14] C. Gillberg, U. Rosenhall, E. Johansson, Auditory brainstem responses in childhood

Additionally, no signiﬁcant differences existed between children psychosis, J. Autism Dev. Disord. 13 (June (2)) (1983) 181–195.

[15] A. Klin, Auditory brainstem responses in autism: brainstem dysfunction or

with ASD and controls when acoustic stapedial reﬂexes were

peripheral hearing loss? J. Autism Dev. Disord. 23 (March (1)) (1993) 15–35.

analysed [39]. The authors concluded that while many children

[16] V. Wong, S.N. Wong, Brainstem auditory evoked potential study in children with

with ASD are known by their caregivers to display auditory hyper- autistic disorder, J. Autism Dev. Disord. 21 (September (3)) (1991) 329–340.

[17] S.M. Rosenblum, J.R. Arick, D.A. Krug, E.G. Stubbs, N.B. Young, R.O. Pelson,

responsiveness, these behaviours are not a result of hypersensi-

Auditory brainstem evoked responses in autistic children, J. Autism Dev. Disord.

tivities along peripheral auditory pathways. Auditory hyper-

10 (June (2)) (1980) 215–225.

responsiveness in this case could not be quantiﬁed using [18] M. Student, H. Sohmer, Evidence from auditory nerve and brainstem evoked

conventional audiological test methods. responses for an organic brain lesion in children with autistic traits, J. Autism

Child. Schizophr. 8 (1) (1978) 13–20.

Study methodology and subject characteristic variability as

[19] L. Roper, P. Arnold, B. Monteiro, Co-occurrence of autism and deafness: diagnostic

well as the lack of homogeneity in autistic symptoms experienced

considerations, Austim 7 (September (3)) (2003) 245–253.

by individuals with ASD may contribute to the wide prevalence [20] L.J. Anteunis, J.A. Engel, J.J. Hendriks, J.J. Manni, A longitudinal study of the validity

of parental reporting in the detection of otitis media and related hearing im-

range of auditory hyper-responsiveness reported within this

pairment in infancy, Audiology 38 (March–April (2)) (1999) 75–82.

population. Limitations in current research on hyper-responsive-

[21] R. Davis, L. Stiegler, Toward more effective audiological assessment of children

ness are that data are primarily based on caregiver or teacher with autism spectrum disorders, Semin. Hear. 26 (November (4)) (2005) 241.

[22] P. Cloppert, S. Williams, Evaluating an enigma: what people with autism spec-

report [16,31,32] rather than direct measurement. Limited studies

trum disorders and their parents would like audiologists to know, Semin. Hear. 26

have been conducted on the nature of hyper-responsiveness

(November (4)) (2005) 253.

patterns among children with ASD and clinical assessment tools [23] D. Kemp, Stimulated acoustic emissions from within the human auditory system,

J. Acoust. Soc. Am. 64 (November (5)) (1978) 1386–1391.

are not available to objectively measure and differentiate hyper-

[24] T. Balkany, F.F. Telischi, M.J. McCoy, B.L. Lonsbury-Martin, G.K. Martin, Otoacous-

responsiveness from other behaviours [38].

tic emissions in otologic practice, Am. J. Otol. 15 (1994) 29–38.

[25] T.S. Grewe, J.L. Danhauer, K.J. Danhauer, A.R.D. Thornton, Clinical use of otoa-

coustic emissions in children with autism, Int. J. Pediatr. Otorhinolaryngol. 30

4. Conclusion

(August (2)) (1994) 123–132.

[26] D.R. Stapells, J.S. Gravel, B.A. Martin, Thresholds for auditory brain stem responses

Prevalence rates of hearing impairment among individuals with to tones in notched noise from infants and young children with normal hearing or

ASD continue to be debated. At present there is no conclusive sensorineural hearing loss, Ear Hear. 16 (August (4)) (1995) 361–371.

[27] D.L. Jewett, J.S. Williston, Auditory-evoked far ﬁelds averaged from the scalp of

evidence that children with ASD are at increased risk of peripheral

humans, Brain 94 (1971) 681–696.

hearing loss. A complete audiological assessment is recommended

[28] Joint Committee on Infant Hearing, From the American Academy of Pediatrics:

in all cases where ASD is suspected or conﬁrmed, so as not to delay Year 2007 position statement: principles and guidelines for early hearing detec-

tion and intervention programs, Pediatrics. 120 (October (4)) (2007) 898–921.

the diagnosis of hearing impairment in the event that hearing loss

[29] D.R. Stapells, T.W. Picton, A. Durieux-Smith, C.G. Edwards, L.M. Moran, Thresholds

and ASD co-exist. Objective assessment measures should be used

for short-latency auditory-evoked potentials to tones in notched noise in normal-

to conﬁrm behavioural testing in order to ensure reliability of hearing and hearing-impaired subjects, Audiology 29 (5) (1990) 262–274.

A.N. Beers et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 96–101 101

[30] S. Khalfa, N. Bruneau, B. Roge, N. Georgieff, E. Veuillet, et al., Increased perception [36] G.T. Baranek, L.D. Parham, J.W. Bodﬁsh, Sensory and motor features in autism:

of loudness in autism, Hear. Res. 198 (September) (2004) 87–92. assessment and intervention, in: F. Volkmar, A. Klin, R. Paul (Eds.), 3rd ed.,

[31] F.R. Volkmar, D.J. Cohen, R. Paul, An evaluation of DSM-IIl criteria for infantile Handbook of Autism and Pervasive Developmental Disorders: Assessment, Inter-

autism, J. Am. Acad. Child Psychiatry 25 (March (2)) (1986) 190–197. ventions and Policy, vol. 2, John Wiley &Sons, Hoboken, NJ, 2005, pp. 831–857.

[32] G.T. Baranek, L.E. Gutermuth, G. Berkson, Sensory defensiveness in persons [37] G.T. Baranek, Y.H. Chin, L.M. Greiss, J.G. Yankee, D.D. Hatton, S.R. Hooper, Sensory

with developmental disabilities, Occup. Ther. J. Res. 17 (February (2)) (1997) processing correlates of occupational performance in children with fragile X

173–185. syndrome: preliminary ﬁndings, Am. J. Occup. Ther. 56 (September-October

[33] A.I. Donaldson, K.S. Heavner, T.A. Zwolan, Measuring progress in children with (5)) (2002) 538–546.

autism spectrum disorder who have cochlear implants, Arch. Otolaryngol. Head [38] G.T. Baranek, F.J. David, M.D. Poe, W.L. Stone, L.R. Watson, Sensory experiences

Neck Surg. 130 (May (5)) (2004) 666–671. questionnaire: discriminating sensory features in young children with autism,

[34] A. Daneshi, S. Hassanzadeh, Cochlear implantation in prelingually deaf persons developmental delays, and typical development, J. Child Psychol. Psychiatry 47

with additional disability, J. Laryngol. Otol. 121 (July (7)) (2007) 635–638. ((June) 6) (2006) 591–601.

[35] G.T. Baranek, B.A. Boyd, M.D. Poe, F.J. David, L.R. Watson, Hyperresponsive sensory [39] E. Gomes, N.T. Rotta, F.S. Pedrosa, P. Sleifer, M.C. Danesi, Auditory hypersensitivity

patterns in young children with autism, developmental delay, and typical devel- in children and teenagers with autistic spectrum disorder, Arq. Neuropsiquiatr. 62

opment, Am. J. Ment. Retard. 112 (July (4)) (2007) 233–245. (3-B) (2004) 797–801. International Journal of Audiology

ISSN: 1499-2027 (Print) 1708-8186 (Online) Journal homepage: http://www.tandfonline.com/loi/iija20

Evidence of binaural integration benefits following ARIA training for children and adolescents diagnosed with amblyaudia

Deborah Moncrieff, William Keith, Maria Abramson & Alicia Swann

To cite this article: Deborah Moncrieff, William Keith, Maria Abramson & Alicia Swann (2017) Evidence of binaural integration benefits following ARIA training for children and adolescents diagnosed with amblyaudia, International Journal of Audiology, 56:8, 580-588, DOI: 10.1080/14992027.2017.1303199 To link to this article: https://doi.org/10.1080/14992027.2017.1303199

Published online: 27 Mar 2017.

Submit your article to this journal

Article views: 154

View related articles

View Crossmark data

Citing articles: 1 View citing articles

Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=iija20 International Journal of Audiology 2017; 56: 580–588

Original Article

Evidence of binaural integration benefits following ARIA training for children and adolescents diagnosed with amblyaudia

Deborah Moncrieff1, William Keith2, Maria Abramson3 & Alicia Swann4

1Department of Communication Science and Disorders, School of Health and Rehabilitation Sciences, University of Pittsburgh, Pittsburgh, PA, USA, 2SoundSkills APD Clinic, Auckland, New Zealand, 3Hear Now/Abramson Audiology, Laguna Niguel, CA, USA, and 4Auditory Processing Center, LLC, Clinton, MS, USA

Abstract Objective: The purpose of this study was to demonstrate the efficacy of Auditory Rehabilitation for Interaural Asymmetry (ARIA) to improve dichotic listening scores in children and adolescents diagnosed with amblyaudia and other binaural integration deficits. Design: The study is a field experiment without randomisation. Study: Participants placed into groups based on dichotic listening test scores received four sessions of ARIA training. Baseline scores were compared to performance during the final session of training and to scores obtained 2 or more months after completion of ARIA. Sample: A total of 125 children participated at five different clinical sites. Results: Dichotic listening scores improved across all participants. Post hoc analyses demonstrated highly significant gains in non-dominant ear performance and reductions of interaural asymmetry among participants diagnosed with amblyaudia at both post-ARIA measurements. Participants in other diagnostic groups also showed significant benefits for some post-ARIA measures. Conclusions: Results demonstrate that ARIA training is an effective method for improving binaural integration skills among children and adolescents identified with dichotic listening weaknesses during assessments for auditory processing disorder (APD), especially for those diagnosed with amblyaudia. Benefits achieved following ARIA training remain stable across several months.

Key Words: Auditory processing; amblyaudia; dichotic listening; behavioural measures; paediatric; psychoacoustics/hearing science; speech perception

Amblyaudia is a type of auditory processing disorder (APD) hemisphere, which is the right ear in 65–80% of normal listeners characterised by deficits in the binaural integration of verbal (Hiscock et al, 2000; Moncrieff, 2011) and the left ear in others information (Moncrieff, 2010). The hallmark pattern of amblyaudia (Denes & Caviezel, 1981). Because either the left or right ear can be is an abnormally large asymmetry between the two ears during dominant during DL tests, a diagnosis of amblyaudia is based on dichotic listening (DL) tasks with either normal or below normal comparison to norms developed without regard to which ear is performance in the dominant ear (Moncrieff et al, 2016). Children superior (Moncrieff, 2011; Moncrieff et al, 2016). with listening, learning and reading difficulties have produced When assessed under earphones, normal listeners can readily symmetrical deficits in binaural integration (Hynd et al, 1979; identify information presented to their non-dominant ear during DL Keefe & Swinney, 1979; Pelham, 1979; Tobey et al, 1979; Harris et tasks, long thought to be primarily through cortical connections via al, 1983; Roush & Tait, 1984; Grogan, 1986; Vanniasegaram et al, the corpus callosum because after sectioning of the corpus 2004; Pinheiro et al, 2010) as well as an abnormally large callosum, a listener’s ability to identify material presented to the asymmetry during DL tasks due to low performance in their non- non-dominant ear is extinguished (Sparks & Geschwind, 1968). In dominant ears (Ayres, 1977; Johnson et al, 1981; Dermody et al, those patients, however, the performance improves following a 1983; Aylward, 1984; Berrick et al, 1984; Asbjornsen et al, 2000; reorganisation of the auditory pathways despite the absence of Vanniasegaram et al, 2004; Moncrieff & Black, 2008). The callosal transmission (Milner et al, 1968; Levitsky & Geschwind, structural model of DL (Kimura, 1961) predicts enhanced perform- 1968; Springer et al, 1978). This suggests that binaural integration ance in the ear that is contralateral to the language dominant cortical of dichotic material engages both contralateral and ipsilateral

Correspondence: Deborah Moncrieff, Ph.D., Department of Communication Science and Disorders, University of Pittsburgh, 6035 Forbes Tower, Pittsburgh, PA 15260. Tel: (412) 648-0156, Fax: (412) 383-6555. E-mail: [email protected]

(Received 15 July 2016; revised 4 February 2017; accepted 15 February 2017) ISSN 1499-2027 print/ISSN 1708-8186 online ß 2017 British Society of Audiology, International Society of Audiology, and Nordic Audiological Society DOI: 10.1080/14992027.2017.1303199 Evidence of binaural integration benefits 581 ascending pathways, probably starting in the brainstem. During therapies for stroke patients and visual training for patients with most listening experiences, individuals must fuse acoustic cues that amblyopia whereby performance from the dominant side is are similar and separate cues that are different between the two ears suppressed during targeted exercises, resulting in enhancement on in a process that is similar to binaural integration because we rarely the non-dominant side. The relative intensity between the two ears process sounds from an isolated single source without the influence must be carefully controlled throughout training to avoid over- of competing acoustic signals. We use interaural timing and training to a reversed condition of amblyaudia whereby the non- intensity differences from binaural cues for spatial listening and dominant ear becomes excessively dominant over the formerly localisation, the capacity of our auditory system to interpret dominant ear as inadvertently demonstrated in one study utilising a information arriving along different paths, through processes that similar method (Denman et al, 2015). Because of a risk of over- are mediated by excitatory and inhibitory mechanisms in the training to a reversed abnormal interaural asymmetry, ARIA superior olivary complex of the brainstem (Middlebrooks & Green, requires individualised treatment by an audiologist with access to 1991; Blauert, 1997). Binaural spatial hearing is a process that is audiometric equipment and appropriate training in the labile during early development (Keating & King, 2013) but may methodology. stabilise by age 4 to 5 years (Litovsky, 2011), which could be why The purpose of this study was to evaluate the efficacy of ARIA children begin to perform DL tasks at age 5 (Moncrieff, 2011). By at several clinical sites in the hands of trained audiologists. At each young adulthood, normal binaural integration is characterised by site, participants were assessed for APD and were enrolled in ARIA strong performance in both ears with only a small interaural treatment based on results from DL tests. We hypothesised that asymmetry, indicating that cues from both ears are processed participants would demonstrate benefits from ARIA intervention effectively during these challenging listening tasks. DL perform- with improvements in non-dominant ear performance after training. ance is also related to global top-down factors such as verbal working memory, attention, receptive language and neurophysiolo- Methods gic maturation that depend on bottom-up auditory processing. Management recommendations for APD have included a variety Children and adolescents ages 5 to 19 years (n ¼ 125) were enrolled of bottom-up auditory skill training strategies focussed on sensory in ARIA at five clinical sites: Children’s Hospital of Pittsburgh, detection and discrimination without specificity for the type of Pittsburgh, PA; the Auditory Neurophysiology Laboratory at the auditory deficit diagnosed (Chermak et al, 1999). Critics of a University of Pittsburgh, Pittsburgh, PA; SoundSkills APD Clinic in speech-based auditory training programme proposed for children Auckland, New Zealand; HearNow Abramson Audiology in Laguna with any type of APD and/or language difficulties argued that those Niguel, CA; and Auditory Processing Center in Clinton, MS. kinds of programme primarily influence sustained attention and fail Demographic information regarding the participants enrolled in the to generalise beyond trained skills (Moore, 2011). Auditory study is shown in Table 1. All were enrolled in ARIA following Rehabilitation for Interaural Asymmetry (ARIA) is a clinical APD assessment if they demonstrated weaknesses across at least training regimen designed to specifically rehabilitate the DL deficit two tests of auditory processing (including at least one DL test), had behind amblyaudia. In Phase I clinical trials conducted at the reported difficulties listening and learning at school, and had normal University of Florida in 2000–2001 (Moncrieff & Wertz, 2008), hearing sensitivity for pure tones525 dB HL from 500 to 4000 Hz. participation in ARIA led to reduced interaural asymmetry from DL All children were proficient speakers of English. tasks in children with binaural integration deficits consistent with Participants were assessed with the Randomized Dichotic Digits amblyaudia. In addition, some children also attained grade-level Test (RDDT) (Strouse & Wilson, 1999), the Dichotic Words Test performance in listening comprehension after ARIA, a skill that was (Moncrieff, 2011) and/or the Dichotic Digits Test (Musiek, 1999), not specifically trained. ARIA initially involved a 30-minute all administered under earphones through a clinical audiometer set training session three times per week for a period of 4–6 weeks. to 50 dB HL bilaterally. Results were compared to normative To increase access during the school year, ARIA was modified in information according to the protocol used to diagnose amblyaudia 2008 to provide two 20 min sessions divided by a 20 min rest break (AMB), dichotic dysaudia (DD), and amblyaudia plus (AMB+) at four weekly appointments. A comparison of the three-per-week (Moncrieff et al, 2016). 81 (65%) were given diagnostic labels versus one-per-week modes of conducting ARIA demonstrated AMB (n ¼ 58), AMB + (n ¼ 16), or DD (n ¼ 7). Of the remaining 44 results that were similar to and in some cases, better, under the new participants, 19 of them produced abnormal results on two DL tests, mode of administration (Russo et al, 2014). but performance patterns were mixed (MIX). The remaining 25 Researchers have reported improvements in DL scores in normal young adults (Tallus et al, 2015) and in children identified with Table 1. Demographic information for subjects enrolled in ARIA. APD (Tawfik et al, 2015) following a training protocol that presented dichotic material at the same intensity to both ears. In Age n M F AMB AMB+ DD MIX UND another study, children who received integrative auditory training 5–6 11 6 5 7 1 0 2 1 with diotic and dichotic speech-based materials in quiet and in noise 725151084427 showed improvements compared to children who did not receive the 820164102134 treatment (Putter-Katz et al, 2008). During ARIA, a clinician 9251411135034 systematically adjusts the relative intensity of input to the two ears 10 16 12 4 9 0 0 4 3 during DL exercises presented through sound-field speakers. The 11–12 14 14 0 4 2 2 2 4 training is conducted in sound field to increase ecologic validity 13–15 8 6 2 4 1 0 2 1 with situations similar to those encountered in daily listening 16–19 6 4 2 3 1 0 1 1 Total 125 87 38 58 16 7 19 25 experiences (Jerger et al, 2000) and to differentiate the exercises from presentations made under earphones during diagnostic AMB: amblyaudia; AMB+:amblyaudia plus dichotic dysaudia; DD: assessment. The approach is similar to constraint-induced physical dichotic dysaudia; MIX: mixed pattern; UND: undiagnosed. 582 D. Moncrieff et al. produced abnormal results on only one DL test and were therefore Table 2. Significant effects of age group on individual scores. undiagnosed with respect to binaural integration alone (UND). Test Score J–T statistic Levelsa p Participants in the UND category also performed below normal on another test of auditory processing so they qualified for a diagnosis RDDT Non-dominant ear 5.20 6 50.001 5 of APD based on ASHA (2005) and AAA (2010) standards. Dominant ear 4.65 6 0.001 Interaural asymmetry 2.92 6 0.004 All participated in ARIA during four weekly one-hour appointments that involved 20 min of dichotic training followed by 20 min DWT Non-dominant ear 2.66 4 0.008 of rest and then followed with another 20 min of dichotic training. Dominant ear 1.28 4 0.201 Interaural asymmetry 1.95 4 0.051 Dichotic training material was comprised of words (digits, mono- syllables and spondees) spoken in standard American English RDDT: Randomized Dichotic Digits Test; DWT: Dichotic Words presented in a sound-treated booth or quiet room through sound- Test; J-T Statistic is from the Jonckheere–Terpstra Test. field speakers. Each dichotic presentation was aligned at onset and aLevels refers to the number of age groups in the analysis. normalised for average RMS amplitude across its duration. The intensity of output directed toward the non-dominant ear was held at 50 dB HL while the intensity of output directed toward the dominant different only for digits and scores for interaural asymmetry were ear was adjusted throughout each training session. As the purpose of minimally significant for words. As shown in Figure 1, dominant the training is to minimise interaural asymmetry while listening to (dom) and non-dominant (nondom) ear scores increased and values dichotic material, the goal with each presentation was to maintain for interaural asymmetry decreased with maturation. Results are differences between the two ears close to or below 10%. Clinicians displayed within age groups defined for establishing normative data selected a list of dichotic words and measured performance after for scores from each test. Average ear scores and interaural 15–25 presentations. The order of material was not specified and if asymmetry are separated by diagnostic category and displayed in a child had more difficulty with one type of material (words, digits), Figure 2. The large asymmetry between the two ears that is the training was usually more focussed on that material than the other hallmark pattern of amblyaudia is apparent in the lower non- (digits, words), but both types of material were always used. dominant ear scores and large values for interaural asymmetry in Intensity was reduced for the dominant ear when relative perform- the AMB and AMB + groups. Participants in the other diagnostic ance was better on that side by more than 10% and increased when groups (DD, MIX and UND) demonstrated abnormal but higher relative performance on the other side was better by more than 10%. non-dominant ear scores and lower values of interaural asymmetry. Adjustments to intensity were made in steps of 1 dB while continually monitoring performance differences in the two ears as detailed previously (Moncrieff & Wertz, 2008). Effect of ARIA training on DL scores at fourth week At the fourth training session, scores for most of the participants Scores from the pre-ARIA evaluation were compared to scores were recorded from one list of dichotic words (n ¼ 119) and one list during the fourth session by the Wilcoxon signed ranks test. Scores of two-pair dichotic digits (n ¼ 116). A subset of these (n ¼ 70) were significantly different except for dominant ear scores during returned to the clinic at least 2 months following the end of the the words test as detailed in Table 3. A separate analysis for treatment and were reassessed for their DL skills. Scores from the significant differences within each diagnostic group was done by pre-ARIA assessment, the fourth training session, and the post- the Wilcoxon signed ranks test with Bonferroni’s correction to ARIA assessment were used to measure task-specific outcomes adjust significance level to p50.01 for five separate groups. As from training. shown in the left column of Figure 3, non-dominant ear scores improved significantly for participants in the AMB, AMB + and MIX groups for both words and digits. Participants in the UND Statistical methods diagnostic group demonstrated significant improvement for digits only. The goal of ARIA training was to improve non-dominant ear The Kolmogorov–Smirnoff test determined that DL scores were not performance, especially among those whose interaural asymmetry normally distributed, even after applying rationalised arcsine was largest because of non-dominant ear weakness. As expected, transforms to the raw data. Therefore, DL scores for the dominant the greatest gains in non-dominant ear performance and reductions and non-dominant ears and values of interaural asymmetry were in interaural asymmetry were seen in the AMB and AMB + group subjected to nonparametric analyses of variance. Between-partici- whose non-dominant ear performance was lowest at diagnosis (see pant factors of age were compared for significant differences prior Figure 2). The only group that showed a significant improvement in to ARIA training. Scores before and after ARIA treatment were their dominant ears (not shown) were those in the UND group who compared across all participants and within each diagnostic group demonstrated a significant increase with digits at the fourth training for significant effects at the fourth training session and at the post- session, Z ¼2.70, p ¼ 0.007. ARIA assessment. Comparisons were made between scores obtained at the fourth session and the post-ARIA assessment to determine if gains were maintained or if regression had occurred. Effect of ARIA training on DL scores after several months A subset of 70 participants returned to three clinical sites for a post- Results ARIA follow-up evaluation (35 AMB, 11 AMB+, 3 DD, 7 MIX and 14 UND). All were assessed with 2-pair digits and 67 were assessed Effect of age and diagnosis on pre-ARIA DL scores with dichotic words at a time between 2 and 12 months following Based on results from the Jonckheere–Terpstra test, non-dominant completion of training, with most done between 3 and 6 months. ear scores and interaural asymmetry were significantly different for Post-ARIA scores were significantly higher than pre-ARIA diag- age group (Table 2). Dominant ear scores were significantly nostic scores for dominant ear and non-dominant ear and were Evidence of binaural integration benefits 583

Figure 1. Age-related changes in scores in the non-dominant (nondom) ears (dashed lines) and dominant (dom) ears (solid lines) for the two-pairs condition of the Randomized Dichotic Digits Test (RDDT) (top) and the Dichotic Words Test (DWT) (bottom). Age- related changes in interaural asymmetry (earadv) for both tests are displayed by the dash-dot lines. Age groups are defined by the ranges used for establishing normative values for each t.

significantly lower for interaural asymmetry across both tests as shown in Table 3. Results within each diagnostic group demonstrated significantly better post-ARIA non-dominant ear scores among the AMB, AMB + and UND groups for digits and among the AMB and AMB + groups for words as shown on the right side of Figure 3. Post-ARIA improvements in the dominant ear were also Figure 2. Average scores in the non-dominant (nondom) (grey significant for the UND group with digits, Z ¼2.67, p ¼ 0.007, bars) and dominant (dom) (black bars) for the digits test (top) and and words, Z ¼2.95, p ¼ 0.003 (not shown). Participants in the words test (bottom) for participants in each diagnostic group. AMB: AMB group showed significant reductions in interaural asymmetry amblyaudia; AMB+: amblyaudia plus dichotic dysaudia; DD: with both digits and words, but those in the AMB + group showed dichotic dysaudia; MIX: mixed; UND: undiagnosed. significant reductions only with words. There were no significant post-ARIA changes in interaural asymmetry in the DD, MIX or UND groups. by the Friedman test, a non-parametric analysis of repeated measures. Significant differences were found across all measures as shown in Table 4. To determine which intervals resulted in Comparison of scores at initial diagnosis, fourth session and significant differences, post hoc analyses with Wilcoxon signed at post-ARIA evaluation ranks tests with Bonferroni’s adjustment were run with the For those whose performance was measured on all three occasions significance level adjusted to p50.017 to accommodate three (pre-ARIA, fourth session and post-ARIA), scores were compared intervals. As detailed in Table 5, non-dominant ear and interaural 584 D. Moncrieff et al.

Table 3. Main effects of ARIA on dichotic listening scores. recommend testing with ‘‘Dichotic Listening (Speech) Tests’’ for Test Score Z score n p individuals suspected of having an APD. A survey of audiologists reported that a majority use DL tests to assess patients (Emanuel et (a) Significant effects of ARIA on scores at fourth session. al, 2011). DL tests have been utilised for over 50 years and new RDDT Non-dominant ear 8.24 116 50.001 normative information is available for assessing performance in the Dominant ear 2.54 116 0.011 non-dominant and dominant ears so that direction of ear advantage Interaural asymmetry 7.34 116 50.001 does not influence measures of interaural asymmetry (Moncrieff, DWT Non-dominant ear 8.18 119 50.001 2011; Moncrieff, 2015). All study participants demonstrated Dominant ear 0.128 119 0.898 binaural integration weaknesses and 65% of them were diagnosed Interaural asymmetry 8.14 119 50.001 following consistent patterns from two DL tests: 46% as AMB, 13% (b) Significant effects of ARIA on scores at post-ARIA evaluation. as AMB + and 6% as DD. Another 15% who demonstrated RDDT Non-dominant ear 6.71 70 50.001 inconsistent abnormal performance were designated MIX and Dominant ear 2.67 70 0.008 20% who demonstrated abnormal performance on only one DL Interaural asymmetry 5.99 70 50.001 test were designated UND. The high prevalence of scores DWT Non-dominant ear 6.01 67 50.001 designating AMB or AMB + demonstrates that clinically significant Dominant ear 3.92 67 50.001 DL deficits in the non-dominant ear are common among children Interaural asymmetry 5.65 67 50.001 and adolescents referred for APD assessment. Since the primary RDDT: Randomized Dichotic Digits Test; DWT: Dichotic Words purpose of clinical assessment should be to direct remediation, Test. training methods designed to improve binaural integration perform- Z score is from the Wilcoxon signed ranks test. ance with competing signals are useful to treat the large number of n refers to the number of participants whose scores were analysed. children and adolescents suffering from the effects of amblyaudia. Participants in other diagnostic groups also benefitted from participation in ARIA. Those diagnosed as DD from relatively symmetrical but weak performance in both ears during DL tests asymmetry measures were significantly different between the pre- demonstrated higher ear scores and lower interaural asymmetry, but ARIA evaluation and the fourth session in this subgroup of none of their results achieved significance. As there were only participants, with results that are highly similar to results obtained seven participants diagnosed with DD and only three for whom from the larger group of participants enrolled in the study (see Table post-ARIA scores were measured, it is not known whether a larger 3). Scores between pre- and post-ARIA evaluations were also number of participants with the DD diagnosis would have shown highly significant across all measures and similar to results from the significant improvements following training. Those designated MIX larger group (see Table 3). The only scores that showed a significant demonstrated inconsistent but abnormal performance on two DL change between the fourth session and post-ARIA were the tests and showed significant improvements in their non-dominant dominant ear scores with words. This significant change in ears at the fourth session, but not at the post-ARIA assessment when dominant ear performance with words did not represent post- there were scores available for only seven of them. When examined training regression, however, but instead reflected a continued individually, however, only one of them continued to demonstrate improvement in the participants’ ability to process dichotic words in an abnormal asymmetry of 20% at the post-ARIA evaluation with their dominant ears as shown in Figure 4. words, a difference that was only 2% above the normal cut-off The post-ARIA improvement in the dominant ear with words criterion for that age. This would suggest that for children and also led in some cases to an increase in interaural asymmetry. adolescents whose abnormal performance is inconsistent, ARIA Within this subgroup for whom measures were obtained at all three may be able to provide improvement for their binaural integration times, average interaural asymmetry increased from under 12% to skills. More data from a larger subset of participants with the DD over 18% between the fourth session and the post-ARIA evaluation. diagnosis and MIX designation may better determine if they may Among those diagnosed as AMB and AMB+, nearly half of them significantly benefit from ARIA training. demonstrated interaural asymmetry with dichotic words greater than Those designated UND demonstrated abnormal performance on 10% at their post-ARIA evaluations. This same pattern did not only one DL test, either with words or with digits. They occur for participants in the DD, MIX or UND groups. This demonstrated significant improvements in non-dominant ears with suggests that despite gains in non-dominant ears, there may be a digits at the fourth training session and the smaller subgroup of residual degree of amblyaudia present among some of these them who returned for a post-ARIA assessment demonstrated participants resulting in a tendency for the dominant ear to suppress significant improvements in non-dominant ears with both digits and non-dominant ear performance. Pre- and post-ARIA values of words. It was anticipated that significant gains would be observed interaural asymmetry for participants with amblyaudia when tested only among those participants who demonstrated a consistent with dichotic words are displayed in Figure 5. All but three of the pattern of weakness across both DL tests and not among those participants with amblyaudia showed reductions in interaural whose weakness was apparent on only one DL test. A majority of asymmetry, two participants (one 7-year-old and one 16-year-old) UND group participants who returned for post-ARIA evaluations showed an increase in interaural asymmetry after training, and one had produced the AMB or AMB + pattern on the one DL test that 8-year-old showed the same value as before ARIA. was abnormal (9 of the 14). The significant improvements seen in this subgroup suggests that even when DL testing falls below Discussion normal for only one test, children and adolescents diagnosed with APD who show the AMB or AMB + pattern on one DL test may still ASHA (2005) recommends assessment of ‘‘auditory performance benefit from enrolment in ARIA. Others in the UND group in competing signals’’ and the AAA Clinical Practice Guidelines demonstrated the DD pattern on one DL test and all showed large Evidence of binaural integration benefits 585

Figure 3. Left column displays results from the group of participants whose scores were measured at the fourth session of ARIA (words, n ¼ 119; digits, n ¼ 117): Outcomes measured during the pre-ARIA evaluation (grey bars) and during the 4th ARIA session (black bars) for participants in each diagnostic group (a) for non-dominant ears from the 2-pairs condition of dichotic digits; (b) for non-dominant ears from dichotic words and (c) for interaural asymmetry measured from digits and words; Right column displays results from the subgroup of participants whose scores were measured at pre-ARIA (grey bars) and post-ARIA (black bars) evaluation (words, n ¼ 67; digits, n ¼ 70); (d) for non-dominant ears from the 2-pairs condition of dichotic digits; (e) for non-dominant ears from dichotic words; (e) for interaural asymmetry measured from digits and words. Significance values are represented by bars, p50.001***, p50.01**, p50.05*. gains in both ears following ARIA training, suggesting that children Benefits are maximal, though, for children and adolescents whose and adolescents who perform poorly and symmetrically on DL tests binaural integration skills are consistently weak across two different may also benefit from participation. DL tests, especially when the pattern leads to the diagnosis of Results from this study suggest that ARIA training benefits amblyaudia. As the ARIA training paradigm requires that the individuals whose performance on a DL test falls below normal clinician continually adjust the relative intensity of material which is not surprising since the training focuses on dichotic tasks. presented through the sound-field speakers, participants with poor 586 D. Moncrieff et al.

Table 4. Significant differences between scores at initial evaluation, fourth session and post-ARIA. Test Condition V 2 df np RDDT Non-dominant ear 67.99 2 70 50.001 Dominant ear 9.33 2 70 0.009 Interaural asymmetry 26.82 2 70 50.001 DWT Non-dominant ear 51.54 2 67 50.001 Dominant ear 18.73 2 67 50.001 Interaural asymmetry 54.87 2 67 50.001 RDDT: Randomized Dichotic Digits Test; DWT: Dichotic Words Test. V 2 score is from the Friedman test, a nonparametric alternative to repeated measures ANOVA.

Table 5. Post hoc analyses of differences between scores at initial evaluation, fourth session and post-ARIA. Test Score Test interval Z score n p RDDT Non-dominant ear Pre-ARIA to session 4 5.61 66 50.001 Dominant ear 2.23 0.026 Ear advantage 5.12 50.001 DWT Non-dominant ear 5.99 66 50.001 Dominant ear 0.99 0.320 Ear advantage 6.08 50.001 RDDT Non-dominant ear Pre-ARIA to post-ARIA 6.71 70 50.001 Dominant ear 2.67 0.008 Ear advantage 5.99 50.001 DWT Non-dominant ear 6.07 68 50.001 Dominant ear 3.89 50.001 Ear advantage 5.71 50.001 RDDT Non-dominant ear Session 4 to post-ARIA 0.75 66 0.453 Dominant ear 0.42 0.676 Ear advantage 0.33 0.743 Figure 4. Average results for participants who were assessed at all three intervals. Top panel shows non-dominant ear scores for digits DWT Non-dominant ear 0.19 64 0.852 and words at each test time, pre-ARIA, fourth session and post- Dominant ear 4.10 50.001 Ear advantage 3.34 0.001 ARIA. Bottom panel shows dominant ear scores for digits and words at each test time. Significance values are represented by bars, RDDT: Randomized Dichotic Digits Test; DWT: Dichotic Words p50.001***, p50.01**, p50.05*. Test. Z score is from the Wilcoxon signed-ranks test. colliculus, medial geniculate body, and auditory cortex, are but symmetrical DL performance can do most of their training work sensitive to the neural patterns generated by ILDs and use them at relatively equal intensities throughout the training sessions. This to encode spatial sensitivity and location. We hypothesise that the individualised and adaptive approach allows the clinician to ILDs used during ARIA training promote increased activation along structure the training regimen to the specific performance charac- the non-dominant ear’s auditory pathway, thereby inducing teristics of each participant in order to maximise gains across tasks neuroplastic changes that lead toward more symmetrical binaural with dichotic digits and words. integration of verbal material. The systematic adjustments to intensity of presentations to the Sound source location deficits have been reported following dominant ear throughout ARIA depended on the performance in the conductive hearing loss in several animal studies (Slattery & participant’s non-dominant ear. The rationale behind this approach Middlebrooks, 1994; Hartley et al, 2003; Lupo et al, 2011). Even is based on the anatomical and physiological correlates of the long after conductive hearing loss has cleared, children have been duplex theory of sound localisation (Tollin & Yin, 2002). Interaural shown to have binaural localisation deficits (Hall et al, 1998; level differences (ILDs) produced by presenting information to the Roberts et al, 2004). Sound localisation depends on the integration non-dominant ear at a higher relative intensity level should result in of binaural cues, especially in environments with varied sound higher discharge rates in neurons in the ipsilateral lateral superior sources (Blauert, 1997). However, a clear relationship between olive (LSO), leading to an enhanced sensitivity to information chronic otitis media, sound localisation deficits, and performance on arriving at that side and reduced sensitivity to presentations at the DL tests has not been established. Whether participants in this study contralateral ear (Boudreau & Tsuchitani, 1968). Neurons in higher had histories of chronic otitis media is not known, but information level structures along the auditory pathway, i.e. the inferior relating significant histories of conductive hearing loss with Evidence of binaural integration benefits 587

(Moncrieff & Wertz, 2008) in order to evaluate listening and learning skills outside of those used during a standard APD assessment and/or during ARIA training. As this was a clinical study designed to show that multiple practitioners at several clinical sites could produce similar DL outcomes across children diagnosed with amblyaudia, it was beyond its scope to add additional outside evaluations of broader skills needed for classroom performance. A randomised controlled trial is an appropriate next step to evaluate the direct benefits of ARIA on DL performance and to further investigate how improvements might generalise to listening and language skills that can enhance learning in children diagnosed with amblyaudia. In such a trial, all training methods including the number of stimuli presented and the rules regarding changes to interaural intensity throughout ARIA training will also be standardised.

Conclusions Figure 5. Individual scores for interaural asymmetry when tested with dichotic words for participants diagnosed with amblyaudia pre- ARIA training produced significant gains in DL test scores across ARIA (grey bars) and post-ARIA (black bars). participants diagnosed with an APD with the greatest benefits observed among those diagnosed with amblyaudia. Given that the primary focus of ARIA is to enhance performance in the listener’s binaural integration test results would be of interest in managing non-dominant ear, it is not surprising that listening to dichotically deficits in children suspected of having APD. presented words throughout 4 weeks of training led to better scores Children with normal post-ARIA performance should be in these tasks. Scores at the fourth session are likely to reflect the discharged from further treatment and those whose performance highest levels of performance because they are derived from direct remains below normal should receive appropriate recommenda- training-to-the-test, but even after intervals from 2 to 12 months, tions. Even with significant improvements following 4 weeks of significant improvements in non-dominant ear scores were main- ARIA, some scores may not achieve normal levels in children with tained. With words, dominant ear scores continued to improve severe pre-ARIA deficits. If interaural asymmetry remains high following the end of ARIA training suggesting enhanced ability to because of abnormally low performance in the non-dominant ear or process verbal material. Maintenance of binaural integration skills abnormally high performance in the dominant ear, another 4-week for months after training, as seen in those who returned for post- session of ARIA is recommended. If interaural asymmetry is normal ARIA assessments, suggests that the training led to continuing but performance is abnormally low in both ears, use of a remote benefit with minimal or no regression. microphone hearing aid (‘‘FM’’) system at school is recommended (Reynolds et al, 2016). A bilateral hearing assistive device is likely to be more beneficial for a child who does not have a large Declaration of interest: The authors report no conflicts of interest. asymmetry when the two ears are placed in competition, so greater The authors alone are responsible for the content and writing of this benefit may be achieved once the two ears are integrating binaural article. information more symmetrically. This recommendation for use of a bilateral hearing assistive device is based on clinical experience, so research evidence of greater benefit from remote microphone assistive devices following treatment with ARIA is needed. References Benefits derived from participation in ARIA across other auditory processing measures will be addressed in a subsequent American Academy of Audiology (AAA). 2010. Practice guidelines for the diagnosis, treatment, and management of children and adults with manuscript. It is hypothesised that binaural integration deficits may central auditory processing disorder (CAPD). http://www.audi- underlie listening and learning difficulties and that ARIA training ology.org/publications-resources/document-library/central-auditory-pro- may result in improvements in other auditory processing skills that cessing-disorder were found to be deficient during an initial evaluation for APD. American Speech-Language-Hearing Association (ASHA). 2005. (Central) Follow-up research will assess other auditory processing skills such auditory processing disorders [Technical Report]. www.asha.org/policy. as temporal resolution, speech perception in noise, spatial stream Aylward, E.H. 1984. Lateral asymmetry in subgroups of dyslexic children. segregation, and pattern recognition for outcomes following ARIA Brain Lang, 22, 221–231. training. Asbjornsen, A., Holmefjord, A., Reisaeter, S., Moller, P., Klausen, O., et al. A limitation of this study is that not all of the clinical sites 2000. Lasting auditory attention impairment after persistent middle performed post-ARIA assessments. Clinicians providing ARIA ear infections: A dichotic listening study. Dev Med Child Neurol, 42, should have patients return for a follow-up assessment at a time 481–486. Ayres, A.J. 1977. Dichotic listening performance in learning-disabled between 3 and 6 months following the end of the treatment and they children. Am J Occup Ther, 31, 441–446. should use the same tests at the diagnostic evaluation and the post- Berrick, J.M., Shubow, G.F., Schultz, M.C., Freed, H., Fournier, S.R., et al. ARIA assessment in order to adequately compare outcomes across a 1984. Auditory processing tests for children: Normative and clinical variety of auditory processing skills. In the second initial trial of results on the SSW test. J Speech Hear Disorders, 49(3), 318–325. ARIA, the Brigance battery was used to assess listening compre- Blauert, J. 1997 Spatial Hearing: The Psychophysics of Human Sound hension, oral reading, and word recognition following ARIA Localization. Cambridge, MA; London: MIT Press. 588 D. Moncrieff et al.

Boudreau, J.C. & Tsuchitani, C. 1968. Binaural interaction in the cat Moncrieff, D. 2015. Age- and gender-specific normative information superior olive S segment. J Neurophysiol, 31, 442–454. from children assessed with a dichotic words test. J Am Acad Audiol, Chermak, G.D., Hall, J.W. 3rd, & Musiek, F.E. 1999. Differential diagnosis 26, 632–644. and management of central auditory processing disorder and attention Moncrieff, D.W. 2011. Dichotic listening in children: Age-related changes in deficit hyperactivity disorder. J Am Acad Audiol, 10, 289–303. direction and magnitude of ear advantage. Brain Cogn, 76, 316–322. Denes, G. & Caviezel, F. 1981. Dichotic listening in crossed aphasia: Moncrieff, D.W. & Black, J.R. 2008. Dichotic listening deficits in children ‘‘paradoxical’’ ipsilateral suppression. Arch Neurol, 38, 182–185. with dyslexia. Dyslexia, 14, 54–75. Denman, I., Banajee, M. & Hurley, A. 2015. Dichotic listening training in Moncrieff, D.W. & Wertz, D. 2008. Auditory rehabilitation for interaural children with autism spectrum disorder: a single subject design. Int J asymmetry: Preliminary evidence of improved dichotic listening Audiol, 54, 991–996. performance following intensive training. Int J Audiol, 47, 84–97. Dermody, P., Mackie, K. & Katsch, R. 1983. Auditory processing limitations Moncrieff, D., Keith, W., Abramson, M. & Swann, A. 2016. Diagnosis of in low verbal children: Evidence from a two-response dichotic listening amblyaudia in children referred for auditory processing assessment. Int J task. Ear Hear, 4, 272–277. Audiol, 55, 333–345. Emanuel, D.C., Ficca, K.N. & Korczak, P. 2011. Survey of the diagnosis Moore, D.R. 2011. The diagnosis and management of auditory processing and management of auditory processing disorder. Am J Audiol, 20, 48–60. disorder. Lang Speech Hear Serv Sch, 42, 303–308. Grogan, S. 1986. Hemispheric lateralisation for verbal analysis: Do children Musiek, F.E. 1999. Central auditory tests. Scand Audiol Suppl, 51, 33–46. with reading problems differ from controls? Percept Mot Skills, 63, Pelham, W.E. 1979. Selective attention deficits in poor readers? Dichotic 1265–1266. listening, speeded classification, and auditory and visual central and Hall, J.W., 3rd, Grose, J.H., Dev, M.B. & Ghiassi, S. 1998. The effect of incidental learning tasks. Child Dev, 50, 1050–1061. masker interaural time delay on the masking level difference in children Pinheiro, F.H., Oliveira, A.M., Cardoso, A.C. & Capellini, S.A. 2010. with history of normal hearing or history of otitis media with effucsion. Dichotic listening tests in students with learning disabilities. Braz J Ear Hear, 19, 429–433. Otorhinolaryngol, 76, 257–262. Harris, V.L., Keith, R.W. & Novak, K.K. 1983. Relationship between Putter-Katz, H., Adi-Bensaid, L., Feldman, I., & Hildesheimer, M. 2008. two dichotic listening tests and the Token test for children. Ear Hear,4, Effects of speech in noise and dichotic listening intervention programs 278–282. on central auditory processing disorders. J Basic Clin Physiol Hartley, D.E.H., Hill, P.R. & Moore, D.R. 2003. The auditory basis Pharmacol, 19, 301–316. of language impairments: Temporal processing versus processing Reynolds, S., Kuhaneck, H.M. & Pfeiffer, B. 2016. Systematic review of the efficiency hypotheses. Int J Pediatr Otorhinolaryngol, 67 Suppl 1, effectiveness of frequency modulation devices in improving academic S137–S142. outcomes in children with auditory processing difficulties. Am J Occup Hiscock, M., Cole, L.C., Benthall, J.G., Carlson, V.L. & Ricketts, J.M. 2000. Ther, 70, 1–11. Toward solving the inferential problem in laterality research: Effects of Roberts, J., Hunter, L., Gravel, J., Rosenfeld, R., Berman, S., et al. 2004. increased reliability on the validity of the dichotic listening right-ear Otitis media, hearing loss, and language learning: Controversies and advantage. J Int Neuropsychol Soc, 6, 539–547. current research. J Dev Behav Pediatr, 25, 110–122. Hynd, G.W., Obrzut, J.E., Weed, W. & Hynd, C.R. 1979. Development of Roush, J. & Tait, C.A. 1984. Binaural fusion, masking level differences, and cerebral dominance: Dichotic listening asymmetry in normal and auditory brain stem responses in children with language-learning learning-disabled children. J Exp Child Psychol, 28, 445–454. disabilities. Ear Hear, 5, 37–41. Jerger, J., Greenwald, R., Wambacq, I., Seipel, A. & Moncrieff, D. 2000. Russo, A., Snyder, M. & Moncrieff, D. 2014 ARIA: Auditory rehabilitation Toward a more ecologically valid measure of speech understanding in for interaural asymmetry. Research poster presented at the American background noise. J Am Acad Audiol, 11, 273–282. Academy of Audiology annual convention; Orlando, FL; 2014 Mar 26–29. Johnson, D.W., Enfield, M.L. & Sherman, R.E. 1981. The use of the Slattery, W.H. III. & Middlebrooks, J.C. 1994. Monaural sound localization: Staggered Spondaic Word and the competing environmental sounds tests Acute versus chronic unilateral impairment. Hear Res, 75, 38–46. in the evaluation of central auditory function of learning disabled Sparks, R. & Geschwind, N. 1968. Dichotic listening in man after section of children. Ear Hear, 2, 70–77. neocortical commissures. Cortex, 4, 3–16. Keating, P. & King, A.J. 2013. Developmental plasticity of spatial hearing Springer, S.P., Sidtis, J., Wilson, D. & Gazzaniga, M.S. 1978. Left ear following asymmetric hearing loss: Context-dependent cue integration performance in dichotic listening following commissurotomy. and its clinical implications. Front Syst Neurosci, 7, 123. Neuropsychologia, 16, 305–312. Keefe, B. & Swinney, D. 1979. On the relationship of hemispheric Strouse, A. & Wilson, R.H. 1999. Recognition of one-, two-, and three-pair specialization and developmental dyslexia. Cortex, 15, 471–481. dichotic digits under free and directed recall. J Am Acad Audiol, 10, Kimura, D. 1961. Some effects of temporal-lobe damage on auditory 557–571. perception. Can J Psychol, 15, 156–165. Tallus, J., Soveri, A., Ha¨ma¨la¨inen, H., Tuomainen, J. & Laine, M. 2015. Levitsky, W. & Geschwind, N. 1968. Asymmetries of the right and left Effects of auditory attention training with the dichotic listening task: hemisphere in man. Trans Am Neurol Assoc, 93, 232–233. Behavioural and neurophysiological evidence. PLoS One, 10(10), Litovsky, R.Y. 2011. Review of recent work on spatial hearing skills in e0139318. children with bilateral cochlear implants. Cochlear Implants Int,12 Tawfik, S., Mohamed Hassan, D. & Mesallamy, R. 2015. Evaluation of Suppl 1, S30–S34. long term outcome of auditory training programs in children Lupo, J.E., Koka, K., Hyde, B.J., Jenkins, H.A. & Tollin, D.J. 2011. with auditory processing disorders. Int J Pediatr Otorhinolaryngol, 79, Physiological assessment of active middle ear implant coupling to the 2404–2410. round window in chinchilla lanigera. Otolaryngol Head Neck Surg, 145, Tobey, E.A., Cullen, J.K., Jr. & Rampp, D.L. 1979. Effects of stimulus-onset 641–647. asynchrony on the dichotic performance of children with auditory- Middlebrooks, J.C. & Green, D.M. 1991. Sound localization by human processing disorders. J Speech Hear Res, 22, 197–211. listeners. Annu Rev Psychol, 42, 135–159. Tollin, D.J. & Yin, T.C. 2002. The coding of spatial location by Milner, B., Taylor, L. & Sperry, R.W. 1968. Lateralized suppression of single units in the lateral superior olive of the cat. II. The dichotically presented digits after commissural section in man. Science, determinants of spatial receptive fields in azimuth. J Neurosci, 161, 184–186. 22, 1468–1479. Moncrieff, D. 2010. Amblyaudia: Evidence of indistinct processing of Vanniasegaram, I., Cohen, M. & Rosen, S. 2004. Evaluation of selected binaural information in children. Research presentation at American auditory tests in school-age children suspected of auditory processing Auditory Society annual meeting; Phoenix, AZ; 2010 Mar 4–6. disorders. Ear Hear, 25, 586–597. Hearing, Cognition, and Healthy Aging: Social and Public Health Implications of the Links between Age-Related Declines in Hearing and Cognition

M. Kathleen Pichora-Fuller, Ph.D.,1,2,3 Paul Mick, MD, MPH, FRCSC,4,5 and Marilyn Reed, M.Sc.6

ABSTRACT

Sensory input provides the signals used by the brain when listeners understand speech and participate in social activities with other people in a range of everyday situations. When sensory inputs are diminished, there can be short-term consequences to brain functioning, and long-term deprivation can affect brain neuroplasticity. Indeed, the association between hearing loss and cognitive declines in older adults is supported by experimental and epidemiologic evidence, although the causal mechanisms remain unknown. These interactions of auditory and cognitive aging play out in the challenges confronted by people with age- related hearing problems when understanding speech and engaging in social interactions. In the present article, we use the World Health Organization’s International Classification of Functioning, Disability and Health and the Selective Optimization with Compensation models to highlight the importance of adopting a healthy aging perspective that focuses on facilitating active social participation by older adults. First, we examine epidemiologic evidence linking ARHL to cognitive declines and other health issues. Next, we examine how social factors influence and are influenced by auditory and cognitive aging and if they may provide a possible explanation for the association between ARHL and cognitive decline. Finally, we outline how audiologists could reposition hearing health care within the broader context of healthy aging. This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.

1Department of Psychology, University of Toronto, Tor- Toronto, 3359 Mississauga Rd N., Mississauga, ON, onto, Ontario, Canada; 2Toronto Rehabilitation Institute, Canada L5L 1C6 (e-mail: [email protected]). University Health Network, Toronto, Ontario, Canada; Cognition and the Aging Auditory System; Guest 3Rotman Research Institute, Toronto, Ontario, Canada; Editor, Lindsey E. Jorgensen, Au.D., Ph.D. 4Department of Surgery, University of British Columbia, Semin Hear 2015;36:122–139. Copyright # 2015 by Vancouver, British Columbia, Canada; 5Kelowna General Thieme Medical Publishers, Inc., 333 Seventh Avenue, Hospital, Kelowna, British Columbia, Canada; and; New York, NY 10001, USA. Tel: +1(212) 584-4662. 6Baycrest Health Sciences, Toronto, Ontario, Canada. DOI: http://dx.doi.org/10.1055/s-0035-1555116. Address for correspondence: M. Kathleen Pichora- ISSN 0734-0451. Fuller, Ph.D., Department of Psychology, University of 122 HEARING, COGNITION, AND HEALTHY AGING/PICHORA-FULLER ET AL 123

KEYWORDS: Social isolation, social support, healthy aging, age- related hearing loss (ARHL), cognitive aging

Learning Outcomes: As a result of this activity, the participant will be able to (1) discuss the evidence regarding the associations between age-related hearing loss (ARHL) and other age-related health issues, and (2) describe how social factors inﬂuence and are inﬂuenced by ARHL to guide new approaches to audiologic practice.

HEALTH, HEALTHY AGING, AND context and can be modulated by personal and THE IMPORTANCE OF SOCIAL environmental factors. All components of the FUNCTIONING system are linked to each other such that change All too often, old age is associated with diffi- in one will yield changes in others. Importantly, culties such as multiple health problems, in- modifying contextual factors by removing bar- cluding declines in auditory and cognitive riers and/or enhancing supports can facilitate abilities, and social isolation. Sensory input healthy functioning for an individual. Ideally, provides the signals used by the brain when healthy functioning would result if personal listeners understand speech, enjoy music, detect resources and supportive physical and social warning signals, react to environmental sounds, environments were optimized to offset the and participate in social activities with other potentially negative consequences of biological people in a range of everyday situations. When impairments. From an ecological perspective, sensory inputs are diminished, there can be an optimized person-context fit would promote short-term consequences to brain functioning, healthy adaptation,4 enabling the person to and long-term deprivation can affect brain cope successfully and achieve their goals with- neuroplasticity. Importantly, the interactions out stress. of auditory and cognitive aging play out in According to the SOC model, “successful” everyday life as people with age-related hearing aging involves modifying the balance between problems communicate, participate in social losses and gains such that, as older adults activities, and maintain social roles and continue to develop in their later life, it remains relationships. possible to have a positive balance. The SOC In the present article, we consider how age- model assumes that as adults age there is intra- related sensory, cognitive, and social function- individual plasticity and interindividual vari- ing may be interrelated and the implications of ability (i.e., all individuals can change with age, such interrelationships for rehabilitative audi- but they may differ in how they change). The ology. In particular, we use the World Health model describes three key processes involved in Organization’s International Classification of successful aging: (1) selection and prioritization Functioning, Disability and Health (WHO of goals to be achieved with limited resources; ICF) and the Selective Optimization with (2) optimization of how limited resources are

Compensation (SOC) model of Baltes and deployed to achieve selected goals (e.g., devel- This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. Baltes to highlight the importance of adopting oping new skills can improve the efﬁciency of a healthy aging perspective that focuses on resource use); (3) compensation by drawing on facilitating active social participation by older relatively intact abilities (or using assistive adults.1,2 technologies) to offset declining abilities so The WHO ICF provides a familiar frame- that selected goals can be achieved in new work that has been used by audiologists.3 Two ways. The SOC model can be applied in key features of the WHO ICF are that func- rehabilitation to understand how older adults tioning is conceptualized with bidirectional cope successfully with declines and various inﬂuences across a trio of levels (impairment, disabilities (e.g., recovery from stroke).5 The activity, and participation) spanning biological model can also be used to understand how to social factors and that functioning occurs in younger and middle-aged people can prevent 124 SEMINARS IN HEARING/VOLUME 36, NUMBER 3 2015

declines and prepare for successful aging by Nonauditory age-related health conditions engaging proactively in health-promoting may influence hearing loss and hearing health lifestyles.6 care. For example, hearing loss has been linked Health has been defined as a state of to other major chronic health conditions that complete physical, mental, and social well- may be risk factors for hearing loss, including being and not merely the absence of disease diabetes,15 cardiovascular disease,16 and hyper- or infirmity,7 but from the perspective of health tension.17 Furthermore, hearing rehabilitation, promotion, health has been redefined as the including use of hearing aids, may be compli- capacity of people to adapt to, respond to, or cated by nonauditory age-related conditions, control life’s challenges and changes.8 Impor- such as visual impairment or loss of manual tantly, hearing health needs to be thought of as dexterity due to arthritis.18–20 Conversely, hear- part of this capacity for people to live their lives ing loss and hearing health care may influence optimally. We are poised to reinvent hearing other age-related health conditions. Clearly, health care for older adults by adopting a the association between hearing loss and inci- healthy aging approach. dent dementia has become a concern. However, like many geriatric health conditions, dementia rarely has a single cause; although various types LINKS BETWEEN HEARING LOSS, of dementia have been identified, their etiolo- COGNITIVE DECLINE, AND THE gies are not yet well understood and likely HEALTH OF OLDER ADULTS involve a complex interaction of many genetic, The population is aging rapidly; a historically lifestyle, and environmental factors.21 Further- unprecedented large proportion of the popula- more, hearing loss is associated with and may tion is becoming seniors as those in the “baby precede a broad range of other age-related boomer” generation turn 65 years old. Another mental and physical health issues besides de- reason is that longevity has increased, with mentia, including depression,22 difficulties more people living to older ages, including walking,23 falls,24,25 poorer physical function- many who are centenarians.9–11 Indeed, the ing and increased risk in women of incident most rapid reductions in mortality are occurring nursing care requirements,26 frailty,27 and even in the “oldest old” age group of octogenarians mortality.28–32 Similar to hearing loss, demen- and nonagenarians.12 Accordingly, in the com- tia and cognitive dysfunction also contribute to ing years, audiologists should expect to see more falls,33 length of stay in hospital,34 more inten- people who are living to older ages and whose sive nursing care,35 and poorer recovery after needs differ from those of younger adults. surgery,36 and hospital stays and surgery may In general, as the population ages, the increase rates of cognitive declines in older burden of disease and mortality is shifting individuals with and without dementia on with increases in chronic, noncommunicable, admission.37 In addition, provocative findings and geriatric illnesses; specifically, a recent indicate that hearing loss may undermine the study of projected global disease burden, using quality and costs of health care, and that estimates based on trends in demographic and hospital costs for patients with dementia are socioeconomic factors, suggested that demen- higher compared to costs for peers without This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. tias and adult-onset hearing loss will be among dementia,38,39 even in analyses that control the top 10 causes of total disability in high- for severity of medical illness, functional im- income countries by the year 2030.13 Not pairment, and other potentially confounding surprisingly, given the increasing prevalence variables. Thus, the implications of hearing loss of hearing loss, dementia, and other chronic in older adults, especially in those who have or health conditions, it is becoming more common may develop dementia, extend to many aspects for older adults to have multiple comorbidities. of health and health care. Indeed, there is growing awareness that hearing Of course, many older people with hearing loss must be factored into social policies to loss are cognitively normal, and not all people promote healthy aging and programs to provide with dementia have hearing loss. Nevertheless, a wide range of health services to older adults.14 the increasingly common co-occurrence of HEARING, COGNITION, AND HEALTHY AGING/PICHORA-FULLER ET AL 125 declines in sensory loss and cognitive loss as shown faster rates of cognitive declines among people get older does not seem to be merely a patients who already had dementia with hear- result of increasing prevalence in unrelated ing loss compared with those with normal conditions, but rather that these conditions hearing.44,45 In addition to associations be- are interrelated.40 Convincing evidence has tween pure tone hearing loss and dementia, emerged suggesting that cognitive loss is other studies have also demonstrated that more prevalent or may progress more quickly performance on tests of central auditory proc- in people with hearing loss than in their peers essing (e.g., dichotic tests of speech in noise) with good hearing. Audiologists will need to be are associated with cognitive status and inci- prepared to help an increasing number of older dent dementia.46–49 Taken together, these adults who are living with hearing loss in studies establish that there is a link between combination with other chronic health issues, auditory and cognitive aging, but further re- but especially those who have both hearing loss search is required to determine why there is a and dementia.41 link or if the link can be modified by interven- The strongest epidemiologic evidence link- tion. The explanations for the link may be ing pure tone hearing loss to accelerated cogni- based on biological, cognitive, and/or social tive decline and dementia comes from two mechanisms. In the present article, the biolog- large, population-based cohort studies with ical and cognitive mechanisms are mentioned long follow-up periods. The first study included briefly, but there is greater focus is on the a cohort of 639 dementia-free older individuals possible social mechanisms that may be associ- who underwent audiometric pure tone thresh- ated with the link between auditory and cog- old testing in the early 1990s.42 The partici- nitive aging. pants returned for follow-up every 2 years with a median follow up of over 11 years. Whether or not the time to incident all-cause dementia Possible Biological Mechanisms depended on the severity of hearing loss was A common neurodegenerative pathology could investigated using Cox proportional hazards conceivably lead to both hearing loss and de- regression models adjusted for age, sex, race, mentia. The pathognomonic b-amyloid pla- education, diabetes, smoking, and hyperten- ques, neurofibrillary tangles, and neuronal sion. Strikingly, the risk of incident dementia atrophy of Alzheimer disease have been ob- increased by 1.27 times per 10-dB increase in served in the peripheral visual neural and olfac- hearing thresholds. Relative to people with tory sensory systems at autopsy, and reduced normal hearing, the incidence of all-cause de- smell is an early clinical symptom of the disease; mentia was 1.89 times higher in people with however, Alzheimer pathology, although af- mild hearing loss, 3 times higher in people with fecting the central auditory system,50 has not moderate hearing loss, and 4.94 times higher in been demonstrated in peripheral structures like people with severe hearing loss. To put the the cochlea or cochlear nerve.40 Other possible effect size into context, the authors calculated biological risk factors common to both hearing that the cognitive reduction associated with a loss and dementia could be a variety of genetic, 25-dB loss in hearing was equivalent to the inflammatory, or immunologic and/or other This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. reduction associated with an increase of 7 years health conditions (e.g., cardiovascular disease, in age. Similar results were found in the second hypertension, diabetes, stress) that are often study of nearly 2,000 people in the Health ABC controlled in epidemiologic studies as possible cohort who were 70 to 79 years old.43 In that confounders.43,51 study, participants from Pittsburgh and Mem- phis with mild to severe hearing loss had faster rates of cognitive decline compared with normal Possible Cognitive Mechanisms hearing participants, even after adjusting for Cognitive mechanisms may contribute to the possible confounders due to demographic or link between hearing and dementia. Evidence health factors. Furthermore, at least two earlier for cognitive mechanisms comes from research studies conducted by other researchers have on age-related changes in brain structure, 126 SEMINARS IN HEARING/VOLUME 36, NUMBER 3 2015

functional brain networks, and behavioral certain point, however, even individuals who performance. have greater cognitive or brain reserves can no A link between hearing loss and changes in longer compensate for neural degeneration and brain structure is suggested by recent radio- the clinical manifestation of disease becomes graphic evidence from a brain imaging study of apparent, even though symptoms have been participants in the Baltimore Longitudinal delayed relative to others who have less reserve. Study of Aging.52 Among a cohort of 126 Cognitive reserve is thought to be greater for dementia-free individuals, the mean rate of those who are more educated, have higher IQs, brain volume reduction over a 6.4-year fol- work in occupations or engage in activities with low-up was greater among participants with rich opportunities for mental and social engage- hearing loss. The size of this effect was approx- ment, and adopt healthy lifestyles in terms of imately equivalent to the average difference diet and physical activity.65,66 Thus, there may seen between people with normal cognition be interindividual differences insofar as older and people with mild cognitive impairment. adults may begin the process of adjusting to In particular, hearing loss was related to greater hearing loss having accumulated more or less changes in the temporal lobe structures, which cognitive reserve over their lifetimes. In the are important for spoken language processing, long term, if hearing loss compromises an semantic memory, and sensory integration, and individual’s ongoing lifestyle choices, resulting atrophy in the medial temporal lobe is found in in reduced engagement in physical, mental, and the early stages of Alzheimer’s disease.53–58 social activity, then the maintenance of the Beyond changes in brain structures, it is person’s cognitive reserve may be compromised. even more important to understand more about In this way, over many years, hearing loss may changes in brain function and associated be- accelerate the depletion of cognitive reserve as haviors. Notably, in addition to biological dementia progresses. For a given person at a changes in the brain and reduced performance given point in time, there are also intraindivid- on behavioral tests of memory, a necessary ual differences in how brain networks are condition of dementia diagnosis is loss of ability activated depending on the cognitive load de- to function independently in everyday life.59,60 manded by various tasks. In studies of brain Building on what has been learned about age- function, increasing brain activation as the related changes in functional brain networks,61 cognitive load demanded by a task increases further research using functional brain meas- has been interpreted as evidence of cognitive ures is needed to determine how sensory-cog- compensation.61 nitive brain networks may change over time and The notions of cognitive reserve and cog- how these changes relate to functioning in nitive compensation are compatible with be- different everyday communication situations.62 havioral findings concerning interindividual Two main themes that have emerged in re- and intraindividual differences in listening per- search on the cognitive neuroscience of aging formance.67,68 The cognitive demands encoun- are that there are individual differences in tered by a listener performing tasks in everyday cognitive reserve and that there is cognitive life vary depending on a variety of interacting compensation such that patterns of brain acti- factors related to the characteristics of the This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. vation for a given individual vary depending on listener and the signal(s) produced by one or the cognitive load demanded by a task. more sources in a context.69 Listener character- The concept of cognitive reserve refers to istics, including cognitive reserve, contribute to individual differences in cognition that seem to interindividual differences in performance. In- moderate the extent to which dementia pathol- dividuals of the same age can differ in their ogy manifests in symptoms of disease.63 People auditory and cognitive processing abilities, and with greater cognitive reserve are thought to in their linguistic and world knowledge; how- have neural networks that are less susceptible to ever, in general, processing abilities decline disruption such that they can compensate better whereas knowledge increases or is maintained for pathologic changes in the brain and thereby as adults get older.70 Finally, consistent with the maintain functioning in everyday life.64 At a SOC model, listeners may differ in their goals HEARING, COGNITION, AND HEALTHY AGING/PICHORA-FULLER ET AL 127 or selection of priorities for allocating cognitive item–item and item–context associative mem- resources, especially when there are multiple ory, with diminished ability to compensate by competing tasks. These listener characteristics using context when information is encoded and interact with signal and context factors that with disruptions of auditory associations that contribute to situation-specific intraindividual are temporally defined.78,79 Insofar as those differences in performance. In the terms of the with age-related hearing loss (ARHL) may SOC model, optimization could reduce and/or habitually experience even greater cognitive compensation could offset-increased cognitive loads in typical listening situations, which load. The signal properties affecting the cogni- might accelerate changes in brain networks, it tive load demanded in a specific listening is possible that they could show sooner or faster situation may include not only the acoustic cognitive declines than peers with better (with or without optimization by the use of hearing. listening technologies) and the linguistic properties of a target speech signal, but also the properties of energetic and informational HOW SOCIAL FACTORS maskers, and converging or competing infor- INFLUENCE AND ARE INFLUENCED mation from other sensory modalities. In addi- BY AUDITORY AND COGNITIVE tion, the cognitive load of a listening task will AGING depend on many aspects of the context that may Social factors affect many aspects of health, facilitate the use of semantic, social, and inter- including how people live with age-related personal knowledge and expectancies to inter- auditory and cognitive declines; conversely, pret incoming signals. health and age-related auditory and cognitive In general, congruent or supportive con- declines affect social interactions and quality of texts make the task easier and lessen cognitive life. The connections between auditory, cogni- load, and incongruent contexts make the task tive, and social factors are illustrated by the more difficult and increase cognitive load. Even comment of a doctor who summed up his if the acoustical conditions are the same, in- experience with hearing loss as follows: “It creased cognitive load (e.g., by increasing task affected me meeting girls and having girl- demands) causes listeners to rely more on lexical friends. Being made fun of for not hearing knowledge and less on phonetic detail.71 Nota- things, where people were making light of it. bly, younger adults rely relatively more on It hurt, but you’d never admit to it. People bottom-up processing of signal cues, whereas would say, ‘You can hear, you just didn’t pay older adults rely relatively more on top-down attention.’ And that was partly accurate. I processing of context cues.72 In effect, com- stopped paying attention because I couldn’t pared with younger listeners, older listeners hear.”80 How successfully an older adult selects, may be chronically operating with more cogni- optimizes, and compensates in everyday life tive load and more frequently engaging com- may depend on several relevant social factors, pensatory patterns of brain activation.73 Over including stigma, self-efficacy, social support, many decades, the compensatory shift in the use social networks, and participation in social of different types of information during every- activities. This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. day listening may accompany permanent changes in patterns of activation in brain networks, such as changes in hemispheric asym- Stigma, Stereotypes, and Self- metry or shifts to more frontal lobe Perceptions activation.74,75 In addition, converging evi- Stigma involves the (self-)identification of a dence suggests that the hippocampus is in- person as having a characteristic that is devalued volved not only in memory but also in in a social context.81 Negative stereotypes of associative memory or the binding of informa- aging, as well as negative self-perceptions, may tion about items and contexts during the per- affect the health of older adults and how they ception of complex stimuli.76,77 Experimental function in everyday activities, such as commu- studies demonstrated age-related declines in nication and social interaction, which depend 128 SEMINARS IN HEARING/VOLUME 36, NUMBER 3 2015

on both hearing and memory.82–84 Notably, adaptation to ARHL. Importantly, audiologists memory and hearing are two domains in which could use techniques to increase the self-efficacy older people are often negatively stereo- of older adults at various stages of the rehabili- typed.85–90 Age-related declines in memory tative process.101 and hearing may be exacerbated when older adults fall prey to negative stereotypes of aging, and negative views of aging may be one reason Social Support why older people’s self-perceptions of ability do Social support refers to the perceived quality, not always accurately reflect their actual func- rather than the quantity, of relationships pro- tion.91,92 The stigma associated with hearing viding emotional or affective support (e.g., loss is entangled with ageism,93 and perceptions empathy), instrumental support (e.g., financial of stigmatization can fuel the denial of hearing aid), and/or informational support (e.g., ad- problems and nonadherence to rehabilitative vice).102,103 Social support can have a powerful treatments.94,95 The perception of stigma seems effect on outcomes for various health conditions to differ between men and women, to vary with by promoting the use of effective coping strate- age, and to be associated more with hearing loss gies and counteracting negative interpretations than with hearing-aid use.96 Importantly, age of adverse events.104,105 The importance of per se is not correlated with negative views of social support is demonstrated in the role played aging,97 and responses to stigma can vary by significant others in hearing rehabilitation depending on context such that some individu- and by caregivers in dementia care.106 Signifi- als are vulnerable while others are resilient to cant others can act as a source of motivation and stigma.81 Thus, there is potential to tailor support for those using hearing aids,107,108 by interventions according to the individual’s re- encouraging the person who is hard-of-hearing sponses to stigma. to pursue and adhere to treatment,109,110 by acting as a facilitator for communication, and by providing information and instruction on the Self-Efficacy proper use and operation of hearing aids111–113; Self-efficacy refers to domain-specific “beliefs however, the negative attitudes of significant in one’s capabilities to organize and execute the others may contribute to delaying help-seeking courses of action required to produce given or rejecting treatment.114,115 The availability of attainments.”98(p.3) In general, individuals the social support seems to differentiate suc- with high self-efficacy put forth greater effort cessful from unsuccessful and satisfied from in achieving a behavior, persevere when diffi- unsatisfied hearing aid users.116,117 Further- culties arise or failures occur, have self-aiding more, social support is a strong predictor of thoughts for achieving the behavior, and cope adjustment to the psychological distress associ- better with emotional, social, and environmen- ated with hearing loss.118 tal demands surrounding the behavior.98 Nu- Social support provided by significant merous studies have demonstrated that others and caregivers may be beneficial to the domain-specific self-efficacy plays an important person who has the impairment(s), but unfor- role in the successful management of a various tunately, providing social support may strain This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. chronic health conditions; for example, patients relationships, increase stress, and compromise with high self-efficacy for managing diabetes the health and quality of life of those who have more positive outcomes, higher health- provide the support. Hearing loss affects not related quality of life, increased compliance only the person who has hearing loss but also with treatment recommendations, and better significant others,119–130 reducing their physi- long-term maintenance of the modified behav- cal, psychological, and social well-being. In ior compared with patients with low self-effi- particular, third-party disability manifests in cacy.98–100 Thus, it seems that sufficient self- wide-ranging effects on the spouses of persons efficacy is critical if older adults are to seek help, with hearing impairment,131 including commu- take action, and modify and maintain the nication difficulties, emotional sequelae, and behavior changes required for successfully effects on relationships and everyday HEARING, COGNITION, AND HEALTHY AGING/PICHORA-FULLER ET AL 129 activities.127,129 Similarly, the well-known bur- physical capabilities and instrumental activities den of caregiving for an individual with demen- of daily living, such as managing medication.151 tia can result in changes in caregivers’ own A recent population-based study conducted physical health, mental health, financial using nationally representative data for the resources, and social participation.132,133 United States confirmed that older people Dementia can result in difficulties with lan- with hearing loss, especially women, had guage and cognition,134,135 and hearing loss in smaller social networks compared with people this population can also lead to communication with normal hearing.152 Isolation may increase breakdowns. Importantly, breakdowns in com- the risks of dementia onset and progression. munication increase caregiver burden and have Epidemiologic studies indicate that weaker been identified as one of the most distressing social networks, reduced social support and problems for caregivers of people with loneliness increase the risk of incident dementia dementia.136–139 For example, many of the among those who are initially free of the behavioral symptoms of AD and other demen- disease,153,154 after controlling for potentially tias, such as frequent requests for repetition and confounding variables. Again, age-related de- difficulty following conversations, can lead to clines in hearing and cognition both increase withdrawal from social situations and increased risk of social isolation and in combination the feelings of caregiver burden. The combination risk is likely to be even greater. Preventing social of declines in hearing and cognition may be isolation must be a target for audiologic reha- overwhelming for caregivers,41 but audiologic bilitation designed to promote healthy aging. rehabilitation of older adults with cognitive impairment and their significant others may help alleviate burden, increasing the efficiency PARTICIPATION IN SOCIAL of home care and delaying the client’s move to ACTIVITIES institutional care.140 Beyond merely preventing social isolation, effective selection, optimization, and compensation would enable a person who is aging Social Networks successfully to participate actively in enriching Significant others and caregivers are the core of activities in everyday life. In models based on an older person’s social network. Indeed, as population data, there are significant paths from people age, their socioemotional priorities shift age to hearing to intelligence to participation in toward a focus on emotional regulation and activities of daily living.155 In samples of people having fewer, high-quality close relationships with hearing impairment (or vision im- and away from a focus on knowledge acquisi- pairment), relationships have been found be- tion and having larger social networks.141 Im- tween cognition and everyday functioning.156 portantly, maintaining social connections is Again, there are reciprocal issues insofar as not critical to healthy aging. Indeed, social isolation only do auditory and cognitive declines com- and loneliness are particularly common in older promise active participation in everyday life, but adults and increase their risks of mortality, reduced opportunities for social participation mental and physical health issues, disabilities, may accelerate further declines. Animal studies This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. and difficulty in making life-course transi- showed that social and sensory stimulation can tions.142,143 Stronger social networks may re- strengthen the structure and function of the duce mortality, cardiovascular disease, central nervous system at the cellular and mo- depression, and anxiety,144–147 with the risk lecular levels; for example, compared with con- of mortality associated with social isolation trols isolated in sensory-deprived conditions, being as great as that of other notorious clinical rats randomized to live among their peers in risk factors such as cigarette smoking and cages containing abundant sensory stimuli had hypertension.148,149 Hearing loss in cognitively lower concentrations of inflammatory cytokines normal older adults has been related to reduced and higher levels of protective neurotrophic quality of life and increased social isolation and factors in the hippocampus, with greater num- emotional distress,150 as well as reductions in bers of synapses per dendrite, larger neurons, 130 SEMINARS IN HEARING/VOLUME 36, NUMBER 3 2015

and greater neurogenesis, as well as better programs to prevent, assess, treat, and increase spatial memory and learning behaviors.157 Sim- accessibility for older adults who have ARHL. ilar molecular and cellular changes have been observed in animals exposed to greater levels of physical activity158 or reduced caloric in- Prevention take,159 and these changes may mediate a path Fortunately, over the past 20 to 30 years, total between healthy lifestyles and cognitive resil- disability among Americans 65 years and older ience. Of many studies conducted in humans, has declined by 25%, possibly in response to one of the most intriguing evaluated the Expe- rising levels of education and standards of rience Corps,160 a social intervention in which living, better treatment of cardiovascular dis- at-risk older adults are trained and work as eases, reduced rates of smoking, greater avail- volunteers with children in classrooms; com- ability of assistive devices, and less physically pelling evidence suggests that increasing mean- demanding jobs.163 Similarly, risk of ARHL ingful social participation for the volunteers decreased throughout the 20th century in resulted in numerous positive outcomes, in- North America, possibly in response to lower cluding improved physical performance (walk- levels of industrial noise and better control of ing speed), improved cognitive performance cardiovascular disease.164 Prevention of ARHL (memory), and increased size of social net- may be achieved by addressing risk factors like works. Furthermore, functional brain imaging cardiovascular disease and noise exposure, par- provided evidence of intervention-specific in- ticularly among young people whose ears may creases in brain activity in the left prefrontal be particularly susceptible to subclinical noise cortex and anterior cingulate cortex over the 6- damage.165 These findings are inspiring public month interval relative to matched controls, health researchers to find more ways to slow or with matched gains in executive inhibitory prevent age-related health issues, especially ability.161 In general, longitudinal population- cognitive impairment and dementia, and to based studies suggest that greater participation improve the independence and well-being of by older adults in social activities may be older adults. On the one hand, health promo- associated with better cognition.162 These find- tion initiatives that help older adults to main- ings point to active and meaningful social tain good health and active social participation participation as another target for audiologic could moderate some of the negative conse- rehabilitation designed to promote healthy quences of age-related declines in hearing and aging. cognition. On the other hand, if hearing loss is a modifiable risk factor, especially for dementias and cognitive decline, then sooner and better IMPLICATIONS FOR AUDIOLOGIC hearing health care could moderate some of the PRACTICE negative consequences of several age-related As described above, across time and at any given health issues. point in time, the health and everyday social functioning of older adults can affect and be affected by age-related declines in hearing and Assessment This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. cognition. Consistent with the WHO ICF Audiometric threshold testing has been the framework, there is a pressing need to reinvent cornerstone of traditional audiologic assess- hearing health care for older adults by adopting ment, and hearing aid fitting has been the a healthy aging approach that emphasizes social cornerstone of traditional audiologic rehabili- participation. The processes for achieving suc- tation. Going beyond the information gathered cessful aging described in the SOC model could in a traditional audiologic assessment, informa- be used as a basis for designing new approaches tion about nonaudiometric factors concerning to rehabilitative audiology and for designing relevant aspects of an individual’s physical, other health-related programs delivered to mental, and social health would be needed to older adults who have hearing problems. The plan effective hearing health care in the broader WHO ICF and SOC model could be applied in context of healthy aging. Relevant aspects of HEARING, COGNITION, AND HEALTHY AGING/PICHORA-FULLER ET AL 131 physical health would include background on Rehabilitation other sensory modalities (e.g., vision, balance, Current rehabilitation options for individuals touch), motor functioning (e.g., dexterity, mo- with ARHL include technologies (e.g., hearing bility, falls), and chronic health conditions (e.g., aids, assistive devices, cochlear implants), audi- diabetes, cardiovascular disease, hypertension). tory or communication training, counseling, Relevant aspects of mental health would in- and group programs. Unfortunately, there is clude background on cognitive functioning no simple, one-size-fits-all solution for adults (e.g., screening or history of diagnosis of mild with normal cognition, and more specialized cognitive impairment or dementia) and mood interventions for those at various stages of (e.g., depression) or other psychological or cognitive decline are rarely implemented in psychiatric disorders (e.g., anxiety). Audiolo- typical audiology clinical settings.41,169 Some gists would not typically gather information barriers to successful rehabilitation are related about physical and mental health issues, and to technology that falls short of user expect- other health professionals, even those diagnos- ations or is expensive and/or ergonomically ing dementia, seldom gather information about suboptimal. However, there are many other hearing,166 even though hearing problems barriers to successful rehabilitation that are could affect the results of testing.167 However, primarily related to social factors. in the context of interprofessional teams, those Many older adults delay seeking help for involved in the care of older adults with multi- hearing problems and, by the time they first try ple health issues could benefit from cooperating a hearing aid, they are already affected by and sharing relevant information to ensure that multiple physical and cognitive health issues. the best quality of health care is provided. For decades, hearing health professionals have In addition to information about physical advocated for fitting hearing aids as soon as and mental health, information about relevant possible and certainly while older adults are aspects of social functioning would be needed physically and psychologically able to learn to to plan hearing health care designed to pro- use the technology.170,171 Although some mote successful aging. Rehabilitation planning health professionals have questioned the value could be greatly enhanced if audiologists had of hearing rehabilitation for older adults with information about the social factors that are dementia, it seems that hearing rehabilitation most likely to influence an older adult’s readi- may be of extreme importance in helping ness to seek help, take action, and maintain these individuals and their family care- behavior change to live successfully with hear- givers.43,170,172 There is strong evidence that ing loss. These measures would provide in- hearing aids and auditory rehabilitation im- sights into the stigma, self-efficacy, social prove communication and hearing-related support, social isolation, and social participa- quality of life.173,174 If poorer communication tion issues of the individual with hearing loss. increases risk for social isolation and with- Planning rehabilitation designed to help an drawal from social participation, which in turn older adult achieve a good person–environment contribute to the link between hearing loss and fit would also require information about the cognitive decline, then it is possible that im- contextual barriers and facilitators specific to proving communication by using hearing aids This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. the individual’s physical and social communi- might weaken the link. In epidemiologic stud- cation ecologies. Such information would be ies, cognitive declines were associated with used to assess the accessibility needs of the hearing loss but did not differ between partic- person, in particular communication situations, ipants who reported using hearing aids and as well as to characterize the availability of those who did not42,43; however, minimal social support and possible concerns regarding information on hearing aid use was available. third-party disability or caregiver burden. For- High-quality, long-duration randomized con- tunately, there has been a recent increase in trolled trials are needed to determine if older research on applying the principles of health adults with clinically significant cognitive and social psychology in audiologic declines benefit as much as peers with normal rehabilitation.168 cognition from current best practices in 132 SEMINARS IN HEARING/VOLUME 36, NUMBER 3 2015

audiologic rehabilitation and if such rehabili- predicted the frequency of participation in tation can slow cognitive declines.14 and subjective benefit from an online self- Some older adults are successful in adapt- help group for hearing loss.178 Finally, new ing to hearing loss. They identify and accept rehabilitative approaches to address the effects that they have hearing-related problems; they of hearing loss on significant others could also select the situations and relationships where help to predispose uptake and reinforce out- communication has the most value for them; comes of audiologic rehabilitation, especially they optimize by learning new listening skills for caregivers of older adults who have hearing (e.g., through auditory training and practice), loss and clinically significant cognitive declines. using technologies (e.g., hearing aids and assistive devices), and modifying environments (e.g., by reducing background noise); and they com- Accessibility pensate by speech-reading and using commu- Reduction of the risk of developing dementias nication strategies in conversation (e.g., asking may depend on lifestyle changes and improved communication partners to speak clearly). But treatment or prevention of chronic health con- other people have more difficulty making ditions, including hearing loss, that confer changes to adjust to life as a person with hearing additional risk. The most important modifiable loss. They may not recognize or might deny that lifestyle factors include cognitive reserve, phys- they have hearing problems; they might never ical activity and exercise, dietary factors, alcohol have their hearing tested; they might avoid intake, and smoking. The most obvious treat- social situations out of fear of embarrassment, able chronic health conditions include cardio- or take out their frustrations in ways that may vascular diseases, stroke, midlife hypertension, damage relationships with friends or family, diabetes, and midlife hypercholesterolemia.179 jeopardize social support, and/or reduce oppor- Sadly, many of the potentially modifiable risk tunities for interaction.130 factors for dementia, such as socialization, New approaches may be needed to over- physical activity, and prevention or treatment come barriers arising from social factors such as of chronic health conditions, including hearing stigma and negative views of aging, poor self- loss as a recent addition, are poorly addressed at efficacy, lack of social support, and social isola- the population level. Too many people are tion and/or withdrawal from social interaction. isolated, sedentary, and do not do anything For example, Internet screening may provide an about hearing impairment. There may be im- opportunity to reach those who would not come portant opportunities to reduce the incidence of to a clinic for a hearing test. However, the cognitive loss if these factors, and others, are identification of hearing loss by screening may addressed in a comprehensive manner in com- be insufficient to predispose further action; new munities, hospitals, and clinics. The current approaches to increase readiness for hearing model of health care, however, which is subju- rehabilitation will likely be needed to predis- gated to the “tyranny of the acute” and divided pose uptake of hearing aids and other changes into professional silos, is not an ideal platform in behavior to improve hearing-related quality upon which to build a holistic program that of life.175,176 Other new approaches, especially aims to prevent chronic illness. This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. those involving social support, may be needed New interprofessional and public health to reinforce and maintain new behaviors and approaches to population health are needed. coping; for example, in one study, harnessing In particular, such approaches could increase social support by including significant others in awareness of the importance of hearing rehabilitation classes for individuals with hear- health, better integrate hearing health care ing loss resulted in larger reductions in hearing into the larger health care picture, increase handicap scores for those who attended with a uptake of amplification devices and other significant other compared with those who forms of audiologic rehabilitation, and man- attended alone.177 The Internet may also pro- date public policies and environmental ac- vide new opportunities to overcome these bar- commodations such as ambient noise controls riers; for example, lack of social support and access to written information in public HEARING, COGNITION, AND HEALTHY AGING/PICHORA-FULLER ET AL 133 spaces. At the level of global health policy, 4. Stokols D. Establishing and maintaining healthy within the last decade, the World Health environments. Toward a social ecology of health Organization has spearheaded an interna- promotion. Am Psychol 1992;47(1):6–22 tional “age-friendly cities” agenda to foster 5. Donnellan C, O’Neill D. Baltes’ SOC model of successful ageing as a potential framework for environmental and social initiatives for “ac- stroke rehabilitation. Disabil Rehabil 2014;36(5): tive aging,” including community-level pro- 424–429 grams to promote the health, security, and the 6. Ouwehand C, de Ridder DT, Bensing JM. A social participation of older adults in socie- review of successful aging models: proposing ty.180 A challenge to audiologists is to begin proactive coping as an important additional strat- to contribute to such initiatives.181 egy. Clin Psychol Rev 2007;27(8):873–884 7. World Health Organization (WHO). WHO definition of health. Preamble to the Constitution CONCLUSION of the World Health Organization as adopted by the International Health Conference, New York, Greater attention to improving lifestyle and June 19–22 1946; signed on July 22, 1946, by the medical risk factors for cognitive declines is representatives of 61 States (Official Records of urgently needed across adulthood. Prevention the World Health Organization, no. 2, p. 100) and and mitigation of hearing loss may play a entered into force on April 7, 1948. Available at: significant role in these efforts, in combination http://www.who.int/about/definition/en/print. html. Accessed October 1, 2014 with other ways to promote healthy physical, 8. Frankish CJ, Green LW, Ratner PA, Chomik T, mental, and social aging. Even if dementias Larsen C. Health impact assessment as a tool for cannot be entirely prevented by sooner and population health promotion and public policy: a better hearing health care, simply delaying the report submitted to the health promotion devel- onset of disease would result in significant opment division of Health Canada. Vancouver, benefits to individuals and society. It is estimat- BC: University of British Columbia; 1996. Avail- ed that a 1-year delay would lead to a 10% able at: http://catalogue.iugm.qc.ca/GEIDEFile/ 182 healthimpact.PDF?Archive=192469191064. Ac- reduction in prevalence by 2050, and there cessed April 2015 would likely be additional, noncognitive bene- 9. Christensen K, Doblhammer G, Rau R, Vaupel fits to the health and well-being of older adults. JW. Ageing populations: the challenges ahead. The alternative of doing nothing will almost Lancet 2009;374(9696):1196–1208 certainly result in growing strains on families, 10. Kannisto V, Lauritsen J, Thatcher A, Vaupel J. health care systems, and aging societies. Reductions in mortality at advanced ages: several Audiologists have a valuable contribution to decades of evidence from 27 countries. Popul Dev Rev 1994;20:793–810 make by reinventing hearing care in the context 11. Vaupel JW. Biodemography of human ageing. of healthy and successful aging. Nature 2010;464(7288):536–542 12. Suzman R, Haaga J. World demography of aging. In: Longo D, Fauci A, Kasper D, Hauser S, REFERENCES Jameson J, Loscalzo J, eds. Harrison’s Principles of Internal Medicine, 18th ed. New York, NY: 1. World Health Organization (WHO). Interna- McGraw-Hill; 2012

tional Classiﬁcation of Functioning, Disability 13. Mathers CD, Loncar D. Projections of global This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. and Health (ICF). Geneva, Switzerland: WHO mortality and burden of disease from 2002 to Press; 2001. Available at: http://www.who.int/ 2030. PLoS Med 2006;3(11):e442 classiﬁcations/icf/en/. Accessed April 15, 2015 14. Lustig TA, Olson S. Hearing loss and healthy 2. Baltes P, Baltes M. Psychological perspectives on aging: workshop summary. Washington, DC: successful aging: the model of selective optimiza- The National Academies Press; 2014. Available tion with compensation. In: Baltes P, Baltes M, at: http://www.nap.edu/catalog/18735/hearing- eds. Successful Aging: Perspectives for the Behav- loss-and-healthy-aging-workshop-summary. Ac- ioral Sciences. New York, NY: Cambridge Uni- cessed April 2015 versity Press; 1990:1–34 15. Mitchell P, Gopinath B, McMahon CM, et al. 3. Worrall L, Hickson L. Communication Disability Relationship of type 2 diabetes to the prevalence, in Aging: From Prevention to Intervention. New incidence and progression of age-related hearing York, NY: Delmar Publishers; 2003 loss. Diabet Med 2009;26(5):483–488 134 SEMINARS IN HEARING/VOLUME 36, NUMBER 3 2015

16. Lin HC, Chao PZ, Lee HC. Sudden sensorineu- based study. J Clin Epidemiol 2012;65(7): ral hearing loss increases the risk of stroke: a 5-year 764–777 follow-up study. Stroke 2008;39(10):2744–2748 31. Fisher D, Li CM, Chiu MS, et al. Impairments in 17. Kiely KM, Gopinath B, Mitchell P, Luszcz M, hearing and vision impact on mortality in older Anstey KJ. Cognitive, health, and sociodemo- people: the AGES-Reykjavik Study. Age Ageing graphic predictors of longitudinal decline in hear- 2014;43(1):69–76 ing acuity among older adults. J Gerontol A Biol 32. Genther DJ, Betz J, Pratt S, et al; Health ABC Sci Med Sci 2012;67(9):997–1003 Study. Association of hearing impairment and 18. Smith SL, Bennett LW, Wilson RH. Prevalence mortality in older adults. J Gerontol A Biol Sci and characteristics of dual sensory impairment Med Sci 2015;70(1):85–90 (hearing and vision) in a veteran population. J 33. van Doorn C, Gruber-Baldini AL, Zimmerman Rehabil Res Dev 2008;45(4):597–609 S, et al; Epidemiology of Dementia in Nursing 19. Erber NP. Use of hearing aids by older people: Homes Research Group. Dementia as a risk factor inﬂuence of non-auditory factors (vision, manual for falls and fall injuries among nursing dexterity). Int J Audiol 2003;42(Suppl 2):S21–S25 home residents. J Am Geriatr Soc 2003;51(9): 20. Singh G, Pichora-Fuller MK, Hayes D, von 1213–1218 Schroeder HP, Carnahan H. The aging hand 34. Saravay SM, Kaplowitz M, Kurek J, et al. How do and the ergonomics of hearing aid controls. Ear delirium and dementia increase length of stay of Hear 2013;34(1):e1–e13 elderly general medical inpatients? Psychoso- 21. Ferrucci L, Studenski S. Clinical Problems of matics 2004;45(3):235–242 Aging. In: Longo D, Fauci A, Kasper D, Hauser 35. Erkinjuntti T, Autio L, Wikstro¨m J. Dementia in S, Jameson J, Loscalzo J, eds. Harrison’s Principles medical wards. J Clin Epidemiol 1988;41(2): of Internal Medicine; 18th ed. New York, NY: 123–126 McGraw-Hill; 2012 36. Magaziner J, Simonsick EM, Kashner TM, Hebel 22. Huang CQ, Dong BR, Lu ZC, Yue JR, Liu QX. JR, Kenzora JE. Predictors of functional recovery Chronic diseases and risk for depression in old age: one year following hospital discharge for hip a meta-analysis of published literature. Ageing fracture: a prospective study. J Gerontol 1990; Res Rev 2010;9(2):131–141 45(3):M101–M107 23. Viljanen A, Kaprio J, Pyykko¨ I, Sorri M, Kosken- 37. Etzioni DA, Liu JH, Maggard MA, Ko CY. The vuo M, Rantanen T. Hearing acuity as a predictor aging population and its impact on the surgery of walking difﬁculties in older women. J Am workforce. Ann Surg 2003;238(2):170–177 Geriatr Soc 2009;57(12):2282–2286 38. Mick P, Foley DM, Lin FR. Hearing loss is 24. Viljanen A, Kaprio J, Pyykko¨ I, et al. Hearing as a associated with poorer ratings of patient-physician predictor of falls and postural balance in older communication and healthcare quality. J Am female twins. J Gerontol A Biol Sci Med Sci 2009; Geriatr Soc 2014;62(11):2207–2209 64(2):312–317 39. Foley DM, Frick KD, Lin FR. Association be- 25. Lin FR, Ferrucci L. Hearing loss and falls among tween hearing loss and healthcare expenditures in older adults in the United States. Arch Intern Med older adults. J Am Geriatr Soc 2014;62(6): 2012;172(4):369–371 1188–1189 26. Chen DS, Betz J, Yaffe K, et al; Health ABC 40. Albers MW, Gilmore GC, Kaye J, et al. At the study. Association of hearing impairment with interface of sensory and motor dysfunctions and declines in physical functioning and the risk of Alzheimer’s disease. Alzheimers Dement 2015; disability in older adults. J Gerontol A Biol Sci 11(1):70–98 Med Sci 2015;70(5):654–661 41. Pichora-Fuller MK, Dupuis K, Reed M, Lemke

27. Kamil RJ, Li L, Lin FR. Association between U. Helping older people with cognitive decline This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. hearing impairment and frailty in older adults. J communicate: hearing aids as part of a broader Am Geriatr Soc 2014;62(6):1186–1188 rehabilitation approach. Semin Hear 2013; 28. Appollonio I, Carabellese C, Magni E, Frattola L, 34:308–330 Trabucchi M. Sensory impairments and mortality 42. Lin FR, Metter EJ, O’Brien RJ, Resnick SM, in an elderly community population: a six-year Zonderman AB, Ferrucci L. Hearing loss and follow-up study. Age Ageing 1995;24(1):30–36 incident dementia. Arch Neurol 2011;68(2): 29. Karpa MJ, Gopinath B, Beath K, et al. Associa- 214–220 tions between hearing impairment and mortality 43. Lin FR, Yaffe K, Xia J, et al; Health ABC Study risk in older persons: the Blue Mountains Hearing Group. Hearing loss and cognitive decline in older Study. Ann Epidemiol 2010;20(6):452–459 adults. JAMA Intern Med 2013;173(4):293–299 30. Feeny D, Huguet N, McFarland BH, Kaplan MS, 44. Peters CA, Potter JF, Scholer SG. Hearing im- Orpana H, Eckstrom E. Hearing, mobility, and pairment as a predictor of cognitive decline in pain predict mortality: a longitudinal population- dementia. J Am Geriatr Soc 1988;36(11):981–986 HEARING, COGNITION, AND HEALTHY AGING/PICHORA-FULLER ET AL 135

45. Uhlmann RF, Larson EB, Rees TS, Koepsell TD, Alzheimer’s disease: recommendations from the Duckert LG. Relationship of hearing impairment National Institute on Aging-Alzheimer’s Associ- to dementia and cognitive dysfunction in older ation workgroups on diagnostic guidelines for adults. JAMA 1989;261(13):1916–1919 Alzheimer’s disease. Alzheimers Dement 2011; 46. Gates GA, Anderson ML, McCurry SM, Feeney 7(3):270–279 MP, Larson EB. Central auditory dysfunction as a 60. McKhann GM, Knopman DS, Chertkow H, et al. harbinger of Alzheimer dementia. Arch Otolar- The diagnosis of dementia due to Alzheimer’s yngol Head Neck Surg 2011;137(4):390–395 disease: recommendations from the National In- 47. Gates GA, Beiser A, Rees TS, D’Agostino RB, stitute on Aging-Alzheimer’s Association work- Wolf PA. Central auditory dysfunction may pre- groups on diagnostic guidelines for Alzheimer’s cede the onset of clinical dementia in people with disease. Alzheimers Dement 2011;7(3):263–269 probable Alzheimer’s disease. J Am Geriatr Soc 61. Grady C. The cognitive neuroscience of ageing. 2002;50(3):482–488 Nat Rev Neurosci 2012;13(7):491–505 48. Gates GA, Gibbons LE, McCurry SM, Crane 62. Pichora-Fuller MK, Singh G. Effects of age on PK, Feeney MP, Larson EB. Executive dysfunc- auditory and cognitive processing: implications for tion and presbycusis in older persons with and hearing aid ﬁtting and audiologic rehabilitation. without memory loss and dementia. Cogn Behav Trends Amplif 2006;10(1):29–59 Neurol 2010;23(4):218–223 63. Fratiglioni L, Wang HX. Brain reserve hypothesis 49. Idrizbegovic E, Hederstierna C, Dahlquist M, in dementia. J Alzheimers Dis 2007;12(1):11–22 Ka¨mpfe Nordstro¨m C, Jelic V, Rosenhall U. 64. Nyberg L, Lo¨vden M, Riklund K, Lindenberger Central auditory function in early Alzheimer’s U, Ba¨ckman L. Memory aging and brain mainte- disease and in mild cognitive impairment. Age nance. Trends Cogn Sci 2012;16(5):292–305 Ageing 2011;40(2):249–254 65. Scarmeas N, Stern Y. Cognitive reserve and 50. Sinha UK, Hollen KM, Rodriguez R, Miller CA. lifestyle. J Clin Exp Neuropsychol 2003;25(5): Auditory system degeneration in Alzheimer’s dis- 625–633 ease. Neurology 1993;43(4):779–785 66. Scarmeas N, Luchsinger JA, Schupf N, et al. 51. Yamasoba T, Lin FR, Someya S, Kashio A, Physical activity, diet, and risk of Alzheimer Sakamoto T, Kondo K. Current concepts in disease. JAMA 2009;302(6):627–637 age-related hearing loss: epidemiology and mech- 67. Pichora-Fuller MK. Audition and cognition: what anistic pathways. Hear Res 2013;303:30–38 audiologists need to know about listening. In: 52. Lin FR, Ferrucci L, An Y, et al. Association of Palmer C, Seewald R, eds. Hearing Care for hearing impairment with brain volume changes in Adults. Sta¨fa, Switzerland: Phonak; 2007:71–85 older adults. Neuroimage 2014;90:84–92 68. Pichora-Fuller MK. Using the brain when the ears 53. Davis MH, Gaskell MG. A complementary sys- are challenged helps healthy older listeners com- tems account of word learning: neural and behav- pensate and preserve communication function. In: ioural evidence. Philos Trans R Soc Lond B Biol Hickson L, eds. Hearing Care for Adults. Sta¨fa, Sci 2009;364(1536):3773–3800 Switzerland: Phonak; 2010:53–65 54. Peelle J. The hemispheric lateralization of speech 69. Pichora-Fuller MK. Auditory and cognitive proc- processing depends on what “speech” is: a hierar- essing in audiologic rehabilitation. In Spitzer J, chical perspective. Front Hum Neurosci Montano J, eds. Adult Audiologic Rehabilitation: 2012;6:309. Available at: http://journal.frontier- Advanced Practices, 2nd ed. San Diego, CA: sin.org/article/10.3389/fnhum.2012.00309/full. Plural Publishing; 2013:519–536 Accessed April 2015 70. Park DC, Lautenschlager G, Hedden T, David- 55. Chetelat G, Landeau B, Eustache F, et al. Using son NS, Smith AD, Smith PK. Models of visuo-

voxel-based morphometry to map the structural spatial and verbal memory across the adult life This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. changes associated with rapid conversion in MCI: span. Psychol Aging 2002;17(2):299–320 a longitudinal MRI study. Neuroimage 2005; 71. Mattys S, Wiget L. Effects of cognitive load on 27(4):934–946 speech recognition. J Mem Lang 2011;65: 56. Kantarci K, Jack CR Jr. Quantitative magnetic 145–160 resonance techniques as surrogate markers of 72. Goy H, Pelletier M, Coletta M, Pichora-Fuller Alzheimer’s disease. NeuroRx 2004;1(2):196–205 MK. The effects of semantic context and the type 57. Mesulam MM. From sensation to cognition. and amount of acoustic distortion on lexical deci- Brain 1998;121(Pt 6):1013–1052 sion by younger and older adults. J Speech Lang 58. Tranel D, Damasio H, Damasio AR. A neural Hear Res 2013;56(6):1715–1732 basis for the retrieval of conceptual knowledge. 73. Schneider BA, Pichora-Fuller MK, Daneman M. Neuropsychologia 1997;35(10):1319–1327 The effects of senescent changes in audition and 59. Albert MS, DeKosky ST, Dickson D, et al. The cognition on spoken language comprehension. In: diagnosis of mild cognitive impairment due to Gordon-Salant S, Frisina R, Popper A, Fay D, 136 SEMINARS IN HEARING/VOLUME 36, NUMBER 3 2015

eds. The Aging Auditory System: Perceptual 88. Hummert ML, Garstka TA, Shaner JL, Strahm Characterization and Neural Bases of Presbycusis, S. Stereotypes of the elderly held by young, Springer Handbook of Auditory Research. Berlin, middle-aged, and elderly adults. J Gerontol Germany: Springer; 2010:167–210 1994;49(5):240–249 74. Cabeza R. Hemispheric asymmetry reduction in 89. Levy B. Stereotype embodiment: a psychosocial older adults: the HAROLD model. Psychol Ag- approach to aging. Curr Dir Psychol Sci 2009; ing 2002;17(1):85–100 18(6):332–336 75. Davis SW, Dennis NA, Daselaar SM, Fleck MS, 90. Levy BR, Slade MD, Gill TM. Hearing decline Cabeza R. Que PASA? The posterior-anterior predicted by elders’ stereotypes. J Gerontol B shift in aging. Cereb Cortex 2008;18(5): Psychol Sci Soc Sci 2006;61(2):82–87 1201–1209 91. Hertzog C, Dunlosky J. Metacognition in later 76. Olsen RK, Moses SN, Riggs L, Ryan JD. The adulthood: spared monitoring can benefit older hippocampus supports multiple cognitive process- adults’ self-regulation. Curr Dir Psychol Sci 2011; es through relational binding and comparison. 20(3):167–173 Front Hum Neurosci 2012;6:146 92. Hertzog C, Hultsch DF. Metacognition in adult- 77. Yonelinas AP. The hippocampus supports high- hood and old age. In: Craik F, Salthouse T, eds. resolution binding in the service of perception, The Handbook of Aging and Cognition. Mah- working memory and long-term memory. Behav wah, NJ: Lawrence Erlbaum Associates; 2000: Brain Res 2013;254:34–44 417–466 78. Li SC, Naveh-Benjamin M, Lindenberger U. 93. Wallhagen MI. The stigma of hearing loss. Ger- Aging neuromodulation impairs associative bind- ontologist 2010;50(1):66–75 ing: a neurocomputational account. Psychol Sci 94. Hetu R. The stigma attached to hearing im- 2005;16(6):445–450 pairment. Scand Audiol Suppl 1996;43:12–24 79. Howard MW, Kahana MJ, Wingfield A. Aging 95. Gagne JP, Southall K, Jennings M. The psycho- and contextual binding: modeling recency and lag logical effects of social stigma: applications to recency effects with the temporal context model. people with an acquired hearing loss. In: Montano Psychon Bull Rev 2006;13(3):439–445 J, Spitzer J, eds. Advanced Practice in Adult 80. Bouton K. Shouting Won’t Help: Why I—and 50 Audiologic Rehabilitation: International Perspec- Million Other Americans—Can’t Hear You. New tive. New York, NY: Plural Publishing; 2009: York, NY: Sarah Crichton Books; 2013 63–92 81. Southall K, Gagne J-P, Jennings MB. Stigma: a 96. Erler SF, Garstecki DC. Hearing loss- and hear- negative and a positive influence on help-seeking ing aid-related stigma: perceptions of women with for adults with acquired hearing loss. Int J Audiol age-normal hearing. Am J Audiol 2002;11(2): 2010;49(11):804–814 83–91 82. Levy BR, Slade MD, Kasl SV. Longitudinal 97. Kang SK, Chasteen AL. The development and benefit of positive self-perceptions of aging on validation of the age-based rejection sensitivity functional health. J Gerontol B Psychol Sci Soc questionnaire. Gerontologist 2009;49(3):303–316 Sci 2002;57(5):409–417 98. Bandura A. Self Efficacy: The Exercise of Con- 83. Ryan EB, Meredith SD, MacLean MJ, Orange trol. New York, NY: Freeman; 1997 JB. Changing the way we talk with elders: pro- 99. van der Bijl JJ, Shortridge-Baggett LM. The moting health using the communication enhance- theory and measurement of the self-efficacy con- ment model. Int J Aging Hum Dev 1995;41(2): struct. Sch Inq Nurs Pract 2001;15(3):189–207 89–107 100. Plack K, Herpertz S, Petrak F. Behavioral medi- 84. Ryan EB, Giles H, Bartolucci G, Henwood K. cine interventions in diabetes. Curr Opin Psychi-

Psycholinguistic and social psychological compo- atry 2010;23(2):131–138 This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. nents of communication by and with the elderly. 101. Smith SL, West RL. The application of self- Lang Commun 1986;6:1–24 efﬁcacy principles to audiologic rehabilitation: a 85. Chasteen AL, Schwarz N, Park DC. The activa- tutorial. Am J Audiol 2006;15(1):46–56 tion of aging stereotypes in younger and older 102. Broadhead WE, Gehlbach SH, de Gruy FV, adults. J Gerontol B Psychol Sci Soc Sci 2002; Kaplan BH. The Duke-UNC Functional Social 57(6):540–547 Support Questionnaire. Measurement of social 86. Cuddy A, Norton M, Fiske S. This old stereotype: support in family medicine patients. Med Care the pervasiveness and persistence of the elderly 1988;26(7):709–723 stereotype. J Soc Issues 2005;61:267–285 103. Cohen S. Social relationships and health. Am 87. Goffman E. Stigma: Notes on the Management of Psychol 2004;59(8):676–684 Spoiled Identity. Englewood Cliffs, NJ: Prentice- 104. Broadhead WE, Kaplan BH, James SA, et al. The Hall; 1963 epidemiologic evidence for a relationship between HEARING, COGNITION, AND HEALTHY AGING/PICHORA-FULLER ET AL 137

social support and health. Am J Epidemiol 1983; 120. Stephens D, France L, Lormore K. Effects of 117(5):521–537 hearing impairment on the patient’s family and 105. Cohen S, Wills TA. Stress, social support, and the friends. Acta Otolaryngol 1995;115(2):165–167 buffering hypothesis. Psychol Bull 1985;98(2): 121. Stephens D. Hearing rehabilitation in a psycho- 310–357 social framework. Scand Audiol Suppl 1996; 106. Erber N. Communication Therapy for Adults 43:57–66 with Sensory Loss. Melbourne, Australia: Clavis; 122. Heine C, Erber NP, Osborn R, Browning CJ. 1996 Communication perceptions of older adults with 107. Carson AJ. “What brings you here today?” The sensory loss and their communication partners: role of self-assessment in help-seeking for age- implications for intervention. Disabil Rehabil related hearing loss J Aging Stud 2005; 2002;24(7):356–363 19:185–200 123. Kramer SE, Kapteyn TS, Kuik DJ, Deeg DJ. The 108. Lockey K, Jennings MB, Shaw L. Exploring association of hearing impairment and chronic hearing aid use in older women through narra- diseases with psychosocial health status in older tives. Int J Audiol 2010;49(8):542–549 age. J Aging Health 2002;14(1):122–137 109. Duijvestijn JA, Anteunis LJ, Hoek CJ, Van Den 124. Danermark B, Gellerstedt LC. Psychosocial work Brink RH, Chenault MN, Manni JJ. Help-seek- environment, hearing impairment and health. Int ing behaviour of hearing-impaired persons aged > J Audiol 2004;43(7):383–389 or ¼ 55 years; effect of complaints, significant 125. Stark P, Hickson L. Outcomes of hearing aid others and hearing aid image. Acta Otolaryngol fitting for older people with hearing impairment 2003;123(7):846–850 and their significant others. Int J Audiol 2004; 110. Meyer C, Hickson L, Lovelock K, Lampert M, 43(7):390–398 Khan A. An investigation of factors that influence 126. Scarinci N, Worrall L, Hickson L. The effect of help-seeking for hearing impairment in older hearing impairment in older people on the spouse. adults. Int J Audiol 2014;53(Suppl 1):S3–S17 Int J Audiol 2008;47(3):141–151 111. Mahoney CF, Stephens SD, Cadge BA. Who 127. Scarinci N, Worrall L, Hickson L. The effect of prompts patients to consult about hearing loss? Br hearing impairment in older people on the spouse: J Audiol 1996;30(3):153–158 development and psychometric testing of the 112. van den Brink RH, Wit HP, Kempen GI, van significant other scale for hearing disability Heuvelen MJ. Attitude and help-seeking for (SOS-HEAR). Int J Audiol 2009;48(10): hearing impairment. Br J Audiol 1996;30(5): 671–683 313–324 128. Kamil RJ, Lin FR. The effects of hearing im- 113. Manchaiah VKC, Stephens D, Lunner T. Com- pairment in older adults on communication part- munication partners’ journey through their partners: a systematic review. J Am Acad Audiol 2015; ner’s hearing impairment. Int J Otolaryngol 2013; 26(2):155–182 2013:707910 129. Wallhagen MI, Strawbridge WJ, Shema SJ, Ka- 114. Brooks DN. The effect of attitude on benefit plan GA. Impact of self-assessed hearing loss on a obtained from hearing aids. Br J Audiol 1989; spouse: a longitudinal analysis of couples. J Ger- 23(1):3–11 ontol B Psychol Sci Soc Sci 2004;59(3): 115. Kochkin S. MarkeTrak VII: Obstacles to adult S190–S196 non-user adoption of hearing aids. Hear J 2007; 130. Hetu R, Jones L, Getty L. The impact of acquired 60:24–51 hearing impairment on intimate relationships: 116. Hickson L, Meyer C, Lovelock K, Lampert M, implications for rehabilitation. Audiology 1993; Khan A. Factors associated with success with 32(6):363–381

hearing aids in older adults. Int J Audiol 2014; 131. Scarinci N, Worrall L, Hickson L. The ICF and This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. 53(Suppl 1):S18–S27 third-party disability: its application to spouses of 117. Singh G, Lau S-T, Pichora-Fuller MK. Social older people with hearing impairment. Disabil support predicts hearing aid satisfaction. Ear Rehabil 2009;31(25):2088–2100 Hear, doi: 10.1097/AUD.0000000000000182 132. George LK, Gwyther LP. Caregiver well-being: a 118. Frankel B, Turner R. Psychological adjustment in multidimensional examination of family care- chronic disability: the role of social support in the givers of demented adults. Gerontologist 1986; case of the hearing impaired. Can J Sociol 1983; 26(3):253–259 8:273–291 133. Dunkin JJ, Anderson-Hanley C. Dementia care- 119. Hetu R, Lalonde M, Getty L. Psychosocial dis- giver burden: a review of the literature and guide- advantages associated with occupational hearing lines for assessment and intervention. Neurology loss as experienced in the family. Audiology 1987; 1998;51(1, Suppl 1):S53–S60, discussion S65– 26:141–152 S67 138 SEMINARS IN HEARING/VOLUME 36, NUMBER 3 2015

134. Zientz J, Rackley A, Chapman SB, et al. Evi- 150. Arlinger S. Negative consequences of uncorrected dence-based practice recommendations for de- hearing loss—a review. Int J Audiol 2003;42 mentia: educating caregivers on Alzheimer’s (Suppl 2):S17–S20 disease and training communication strategies. J 151. Crews JE, Campbell VA. Vision impairment and Med Speech-Lang Pathol 2007;15(1):53–64 hearing loss among community-dwelling older 135. Kempler D. Neurocognitive Disorders in Aging. Americans: implications for health and function- Thousand Oaks, CA: Sage Publications; 2005 ing. Am J Public Health 2004;94(5):823–829 136. Savundranayagam MY, Hummert ML, Mont- 152. Mick P, Kawachi I, Lin FR. The association gomery RJV. Investigating the effects of commu- between hearing loss and social isolation in older nication problems on caregiver burden. J Gerontol adults. Otolaryngol Head Neck Surg 2014;150(3): B Psychol Sci Soc Sci 2005;60(1):S48–S55 378–384 137. Orange JB. Perspectives of family members re- 153. Seeman TE, Kaplan GA, Knudsen L, Cohen R, garding communication changes. In: Lubinski R, Guralnik J. Social network ties and mortality eds. Dementia and Communication. Philadel- among the elderly in the Alameda County Study. phia, PA: Mosby; 1991:168–186 Am J Epidemiol 1987;126(4):714–723 138. Ripich DN, Horner J. The neurodegenerative 154. Shankar A, Hamer M, McMunn A, Steptoe A. dementias: diagnoses and interventions. ASHA Social isolation and loneliness: relationships with Leader 2004;9:4–5 cognitive function during 4 years of follow-up in 139. Kinney JM, Stephens MA. Hassles and uplifts of the English Longitudinal Study of Ageing. Psy- giving care to a family member with dementia. chosom Med 2013;75(2):161–170 Psychol Aging 1989;4(4):402–408 155. Marsiske M, Klumb P, Baltes MM. Everyday 140. Yaffe K, Fox P, Newcomer R, et al. Patient and activity patterns and sensory functioning in old caregiver characteristics and nursing home place- age. Psychol Aging 1997;12(3):444–457 ment in patients with dementia. JAMA 2002; 156. Heyl V, Wahl HW. Managing daily life with age- 287(16):2090–2097 related sensory loss: cognitive resources gain in 141. Carstensen LL, Isaacowitz DM, Charles ST. importance. Psychol Aging 2012;27(2):510–521 Taking time seriously. A theory of socioemotional 157. Pham TM, Winblad B, Granholm AC, Mo- selectivity. Am Psychol 1999;54(3):165–181 hammed AH. Environmental inﬂuences on brain 142. Tomaka J, Thompson S, Palacios R. The relation neurotrophins in rats. Pharmacol Biochem Behav of social isolation, loneliness, and social support to 2002;73(1):167–175 disease outcomes among the elderly. J Aging 158. Neeper SA, Go´mez-Pinilla F, Choi J, Cotman Health 2006;18(3):359–384 CW. Physical activity increases mRNA for brain- 143. House JS. Social isolation kills, but how and why? derived neurotrophic factor and nerve growth Psychosom Med 2001;63(2):273–274 factor in rat brain. Brain Res 1996;726(1-2):49–56 144. House JS, Robbins C, Metzner HL. The associa- 159. Lee J, Seroogy KB, Mattson MP. Dietary restriction of social relationships and activities with tion enhances neurotrophin expression and neuro- mortality: prospective evidence from the Tecum- genesis in the hippocampus of adult mice. J seh Community Health Study. Am J Epidemiol Neurochem 2002;80(3):539–547 1982;116(1):123–140 160. Fried LP, Carlson MC, McGill S, et al. Experi- 145. Green AF, Rebok G, Lyketsos CG. Inﬂuence of ence Corps: a dual trial to promote the health of social network characteristics on cognition and older adults and children’s academic success. Con- functional status with aging. Int J Geriatr Psychi- temp Clin Trials 2013;36(1):1–13 atry 2008;23(9):972–978 161. Carlson MC, Erickson KI, Kramer AF, et al. 146. Oxman TE, Berkman LF, Kasl S, Freeman DH Evidence for neurocognitive plasticity in at-risk

Jr, Barrett J. Social support and depressive symp- older adults: the experience corps program. J This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. toms in the elderly. Am J Epidemiol 1992;135(4): Gerontol A Biol Sci Med Sci 2009;64(12): 356–368 1275–1282 147. Strogatz DS, James SA. Social support and hy- 162. Fratiglioni L, Paillard-Borg S, Winblad B. An pertension among blacks and whites in a rural, active and socially integrated lifestyle in late life southern community. Am J Epidemiol 1986; might protect against dementia. Lancet Neurol 124(6):949–956 2004;3(6):343–353 148. House JS, Landis KR, Umberson D. Social rela- 163. Schoeni RF, Freedman VA, Martin LG. Why is tionships and health. Science 1988;241(4865): late-life disability declining? Milbank Q 2008; 540–545 86(1):47–89 149. Pantell M, Rehkopf D, Jutte D, Syme SL, Balmes 164. Zhan W, Cruickshanks KJ, Klein BE, et al. J, Adler N. Social isolation: a predictor of mortal- Generational differences in the prevalence of ity comparable to traditional clinical risk factors. hearing impairment in older adults. Am J Epi- Am J Public Health 2013;103(11):2056–2062 demiol 2010;171(2):260–266 HEARING, COGNITION, AND HEALTHY AGING/PICHORA-FULLER ET AL 139

165. Kujawa SG, Liberman MC. Acceleration of age- tematic review of the evidence. J Am Acad Audiol related hearing loss by early noise exposure: evi- 2005;16(7):485–493 dence of a misspent youth. J Neurosci 2006;26(7): 175. Laplante-Levesque A, Hickson L, Worrall L. 2115–2123 Stages of change in adults with acquired hearing 166. Jorgensen L, Palmer C, Fischer G. Evaluation of impairment seeking help for the first time: hearing status at the time of dementia diagnosis. application of the transtheoretical model in Audiol Today 2014;26(1):38–45 audiologic rehabilitation. Ear Hear 2013; 167. Dupuis K, Pichora-Fuller MK, Chasteen AL, 34(4):447–457 Marchuk V, Singh G, Smith SL. Effects of 176. Laplante-Levesque A, Bra¨nnstro¨m KJ, Ingo E, hearing and vision impairments on the Montreal Andersson G, Lunner T. Stages of change in Cognitive Assessment. Neuropsychol Dev Cogn adults who have failed an online hearing screen- B Aging Neuropsychol Cogn 2015;22(4):413–437 ing. Ear Hear 2015;36(1):92–101 168. Fuller P, Kathleen M. Revolutionizing audiologic 177. Preminger JE. Should significant others be en- rehabilitation using approaches from health and couraged to join adult group audiologic rehabili- social psychology to facilitate adjusting to and tation classes? J Am Acad Audiol 2003;14(10): living with hearing loss. Hear J. 2015;68(5):6 545–555 169. Kricos P. Providing hearing rehabilitation to 178. Cummings J, Sproull L, Kiesler S. Beyond hear- people with dementia presents unique challenges. ing: where real-world and online support meet. Hear J 2009;62(11):39–42 Group Dyn 2002;6:78–88 170. Alberti PW. Hearing loss in the elderly: the hidden 179. Ballard C, Gauthier S, Corbett A, Brayne C, handicap. Can Fam Physician 1977;23:91–93 Aarsland D, Jones E. Alzheimer’s disease. Lancet 171. Davis A, Smith P, Ferguson M, Stephens D, 2011;377(9770):1019–1031 Gianopoulos I. Acceptability, benefit and costs 180. World Health Organization (WHO). Global age- of early screening for hearing disability: a study of friendly cities: a guide. Geneva, Switzerland: potential screening tests and models. Health WHO Press; 2007. ISBN 9789241547307. Technol Assess 2007;11(42):1–294 Available at: http://www.who.int/ageing/age_- 172. Gates GA, Mills JH. Presbycusis. Lancet 2005; friendly_cities_guide/en/. Accessed April 15, 366(9491):1111–1120 2015 173. Chisolm TH, Johnson CE, Danhauer JL, et al. A 181. Fuller P, Kathleen M. A successful aging perspec- systematic review of health-related quality of life tive on the links between hearing and cognition. and hearing aids: final report of the American Perspectives on Hearing and Hearing Disorders: Academy of Audiology Task Force on the Health- Research and Diagnostics 2014;18:53–59 Related Quality of Life Benefits of Amplification 182. Brookmeyer R, Johnson E, Ziegler-Graham K, in Adults. J Am Acad Audiol 2007;18(2):151–183 Arrighi HM. Forecasting the global burden of 174. Hawkins DB. Effectiveness of counseling-based Alzheimer’s disease. Alzheimers Dement 2007; adult group aural rehabilitation programs: a sys- 3(3):186–191 This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. International Journal of Audiology 2014; 53: 65–76

Review Article

The ICF Core Sets for hearing loss – researcher perspective. Part I: Systematic review of outcome measures identiﬁ ed in audiological research

Sarah Granberg∗ , ‡ , Jennie Dahlstr ö m∗ , Claes M ö ller∗ , Kim K ä h ä ri † & Berth Danermark∗

∗ Audiological Research Centre, Ö rebro University Hospital, School of Health and Medical Sciences/Swedish Institute for Disability Research, Ö rebro University, Ö rebro, Sweden, † Division of Audiology, Institution for Neuroscience and Physiology, Sahlgrens ’ Academy, University of Gothenburg, Gothenburg, Sweden, and ‡ HEAD Graduate School, Link ö ping University, Link ö ping, Sweden

Abstract Objective: To review the literature in order to identify outcome measures used in research on adults with hearing loss (HL) as part of the ICF Core Sets development project, and to describe study and population characteristics of the reviewed studies. Design: A systematic review methodology was applied using multiple databases. A comprehensive search was conducted and two search pools were created, pool I and pool II. Study sample: The study population included adults ( Ն 18 years of age) with HL and oral language as the primary mode of communication. Results: 122 studies were included. Outcome measures were distinguished by ‘ instrument type ’ , and 10 types were identifi ed. In total, 246 (pool I) and 122 (pool II) different measures were identifi ed, and only approximately 20% were extracted twice or more. Most measures were related to speech recognition. Fifty-one different questionnaires were identifi ed. Many studies used small sample sizes, and the sex of participants was not revealed in several studies. Conclusion: The low prevalence of identifi ed measures refl ects a lack of consensus regarding the optimal outcome measures to use in audiology. Refl ections and discussions are made in relation to small sample sizes and the lack of sex differentiation/descriptions within the included articles.

Key Words: Hearing loss; audiology; ICF; ICF core sets; outcome assessment; systematic literature review For personal use only.

The International Classifi cation of Functioning, Disability and shortcomings of the detailed classifi cation, including how to manage Health (ICF) has gained emerging interest during the last decade. the psychological consequences of hearing loss (HL) in relation to ICF was endorsed by the World Health Organization (WHO) in 2001 the classifi cation, or how to distinguish between activity and par- for use as the international standard for describing and measuring ticipation in the classifi cation, were also highlighted and discussed. health and disability within the member states (WHO, 2001a). ICF Other studies have used ICF as a theoretical framework (i.e. related offers a theoretical framework by integrating the social model research into the ICF framework) and discussed the implications of (wherein disability is viewed as consequences of societal conditions) ICF on different perspectives of audiology (Hickson & Scarinci, and the medical model (for which disability is viewed as being con- 2007; Kiessling et al, 2003; M ö ller, 2003; M ö ller et al, 2009; nected to the bodily dimension of a person, caused by a disease) into Saunders et al, 2005), whereas others investigated the methods of

Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 a bio-psycho-social approach. ICF is also a classifi cation , with classifi cation (i.e. used the different ICF categories in the research) numerical category codes developed for use in clinical settings or in relation to, e.g. Mé ni è re s disorder (Levo et al, 2010), tinnitus research (WHO, 2001b). ICF has previously gained interest within (Ramkumar & Rangasayee, 2010), and third-party disability (Scarinci the audiological research fi eld. Stephens (2001) discussed the advan- et al, 2009). tages and shortcomings of ICF compared to its precursor, the Inter- The classifi cation is based on a hierarchical structure and national Classifi cation of Impairments, Disabilities, and Handicaps consists of two parts: (A) Functioning & Disability and (B) (ICIDH). The author emphasized the strengths of the theoretical Contextual factors . Each part, in turn, consists of two compo- model, such as the transformation from the negative concepts of nents: (A1) Body structures (anatomical parts of the body) & ‘ impairment, disability, and handicap ’ to the more positive terms Body functions (physiological functions of body systems), (A2) ‘ functioning, activity, and participation’ . These new concepts were Activities (execution of a task or an action by an individual) & based on individuals ’ capacities rather than their incapacities. The Participation (involvement in a life situation), (B1) Environ mental

Correspondence: Sarah Granberg, Audiological Research Centre, Ö rebro University Hospital, SE-701 85 Ö rebro, Sweden. E-mail: [email protected]

(Received 20 May 2013 ; accepted 1 October 2013 ) ISSN 1499-2027 print/ISSN 1708-8186 online © 2014 British Society of Audiology, International Society of Audiology, and Nordic Audiological Society DOI: 10.3109/14992027.2013.851799 66 S. Granberg et al.

ranging from genetics to social levels of analysis, and a variety of Abbreviations other types of outcome measures are presumably found when CCT Controlled clinical trial investigating the fi eld. In other core set projects, several other HHIE Hearing handicap inventory for the elderly types of outcome measures have been identifi ed when targeting HL Hearing loss the researcher perspective, such as standard provider-reported or ICIDH International Classifi cation of Impairments, third-party-reported measures (e.g. clinician assessments), non- Disabilities and Handicaps standard measures (e.g. single questions) (Escorpizo et al, 2011), ICD-10 International Classifi cation of Diseases, clinical measures (e.g. joint pain or joint swelling), and technical Tenth revision measures (e.g. X-ray) (Zochling et al, 2006). ICF International Classifi cation of Functioning, The present article focused on the fi rst step (part I) of the Disability and Health researcher perspective by providing a systematic review. In a IOI-HA International outcome inventory for hearing aids review about the psychosocial effects of hearing impairment, sev- PT-S Patient-reported measures – standardized eral weaknesses were identifi ed related to the study and/or popula- RCT Randomized controlled trial tion characteristics of the reviewed studies. The lack of QoL Quality of life longitudinal designs was mentioned, as were the small number of WHO World Health Organization subjects in the included studies, the onset time of HL, the degree of HL, and the lack of sex differentiation (subjects were treated as a unisex group) (Danermark, 2005). Therefore, in addition to outcome measures, study and population characteristics were factors (the physical, social, and attitudinal environment), and extracted from these studies given that comparisons, conclusions, (B2) Personal factors (the particular background of an indivi- and interpretations of research results demand well-described tar- dual ’ s life and living) (WHO, 2001b, pp. 10). Each component get groups. By using an integrative methodology, i.e. by relating (with the exception of personal factors) has associated chapters the fi ndings into a theoretical framework, the researcher perspec- and categories (with assigned numeric category codes) at different tive also provides a unique opportunity for presenting an overview levels. The classifi cation is comprehensive, with more than 1400 of the research fi eld on adults with HL from an ICF perspective categories. To facilitate the applicability of ICF in clinical settings (when combining parts I and II). Given the biopsychosocial or research, the WHO initiated the ICF core sets projects (ICF approach, ICF is useful as a reference model and theoretical Research Branch, 2012; Stucki & Grimby, 2004). A core set is a framework when describing and interpreting trends and para- set of ICF categories of particular relevance for a certain diagno- digms. The typical goal of systematic reviews is otherwise to sis or health condition. Core sets have been developed for various collect evidence for a specifi c research and/or clinical question conditions (for an overview, see ICF Research Branch, 2013). In (Cox, 2005; Hickson, 2009). Several reviews of this variety have 2008, an ICF core sets project targeting adults with HL was initi- been conducted within the audiological area; however, to our For personal use only. ated (Danermark et al, 2010). The general outline of ICF core sets knowledge, this is the fi rst review where audiological research is projects was applied, consisting of a preparatory phase (phase I), referenced in relation to the ICF framework. followed by a consensus conference (phase II) and a validation The objectives of the present study were the following: phase (phase III). Phase I and II of the project have been performed, while phase III remain to be implemented. The objectives 1. Identify outcome measures used in research conducted in adults of the preparatory phase were to collect information about func- with HL as part of the developmental process of the ICF core tioning, disability, and contextual factors relevant to the target sets for HL project. group and to connect this information to ICF categories, thereby 2. Present an overview of research on adults with HL by describing creating a basis for further evaluations (phases II and III) of core the identifi ed outcome measures as well as the study and popula- ICF categories of particular relevance for the given target group. tion characteristics of the included studies. During the preparatory phase, data were collected by conducting four different scientifi c studies targeting the researcher, patient Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 Methods (two studies), and expert perspectives (Danermark et al, 2010). The objective of the researcher perspective was to identify out- Inclusion criteria come measures cited in published studies on adults with HL and The study population was referred to as adults ( Ն 18 years old) to quantify the concepts contained in these measures using ICF as with HL. HL can be a symptom included in various diagnoses and a reference. This task was comprehensive and required several is typically expressed as mild (Ն 25 dB) to profound (Ն 95 dB) steps. In the fi rst step (Part I), a systematic literature review was HL. Patients should also use oral communication as their primary conducted to identify outcome measures used in audiological modes of communication. Study designs: controlled experimental research. In the second step (Part II), the identifi ed outcome mea- studies, i.e. controlled clinical trials (CCTs) and randomized sures were closely investigated to connect them to the different controlled trials (RCTs); observational studies, i.e. longitudinal category codes in ICF. The choice of outcome measures as repre- and cross-sectional studies; chart reviews; economic evaluation sentative of the researcher perspective was based on their roles as analyses based on fi rst-hand data. Sample size: Ն 10 subjects. indicators of the conducted research, refl ecting the objectives of Publication type and language: English-language journal articles. a study. Traditionally, in clinical audiology, there are typically Publication year: 2002 – 2007. Particulars: The focus of the two types of outcome measures: performance-based measures studies should be the target group. Conditions related to HL (such (e.g. pure-tone audiometry) and self-report measures (e.g. the as tinnitus, vertigo, or hyperacusis) were allowed if accompanied abbreviated profi le of hearing aid benefi t, APHAB) (Saunders by HL, as long as the primary focus of as study was issues related et al, 2005). However, the fi eld of audiology research is broad, to HL. Systematic review of outcome measures 67

Exclusion criteria databases contributed proportionally to the total sample. This proce- ® Study populations: non-human populations, subjects Ͻ 18 years old, dure resulted in 431 articles from MEDLINE , 217 from CINAHL, mixed age groups, unclear (or undefi ned) target groups. Study 181 from EMBASE, and 171 from PsycInfo. In the second pool, it designs: Reviews or meta-analyses, psychometric evaluation studies, was possible to manage all located articles. When potentially rele- study protocols, case reports, economic evaluation analysis based on vant articles from the two pools were identifi ed, all of the abstracts reviews or meta-analysis, qualitative studies, primary prevention from these articles were reviewed independently by two researchers. studies, single-subject experimental designs (not crossover or Latin The abstracts were judged with the following designations: “ yes ” to square designs). Sample size, publication type, language, and inclusion, “ no ” to inclusion, or “ ambiguous ” status. An article was year: other than stated. Particulars: Studies wherein the health con- deemed ambiguous if the reviewers disagreed in their decisions or dition of the target population was other than HL but where HL if it was impossible to judge whether the inclusion criteria were met existed comorbid to the condition (e.g. multiple sclerosis) were by reviewing the abstract only. Articles with no available abstract but excluded if the focus was issues not related to HL. with a relevant title were also deemed ambiguous. In cases where the reviewers disagreed about the inclusion of a specifi c article, a discussion took place. If the disagreement still not could be resolved, Inclusion process a third reviewer read the particular abstract and decided whether it D ATABASE SEARCH should be retrieved in full text form or not. All articles for which the A systematic electronic search was conducted in the following nine abstracts were judged as “ yes ” , as well as the remaining “ ambigu- databases: MEDLINE ® (Ovid SP), Cumulative Index to Nursing ous” abstracts, were retrieved in full text (278). All full-text articles and Allied Health Literature (CINAHL) (Ebsco), The Excerpta were reviewed by the fi rst author, and 33% of the total amount were Medica Database (EMBASE) (Ovid SP), PsycInfo (Ovid SP), the divided among and separately reviewed by three of the other research- Allied and Complementary Medicine Database (AMED) (Ovid ers. These steps were taken to enhance the reliability of the data SP), Education Resources Information Center (ERIC) (CSA extraction process. A consensus was reached between the researchers Illumina), Sociological Abstracts (CSA Illumina), PsycArticles regarding the inclusion criteria for articles as well as the strategies (CSA Illumina), and the Cochrane Central Register of Controlled for identifying outcome measures used in the articles. All full-text Trials (CENTRAL) (Cochrane library). Although extensive audio- articles were checked for inclusion. Altogether, 87 articles in pool I logical research can be found in such databases as Medline/PubMed and 35 in pool II (122 total) fulfi lled the inclusion criteria and were and Cinahl (Cox, 2005; Hawkins, 2005; Hickson, 2009), additional thus used in this review. databases were selected to ensure a broad, interdisciplinary sam- pling approach. To fi nd appropriate search terms, possible terms D ATA EXTRACTION for different diagnoses for which HL could be a symptom were Information about country, study design, sex, diagnosis (or type of considered, as well as terminology used for the target group in the HL), and other information about the target group were retrieved, as scientifi c literature (i.e. other systematic literature reviews within were the outcome measures used in each particular study. For personal use only. the area or scientifi c studies). The standardized search terms The term ‘ outcome measures ’ is occasionally used solely for mea- (e.g. the Mesh, Emtree, Cinahl headings) in the different databases sures of intervention studies; however, following the outline of pre- were also used. The strategy was adapted to the conditions of the vious ICF core sets projects, the term is used in a broader sense different databases (e.g. tree structures of subject headings, use of here (e.g. Post et al, 2010; Scheuringer et al, 2005; Zochling et al, Boolean operators, the explode and focus options). Examples of 2006). An outcome measure was defi ned as any measure reported search terms included the following: text words: hearing loss, hear- in the methods or results sections if it provided information neces- ing impairment, hearing disability, hearing disorder, deaf. Examples sary to address the study aims of an included article. The measures of subject headings included the following: hearing loss, otosclero- were extracted independently of the study results. Only measures sis, retrocochlear diseases, hearing disorder, high-frequency hearing related to the target group were extracted, i.e. instruments targeting loss, noise-induced hearing loss, and hearing aids. signifi cant others were not extracted. Measures used only to describe the study population that were unrelated to the study aims were not P ROCEDURE Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 included. The outcome measures were sorted by instrument type , In this systematic review, it was impossible to narrow the search by and 10 different types were identifi ed: (1) patient-reported adding more specifi c search terms as often is the case in other sys- measures – standardized (PT-S) (including generic and condition- tematic reviews, given that this strategy would have excluded poten- specifi c self-report measures, such as the Short Form -36 [SF-36] tially relevant articles. The use of broader search terms resulted in a and APHAB; survey questionnaires wherein the respondent large sample of possible studies to include and was managed by answered standardized questions), (2) health professional-reported dividing the search into two different pools (Figure 1). The databases measures – standardized (HP-S) (the health professional evaluated varied largely according to size, and the fi rst pool contained located the patient’ s performance using standardized forms), (3) standardized ® articles from large databases, i.e. MEDLINE , CINAHL, EMBASE, tests (quasi-objective measures following a standardized procedure, and PsycInfo (8355), to avoid any disadvantages due to database i.e. the test instructions, performance, evaluation, scoring, and inter- size. The second pool contained articles located in smaller databases; pretation of the achievement, were standardized. Subject participa- i.e. AMED, ERIC, Sociological Abstracts, PsycArticles, and CEN- tion in the procedure was required, e.g. pure-tone audiometry, TRAL (1038). All potentially relevant articles were handled through speech audiometry, Stroop color word test), (4) technical measures a reference system (EndNote ® Windows Version X5, Copyright © (objective measures following a standardized procedure, e.g. tym- 1988– 2011 Thomson Reuters) to identify duplicates. After the dupli- panometry, auditory brainstem response [ABR], vestibular evoked cate check, 5715 and 832 potentially relevant articles remained in myogenic potential [Vemp]), (5) laboratory measures (e.g. pools I and II, respectively. From the fi rst pool, a simple random measures of blood lipids, blood count, immune tests), (6) imaging sample (1000) was selected. The sample was constructed so that the techniques (e.g. magnetic resonance imaging [MRI] or computed 68 S. Granberg et al. For personal use only. Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14

Figure 1. Process of inclusion in the review.

tomography [CT]), (7) health professional observations (e.g. of persons with HL using oral language as the primary mode of physical examination) , (8) patient-reported measures – non communication and culturally deaf persons using sign language, standardized (e.g. patients ’ answers to single questions), (9) all of whom were referred to as deaf. Such discrepancies made it healthcare delivery indicators (e.g. treatment costs), and (10) impossible to determine whether the target group was appropriate; register data (e.g. data from national statistical databases). thus, the studies were excluded. In pool I, another common reason for exclusion was mixed age groups, i.e. the population was mixed E XCLUDED STUDIES according to age and not stratiﬁ ed by age in the analyses. This In this review, 156 articles were excluded (Figure 1). An analysis approach made it impossible to judge whether the results were valid of the excluded articles indicated that the most common reason for for children or adults. In pool II, the wrong publication type was exclusion in both pools was use of an inappropriate/ambiguous the second most common reason for exclusion. Some databases did target group, such as in studies where the target group was restricted not have ‘ journal articles’ as a ﬁ ltering option for searches. Thus, to culturally deaf persons with sign language as their primary form articles labeled with the wrong publication type were included of communication or target groups for which the primary condition in the total number of located articles but were excluded after was not related to HL. In some studies, there was also a mixture reviewing them. Systematic review of outcome measures 69

Results their populations, whereas 6% used male-only populations. In pool II (n ϭ 35), 46% of the studies did not specify the sex of the study Of the 122 included studies, 32 were extracted from MEDLINE ® , population. Fifty-two percent used a mixed population, and 2% used 27 from CINAHL, 17 from CENTRAL, 15 from EMBASE, 14 males only. None of the studies (in pools I and II) used a female-only from AMED, 13 from PsycInfo, three from ERIC, and one from population. PsycArticles.

H EARING LOSS Study characteristics Types of HL or the diagnosis of the respondents described in the As displayed in Table 1, the included studies were largely concen- included articles were categorized according to ICD-10 (WHO, trated in North America and Europe. Several studies used very few 2009) based on information about the target group found in participants. For instance, of the group sample size 10 – 25, 44% in the articles (Table 2). This information was provided in 67% of the pool I and 75 % in pool II were experimental studies, i.e. many articles in pool I and in 69% in pool II. The most common type/ studies with few participants were also controlled studies where the diagnosis described was sensorineural HL (H90.3) (defi ned in arti- participants were divided into comparative groups (or where indi- cles as e.g. sensorineural, cochlear, or high-frequency HL). The label viduals acted as their own control). Most studies in the fi rst pool ‘ Various ’ indicates a mixed target group according to diagnosis. were judged to be observational studies. Of these, only 8% were Regarding descriptions of the target groups, very little information longitudinal studies. In pool II, experimental studies were the most was revealed. The participants were mostly described by hearing- common, and 49% of these studies were judged to be RCTs. Exam- related information, such as being a ‘ hearing-aid user’ (25% pool I; ples of experimental studies were cross-over studies in which the 26% pool II), or a ‘ CI user ’ (15% in pool I; 9% in pool II). individual acted as his or her own control when evaluating specifi c features of hearing aids or Latin square designs, such as a compari- Outcome measures son of three or more stimulation rates in cochlear implant (CI) sys- Altogether, an extensive number of outcome measures were extracted tems, using a cross-over design. All observational studies in the from the articles under consideration. Two hundred and forty-six dif- second pool were cross-sectional. Intervention types within experi- ferent outcome measures were identifi ed in pool I, and 122 were mental studies were categorized, and the most common type in pool identifi ed in pool II (Table 3). Note the low prevalence of the identi- I was interventions related to hearing aids (52%). The corresponding fi ed outcome measures; most measurements occurred only once in category in pool II was interventions related to drugs (40%). this review. In fact, only 21.1% of the measures in pool I and 17.2% in pool II were extracted as outcome measures twice or more . Selected Population characteristics types of outcome measures are presented in the results section. S EX All extracted outcome measures are presented in a Supplementary Information about the sex of a population was retrieved from the Appendix 1, which is available in the online version of the journal.

For personal use only. included articles. Of the 87 articles in pool I, 31% did not describe Please fi nd this material with the direct link to the article at: http:// their population according to sex, nor was sex used as a variable in informahealthcare.com/doi/abs/10.3109/14992027.2013.851799. the analyses. Sixty-three percent used both males and females in P ATIENT-REPORTED MEASURES – STANDARDIZED (PT-S) The results from pool I revealed 39 different PT-S that were used as Table 1. Study characteristics of the 122 included studies. outcome measures. Of these, only 11 PT-S appeared twice or more Pool I Pool II in this sample. Sixty-nine percent of the PT-S were condition specifi c (n ϭ 8 7 ) (n ϭ 3 5 ) (i.e. questionnaires designed for audiology). The questionnaire most Frequency Frequency frequently detected in this sample was the the Hearing Handicap Inventory for the Elderly (HHIE) (Ventry & Weinstein, 1982), Continent which was identifi ed fi ve times (2.0%). In the second pool, 23 Asia 8 (9.2%) 3 (8.6%) different PT-S were found, yet only two appeared twice or more. Australia 3 (3.4%) 1 (2.9%) Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 Seventy percent were condition specifi c. In this group, the HHIE and Europe 39 (44.8%) 14 (40.0%) North America 36 (41.4%) 17 (48.6) South America 1 (1.1%) – Table 2. Types of hearing loss classifi ed into ICD-10 categories. Sample size 10 – 25 31 (35.6%) 8 (22.9%) Pool I Pool II 26 – 40 15 (17.2%) 6 (17.1%) (n ϭ 8 7 ) (n ϭ 3 5 ) 41 – 60 11 (12.6%) 3 (8.6%) Frequency Frequency 61 – 100 11 (12.6%) 7 (20.0%) 101 – 150 9 (10.3%) – Sensorineural HL (H90.3) 32 (36.8%) 15 (42.9%) 151 – 200 4 (4.6%) 3 (8.6%) M é ni è re disease (H81.0) 6 (6.9%) – 201 – 500 5 (5.7%) 3 (8.6%) Sudden idiopathic HL (H91.2) 6 (6.9%) 4 (11.4%) Ն 5 0 1 1 (1.1%) 5 (14.3%) Otosclerosis (H80.9) 4 (4.6%) – Study design Other disorders of ear, not elsewhere classifi ed Chart reviews 7 (8.0%) 2 (5.7%) (H93) (central auditory processing disorder) 1 (1.1%) – Economic evaluations 1 (1.1%) 1 (2.9%) Presbyacusis (H91.1) 1 (1.1%) – Experimental studies 25 (28.7%) 21 (60.0%) Noise-induced HL (H83.3) 1 (1.1%) – Mixed quantitative design 2 (2.3%) – Various 7 (8.0%) 5 (14.3%) Observational studies 52 (59.8%) 11 (31.4%) Not defi ned 29 (33.3%) 11 (31.4%) 70 S. Granberg et al.

Table 3. Outcome measures divided into instrument types from the of speech sounds, with no semantic processing required; patient included studies. repeats the stimuli), and speech comprehension (the patient is required to attach meaning to the stimulus). This further division Pool I Pool II was necessary due to indistinctive labeling of the different speech/ ϭ ϭ (n 246) (n 122) words measures in the included articles. When dividing and Frequency Frequency labeling the different speech/word measures, attention was paid to PT-S 39 (15.8%) 22 (18.0%) the descriptions of the measurement objectives and test procedures Standardized 87 (35.4%) 25 (20.5%) within the included studies. In total, 35 different speech/word mea- HP-S 4 (1.6%) – sures with/without noise were identifi ed in pool I, all labeled as Technical 22 (8.9%) 3 (2.5%) speech recognition tests. Speech/word measures comprised 40.2% Laboratory 24 (9.8%) 33 (27.1%) of all standardized outcome measures. ‘ Hearing in noise test’ (HINT) Ͻ Imaging 3 (1.2%) 1 ( 1.0%) (Nilsson et al, 1994) was the one most frequently identifi ed indices HPO 7 (2.9%) 4 (3.3%) in this sample (2.0%), followed by the ‘ Connected speech test’ (CST) PT-not S 56 (22.8%) 26 (21.3%) (Cox et al, 1987) and ‘ undefi ned test of speech recognition ’ (1.6% Health care delivery indicators 4 (1.6%) 1 (Ͻ 1.0%) Register data – 7 (5.7%) each). The second largest group was labeled as cognitive measures . This group constituted 21.8% of all standardized measures and contained 19 different measurements, each with a prevalence of one. the Hearing Disability Handicap Scale (HDHS) (H é tu et al, 1994) Examples of these identifi ed measurements included the ‘ Stroop were identifi ed most frequently (1.6%, equal to twice each). color word test ’ (Stroop, 1935), the ‘ Concept shifting task ’ (CST) Altogether, 51 different PT-S were found in this review containing (Vink & Jolles, 1985), and the ‘ Benton visual retention test’ (Benton, 122 included articles. Only 16 of them were used twice or more 1978). The measures with high prevalence were tests used to defi ne when combining pools I and II (Table 4). the hearing levels, i.e. ‘ pure-tone audiometry ’ (11.4%), ‘ B é k è sy audiometry ’ , and ‘ free-fi eld audiometry’ (once each). Within pool II, S TANDARDIZED MEASURES seven categories were established out of the 25 identifi ed standard- The standardized measures were divided into subgroups according ized measures. Speech/word measures with/without noise comprised to the overall aims of each specifi c measure. Twenty-fi ve subgroups the largest group in this pool as well, containing 64.0% of all were constructed out of the 87 different standardized measures found standardized measures in this pool. All of the measures in this in pool I. Seven of these subgroups contained outcome measures that subgroup, except one, were labeled as measures of speech have been identifi ed twice or more in this review (Table 5). The recognition (16 different measures). ‘ CST ’ and ‘ City University of largest subgroup (i.e. the group with the most measures) was NY sentences in quiet’ (CUNY) (Boothroyd et al, 1985) were iden- speech/word measures with/without noise. This subgroup was fur- tifi ed most frequently (2.5%). One measure was labeled speech ther divided into four subdomains according to the hierarchical discrimination, namely, the ‘ Tone discrimination test’ (TDT), which For personal use only. organization of levels of auditory processing (Erber, 1982; demands that subjects discriminate between tonal patterns of Thibodeau, 2007), including the following: speech awareness (the pairs of syllables. The test is part of the Hong Kong Speech Percep- detection of speech sounds), speech discrimination (the detection of tion Test Manual (HKSPTM) (described in Au, 2003). As in pool I, changes in the acoustic stimuli), speech recognition (the recognition cognitive measures were the second largest group. This subgroup

Table 4. Overview of the most frequently identiﬁ ed PT-S in this review. The references are available as a Supplementary Appendix 2 in the online version of the journal. Please ﬁ nd this material using the direct link to the article at: http://informahealthcare.com/ doi/abs/10.3109/14992027.2013.851799.

Pool I Pool II Instrument (n ϭ 246) (n ϭ 122) type ∗ Frequency Frequency Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 The Hearing Handicap Inventory for the Elderly (HHIE). (Ventry & Weinstein, 1982) CS 5 (2.0%) 2 (1.6%) The Abbreviated Profi le of Hearing Aid Benefi t (APHAB). (Cox & Alexander, 1995) CS 3 (1.2%) 1 (Ͻ 1.0%) Hearing Disability and Handicap Scale (HDHS). (H é tu et al, 1994) CS 2 (Ͻ 1.0%) 2 (1.6%) Hearing Handicap Inventory for the adults (HHIA). (Newman et al, 1990) CS 2 (Ͻ 1.0%) 1 (Ͻ 1.0%) Satisfaction with amplifi cation in daily life (SADL). (Cox & Alexander, 1999) CS 2 (Ͻ 1.0%) 1 (Ͻ 1.0%) Short Form – 36 (SF-36). (Ware et al, 1993) G 2 (Ͻ 1.0%) 1 (Ͻ 1.0%) The Communication Profi le for the Hearing Impaired (CPHI). (Demorest & Erdman, 1987) CS 2 (Ͻ 1.0%) – Communication Scale for Older Adults (CSOA). (Kaplan et al, 1997) CS 1 (Ͻ 1.0%) 1 (Ͻ 1.0%) Glasgow Benefi t Inventory (GBI). (Robinson et al, 1996) G 1 (Ͻ 1.0%) 1 (Ͻ 1.0%) The Hearing Aid Performance Inventory (HAPI). (Walden et al, 1984) CS 1 (Ͻ 1.0%) 1 (Ͻ 1.0%) The Hearing Handicap and Disability Inventory (HHDI). (van den Brink et al, 1996) CS 1 (Ͻ 1.0%) 1 (Ͻ 1.0%) Health Utilities Index Mark III (HUI3). (Feeny et al, 1995) G 2 (Ͻ 1.0%) – The International Outcome Inventory for Hearing Aids (IOI-HA). (Cox et al, 2000) CS 2 (Ͻ 1.0%) – Performance Inventory for Profound and Severe Loss (PIPSL). (Owens & Raggio, 1988) CS 2 (Ͻ 1.0%) – Short Form -12 (SF-12). (Ware et al, 1996) G 1 (Ͻ 1.0%) 1 (Ͻ 1.0%) The Speech, Spatial and Qualities of Hearing Scale (SSQ). (Gatehouse & Noble, 2004) CS 2 (Ͻ 1.0%) –

∗ CS : condition-speciﬁ c instrument; G : generic instrument. Systematic review of outcome measures 71

Table 5. Overview of standardized outcome measures identifi ed contained single questions, and every unique question was counted twice or more in this review. as a separate outcome measure. The outcome measure in pool I with the highest prevalence was ‘ MRI ’ (imaging technique ) (3.3%), Pool I Pool II followed by ‘ preferred HA setting ’ (PT-not S ) (2.0%) and then ϭ ϭ (n 246) (n 122) ‘ age ’ , ‘ family history of HL ’ , ‘ education ’ , ‘ diffi culties associated Frequency Frequency with HL ’ , and ‘ onset time of HL ’ , all belonging to PT-not S and Speech measures with/without noise identifi ed three times (1.2%) each. The outcome measure ‘ otoscopic Hearing in noise test (HINT) 5 (2.0%) 2 (1.6%) examination ’ (HPO ), ‘ civil status ’ (PT-not S ), ‘ subjective experience Speech recognition test 4 (1.6%) 2 (1.6%) of HL ’ (PT-not S ), ‘ employment ’ (PT-not S ), ‘ smoking status ’ Connected speech test (CST) 4 (1.6%) 3 (2.5%) (PT-not S ), ‘ presence of tinnitus ’ (PT-not S ), ‘ associated symptoms ’ Nonsense syllable test (NST) 3 (1.2%) – (PT-not S ), ‘ gender ’ (PT-not S ), and ‘ alcohol consumption ’ (PT-not S ) Speech perception in noise (SPIN) 3 (1.2%) – were identifi ed as outcome measures twice (Ͻ 1%) each. In pool II, Ͻ Speech perception 2 ( 1.0%) – the most prevalent outcome measure was ‘ presence of tinnitus’ Speech discrimination 2 (Ͻ 1.0%) – (PT-not S) (2.5%), followed by ‘ duration of HL’ (PT-not S ), ‘ educa- CNC words in quiet 2 (Ͻ 1.0%) 2 (1.6%) tion ’ ( ), ‘ use of hearing aids ’ ( ), ‘ hearing-aid expe- City University of NY (CUNY) sentences PT-not S PT-not S in quiet – 3 (2.5%) rience ’ (PT-not S ), ‘ presence of vertigo ’ (PT-not S ), and ‘ subjective Defi ne hearing level severity of HL ’ (PT-not S ), all identifi ed twice (1.6%) each. All Pure-tone audiometry 28 (11.4%) 14 (11.5%) other outcome measures in each pool occurred only once. Intelligence test Groningen intelligence test 2 (Ͻ 1.0%) – Discussion Identifi cation of dead regions in the cochlea Threshold equalizing noise (TEN) test 2 (Ͻ 1.0%) – Outcome measures Sound localization This review focuses on outcome measures used in audiological Sound localization performance 2 (Ͻ 1.0%) – research. It is clear that the fi eld is broad and comprehensive, and the Acceptable noise level judgment identifi cation of 10 different instrument types is a clear indication of Acceptable noise level (ANL) test 2 (Ͻ 1.0%) – this complexity. The dataset consisting of identifi ed outcome mea- Loudness measure sures is extremely large, yet no prevalence limits of specifi c measure- Ͻ Paired comparison judgment 2 ( 1.0%) – ments (e.g. to only report measurements identifi ed in Ն 5% of the included studies) have been established in this review. The reason for contained four different measurements, each with a prevalence this lack of limits is the extremely low occurrence of the identifi ed of one (Ͻ 1%). In this group, the ‘ Stroop color word test ’ was iden- measures. Because only 21.1% (pool I) and 17.2% (pool II) outcome tifi ed, as were e.g. the ‘ Listening span test ’ (Pichora-Fuller et al, measures were identifi ed twice or more, the setting of a limit as small For personal use only. 1995). Pure-tone audiometry exhibited a high prevalence in this as an ‘ occurrence of two’ would have excluded approximately 80% pool as well (11.5%). of the material in each group and skewed the results. Whereas the identifi cation of 10 instrument types in this review indicates a com- T ECHNICAL MEASURES prehensive and widely varying research fi eld, the high number and In pool I, 22 different technical measurements were extracted low prevalence of the outcome measures within the groups of instru- from the included studies. Several of these were related to vestibular ment types indicates a sprawling research fi eld with poor agreement function, such as measures of caloric testing, different types of nys- on which measures to use, thereby resulting in reduced possibilities tagmus testing, eye tracking, and the velocity step test. The most for proper comparisons between studies and populations. prevalent measurement was oto-acoustic emissions (OAE) testing (2.4%), followed by ABR testing and tympanometry (1.6% each), P ATIENT-REPORTED MEASURES – STANDARDIZED (PT-S) Vemp testing, as well as the caloric testing and electrocochleography Notably, the number of questionnaires (PT-S) identifi ed in the reviewed studies. In both pools, several questionnaires were used, Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 (ECoG) testing, (1.2% each). In pool II, three different measurements were identifi ed, including OAE testing (2.5%), tympanometry whereas the reported prevalence was extremely low. This observation (2.5%), and stapedial refl ex measurement (Ͻ 1%). also applies for established, generic measures, such as the SF-36 (Ware et al, 1993) and HUI3 (Feeny et al, 1995). An overview of L ABORATORY MEASURES available condition-specifi c questionnaires was conducted in 2000 Twenty-four laboratory measures were extracted in pool I and cat- (Bentler & Kramer, 2000). At that time, 33 condition-specifi c instru- egorized according to the aim of the measurements. Eight different ments existed in audiology. Importantly, 42% of those questionnaires types were identifi ed. Immune tests were used most frequently were found in this review, which considered 122 articles. As com- (4.1%), followed by measurements for glucose (2.0%), blood lipids pared to similar reviews of outcome measures used in such areas as (1.6%), and mutations (1.2%). In pool II, 33 different types of labo- acute infl ammatory arthritis (Zochling et al, 2006), depressive dis- ratory measurements were identifi ed and divided into nine sub- orders (Brockow et al, 2004), musculoskeletal disorders, and chronic groups. Measurements of blood count were the most frequently used widespread pain (Brockow et al, 2004), where there is a high preva- (12.3%), followed by those for blood lipids (6.6%), immune tests lence of relying on a few questionnaires, the area of audiology has (2.5%), and electrolyte concentration (2.5%). no consensus regarding the most appropriate questionnaires. The reasons for this lack of consensus are varied, but possible expla- O THER INSTRUMENT TYPES nations include issues related to the types of measurements (e.g. The other instrument types (not stated above) contained 74 (pool I) condition-specifi c versus generic) and the construction of condition- and 39 (pool II) outcome measures. The instrument type PT-not S specifi c instruments. 72 S. Granberg et al.

G ENERIC INSTRUMENTS GHAB, which are questionnaires sensitive to individual variations, The majority of the generic instruments found in this review were to overcome this issue. In a case study by Connington (2005), the related to the notion of health-related quality of life (HRQoL) , and author questioned the usage of hearing handicap scales as outcome several authors have investigated this measure in relation to audiol- measures when assessing the potential benefi t of amplifi cation. ogy. Morgan et al (2002) investigated the impact of hearing impair- Although this study was a case report and may have low scientifi c ment on the QoL of older Australian people using the SF-36 and value, its message is important. The construction of several self- the HHIE. Among other conclusions, the authors found a lack of assessment scales in audiology is problematic both on group and sensitivity of the SF-36 in hearing-impaired populations. Similar individual levels. Using questions that are not applicable to some conclusions have been drawn in other studies targeting other individuals may result in missing or ambiguous answers that are generic QoL measurements in relation to hearing-aid fi tting (Bess, ultimately impossible to interpret (Fayers & Machin, 2007). Another 2000; Vuorialho et al, 2006). Humans are typically communicating limitation of the identifi ed condition-specifi c PT-S in this review is beings, and participation restriction in relation to communication the number of items they contain. Fifty percent of the condition- is likely one of the most important restrictions for any human specifi c questionnaires (non-survey questionnaires) contain 20 being. For persons with HL, this restriction is a highly recognized items or more. Thus, item reduction should be part of a validation negative consequence of the health condition. Choosing a HRQoL process when constructing a questionnaire, and striving towards measurement that is suffi ciently sensitive to this restriction is dif- brief questionnaires is highly desirable (Fayers & Machin, 2007). fi cult because several of the established HRQoL instruments fail The evaluation of the construction of condition-specifi c question- to highlight restrictions in communication as a health component naires is beyond the scope of this review, but an overview has been (Cruice et al, 2000). In a tutorial of HRQoL measurements and conducted wherein this topic has been evaluated, to some extent hearing aids by Abrams et al (2005), the limitation of generic (Noble,1998). However, an updated meta-analysis concerning instruments according to communication was highlighted. The both validity and reliability issues is a desirable target for future authors suggested the usage of WHODAS-II (WHO, 1999) as a investigations. generic HRQoL measure for use within the audiological research Condition-specifi c questionnaires that use statements that are not fi eld. Based on ICF, the WHODAS-II acknowledges communica- dependent upon cultural contexts and contain only a few items have tion as one of six life domains assessed in this 36-item question- been developed within the audiological fi eld. An example is the naire. The WHODAS-II does not appear in this review but could international outcome inventory for hearing aids (IOI-HA) (Cox & be an instrument worth considering when measuring HRQoL Alexander, 2002). This questionnaire has been translated into vari- within audiology. The WHODAS-II has been psychometrically ous languages and was psychometrically evaluated for different evaluated in relation to adults with acquired HL and is recom- cultures (e.g. Br ä nnstr ö m & Wennerstr ö m, 2010; Gasparin et al, mended for use as a valid, generic HRQoL instrument (Chisolm 2010; Kramer et al, 2002; Liu et al, 2011). Despite these advantages, et al, 2005). Furthermore, the responsiveness of the instrument has the IOI-HA was only identifi ed twice ( Ͻ 1.0%) as an outcome ® been further examined in relation to hearing aid interventions for measure in this review. A control search in the MEDLINE For personal use only. adults, thereby proving its utility as a useful generic measure in and CINAHL databases (text word: “ IOI-HA ” OR “ International these types of interventions (McArdle et al, 2005). Outcome Inventory for Hearing Aids” , including limitations to English language, use of only adults as target group, and a rejection C ONDITION-SPECIFIC INSTRUMENTS of psychometric evaluations) reveals that the instrument appears in An examination of the items used in the condition-specifi c ques- scientifi c studies 22 times up to the present (week 2, 2013). Thus, tionnaires demonstrates that the item construction of the different the true prevalence in audiological research is also quite low. questionnaires is quite similar. There appears to be a clear tradition One possible explanation for this low prevalence is that this instru- to construct statements upon which the respondent should refl ect. ment was designed to evaluate the effectiveness of hearing aid treat- Sixty-four percent of the included questionnaires contain statements, and these types of studies may be underrepresented within ments involving an activity (such as conversing with one person) the audiology research fi eld. However, the construction of the and a specifi c context (e.g. a noisy grocery store). When assessing questionnaire (i.e. few items, nondependent cultural activities and e.g. participation restrictions, there appears to be a clear idea of Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 contexts) should be used as a model when developing new condi- what is relevant to measure (e.g. listening in noisy situations) tion-specifi c questionnaires that target other latent variables. because many of the items across questionnaires share the same latent variable. However, a limitation to consider with reference to S TANDARDIZED MEASURES this type of statement is that these statements often consider spe- Measures related to speech recognition were the outcome measure cifi c target groups in specifi c cultural contexts, resulting in people most frequently identifi ed in this review. These types of measures who are not part of a given group and context; as a result, they may are clearly highly valued and important within the audiological have diffi culties identifying with the current assertion. Such con- research fi eld. However, the amount of different speech recognition sideration makes it diffi cult to conduct cross-cultural (and cross- measures extracted is noteworthy (35 in pool I, 16 in pool II). Sev- country) comparisons, thereby resulting in items with low eral of the identifi ed speech measurements were labeled ‘ unspeci- international equivalence (Arlinger, 2000). Even within a particular fi ed’ due to the lack of references to the literature in their descriptions. culture, there may be individual differences regarding the type of In this review, we assume that these measurements are standardized activities to consider relevant as well as the appropriate contexts. based on clinical experience of the testing procedure, but we cannot This issue has also been highlighted by other authors. Gagn é (2003) be certain because no validity/reliability data are presented for the discussed condition-specifi c questionnaires as outcome variables measurement and no reference to any development procedure is in audiological interventions and acknowledged the problem with made. This lack of information is a disadvantage with these mea- individual variations concerning relevant activities and contexts. sures and of course a major obstacle to comparisons between stud- The author suggested the usage of self-reports, such as COSI and ies. In research, it is highly desirable to use standardized, Systematic review of outcome measures 73

established outcome measures for which the development procedure similar to the Erber distinction used in this review, the term ‘ speech is traceable. recognition’ is far too broad to offer a suffi cient labeling of what is In this review, the label ‘ speech recognition ’ is broad, with no actually required. The audiological fi eld would benefi t from a clear consideration of the contextual condition (noise/no noise, fi xed/ consensus regarding this topic where all of the different processing varied noise levels), presentation mode (fi xed/varied speech levels), levels and testing procedures are considered (e.g. open-versus or scoring options (percent correctly identifi ed speech stimuli/SNR closed-set). Such a consensus would also certainly facilitate the at a speech recognition score of 50% correct). Although the major- choice of outcome measures in research projects, as well as the ity of the extracted speech measures represent different levels of interpretation of research fi ndings. Furthermore, a proper nomen- speech recognition (i.e. nonsense syllables, word and sentence rec- clature would facilitate the identifi cation of areas where there are a ognition) and different levels of auditory processing is required, a lack of measurements or where modern outcome measures must be lower stage of processing is still involved (Schneider et al, 2010). developed. A more modern way of viewing this requirement would be to state that these are all measures of bottom-up information processing. In S TUDY CHARACTERISTICS ICF, these measures are all related to the body function and activity Most scientifi c studies on humans require information on the target component of ICF (Saunders et al, 2005). Speech recognition does population; however, this information may vary according to the not reach the level of participation and/or participation restriction, study aim. For instance, in register studies, the information might as expressed in ICF wording. To cover that domain, a higher level be very poor due to the characteristics of the data and the fi ne line of auditory processing and an interaction with the environment between what to consider as population characteristics and what to (simulated or real) are required. However, it is important to stress consider as outcome measures. In other studies, such as hearing-aid that investigating whether the speech recognition measures identi- trials, there might be a signifi cant point in reporting, e.g. the sex of fi ed in this review have been used in combination with other mea- the participants or the hearing-aid experience, given that they may sures (e.g. measures of top-down information processing) is beyond be considerable confounds in the results. In the review about psy- the scope of this study. In recent years, a growing interest in speech chosocial effects of hearing impairment, Danermark (2005) discov- comprehension has emerged where both auditory and cognitive pro- ered that persons with HL were mainly treated as a unisex group. cessing are regarded as crucial (e.g. Besser et al, 2012; Kramer et al, The present study supports that fi nding but also extends the fi ndings 2009; Pichora-Fuller & Singh, 2006). This trend is important, not to make an additional modifi cation to the reasoning by concluding only from the patient perspective, given that the measures of speech that in addition to presenting limitations in analysing according to comprehension (especially in combination with carefully consid- sex, many studies also fail to describe the target group according to ered noise stimuli) could likely simulate (and perhaps predict) com- sex. In this review, the sexes of the participants are not revealed in prehension problems in the real world rather accurately. 31% (pool I) and 46% (pool II) of the included articles. The question We found it necessary to divide the identifi ed word/speech mea- arises whether there are audiological differences between males and sures into different categories due to indistinctive labeling (e.g. of females. A short review of the subject indicates that sex differences For personal use only. the aim of the measurement) within the included articles. There occur even in such a basic domain as body structure. Sato et al, appears to be no consensus in the audiological research fi eld (1991) used a 3D imaginary technique to analyse the length of male regarding how to designate speech/word measures. Such expres- and female cochleae and concluded that the male cochlea was, on sions as speech discrimination, speech identifi cation, speech intel- average, approximately 14% longer than that of the female. How- ligibility, speech perception, speech recognition, speech reception, ever, these fi gures have been questioned, and comparative studies and speech understanding were used indiscriminately, which is have been prompted to confi rm the results (Miller, 2007). In problematic. One must carefully review the objectives and proce- terms of the body function domain, several studies have observed

dure of a speciﬁ c measurement to be able to evaluate it comprehen- differences between the two sexes. In a study of ƒ 1 - and ƒ2 -sweep sively. This is an important consideration when reviewing research DPOAE phase-delay measurements in normal-hearing adults, the and evidence. Different suggestions have been put forth regarding authors concluded that at low frequencies, these measures were lon- this topic. For example, in a review about the factors that affect the ger in male subjects and that this difference was likely connected

Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 speech-understanding abilities of older adults, the term speech to sex-related anatomical differences in cochlear length (Bowman understanding was used when referring to open-set recognition or et al, 2000). When investigating the hearing function assessed closed-set identifi cation of nonsense syllables, words, or sentences. with ABR, several studies concluded that the two sexes exhibited Speech understanding was distinguished from the higher-level audi- differences in terms of ABR wave amplitude and latency values tory process term ‘ speech comprehension ’ , even though the authors (e.g. Don et al, 1993; Jerger & Johnson, 1988). Furthermore, dif- clearly stated that the defi nitions of the two concepts were similar ferences between the two sexes were also observed in the activity (Humes & Dubno, 2010). Although this reasoning is welcomed, the and participation domain. Garstecki & Erler (1999) explored sex term understanding might bring the psychological process term differences in self-perceived hearing handicap among older adults. ‘ understanding ’ (i.e. comprehending) to mind and might therefore For instance, they concluded that older women reported more com- be confusing as a standard nomenclature for this type of measure munication-related problems and negative reactions to their HL in the audiological fi eld. In 2003, an initiative was made to divide compared to the men in the study. External infl uences on function- levels of auditory processing (i.e. hearing, listening, comprehending and disability (i.e. environmental factors) have also been ing, and communicating) according to ICF (Kiessling et al, 2003). explored in relation to sex. In a large survey targeting hearing-aid Speech recognition would, in ICF terms, be labeled as a combina- owners, it was concluded that women were more likely to use their tion of b230 ( hearing functions ), b1560 (auditory perception), and HAs daily, all day long, and more regularly than men (Staehelin d115 (listening ) based on its non-semantic processing requirements, et al, 2011, pp. 33). There are clearly differences between the two whereas speech comprehension would be d310 ( communication sexes that must be considered when conducting studies. However, with-receiving-spoken messages) (Granberg et al, In press). However, a thorough systematic review targeting the issues related to all 74 S. Granberg et al.

ICF components is highly desirable and would benefi t the entire Because the HHIE was the questionnaire identifi ed most fre- research fi eld. quently, a prevalence check was made of that specifi c question- Danermark (2005) also found that many studies used small naire. The result indicated that HHIE has only been used 12 times sample sizes, and this observation was confi rmed by the present in MEDLINE ® (English language as a limitation) and one time study. In fact, most studies used samples sizes of 10– 25 participants. in CINAHL from 2008 up to the present (week 2, 2013). However, Several of these studies were labeled ‘ experimental studies ’ . if one extends the search to further include the HHIE-S (i.e. the Although no further analysis of the included studies was made in screening version), the prevalence increases to 24. In conclusion, this matter, some remarks must be made. Small sample sizes may the frequency is still low, but there is an increase in use of the be used in controlled experimental studies, provided that one has HHIE-S from 2008 and up to present. HHIE-S was not identifi ed control over all possible variables that might infl uence the results. in the 2002 – 2007 sample. Thus, when using small sample sizes in controlled experimental When searching the EMBASE database, which was the fi rst studies, high demands must be placed on design issues. In studies database used in this review, a search fi lter related to study type where the heterogeneity of the population makes comparisons dif- (e.g. RCT, CCT, cross-sectional) was added to the search strategy. fi cult, a fruitful solution would be to let subjects serve as their own This restriction limited the number of search results considerably. controls to increase the reliability of a study. Another solution to Unfortunately, when searching in the other databases, we realized this matter would be to use larger sample sizes. that this limitation excluded records that were potentially relevant. Therefore, this fi lter was excluded when searching the other eight databases. Many studies, particularly non-experimental studies, were Methodological discussion not indexed in the different databases according to study type. How- The search strategy in this review was quite different from that of a ever, EMBASE is a medical database, and many duplicates were traditional systematic review, where a common way of tapering the found between EMBASE and, e.g. MEDLINE ® , CENTRAL, and search is by adding more specifi c search terms. Unfortunately, this CINAHL. It is not likely that many studies were excluded in task was impossible because the entire audiological research fi eld EMBASE due to this limitation. Also, the medical dimension of the was the target of inquiry. No limitations were possible except for the audiological research fi eld is well represented among the studies inclusion criteria. Creating a simple random sample of articles of included in this review, and thus, it is not likely that this limitation large databases was a useful solution to limit the search. Statistics in the search strategy affected the result considerably. would typically be used to assess the reliability of a sample (e.g. In total, 1554 abstracts were rejected for inclusion in this review. confi dence interval). Because this review was conducted in several This high number of rejections is tied to the unspecifi c search strat- steps (i.e. search in databases, duplicate check, random sample, egy, where no specifi c search terms were added to taper the search. abstract reviewing, full text reviewing, fi nal decision of inclusion), Some valuable lessons might be learned regarding this matter. there was no accurate way to statistically evaluate the sample in First, including the search term deaf will undoubtedly extend the relation to all of the articles identifi ed by the search. However, the number of hits in the search. There is a tradition, perhaps in the more For personal use only. face validity of the sample was high. For instance, 1000 abstracts social fi eld of audiology, to refer to persons with HL with the distinc- have been reviewed in pool I, which is a signifi cant number. tion of deaf versus Deaf, where the capital D deafness refers to Measures of speech/word recognition are very common in this the cultural aspects of deafness and deaf refers to an audiometric review, and these types of measurement have a long tradition in loss. Unfortunately, this distinction is not acknowledged in the dif- audiology as valuable outcome measures and are often referred ferent databases, resulting in cultural deafness also being included to when discussing the outcomes measures used in audiology in the search when using the search term deaf . However, excluding (Gatehouse, 1998; Saunders et al, 2005). HHIE is the PT-S identifi ed this search term presents the possibility that articles using this most frequently in this review. In a Swedish study that examined terminology (deaf) would be excluded. which condition-specifi c instruments were used most frequently Although the various search limitations offered in the different within audiological research (2000– 2010), the authors concluded databases were used, such as excluding case reports, children studies, that the APHAB was used most frequently, whereas the HHIE was and psychometric studies, these types of studies were still included in the fi nal result. One must consider that the search limiting func- Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 the second most used (Berget & Karlsson, 2010). In this review, the order was opposite; however, the top two in that study were also the tions in databases are only useful when an article is indexed with a top two in this review. This literature search was conducted as the specifi c search term matching that limitation term. If not, the article fi rst step in the entire ICF core sets for HL development process that will still be considered in the result. was initiated in 2008 (Danermark et al, 2010). Thus, the year limit in the search was set as 2002– 2007. As a reliability check of the Conclusions results, a prevalence search of the PT-S was conducted from year 2008 up to the present (week 2, 2013) in the MEDLINE ® and Several conclusions can be drawn from this investigation: CINAHL databases. These are discriminating databases, i.e. in • Based on the perspective of the ICF core sets for HL, a rich CINAHL, is it possible to exclude MEDLINE ® records, resulting in source of material representing the researcher perspective has no duplicate results. The search was conducted using text words and been obtained that will be linked to the ICF framework by using subject headings of ‘ self-assessment∗ OR ‘ questionnaire∗ ’ O R established linking rules in a subsequent study. ‘ scale∗ ’ in combination with relevant search terms for audiology • A substantial number of outcome measures are available and (used in this review). In total, 11.5% of the hits contained articles used within the audiological research fi eld. Although the wide that used some type of PT-S. Compared to the result in this review variety of instrument types refl ects a broad research fi eld, the (16.2% of pool I), this observation is an indication that PT-S are amount of different outcome measures, as well as the low still rarely used as outcome measures within the audiological prevalence of the identifi ed measures within each instrument research fi eld, thereby reinforcing the reliability of this review. type, refl ects a lack of consensus regarding outcome measures Systematic review of outcome measures 75

in audiology, which in turn compromises both national and Bowman D.M. , Brown D.K. & Kimberley B.P . 2000 . An examination of international individual and group comparisons. gender differences in DPOAE phase delay measurements in normal- • When describing target groups in audiology, these descriptions hearing human adults . Hear Res , 142 , 1 – 11 . were primarily based on auditory premises, such as diagnosis (or Br ä nnstr ö m K.J. & Wennerstr ö m I . 2010 . Hearing-aid fi tting outcome: type of HL), and hearing-related descriptions, such as ‘ hearing- Clinical application and psychometric properties of a Swedish translation of the international outcome inventory for hearing aids (IOI-HA) . aid users ’ . Participants were often treated as a unisex group, and J Am Acad Audiol , 21 , 512 – 521 . several studies did not reveal the sex of the participants, even Brockow T. , Cieza A. , Kuhlow H. , Sigl T. , Franke T. et al . 2004 . Identifying though sex differences are established on very foundational the concepts contained in outcome measures of clinical trials on bases in the auditory system. The sample sizes used in the musculoskeletal disorders and chronic widespread pain using the reviewed studies were often small. When using small sample International Classifi cation of Functioning, Disability, and Health as sizes, the design of the study is critically important to control a reference . J Rehabil Med Suppl , 36 , 30 – 36 . for all possible variations that can occur in human beings . Brockow T.T. , Wohlfahrt K. , Hillert A. , Geyh S. , Weigl M. et al . 2004 . Identifying the concepts contained in outcome measures of clinical trials on depressive disorders using the International Classifi cation Acknowledgements of Functioning, Disability, and Health as a reference . J Rehabil Med Suppl , 36 , 49 – 55 . The authors would like to thank research librarian Margareta Chisolm T.H. , Abrams H.B. , McArdle R. , Wilson R.H. & Doyle P.J . 2005 . The Landin at the Medical library at Ö rebro University Hospital for her WHO-DAS II: Psychometric properties in the measurement of functional help with the database search, Hanna Hagsten and Jonas Karlsson health status in adults with acquired hearing loss. Trends Amplif , 9 , for their assistance on data management, Kathy Pichora-Fuller, 111 – 126 . De Wet Swanepoel, and Stephen Widé n for their comments on the Connington M . 2005 . Hearing aid outcome tools: What are we really manuscript, Alarcos Cieza, Heinrich Gall, and Melissa Selb from the measuring? A case study. Semin Hear , 26 , 170 – 175 . ICF Research Branch (a cooperation partner within the WHO Cox R.M. , Alexander G.C. & Gilmore C . 1987 . Development of the connected speech test (CST) . , 8 (Suppl. 5), 119 – 126 . Collaborating Centre for the Family of International Classifi cations Ear Hear Cox R.M . 2005 . Evidence-based practice in provision of amplifi cation . in Germany [at DIMDI]), and the following members of the ICF J Am Acad Audiol , 16 , 419 – 438 . Core Sets for HL steering committee: Jean-Pierre Gagné , Louise Cox R.M. & Alexander G.C . 2002 . The international outcome inventory for Hickson, Sophia Kramer, Bradley McPherson, Jan Peter Str ö mgren, hearing aids (IOI-HA): Psychometric properties of the English version . and Gerold Stucki. Int J Audiol , 41 , 30 – 35 . Cruice M. , Worrall L. & Hickson L . 2000 . Quality-of-life measurement in speech pathology and audiology . Asia Pac J Speech Lang Hear, 5 , 1 – 20 . Declaration of interest: The authors alone are responsible for the Danermark B . 2005 . A review of the psychosocial effects of hearing content and writing of this paper. The authors report no declarations impairment in the working- age population . In: Stephens & Jones (eds.), of interest. The Impact of Genetic Hearing Impairment. London: Whurr, pp. 106– 136 . For personal use only. This work was funded by grants from the Oticon Foundation and Danermark B. , Cieza A. , Gang é J. , Gimigliano F. , Granberg S. et al . 2010 . the Stinger Foundation International Classifi cation of Functioning, Disability, and Health Core Sets for hearing loss: A discussion paper and invitation . Int J Audiol , 49 , 256 – 262 . Don M. , Ponton C.W. , Eggermont J.J. & Masuda A . 1993 . Gender differences References in cochlear response time: An explanation for gender amplitude Abrams H.B. , Chisolm T.H. & McArdle R . 2005 . Health-related quality of differences in the unmasked auditory brain-stem response . J Acoust Soc life and hearing aids: A tutorial . Trends Amplif , 9 , 99 – 109 . Am , 94 , 2135 – 2148 . Arlinger S . 2000 . Can we establish internationally equivalent outcome Erber N . 1982 . Auditory Training . Washington, USA: Alexander Graham Bell measures in audiological rehabilitation? Ear Hear , 21 (Suppl. 4), Association for the Deaf . 97 – 99 . Escorpizo R. , Finger M. , Gl ä ssel A. , Gradinger F. , L ü ckenkemper M. et al . Au D.K . 2003 . Effects of stimulation rates on Cantonese lexical tone 2011 . A systematic review of functioning in vocational rehabilitation perception by cochlear implant users in Hong Kong. Clin Otolaryngol using the international classifi cation of functioning, disability and health. Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 Allied Sci , 28 , 533 – 538 . J Occup Rehabil , 21 , 134 – 146 . Bentler R.A. & Kramer S.E . 2000 . Guidelines for choosing a self-report Fayers P.M. & Machin D . 2007 . Quality of Life. The Assessment, Analysis and outcome measure . Ear Hear , 21 (Suppl. 4), 37 – 49 . Interpretation of Patient-reported Outcomes ( 2nd ed.) . Chichester: Wiley. Benton A . 1978 . Benton Revised Visual Retention Test (4th ed.) . Helsinki: Feeny D. , Furlong W. , Boyle M. & Torrance G . 1995 . Multi-attribute health- Psykologien Kustannus OY . status classifi cation systems: Health utilities index . Pharmacoeconomics , Berget S. & Karlsson C . 2010 . Sj ä lvskattningsskalor och l ä nkning till ICF: en 7 , 490 – 502 . litteraturstudie [Self-assessment questionnaires and linking to ICF]. Gagn é J.-P . 2003 . Treatment effectiveness research in audiological Degree project . Ö rebro: Ö rebro University, School of Health and rehabilitation: fundamental issues related to dependent variables. Int J Medical Sciences, Sweden . Audiol , 42 (Suppl. 1), 104 – 111 . Bess F.H . 2000 . The role of generic health-related quality of life measures in Garstecki D.C. & Erler S.F . 1999 . Older adult performance on the establishing audiological rehabilitation outcomes. Ear Hear , 21 Communication Profi le for the Hearing Impaired: Gender difference. (Suppl. 4), 74 – 79 . J Speech Lang Hear Res , 42 , 785 – 796 . Besser J. , Zekveld A.A. , Kramer S.E. , R ö nnberg J. & Festen J.M . 2012 . New Gasparin M. , Menegotto I. H. & Cunha C. S . 2010 . Psychometric properties measures of masked text recognition in relation to speech-in-noise of the international outcome inventory for hearing aids . Braz J perception and their associations with age and cognitive abilities . Otorhinolaryngol , 76 , 85 – 90 . J Speech Lang Hear Res , 55 , 194 – 209 . Gatehouse S . 1998 . Speech tests as measures of outcome . Scand Audiol , Boothroyd A. , Hanin L. & Hnath T . 1985 . A Sentence Test of Speech 27 (Suppl. 49), 54 – 60 . Perception: Reliability, Set Equivalence, and Short-term Learning . Granberg S. , M ö ller K. , Skagerstrand Å . , M ö ller C. & Danermark B . 2014 . New York: City University . The ICF Core Sets for hearing loss: researcher perspective, Part II: 76 S. Granberg et al.

Linking outcome measures to the International Classifi cation of Pichora-Fuller M. , Schneider B. & Daneman M . 1995 . How young and old Functioning, Disability and Health (ICF). Int J Audiol , 53 , 77 – 87. adults listen to and remember speech in noise . J Acoust Soc Am , 97 , Hawkins D.B . 2005. Effectiveness of counseling-based adult group aural 593 – 608 . rehabilitation programs: A systematic review of the evidence. J Am Acad Post M.W.M. , Kirchberger I. , Scheuringer M. , Wollaars M.M. & Audiol , 16 , 485 – 493 . Geyh S . 2010 . Outcome parameters in spinal cord injury research: A H é tu R. , Getty L. , Philibert L. , Desilets F. , Noble W. et al . 1994 . Mise au systematic review using the International Classifi cation of Functioning, point d ʼun outil clinique pour la mesure d incapacit é s auditives et de Disability and Health (ICF) as a reference . Spinal Cord , 48 , 522 – 528 . handicaps [Development of a clinical tool for the measurement of the Ramkumar V. & Rangasayee R . 2010 . Studying tinnitus in the ICF severity of hearing disabilities and handicaps] . J Speech Lang Pathol framework . Int J Audiol , 49 , 645 – 650 . Audiol , 18 , 83 – 95 . Sato H. , Sando I. & Takahashi H . 1991 . Sexual dimorphism and development Hickson L . 2009 . Evidence-based practice in adult audiologic rehabilitation . of the human cochlea. Computer 3D measurement . Acta Otolaryngol In: Montano & Spitzer (eds.), Adult Audiologic Rehabilitation . (Stockh) , 111 , 1037 – 1040 . San Diego: Plural Publisher Inc , pp. 367 – 380 . Saunders G.H. , Chisolm T.H. & Abrams H.B . 2005 . Measuring hearing aid Hickson L. & Scarinci N . 2007 . Older adults with acquired hearing outcomes: Not as easy as it seems . J Rehabil Res Dev , 42 , 157 – 168 . impairment: Applying the ICF in rehabilitation . Semin Speech Lang , Scarinci N. , Worrall L. & Hickson L . 2009 . The ICF and third-party disability: 28 , 283 – 290 . Its application to spouses of older people with hearing impairment. Humes L.E. & Dubno J.R . 2010 . Factors affecting speech understanding in Disabil Rehabil , 31 , 2088 – 2100 . older adults . In: Gordon-Salant , Frisina , Popper & Fay (eds.), The Aging Scheuringer M. , Grill E. , Boldt C. , Mittrach R. , Mullner P. et al . 2005 . Auditory System, Springer Handbook of Auditory Research 34 . Systematic review of measures and their concepts used in published New York: Springer , pp. 211 – 257 . studies focusing on rehabilitation in the acute hospital and in early ICF Research Branch . 2012 . ICF Core Sets. Manual for Clinical Practice . post-acute rehabilitation facilities . Disabil Rehabil , 27 , 419 – 429 . G ö ttingen: Hogrefe Publishing . Schneider B. , Pichora-Fuller K.M. & Daneman M . 2010 . Effects of senescent ICF Research Branch . 2013 . ICF Research Branch/ICF Core Sets Projects . changes in audition and cognition on spoken language comprehension . Retrieved 25 March, 2013, from http://www.icf-research-branch.org/icf- In: Gordon-Salant , Frisina , Popper & Fay (eds.) The Aging Auditory core-sets-projects-sp-1641024398 System, Springer Handbook of Auditory Research, 34 . New York: Jerger J. & Johnson K . 1988 . Interactions of age, gender, and sensorineural Springer Science , pp. 167 – 210 . hearing loss on ABR latency . Ear Hear , 9 , 168 – 176 . Staehelin K. , Bertoli S. , Probst R. , Schindler C. , Dratva J. et al . 2011 . Gender Kiessling J. , Pichora-Fuller M.K. , Gatehouse S. , Stephens D. , Arlinger S. and hearing aids: Patterns of use and determinants of nonregular use. et al . 2003 . Candidature for and delivery of audiological services: Ear Hear , 32 , 26 – 37 . Special needs of older people . Int J Audiol , 42(Suppl. 2), 92 – 101 . Stephens D . 2001 . World Health Organization s International Classifi cation Kramer S.E. , Goverts S.T. , Dreschle , W.A. , Boymans M. & Festen J.M . 2002 . of Functioning, Disability and Health: ICF . J Audiol Med , 10 , 7 – 10 . International Outcome Inventory for Hearing Aids (IOI-HA): Results Stroop J.R . 1935 . Studies of interference in serial verbal reactions . from the Netherlands . Int J Audiol , 41 , 36 – 41 . J Exp Psychol , 18 , 643 – 662 . Kramer S.E. , Zekveld A.A. & Houtgast T . 2009 . Measuring cognitive factors Stucki G. & Grimby G . 2004 . Foreword: Applying the ICF in medicine . in speech comprehension: The value of using the Text Reception J Rehabil Med Suppl , 36 , 5 – 6 . For personal use only. Threshold test as a visual equivalent of the SRT test . Scand J Psychol , Thibodeau L.M . 2007 . Speech Audiometry . In: Roeser & Hosford-Dunn 50 , 507 – 515 . (eds.), Audiology: Diagnosis. (2nd ed.) . New York: Thieme Publishing Levo H. , Stephens D. , Poe D. , Kentala E. & Pyykk ö I . 2010 . Use of ICF in Group , pp. 288 – 313 . assessing the effects of M é ni è reʼ s disorder on life . Ann Otol Rhinol Ventry I. & Weinstein B . 1982 . The Hearing Handicap Inventory for the Laryngol , 119 , 583 – 589 . Elderly: A new tool . Ear Hear , 3 , 128 – 134 . Liu H. , Zhang H. , Liu S. , Chen X. , Han D. et al . 2011 . International outcome Vink M. & Jolles J . 1985 . A new version of the Trail-Making Test as an inventory for hearing aids (IOI-HA): Results from the Chinese version. information processing task . J Clin Neuropsychol , 7 , 162 . Int J Audiol , 50 , 673 – 678 . Ware J.E.J. , Snow K.K. , Kosinski M. & Gandek B . 1993 . SF-36 Health McArdle R. , Chisolm T.H. , Abrams H.B. , Wilson R.H. & Doyle P.J . 2005 . Survey Manual and Interpretation Guide. Boston: New England Medical The WHO- DAS II: Measuring outcomes of hearing aid intervention Centre . for adults . Trends Amplif , 9 , 127 – 143 . World Health Organization . 1999 . The World Health Organization Disability Miller J.D . 2007 . Sex differences in the length of the organ of Corti in Assessment Schedule Phase II Field Trial Instrument . Geneva: World humans . J Acoust Soc Am , 121 , 151 – 155 . Health Organization . Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 M ö ller K . 2003 . Deafblindness: A challenge for assessment: Is the ICF World Health Organization . 2001a . Fifty-fourth World Health Assembly, a useful tool? Int J Audiol , 42(Suppl. 1), 140 – 142 . WHA 54.21. Agenda Item 13.9, 22 May 2001. International Classifi cation M ö ller K. , Eriksson K. , Sadeghi A.M. , M ö ller C. & Danermark B . 2009 . of Functioning, Disability and Health. Retrieved 7 January, 2013, from Long-term ophthalmic health care in Usher syndrome type I from an http://www.who.int/classifi cations/icf/wha-en.pdf ICF perspective . Disabil Rehabil , 31 , 1283 – 1292 . World Health Organization . 2001b . International Classifi cation of Morgan A. , Hickson L. & Worrall L . 2002 . The impact of hearing impairment Functioning, Disability and Health (ICF) . Geneva: WHO . on quality of life of older people . Asia Pac J Speech Lang Hear , 7 , 39 – 53 . World Health Organization . 2009 . International Statistical Classifi cation of Nilsson M. , Soli S. & Sullivan J . 1994 . Development of the Hearing in Noise Diseases and Related Health Problems: ICD-10 (10. rev., 2008 ed.) . Test for the measurement of speech reception thresholds in quiet and in Geneva: World Health Organization . noise . J Acoust Soc Am , 95 , 1085 – 1099 . Vuorialho A. , Karinen P. & Sorri M . 2006 . Effect of hearing aids on hearing Noble W . 1998 . Self- Assessment of Hearing and Related Functions . London: disability and quality of life in the elderly . Int J Audiol , 45 , 400 – 405 . Whurr . Zochling J. , Bonjean M. , Grill E. , Scheuringer M. , Stucki G. et al . Pichora-Fuller K.M. & Singh G . 2006 . Effects of age on auditory and 2006 . Systematic review of measures and their concepts used in cognitive processing: Implications for hearing-aid fi tting and audiologic published studies focusing on the treatment of acute infl ammatory rehabilitation . Trends Amplif , 10 , 29 – 59 . arthritis . Clin Rheumatol , 25 , 807 – 813 .

Supplementary material available online Supplementary Appendices 1 and 2. International Journal of Audiology 2014; 53: 77–87

Review Article

The ICF Core Sets for hearing loss: researcher perspective, Part II: Linking outcome measures to the International Classiﬁ cation of Functioning, Disability and Health (ICF)

Sarah Granberg∗ , † , Kerstin M ö ller∗ , Å sa Skagerstrand∗ , † , Claes M ö ller∗ & Berth Danermark∗

Abstract Objective: To link outcome measures used in audiological research to the ICF classifi cation and thereby describe audiological research from the ICF perspective. Design: Through a peer-reviewed or a joint linking procedure, link outcome measures to the ICF classifi cation system using standardized ICF linking rules. Additional linking rules were developed in combination with the established rules to overcome diffi culties when connecting audiological data to ICF. Absolute and relative frequencies of ICF categories were reported. Study sample: The identifi ed outcome measures from the previous study (Part I) constituted the empirical material. Results: In total, 285 ICF categories were identifi ed. The most prevalent categories were related to listening, hearing functions, auditory perceptions, emotions and the physical environment, such as noise and hearing aids. Categories related to communication showed lower relative frequencies, as did categories related to the social and attitudinal environment. Conclusions: Based on the linked outcome measures, communication as a research topic is subordinated to other research topics. The same conclusion can be drawn for research targeting the social and attitudinal environment of adults with HL. Diffi culties in the linking procedure were highlighted and discussed, and suggestions for future revisions of the ICF from the audiological perspective were described.

Key Words: Hearing loss; audiology; ICF; linking; ICF core sets For personal use only.

In 2001, the World Health Organization (WHO) classifi cation disability, and contextual factors of adult HL from the researchers’ ‘ International Classifi cation of Functioning, Disability and Health point of view by conducting a systematic review. This preparatory (ICF) ’ was adopted as the international standard when assessing study is discussed in two articles (Part I and Part II). In the previous functioning and health within the member states (WHO, 2001a). article about this perspective (Part I; see this issue), outcome mea- However, due to the comprehensiveness of the classifi cation, the sures used in audiological research between 2002– 2007 were identi- WHO initiated the ‘ core sets project’ to facilitate the use of ICF. The fi ed. In the current study (Part II), all of the outcome measures were objective of a core sets project is to identify ICF categories of par- connected to the ICF classifi cation. The key concepts here are to link ticularly relevance to a specifi c health condition, diagnosis or situa- or interpret data (e.g. outcome measures), to translate the data to the tion (ICF Research Branch, 2012). The ICF core sets for hearing loss ICF nomenclature, and to thereby describe the fi ndings from the ICF

Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 project was initiated in 2008, aiming at identifying ICF categories perspective. The ICF is a particularly useful classifi cation tool for of particular relevance for adults with hearing loss for use in clinical this matter given its organized and hierarchical structure (Figure 1). encounters or in research. This aim was being achieved through The ICF is based on the bio-psycho-social model, where bodily preparatory studies, by collecting empirical evidence for the func- dimensions of functioning, aspects of activities, and participation, tioning and health of adults with hearing loss, by valuing and mod- and the infl uence of contextual factors are recognized as important ifying this evidence through a Delphi exercise, by creating a fi rst features of human functioning and health (WHO, 2001b). Linking version of ICF core sets for hearing loss and, fi nally, through fi eld data to the classifi cation creates a reference tool for other types of trials to validate the ICF core sets for hearing loss (Danermark et al, data (e.g. self-assessment questionnaires or transcribed interviews) 2010). The present article focuses of one of the preparatory studies and allows for comparisons among other types of health data. in the project termed the ‘ researcher perspective ’ . In this perspective, Linking requires rules to assure quality and reliability in the link- the objectives were to identify relevant aspects of functioning, ing process. The concept of ICF linking rules was introduced in

Correspondence: Sarah Granberg, Audiological Research Centre, Ö rebro University Hospital, SE-701 85 Ö rebro, Sweden. E-mail: [email protected]

( R e c e i v e d 2 0 M a y 2 0 1 3 ; a c c e p t e d 1 9 O c t o b e r 2 0 1 3 ) ISSN 1499-2027 print/ISSN 1708-8186 online © 2014 British Society of Audiology, International Society of Audiology, and Nordic Audiological Society DOI: 10.3109/14992027.2013.858279 78 S. Granberg et al.

The fi rst article on the ‘ researcher perspective’ analysis (Part I) Abbreviations outlined the possibility of describing the audiological research fi eld HL Hearing loss using ICF as a reference tool. This can only be achieved when all ICF International Classifi cation of functioning, disability outcome measures have been linked to the ICF. The objectives of the and health present article (Part II) were as follows: WHO World Health Organization 1. To link the identifi ed outcome measures to the ICF classifi cations using established linking rules, and 2. To describe audiological research from the ICF perspective. 2002, when ten rules were developed to describe the relationship The identifi ed ICF categories are used to represent the ‘ researcher between health-status measurements and the ICF. The rules were perspective’ in the ICF core sets for HL project (Danermark developed in a dynamic process by a group of ICF and quality of et al, 2010). life (QoL) experts, who linked approximately 300 items from 20 condition-specifi c and generic health instruments to the ICF (Cieza Methods et al, 2002). The linking rules were further developed in 2005, when special rules for technical and clinical measures were introduced Materials (Cieza et al, 2005). All identifi ed outcome measures from pool I (246) and pool II (122) Linking is a well-established way of analysing and describing that were identifi ed in study I constituted the empirical material existing data from a health perspective. In a review of the scientifi c linked to the ICF in this study. When linking questionnaires, every use of ICF, Cerniauskaite and colleagues (2011) identifi ed 73 item of a specifi c questionnaire was linked. ‘ linking papers’ . Most of these papers had used established linking rules in different ways, proving the scientifi c recognition of the concept. In the area of audiology, Scarinci et al (2009) described Procedure third-party disability of spouses of older people with hearing When linking data to the ICF, standardized linking rules were used impairment from the ICF perspective. The authors successfully (Cieza et al, 2002, 2005). Some key points from the linking rules used the established linking rules but also highlighted some impor- are as follows: (1) always link to the most precise ICF category, tant linking issues, such as problems with overlapping categories (2) do not use so-called ‘ other specifi ed ’ or ‘ unspecifi ed ’ ICF catego- within components (e.g. between conversing and discussing in the ries (identifi ed by the fi nal code 8 and 9) and, (3) personal factors Activities & Participation [d] component), and overlapping cate- are assigned the code ‘ pf ’ . gories between components (e.g. between attention functions in The procedure started with the linking of all measures containing the Body functions [b] component, and focusing attention in the statements or questions, i.e. patient-reported measures - standardized Activities & Participation [d] component). (PT-S) or patient-reported measures - non standardized (PT-not S) . For personal use only. Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14

Figure 1. The hierarchical structure of ICF with examples from each level is provided. Note that all levels are connected to each other; the deeper category contains a more detailed speciﬁ cation of the previous category. The personal factors component lacks categories. ICF Core Sets for HL, Linking of outcome measures 79

The intention was to link all measures separately by two linkers and et al, 2003, p. S93). In the ICF, communication between two or to perform an inter-rater reliability analysis on the linking. However, more persons is referred to as Conversation (d350), and catego- after linking the fi rst questionnaire, it was clear that the linking ries related to conversation were used when bi-directional com- agreement between the two linkers was low. The main reason for munication was linked. Even though this category closely this fi nding was that the established linking rules were not suffi cient resembles the d355 Discussion category, the latter category was when linking audiological measures and allowed too much indi- used when a meaningful concept explicitly highlighted the argu- vidual interpretation. There were also ambiguities regarding the mentation dimension of a conversation or if a conversation was descriptions of specifi c category codes in the ICF. It was therefore expressed as very intense. decided that additional rules, specially designed for the audiological An example of how these concepts were operationalized is the fi eld, needed to be developed as a complement to the existing rules. hearing handicap inventory of the elderly (HHIE) (Ventry & The development of these rules continued throughout the linking Weinstein, 1982), where the category for ‘ hearing function ’ was process. The additional linking rules that were used in this study are used when linking. In item no. 2, ‘ Does a hearing problem cause you available in Appendix 1. to feel embarrassed when meeting new people? ’ , the concept ‘ hearing problem ’ was interpreted as ‘ impairment in the hearing L INKING OUTCOME MEASURES CONTAINING STATEMENTS function’ and was therefore linked to b230 Hearing functions . This OR QUESTIONS decision was based on information from the original article, where When linking according to the standardized rules, meaningful the authors described the intentions and rationales within the ques- concepts of a question or a statement should be identifi ed. For exam- tionnaire. Another example is item no. 2 (fi rst part) in the interna- ple, the two meaningful concepts ‘ pain ’ and ‘ walking any distance ’ tional outcome inventory for hearing aids (IOI-HA) (Cox & occur in the statement ‘ Pain doesn’ t prevent me from walking any Alexander, 2002). For the item ‘ Think about the situation where you distance ’ (Cieza et al, 2005, p. 214). Although not discussed further most wanted to hear better ’ , ‘ hear ’ was linked to d115 Listening . In in the linking rules, these examples require only a literal interpreta- this statement, the notion of ‘ hearing ’ refers to a specifi c situation, tion of the concepts. However, several statements or questions in the indicating the intentional aspect of ‘ hearing ’ . In the ICF, this concept present fi eld needed a latent interpretation to be correctly linked. The is related to the Activities & Participation component (d). However, concept of latent interpretation is common in traditional content there is no description in the statement of ‘ speech comprehension ’ , analysis and refers to the identifi cation of underlying meanings of a and category d115 was used. A third example is from the commu- concept or an expression (Graneheim & Lundman, 2004; Kondracki nication profi le for the hearing impaired (CPHI) (Demorest & Erd- et al, 2002). This factor was especially important for the concepts of man, 1987). For item no. 1 of this assessment, ‘ Someone in your hearing, listening, comprehending speech , and conversing . Some family is talking to you while you’ re driving or riding in a car’ , the descriptions of hearing related categories were unclear or overlap- meaningful concept ‘ talking to you ’ was interpreted as ‘ speech com- ping; therefore, the descriptions made by Kiessling et al (2003) and prehension ’ and coded as d310 Communicating with - receiving - Pichora-Fuller & Singh (2006) were used with some additional For personal use only. spoken messages. This type of statement occasionally appeared clarifi cations. when an activity was expressed as ‘ somebody else ’ s activity ’ (e.g. • Hearing is a passive body function that provides access to the ‘ someone in the family is talking ’ ), but the underlying meaning (and auditory world via the perception of sound. It concerns sensing what the statement was meant to convey) was the receiving portion the presence of sounds and discriminating the location, pitch, of the other person ’ s actions. The fourth example comes from the loudness and quality of sounds (Kiessling et al, 2003, p. S93). hearing aid performance inventory (HAPI) (Walden et al, 1984), In the ICF, hearing functions are assigned to b230 Hearing func- item no. 34 that reads ‘ You are ordering food for the family at tions . Note that b2304 Speech discrimination is not described in McDonald ’ s ’ ; in this example, the concept ‘ ordering food ’ was the ICF as the audiological concept ‘ speech recognition’ is linked to d3503 Conversing with one person . The motivation for the described; therefore, this category was not used when linking interpretation was that the concept of ‘ ordering food’ contains this concept in the current study (WHO, 2001b, p. 65). Instead, aspects of both interacting and comprehending (i.e. conversing), the second level b230 classifi cation was used for this matter. which are aspects known to be diffi cult for the target population. Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 • Listening is the process of hearing with intention and attention In several questionnaires, latent interpretations were required in for purposeful activities demanding the expenditure of mental relation to the concepts of coping and communication strategies. effort (Pichora-Fuller & Singh, 2006, p. 30). In the ICF, listening While these two concepts are related in the fi eld of audiology, they is assigned to d115 Listening. Even though the description of are separated in the ICF. ‘ Coping ’ is referred to as a personal factor this category in the ICF is ambiguous regarding listening to (pf) in the ICF, while ‘ Using communication techniques ’ (i.e. speech (i.e. speech comprehension) (WHO, 2001b, p. 125), this communication strategies) is part of the Activities & Participation category was exclusively used for listening where no speech component (d3602). Examples of how these two concepts were dis- comprehension was involved. tinguished are as follows: the communication strategy scale (CSS) • Comprehending is an activity beyond the processes of hearing (Demorest & Erdman, 1987; Hallberg et al, 1992) item no. 13 reads and listening (Kiessling et al, 2003, p. S93) defi ned as the unidi- ‘ If I ’ m sitting where I can ’ t hear, I ’ ll move to another seat ’ and item rectional reception of information, meaning, and intent (Pichora- no. 9 in the communication scale for older adults (CSOA) (Kaplan Fuller & Singh, 2006, p. 30). In the ICF, speech comprehension et al, 1997) reads ‘ You are at a meeting. The speaker says something is best described with d310 Communicating with - receiving - you do not understand. You pretend to understand and hope to get spoken messages. This category was solely used for (as the name the information later ’ . The fi rst statement was viewed as a commu- implies) the receiving portion of speech communication. nication strategy due to its problem-solution characteristics and • Communication requires the bi-directional transfer of informa- was thereby linked to d3602 Using communication techniques . The tion, meaning, or intent between two or more people (Kiessling latter statement (the meaningful concept ‘ pretend to understand ’ ) 80 S. Granberg et al.

was classifi ed as a pf because it was interpreted as an emotion- Attention functions ) and in the Activities & Participation component focused response to the stressful situation, more specifi cally, an (d160 Focusing attention). Again, it was very helpful to identify avoidance strategy. The rationale for this decision was based on primary concepts and to decide whether the statement should be the construct of the coping concept and how this construct is classifi ed as (b) or (d). In summary, when linking statements or operationalized in the audiological area (Lazarus, 1993; Andersson questions, latent interpretations were necessary for many reasons, & Willebrand, 2003). and it was vital to read the original article (when available) to iden- In many cases, latent interpretations regarding the environment tify latent variables (when expressed) for guidance when linking. It were also necessary. Several questionnaires provided examples of was also very helpful to fi rst consider which components a mean- contexts where listening or speech comprehension can be diffi cult. ingful concept should be linked to, and then select the fi nal category An example is item no. 3 in the Glasgow hearing aid benefi t codes. Approaching the material by using primary concepts in the profi le (GHAB; Gatehouse, 1999) that reads ‘ Carrying on a conver- process of linking to the correct components and categories was sation in a busy street or shop ’ . The statement was interpreted as also helpful. ‘ sustaining a conversation in noise’ , resulting in classifi cation in the ICF categories d3501 Sustaining a conversation and e2501 Sound L INKING CLINICAL OR LABORATORY OUTCOME MEASURES quality . A meaningful concept was sometimes interpreted as an When linking other types of outcome measures, such as clinical ‘ arena ’ where a conversation was taking place, such as a restaurant measures, the aim of the measurements should be linked (Cieza et al, environment, shop, or a large offi ce. In those cases, the latent inter- 2005). A specifi c measurement may be used for different purposes pretation was made according to both noise (e2501 Sound quality ) in different studies (as in the case of X-ray). Consequently, the and acoustics (e150 Design, construction and building products and linking can vary from study to study. In this study, several of the technology of buildings for public use ). It was assumed that the clinical measures required an ‘ active patient’ , even though the aim arenas chosen in the questionnaires served as barriers to conversa- with the measurement was to evaluate a body function. This was the tion due to the design of the environments (e.g. large, open areas), case with pure-tone audiometry, which is described in the literature where noise from other people and things becomes problematic. as a sound detection test in which the patient decides when a sound Other latent interpretations were necessary when a concept con- is detected or not (Roeser & Clark, 2007). Therefore, both the aim tained more dimensions than expressed in the statement, such as and procedure would be taken into consideration when linking. item no 1. in the hearing aid performance inventory (HAPI) (Walden In addition, an overlap between categories in the Body functions et al, 1984): ‘ You are sitting alone at home watching the news on component was discovered between the categories of b230 Hearing TV ’ . The meaningful concept ‘ watching the news on TV ’ was inter- functions and b1560 Auditory perception . To solve this overlap, both preted as both ‘ watching ’ (d110 Watching ) and ‘ comprehending categories were used in the linking process. Pure-tone audiometry speech ’ (d310 Communicating with - receiving - spoken messages ), was assigned to b2300 Sound detection, b1560 Auditory perception , while ‘ TV ’ was recognized as an environmental factor (e1250 and d115 Listening , and this classifi cation considered both the aim General products and technology for communication ). and the test procedure. For personal use only. During the linking process, it was important to recognize whether When linking technical measures, it was impossible to assign a concept was part of the Activities & Participation (d) component several of the measurements to categories at the second level or or the Environmental factors (e) component, such as with d3600 more specifi c levels, such as with ‘ Tympanometry ’ , which is used Using telecommunication devices and e1250 General products and to evaluate the function of the middle ear (Clark et al, 2007). Unfor- technology for communication. It was helpful to recognize the pri- tunately, functions of hearing related structures, such as the tym- mary concepts (i.e. what the statement was about) contained in the panic membrane or the ossicles, are not included in the ICF. When statements. In several statements, the primary concept focused on linking this example, the fi rst level category, b2 Chapter 2, Sensory ‘ conversing ’ , while, for example, ‘ telephone ’ was considered a dif- functions and pain was used. In audiology, the auditory system is fi cult context for conversations. In those cases, the category e1250 viewed as a single system that begins at the outer ear and ending at (an environmental category) was used when linking ‘ telephone ’ . the temporal lobe. According to the ICF, this system is divided. In other cases, the statement specifi cally asked about telephone When referring to ‘ structures of the auditory system ’ , the peripheral

Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 usage. In those cases, d3600 (an Activities & Participation cate- and central parts of the auditory system must be separated. The gory) was used when linking ‘ telephone ’ . If only one category peripheral hearing system is found in Chapter 2 Structures of the could not be selected, both categories were used. eye, ear and related structures of the component Body structures, Some diffi culties were experienced with overlapping categories. while the central auditory system (the auditory nerve and beyond) In some cases, there were problems distinguishing between is located in Chapter 1 Structures of the nervous system . As a direct Body functions (b) and Personal factors (pf), which was the case consequence, functions of brain structures (e.g. linking ABR) were with b1265 Optimism . An example of this situation can be observed linked to b1 Chapter 1, Mental functions . with the Glasgow benefi t inventory (GBI) (Robinson et al, 1996) item no. 3: ‘ Since your operation/intervention, have you felt more or less optimistic about the future? ’ The concept ‘ optimistic about Quality assurance the future’ was interpreted as an ability to produce a personal dis- A peer-review process was conducted to assure the quality of the position, ‘ optimism ’ (b1256), as opposed to being an ‘ optimistic linking of outcome measures containing statements or questions. person in general ’ (a personal factor), which could result from other Two linkers (SG, KM) who were familiar with the audiological fi eld, circumstances. These circumstances could include childhood the ICF, and the linking rules conducted all of the linking of these experiences and family relationships in addition to the personal dis- measures. All statements or questions were linked by either one of position and to factors not related to the health condition. Another these linkers and were checked by the other one to assure linking important diffi culty was discovered when linking concepts related agreement. If the linking was not identical, linking agreement was to ‘ attention ’ . Attention can be a body function in the ICF (b140 reached by discussion between the two linkers. All other outcome ICF Core Sets for HL, Linking of outcome measures 81

measures were linked by three linkers (SG, CM, and Å S). These Table 1. Absolute and relative frequencies of ICF categories measures were linked in a joint process where two linkers worked identifi ed Ն 5 times (Ն 0.9%) after the linking of outcome measures together when linking measures of a specifi c instrument type, because extracted from 122 studies (Body functions component) (n ϭ 537). this approach required detailed knowledge of the aim, performance, and procedures of a specifi c test to assure linking to the correct ICF ICF Absolute and categories. The decision of which of the linkers that should link category relative code ICF category code description frequency (%) certain instrument types was based on their area of expertise and knowledge of the ICF category codes. b1 Chapter 1, Mental functions 18 (3.4%) b117 Intellectual functions 5 (0.9%) b1266 Confi dence 14 (2.6%) Data analysis b1300 Energy level 22 (4.1%) The absolute and relative frequency (%) of all identifi ed ICF cat- b140 Attention functions 8 (1.5%) egories (fi rst to fourth level) were reported (relative to the total b1400 Sustaining attention 7 (1.3%) number of outcome measures). When calculating the total number b144 Memory functions 11 (2.0%) of outcome measures, the prevalence of an identifi ed outcome b1440 Short-term memory 8 (1.5%) measure (results from study I) was considered. If an ICF category b152 Emotional functions 65 (12.1%) b1560 Auditory perception 148 (27.6%) was used frequently in the linking process of a specifi c outcome b1561 Visual perception 5 (0.9%) measure, this category was only calculated once to avoid bias. All b164 Higher-level cognitive functions 9 (1.7%) identifi ed ICF categories and the relative frequency of each b167 Mental functions of language 5 (0.9%) category represented the ‘ researcher perspective’ in the project b2 Chapter 2, Sensory functions and pain 32 (6.0%) (Danermark et al, 2010). Contrary to other core sets projects (e.g. b230 Hearing functions 157 (29.2%) Escorpizo et al, 2011; Post et al, 2010; Zochling et al, 2006), all b2300 Sound detection 52 (9.7%) levels used in the linking process (fi rst to fourth) were reported b2301 Sound discrimination 13 (2.4%) rather than only the second level. The rationale for this approach b2302 Localisation of sound source 10 (1.9%) was a fear of losing the rich, detailed information that the more b2303 Lateralisation of sound 7 (1.3%) specifi c categories provided, as this information may be important b2304 Speech discrimination 6 (1.1%) b235 Vestibular functions 9 (1.7%) within the audiological fi eld. In addition, by reporting all levels, it b2400 Ringing in ears or tinnitus 13 (2.4%) was possible to identify categories or areas where more specifi c b2401 Dizziness 10 (1.9%) hearing-related categories need to be developed in future revisions b280 Sensation of pain 9 (1.7%) of the ICF. b430 Haematological system functions 15 (2.8%) b4350 Immune response 13 (2.4%) b5401 Carbohydrate metabolism 6 (1.1%) For personal use only. Results b5403 Fat metabolism 12 (2.2%) b555 Endocrine gland functions 6 (1.1%) In total, 537 outcome measures were identifi ed in the 122 studies b7502 Refl exes generated by other exteroceptive 6 (1.1%) (approximately 4.4 measures per study). Twelve linked generic mea- stimuli sures were obtained from the ICF Research Branch (http://www. icf-research-branch.org/) because they already had been used in other core sets projects. Three self-report measures could not be retrieved and were therefore not linked. All other outcome measures Body structures (s) were analysed and linked. From the 1036 items identifi ed (instru- Only 19 categories were used in this component during the linking ment types PT-S [self-report measures and survey questionnaires] process. Most categories belonged to Chapter 1 Structures of the and PT-not S [i.e. patients ’ answers to single questions]), 3507 mean- nervous system (43.1%) and Chapter 2 The eye, ear and related ingful concepts were extracted, analysed and linked to the ICF. Fur- structures (46.2%). The most prevalent category was s260 Structures of inner ear . The frequency of this category was very low (1.5%), Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 thermore, 285 ICF categories (fi rst to fourth level) were used, although the majority of these categories were used infrequently. indicating that evaluations of structures are infrequently used as out- Tables 1– 4 reveal the categories linked Ն 5 times ( Ն 0.9%) within come measures in audiology. each component. All categories linked less than 0.9% are available in a Supplementary Appendix 1. This material is available with the Table 2. Absolute and relative frequencies of ICF categories direct link to the article at: http://informahealthcare.com/doi/abs/ identifi ed Ն 5 times (Ն 0.9%) after the linking of outcome measures 10.3109/14992027.2013.858279. in 122 studies (Body structures component) (n ϭ 537).

ICF category ICF category code Absolute and relative Body functions (b) code description frequency (%) In total, 111 categories were used in the (b) component. Most categories (52) belonged to Chapter 1 Mental functions. In this chapter, the s1101 Structure of midbrain 6 (1.1%) most prevalent categories were b1560 Auditory perception (27.6%) s1102 Structure of diencephalon 6 (1.1%) and b152 Emotional functions (12.1%). The second most used chap- s1105 Structure of brain stem 6 (1.1%) ter in this component was Chapter 2 Sensory functions and pain , s1106 Structure of cranial nerves 7 (1.3%) where b230 Hearing functions was the most prevalent category s240 Structure of external ear 7 (1.3%) (29.2%). These two chapters were most frequently used when link- s260 Structure of inner ear 8 (1.5%) s2600 Cochlea 5 (0.9%) ing within this component (86.5%). 82 S. Granberg et al.

Table 3. Absolute and relative frequencies of ICF categories Table 4. Absolute and relative frequencies of ICF categories identiﬁ ed Ն 5 times ( Ն 0.9%) after the linking of outcome measures identiﬁ ed Ն 5 times (Ն 0.9%) after the linking of outcome measures in 122 studies (Activities & Participation component) (n ϭ 537). in 122 studies (Environmental factors component) (n ϭ 537).

ICF Absolute and ICF Absolute and category relative category relative code ICF category code description frequency (%) code ICF category code description frequency (%)

d110 Watching 19 (3.5%) e1101 Drugs 6 (1.1%) d115 Listening 190 (35.4%) e1150 General products and technology for d160 Focusing attention 6 (1.1%) personal use in daily living 7 (1.3%) d310 Communicating with - receiving - spoken e1151 Assistive products and technology for messages 43 (8.0%) personal use in daily living 6 (1.1%) d330 Speaking 13 (2.4%) e1200 General products and technology for d350 Conversation 41(7.6%) personal indoor and outdoor mobility d3503 Conversing with one person 21 (3.9%) and transportation 10 (1.9%) d3504 Conversing with many people 23 (4.3%) e1250 General products and technology for d355 Discussion 8 (1.5%) communication 45 (8.4%) d3600 Using telecommunication devices 25 (4.7%) e1251 Assistive products and technology for d3602 Using communication techniques 25 (4.7%) communication 54 (10.1%) d450 Walking 6 (1.1%) e150 Design, construction and building products d4500 Walking short distances 5 (0.9%) and technology of buildings for public use 36 (6.7%) d4551 Climbing 6 (1.1%) e250 Sound 40 (7.4%) d4701 Using private motorized transportation 9 (1.7%) e2500 Sound intensity 45 (8.4%) d4702 Using public motorized transportation 7 (1.3%) e2501 Sound quality 87 (16.2%) d4751 Driving motorized vehicles 8 (1.5%) e3 Chapter 3, Support and relationships 9 (1.7%) d6200 Shopping 23 (4.3%) e310 Immediate family 13 (2.4%) d7 Chapter 7, Interpersonal interactions e320 Friends 6 (1.1%) and relationships 29 (5.4%) e325 Acquaintances, peers, colleagues, d7203 Interacting according to social rules 5 (0.9%) neighbours and community members 12 (2.2%) d730 Relating with strangers 29 (5.4%) e4 Chapter 4, Attitudes 12 (2.2%) d740 Formal relationships 16 (3.0%) e410 Individual attitudes of immediate family d7402 Relating with equals 5 (0.9%) members 8 (1.5%) d750 Informal social relationships 22 (4.1%) e5800 Health services 24 (4.5%) d7500 Informal relationships with friends 32 (6.0%) e5802 Health policies 5 (0.9%)

For personal use only. d760 Family relationships 37 (6.9%) d770 Intimate relationships 5 (0.9%) d850 Remunerative employment 26 (4.8%) Assistive products and technology for communication (e1251) d9 Chapter 9, Community, social and civic life 17 (3.2%) d910 Community life 18 (3.4%) (10.1%). These two categories were primarily used when linking d920 Recreation and leisure 24 (4.5%) noise and hearing aids. Chapters 1 and 2 (i.e. Products and d9202 Arts and culture 14 (2.6%) technology and Natural environment and human-made changes to d9205 Socializing 22 (4.1%) environment) were most frequently used when linking within this d930 Religion and spirituality 19 (3.5%) component (77.0%). Because ICF does not offer speciﬁ c category d9300 Organized religion 7 (1.3%) codes in the personal factors component, data judged to belong to this component were only assigned with the label pf . Examples of personal factors are avoidance, gender, education and smoking.

Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 The relative frequency of pf was 26.4%. Activities & Participation (d) When identifying the categories most frequently used during the In total, 117 categories were used in this component. The most linking process across levels (fi rst to fourth), the categories explicitly prevalent category was d115 Listening (35.4%), which belonged to related to the auditory system (i.e. hearing, listening, and perception) Chapter 1 Learning and applying knowledge. Although this category were the most frequently used (Table 5). The categories related to was more than four times more frequent than the second category ‘ communication ’ or ‘ conversation ’ were ranked as the tenth and (d310), the chapter most frequently used in the linking process eleventh most frequent categories when all of the categories were was Chapter 7 Interpersonal interactions and relationships ranked by occurrence. (50.8%). Several categories in Chapter 3 Communication were also used. The most common category used was d310 Communi- cating with- receiving- spoken messages (8.0%), followed by d350 Summary of the research fi eld from the ICF perspective Conversation (7.6%). All chapters in this component were used Given the hierarchical structure of the ICF, it is possible to analyse in the linking procedure, although with highly variable frequency. the results on different levels. Figure 2 illustrates the overall results when the absolute frequencies of all the used categories are merged into the fi rst level (chapter level) of each component. When Contextual factors (e) & (pf) analysing the results from a broad perspective (i.e. at the component In the (e) component, 37 categories were used. The most frequently level) and comparing the bubble size of (b), (s), (pf), (e), and (d), linked categories belonged to Sound quality (e2501) (16.2%) and most outcome measures are linked to the Activities & Participation ICF Core Sets for HL, Linking of outcome measures 83

Table 5. Absolute and relative frequencies of the 15 most frequently tional Classifi cation of Functioning, Disability and Health (ICF) identifi ed ICF categories (including personal factors) after the linking (WHO, 2001b). of outcome measures extracted from 122 studies (n ϭ 537). Outcomes targeting bodily dimensions, such as different aspects of hearing functions or emotional functions, were dominant. As ICF Absolute and concluded in Part I, the assessment of abilities related to ‘ speech category relative recognition’ or ‘ sound detection’ (measures partly connected to the code ICF category code description frequency (%) hearing functions) were common. These types of measures are also d115 Listening 190 (35.4%) highly valued and frequently used as outcome measures within the b230 Hearing functions 157 (29.2%) audiological fi eld (Gatehouse, 1998; Killion & Gudmundsen, 2005; b1560 Auditory perception 148 (27.6%) Saunders et al, 2005). Even though these types of measurements Pf Personal factors 142 (26.4%) are often used to assess impairments, Body functions in the ICF e2501 Sound quality 87 (16.2%) are not equal to impairments because ICF offers the possibility to b152 Emotional functions 65 (12.1%) express both negative and positive aspects of human functioning. e1251 Assistive products and technology for For example, it was very common for emotions to be reported as communication 54 (10.1%) infl uencing hearing loss, which indicated a ‘ normal ’ function of b2300 Sound detection 52 (9.7%) e1250 General products and technology for emotions. This fi nding agrees with the integrated ICF model, where communication 45 (8.4%) the different components interact (Cieza & Stucki, 2008). There- e2500 Sound intensity 45 (8.4%) fore, even though bodily dimensions of hearing loss are a very d310 Communicating with - receiving - spoken common topic in audiological research, one cannot conclude that messages 43 (8.0%) audiological research focuses on impairments based on the results d350 Conversation 41 (7.6%) from this review. What can be concluded, however, is that com- e250 Sound 40 (7.4%) munication as a research topic is subordinated to other topics d760 Family relationships 37 (6.9%) according to the chosen outcome measures used in the different e150 Design, construction and building products studies. This statement is based on the fact that categories related and technology of buildings for public use 36 (6.7%) to communication were placed far down in the linking hierarchy despite the precautions taken to link different aspects of ‘ hearing ’ to correct components and categories. This result is noteworthy (d) component, followed by Body functions (b), Environmental fac- because communication restrictions are known to be one of the tors (e), Personal factors (pf), and Body structures (s). Analysis on most frequently reported consequences of hearing loss (e.g. Dalton this level favors components in which many chapters are used in et al, 2003; H é tu et al, 1988; Karlsson Espmark & Hansson combination with medium to high relative frequencies of categories Scherman, 2003; Pryce & Gooberman-Hill, 2012). These fi ndings within these chapters. This situation occurred with the (d) compo- also indicate there might be a confl ict between patients’ experience For personal use only. nent, where the multi-use of chapters when linking was a high con- of the health condition compared to the target of research investiga- tributor to the results found for this level. In fact, only one chapter tions. Therefore, it might be benefi cial to involve people with in this component (Chapter 7) displayed a relative frequency of hearing loss in the research (e.g. to discuss research questions) in Ն 50%. If the results are viewed from a narrower perspective (i.e. the future. Former projects of this kind have shown that even at the fi rst level), the interpretation of the results differs slightly. though there were diffi culties related to divergent background The chapters most frequently used in the linking process were b1 experiences and mutual research understandings when involving (75.2%) and b2 (57.7%), both belonging to the component Body other people in the research process; people with disabilities have functions. Here, a chapter belonging to the (d) component appeared been shown to be highly motivated and willing to participate in fi rst as the third most frequently used chapter (d7). The categories the research process, resulting in positive effects for both parties related to Communication (d3) fi rst appeared in the fi fth most fre- (Erdtman et al, 2012; Priestley et al, 2010a, 2010b). quently used chapter in the linking hierarchy. In the (e) component, In the ICF, environmental factors (e) constitute the physical,

Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 only categories related to the physical environment (e1 and e2) social, and attitudinal environment in which people live and conduct were used with a notable frequency. Categories related to Support their lives (WHO, 2001b, p. 16). In this review, categories for noise and relationships (e3); Attitudes (e4) and Services, systems and and hearing aids (i.e. the physical environment) were used fre- policies (e5) exhibited low frequencies. quently during the linking process. This result is not surprising because both of these factors are very important from a hearing loss perspective. However, very little research attention has been Discussion given (according to identifi ed outcome measures) to societal atti- As stated in Part I of this study (Granberg et al, 2014), the ICF tudes (e4) or support and relationships (e3). In a systematic review provides an opportunity to describe trends and paradigms by func- on factors infl uencing help-seeking for hearing impairment and tioning as a reference tool for other types of data. Given the bio- hearing-aid adoption in older adults, Meyer and Hickson (2012) psycho-social approach to functioning and disability in ICF, the identifi ed seven studies that covered different aspects of environ- classifi cation also provides a perspective that acknowledges bodily mental attitudes (e4) during a broad time spectrum (1996 – 2010). dimensions, executive dimensions and contextual infl uences. In The present systematic review only included quantitative studies, this study, the quality of the outcome measures was not valued, but and these topics may be better addressed with qualitative studies. rather, the content of the outcome measures was valued. The con- This situation, together with the evidence from the study by Meyer tent of these outcome measures created the foundation of which and Hickson, could explain the low frequency of these concepts in current research trends on adult HL was described. This description the present results. However, only 18 studies were excluded from was accomplished using the theoretical framework of the Interna- this current review because of an incorrect design (results of 84 S. Granberg et al. For personal use only.

Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 Figure 2. Relative frequencies of ICF chapters (ﬁ rst levels) identiﬁ ed after the linking of outcome measures extracted from 122 studies (n ϭ 537). The sizes of the bubbles are proportional, i.e. a larger bubble indicates a chapter has been used more frequently in the linking process. The frequency also affects the bubble size for components (d), (e), (s), (b) and (pf).

Part I). Even if each of these studies covered the fi rst level catego- based on a specifi c activity (e.g. ‘ talking with someone’ ) that occurs ries e4 and e3, the relative frequency for these chapters would in a specifi c condition (e.g. a ‘ restaurant environment’ ), requiring a increase only marginally, indicating that the social and attitudinal latent interpretation when linking. In this study, several statements environments are likely neglected areas in audiological research. or questions expressed the term ‘ hearing ’ but actually referred to In addition to the established rules, new linking rules were devel- higher level auditory processing, such as ‘ listening ’ or ‘ conversing ’ . oped especially for audiological data. This development was neces- In a consensus paper about audiology and older adults, Kiessling sary because many self-assessment questionnaires have a different et al (2003) acknowledged how the term ‘ hearing ’ often was used to structure than established, generic HRQoL scales, such as the Short describe the auditory system as well as higher level activities related Form-36 (SF-36) (Ware et al, 1993) or the EuroQol (EQ-5D) (Brooks to hearing (e.g. listening or comprehending). These authors recom- & EuroQol group, 1996) from which the established linking rules mended careful use of adequate hearing-related terms. The present were originally developed (Cieza et al, 2002, 2005). Several hearing study supports this approach because ‘ hearing functions ’ are Body condition-specifi c questionnaires have used statements or questions functions (b) in the ICF, while ‘ listening ’ or ‘ conversing ’ belong to ICF Core Sets for HL, Linking of outcome measures 85

the Activities & Participation (d) component. If latent interpretations Other authors have reported (Scarinci et al, 2009) ambiguity had not been made in reference to ‘ hearing ’ in this study, a large regarding the categories b140 Attention functions and d160 Focusing amount of the results undoubtedly would have been placed in the attention , which belong to different components; similar diffi culties category b230 Hearing functions. This situation would represent a were experienced in this study. Reed et al (2005) stated that these serious misinterpretation of the data because the focus would have two items cannot be distinguished clinically, although these factors been on impairment rather than disability. To state it more clearly, can be conceptually differentiated within the ICF. The Body function the bodily dimensions of hearing loss would have been favored category b140 is described as ‘Specifi c mental functions of focusing instead of the effects of hearing loss due to a linguistic defi ciency. on an external stimulus or internal experience for the required Another type of latent interpretation was related to the concepts period of time’ , and the Activities & Participation category d160 as of communication strategies and coping. In the area of audiology, ‘Intentionally focusing on specifi c stimuli, such as fi ltering out dis- communication strategies are commonly grouped into two catego- tracting noises’ . It is a diffi cult task to differentiate these factors ries: facilitation strategies and repair strategies (Tye-Murray, 1994). because the descriptions of the categories are overlapping. Even Facilitation strategies are defi ned as strategies applied in prepara- though a person can intentionally focus attention (as is the defi nition tion for conversation or to manage an ongoing conversation, while of d160), it is highly questionable whether an individual can inten- repair strategies aim to repair communication breakdowns. The tionally block distracting noises. To ‘ block ’ is merely a consequence characteristics of a communication strategy are the solution- of the ‘ focusing part ’ , and fi ltering is a non-intentional human body oriented approach to a potentially stressful communication situa- function (de Boer et al, 2012). Therefore, the description of the cat- tion. Process-oriented coping is often described as either egory proceeds from a body function perspective, resulting in even problem-focused or emotion-focused (Lazarus, 1993). As stated by greater confusion. Moreover, Reed and colleagues stated that the Andersson & Willebrand (2003), communication strategies are a existence of these two categories is more confusing than helpful, and form of problem-focused coping, although not frequently discussed that the distinction is not clinically meaningful (Reed et al, 2005). in the theoretical framework of coping. Other types of reported Other overlapping categories within a component were identifi ed coping strategies, such as avoidance and minimizing strategies, when using Hearing functions (b230s). The third level categories would be acknowledged as strategies to regulate an emotional dis- connected to this component are b2300 Sound detection , b2301 tress (Hallberg & Carlsson, 1991). In the ICF, these two concepts Sound discrimination , b2302 Localization of sound source , b2303 belong to different components, indicating that theoretically, cop- Lateralization of sound , and b2304 Speech discrimination . These ing and communication strategies are viewed as two different categories overlap with b1560 Auditory perception. Even though concepts in the classifi cation. This situation provides an example the solution was simple (using multiple categories when linking), of how theories used in audiology do not always match the ICF. In the main problem is that all categories presented in Hearing func- this case, the theoretical distinctions made in the ICF are arguable, tions are in fact Auditory perception functions. Because auditory but the consequence of the distinctions are positive because the perception is not viewed as part of hearing function but rather as personal factors component do not contain any specifi c categories part of mental functions, these categories are separated (occurring For personal use only. and thereby make it impossible to specify, for example, coping strat- in different chapters) in the ICF. Furthermore, specifi c categories egies not related to communication. According to the WHO, per- connected to b230 Hearing functions are unfortunately missing. It sonal factors are not classifi ed in ICF ‘ because of the large social was not possible to link any functions of auditory structures to and cultural variance associated with them ’ (WHO, 2001b, p. 8). specifi c ICF categories. Compared to Seeing functions , where the In this study, the problem with overlapping categories has been second level category b215 Functions of structures adjoining the highlighted. This problem occurred both between different compo- eye is clearly connected to the eye structures, no such category is nents and within the same component. Diffi culties were experi- found for Hearing functions. The consequences of this situation are enced when trying to distinguish between categories related to that several of the outcome measures used in the audiological fi eld b126 Temperament and personality functions , such as ‘ optimism ’ were diffi cult to link due to poor matching to appropriate ICF cat- (b1265) or ‘ confi dence ’ (b1266), and some personal factors (inter- egories. These topics need to be addressed in future revisions of preted as ‘ being an optimistic person ’ or ‘ being a confi dent or self- the classifi cation.

Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 confi dent person’ ). Threats (2007) suggested a clinical solution for this problem based on personality traits related to the onset of a Conclusions health condition. For example, if a person was pessimistic and negative before the onset of a stroke, this factor would most likely • A comprehensive set of ICF categories (285) was identifi ed in be classifi ed as a personal factor. However, if a person was described this review, which represents the ‘ researcher perspective ’ in the as optimistic before the stroke and after the stroke appeared unmo- ICF core sets for HL project. The majority of these categories tivated to participate in therapy, this factor may be classifi ed as into were used infrequently in the linking process. The most preva- the Body functions (b) component (p. 69). Although this reasoning lent categories were connected to the auditory system, such as can be very helpful, hearing loss rarely has a distinct onset time; listening , hearing functions , and auditory perception . Categories therefore, this differentiation may be diffi cult. In this project, the related to communication had low relative frequencies, indicat- distinction was made in the linguistic context. When statements ing a lower amount of research attention for communication fac- were expressed about personal disposition in relation to the health tors. The physical environment , such as noise and hearing aids, condition, this information provided a clue that the factor was was identifi ed as important in the audiological research fi eld, related to body functions, while statements about personal disposi- while little research attention was paid to the social and attitu- tion not related to hearing loss could be considered as personal dinal environment . factors. This approach aligns with the ICF description where con- • Additional linking rules were developed to combine with the cepts are assigned as personal factors (pf) if they are not part of a established rules. In the area of audiology, latent interpretations health condition or health state (WHO, 2001b, p. 17). of statements or questions were very important to avoid 86 S. Granberg et al.

mislinking (i.e. linking to the wrong components and catego- Dalton D.S. , Cruickshanks K.J. , Klein B.E.K. , Klein R. , Wiley T.L. et al . ries). It was also important to establish primary concepts of state- 2003 . The impact of hearing loss on quality of life in older adults . ments to identify what the expression was meant to convey. Gerontologist , 43 , 661 – 668 . When linking clinical measures, it was deemed necessary to Danermark B. , Cieza A. , Gang é J. , Gimigliano F. , Granberg S. et al . 2010 . International classifi cation of functioning, disability, and health core refl ect upon both the aim and procedure of a specifi c measure- sets for hearing loss: A discussion paper and invitation. Int J Audiol , ment to link to the correct components and categories. 49 , 256 – 262 . • In future revisions of the ICF, from an audiological perspective, De Boer J. , Thornton A.R. & Krumbholz K . 2012 . What is the role of attention should be paid to issues regarding overlapping catego- the medial olivocochlear system in speech-in-noise processing? ries within components (e.g. the third level categories of Hearing J Neurophysiol , 107 , 1301 – 12 . functions and the category for Auditory perception), overlapping Demorest M.E. & Erdman S.A . 1987 . Development of the Communication categories between components (e.g. distinguishing between Profi le for the Hearing Impaired . J Speech Hear Disord , 52 , 129 – 143 . Attention functions [Body functions] and Focusing attention Erdtman E. , Tideman M. , Fleetwood C. & M ö ller K . 2012 . Research initiation [Activities & Participation]), the lack of categories when linking based on idea-circles: From research object to co-actor . Disabil Soc , 27 , functions of structures (e.g. the function of the ossicles or the 879 – 882 . Escorpizo R. , Finger M. , Gl ä ssel A. , Gradinger F. , L ü ckenkemper M. tympanic membrane), and to the descriptions of specifi c ICF et al . 2011 . A systematic review of functioning in vocational rehabili- categories to facilitate the applicability of the classifi cation. tation using the International Classifi cation of Functioning, Disability and Health . J Occup Rehabil , 21 , 134 – 146 . Acknowledgements Gatehouse S . 1998 . Speech tests as measures of outcome . Scand Audiol , 27 (Suppl. 49), 54 – 60 . The authors would like to thank Hans-Erik Fr ö lander, Jean-Pierre Gatehouse S . 1999 . Glasgow Hearing Aid Benefi t Profi le: Derivation and Gagn é , and De Wet Swanepoel for fruitful discussions and suggestions validation of a client-centered outcome measure for hearing aid services . regarding linking matters; Jerome Bickenbach, Alarcos Cieza, J Am Acad Audiol , 10 , 80 – 103 . Heinrich Gall, and Melissa Selb from the ICF Research Branch Granberg S ., Dahlstr ö m J ., M ö ller C ., K ä h ä ri K . & Danermark B . 2014. (a cooperation partner within the WHO Collaborating Centre for the The ICF Core Sets for hearing loss – researcher perspective, Part I: Family of International Classifi cations in Germany [at DIMDI]) for Systematic review of outcome measures identifi ed in audiological fruitful ICF discussions; Agneta Anderzé n Karlsson for discussions research . Int J Audiol , 53 , 65 – 76 . and suggestions on manuscript outline; Tobias Å slund at the Graneheim U.H. & Lundman B . 2004 . Qualitative content analysis in nursing research: Concepts, procedures and measures to achieve trustworthiness. Audiological Research Centre in Ö rebro for stylistic support; and Nurse Educ Today , 24 , 105 – 112 . the following members of the ICF core sets for HL steering Hallberg L.R.-M. & Carlsson S.G . 1991 . A qualitative study of strategies committee: Louise Hickson, Sophia Kramer, Bradley McPherson, for managing a hearing impairment . Br J Audiol , 25 , 201 – 211 . Jan Peter Str ö mgren, and Gerold Stucki. Hallberg L.R.-M. , Eriksson-Mangold M. & Carlsson S.G . 1992 . Psychometric evaluation of a Swedish version of the Communication Strategies For personal use only. Declaration of interest: A declaration of interest statement report- Scale of the Communication Profi le for the Hearing Impaired. J Speech ing no confl ict of interest has been inserted. Please confi rm whether Hear Res , 35 , 666 – 674 . H é tu R. , Riverin L. , Lalande N. , Getty L. & St-Cyr C . 1988 . Qualitative the statement is accurate. analysis of the handicap associated with occupational hearing loss. This work was funded by grants from the Oticon Foundation and Br J Audiol , 22 , 251 – 264 . the Stinger Foundation. ICF Research Branch . 2012 . ICF Core Sets. Manual for Clinical Practice . G ö ttingen: Hogrefe Publishing . References Kaplan H. , Bally S. , Brandt F. , Busacco D. & Pray J . 1997 . Communication Scale for Older Adults (CSOA) . J Am Acad Audiol , 8 , 203 – 217 . Andersson G. & Willebrand M . 2003 . What is coping? A critical review of Karlsson Espmark A.-K. & Hansson Scherman M . 2003 . Hearing confi rms the construct and its application in audiology . Int J Audiol , 42(Suppl. existence and identity: Experiences from persons with presbyacusis. 1) , S97 – S103 . Int J Audiol , 42 , 106 – 115 . Brooks R. & EuroQol Group . 1996 . EuroQol: the current state of play . Health Kiessling J. , Pichora-Fuller M.K. , Gatehouse S. , Stephens D. , Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 Policy , 37 , 53 – 72 . Arlinger S. et al . 2003 . Candidature for and delivery of audiological Cerniauskaite M. , Quintas R. , Boldt C. , Raggi A. , Cieza A. et al . 2011 . services: Special needs of older people . Int J Audiol , 42 (Suppl. 2), Systematic literature review on ICF from 2001 to 2009: Its use, S92 – S101 . implementation, and operationalisation . Disabil Rehabil , 33 , 281 – 309 . Killion M.C. & Gudmundsen G.I . 2005 . Fitting hearing aids using clinical Cieza A. , Brockow T. , Ewert T. , Amman , E. , Kollerits B. et al . 2002 . prefi tting speech measures: An evidence-based review. J Am Acad Linking health-status measurements to the International Classifi cation of Audiol , 16 , 439 – 447 . Functioning, Disability and Health . J Rehabil Med , 34 , 205 – 210 . Kondracki N.L. , Wellman N.S. & Amundson D.R . 2002 . Content analysis: Cieza A. , Geyh S. , Chatterji S. , Kostanjsek N. , Ü st ü n B. et al . 2005 . ICF Review of methods and their applications in nutrition education. linking rules: An update based on lessons learned. J Rehabil Med , 37 , J Nutr Educ Behav , 34 , 224 – 30 . 212 – 218 . Lazarus R.S . 1993 . Coping theory and research: Past, present, and future . Cieza A. & Stucki G . 2008 . The International Classifi cation of Functioning Psychosom Med , 55 , 234 – 247 . Disability and Health: Its development process and content validity . Eur Meyer C. & Hickson L . 2012 . What factors infl uence help-seeking for hearing J Phys Rehabil Med , 44 , 303 – 13 . impairment and hearing aid adoption in older adults? Int J Audiol , 51 , Clark J.L. , Roeser R.J. & Mendrygal M . 2007 . Middle ear measures . In: 66 – 74 . Roeser , Valente & Hosford-Dunn (eds.) Audiology Diagnosis, 2nd ed . Pichora-Fuller K.M. & Singh G . 2006 . Effects of age on auditory and New York: Thieme Medical Publishers Inc ., pp. 380 – 399 . cognitive processing: implications for hearing aid fi tting and audiologic Cox R.M. & Alexander G.C . 2002 . The International Outcome Inventory for rehabilitation . Trend Amplif , 10 , 29 – 59 . Hearing Aids (IOI-HA): psychometric properties of the English version. Post M.W.M. , Kirchberger I. , Scheuringer M. , Wollaars M.M. & Int J Audiol , 41 , 30 – 5 . Geyh S . 2010 . Outcome parameters in spinal cord injury research: A ICF Core Sets for HL, Linking of outcome measures 87

systematic review using the International Classifi cation of Functioning, Scarinci N. , Worrall L. & Hickson L . 2009 . The ICF and third-party disability: Disability and Health (ICF) as a reference . Spinal Cord , 48 , Its application to spouses of older people with hearing impairment. 522 – 528 . Disabil Rehabil , 31 , 2088 – 2100 . Priestley M. , Waddington L. & Bessozi C . 2010a . Towards an agenda Threats T . 2007 . Access for persons with neurogenic communication for disability research in Europe: Learning from disabled people’ s disorders: Infl uences of personal and environmental factors of the ICF. organizations . Disabil Soc , 25 , 731 – 746 . Aphasiology , 21 , 67 – 80 . Priestley M. , Waddington L. & Bessozi C . 2010b . ERRATUM. Towards Tye-Murray N . 1994 . Communication strategies training [monograph] . an agenda for disability research in Europe: Learning from disabled J Acad Rehabil Audiol , 27 , 193 – 207 . people ’ s organizations . Disabil Soc , 25 , 875 . Walden B.E. , Demorest M.E. & Hepler E.L . 1984 . Self-report approach to Pryce H. & Gooberman-Hill R . 2012 . ‘ There ’ s a hell of a noise ’ : living with assessing benefi t derived from amplifi cation . J Speech Hear Res , 27 , 49 – 56 . a hearing loss in residential care . Age Ageing , 41 , 40 – 6 . Ware J.E.J. , Snow K.K. , Kosinski M. & Gandek B . 1993 . SF-36 Health Survey Reed G.M. , Lux J.B. , Bufka L.F. , Trask C. , Peterson D.B. et al . 2005 . Manual and Interpretation Guide . Boston: New England Medical Centre . Operationalizing the International Classifi cation of Functioning, Ventry I. & Weinstein B . 1982 . The hearing handicap inventory for the Disability and Health in clinical settings. Rehabil Psychol , 50 , elderly: A new tool . Ear Hear , 3 , 128 – 134 . 122 – 131 . World Health Organization . 2001a . Fifty-fourth world health assembly, WHA Robinson K. , Gatehouse S. & Browning G.G . 1996 . Measuring patient 54.21. Agenda item 13.9, 22 May 2001. International classifi cation of benefi t from otorhinolaryngological surgery and therapy . Ann Otol functioning, disability and health. Retrieved 7 Jan, 2013, from http:// Rhinol Laryngol , 105 , 415 – 22 . www.who.int/classifi cations/icf/wha-en.pdf Roeser R.J. & Clark J.L . 2007 . Pure-tone tests . In: Roeser , V alente, & World Health Organization . 2001b . International Classifi cation of Hosford-Dunn (eds.) Audiology Diagnosis, 2nd ed . New York: Thieme Functioning, Disability and Health (ICF) . Geneva: WHO . Medical Publishers Inc ., pp. 238 – 260 . Zochling J. , Bonjean M. , Grill E. , Scheuringer M. , Stucki G. et al . 2006 . Saunders G.H. , Chisolm T.H. & Abrams H.B . 2005 . Measuring hearing Systematic review of measures and their concepts used in published aid outcomes: Not as easy as it seems . J Rehabil Res Dev , 42 , studies focusing on the treatment of acute infl ammatory arthritis . 157 – 168 . Clin Rheumatol , 25 , 807 – 813 .

Appendix 1 third level categories related to the second level designations) can be used when linking hearing related concepts. For example, Linking rules especially designed for audiological data Hearing (b230) can be used for descriptions of impairments in These linking rules should be used in combination with the coding the hearing functions, Listening (d115) can be used when guidelines for ICF (WHO, 2001, p. 219) and the established ICF describing the intentional part of ‘ hearing ’ but when no speech linking rules (Cieza et al, 2002, 2005) and are especially applicable comprehension is present, Communicating with receiving - spo- for audiological data. These rules can be used when linking state- ken messages (d310) can be used when linking speech compre-

For personal use only. ments, words, clinical measures, or other types of audiological data. hension (i.e. the receiving portion of communication) and 1. When linking outcome measures, read the origin article (when Conversation (d350) can be used when linking the bi-directional available) to understand the authors’ intentions with the measure- part of communication. ment and to identify any latent variables. 7. Noisy public ‘ arenas ’ (e.g. restaurants, grocery stores, large open 2. Make latent interpretations (identifying the underlying meaning) offi ces, etc.) should be coded with the codes for both ‘ noise ’ of concepts when linking statements to avoid mislinking (e2501) and ‘ acoustics ’ (e150) if not otherwise specifi ed. (i.e. linking to the wrong components and categories). 8. Assign coping as a personal factor (pf) if the meaningful concept 3. First, identify the appropriate component when linking meaningful is not judged as a communication strategy; in those cases, concepts; second, identify the appropriate category/categories. use d3602. 4. When linking statements, identify primary concepts for guidance 9. When an expressed hearing health condition has an ICD-10 code of what a statement is meant to convey. A primary concept (e.g. presbyacusis or sensorineural HL), use the designation describes the main activity/main focus of a statement. An of ‘ health condition ’ (hc ). When statements use expressions Int J Audiol Downloaded from informahealthcare.com by University of Western Australia on 02/18/14 example of this concept is as follows: ‘ I can ’ t hear what my like ‘ hearing impairment ’ , use the category code b230. mom says on the phone ’ , where the primary concept is can ’ t 10. The concept ‘ watching (TV) ’ should be coded with both the hear what my mom says . category code d110 Watching and with d310 Communicating 5. If it is not possible to determine the primary concept and to with - receiving - spoken messages . select the correct categories to link, use multiple linking, i.e. 11. Several concepts commonly addressed in audiology should be choose several categories. For example, it may be diffi cult to assigned as ‘ not covered ’ (nc ). For example, these concepts can distinguish whether a bus is the context (e1200) or whether the include ‘ visual cues ’ , ‘ home ’ , ‘ hearing-aid usage (frequency) ’ , statement is about using public transportation (d4702). ‘ time aspects ’ , and the ‘ number of hearing aids ’ . 6. Distinguish between hearing, listening, comprehending, and 12. When linking clinical measures, consider both the aim and the communicating (conversing). The second level categories (or test procedure when choosing appropriate ICF categories.

Supplementary material available online Supplementary Appendix 1

Semin Hear. 2016 Nov; 37(4): 369–378. doi: 10.1055/s-0036-1593994 PMCID: PMC5179601

Private Practice

Guest Editor, Gyl Kasewurm, Au.D. The Future of Private Practice in Audiology

David A. Fabry, Ph.D.1 Author information ► Copyright and License information ► Go to: Abstract

Although private practice in audiology has evolved during the past 40 years, hearing aids have remained as a central component to success. This article will discuss present and future trends for the next 40 years, including parallels to other professions and the need to innovate beyond technology.

Keywords: Hearing loss, hearing aids, audiology, personal sound amplification products, President's Council of Advisors on Science and Technology, Food and Drug Administration

Learning Outcomes: As a result of this activity, the participant will be able to (1) describe three differences between the professions of audiology, optometry, and dentistry as they relate to private practice trends; (2) list the primary recommendations made by the National Institute on Deafness and Other Communication Disorders Working Group (2009), as they relate to persons with hearing loss; (3) list five ways that technology will impact the diagnosis, treatment, and rehabilitation of hearing loss in the future.

When I graduated with a master's degree in audiology in 1983, one of my professors pulled me aside, wished me well, and said that he was glad that he was in his shoes, rather than mine, as he was concerned for the future of the profession. Nearly 35 years later, I can state unequivocally that I have not regretted one moment of my decision to become an audiologist, and although there are challenges ahead, I also see tremendous opportunity. In contrast to that professor, I only wish that I had the opportunity to start over, as I feel the future for the profession and private practice is bright, as long as we are willing to adapt. To that end, the focus of this article will be to discuss the current status and make some predictions for the future.

Go to: Current Status

Comparison to Other Professions

Audiology has steadily increased in visibility during the past decade, and it has consistently ranked highly on best-careers lists during the past 5 years.1 2 3 This is no doubt due to the transition from the master's degree to the doctorate of audiology, as well as the emergence of the baby boomer generation as the primary patient demographic for hearing and balance disorders. Comparison to other professions, including dentistry and optometry, reveals similar trends, particularly as they adapt to meet the needs of the 78 million boomers. There is more, however, behind that story that provides insight for the future of audiology private practice.

Optometry

In 1980, there were ∼22,000 optometrists practicing in the United States; almost 90% were either owners or partners in a private practice.4 Over the course of 30 years, however, industry consolidation occurred, as the number of vision care product manufacturers was reduced to only a handful of large players that increased market share (and profit margins) through their own retail networks. The impact of the baby boomer generation was significant, as many individuals with no history of visual impairment began experiencing “front of the eyeball” issues requiring correction for refractive eye disorders. The combination of technological improvements, corporate consolidation, and changes in distribution has resulted in a transformation of optometry from employers to employees for more than half of the 35,000 optometrists practicing in the United States,5 as most optometrists are now working either part-time or full-time for retail corporations. It will be interesting to see if this trend continues as optometry prepares for the second wave of their baby boomer experience, as the majority of that generation begin to move from “front of the eyeball” (refractive) to “back of the eyeball” (e.g., glaucoma, macular degeneration, retinal detachment) issues.

Dentistry

There are currently nearly 200,000 practicing dentists in the United States.6 7 Unlike optometry, however, to date, dentistry has remained predominantly an autonomous profession, with fewer than 17% of all active practitioners working as employees.8 There is a trend toward corporate dental practice,9 but many private practitioners are increasingly focusing on specialty and preventative care as a means of differentiating from their competition. Another focus of potential interest to audiology has been the growth of mid- level dental providers, including hygienists, and a focus on balancing third-party and private pay reimbursement.

Table 1 suggests that corporate consolidation is already underway for audiology; from 2005 to 2013, the self-described primary job title clinical staff audiologist grew from 54 to 59%, while the collective job titles of director, owner/partner, or CEO declined by roughly 6%.10 During the same time period, salaries for clinical audiologists rose 21%, while compensation for those working in independent practice increased by 41%. What does the future hold, and what may be learned from the parallels to optometry and dentistry?

Table 1 Self-described Job Title for Employed Audiologists

Urgency for Action

One thing that both dentistry and optometry have accomplished is the need for regular office visits and a sense of urgency when a problem exists. According to the Glaucoma Research Foundation,11 74% of adults surveyed said that they had their eyes examined at least once every 2 years. The U.S. Department of Health and Human Services Centers for Disease Control and Prevention reports that over 63% of adults in the United States visit their dentist annually.12 In comparison, Abrams and Kihm report that only 23% of adults had their hearing screened during their last physical.13 This included those who were simply asked, “How's your hearing?” This is despite the fact that the Centers for Medicare and Medicaid Services added a new benefit (over a decade ago!) for Medicare Part B beneficiaries that provides for hearing and balance screening questionnaires to be administered by a physician or qualified nonphysician provider during the Welcome to Medicare examination.14 It is only available for the first 6 months after enrollment, and limited to questionnaires only, but it is a start toward raising awareness for the importance of hearing, and reflects an age-related prejudice toward hearing loss in the United States. To wit: in 2013, Centers for Disease Control and Prevention data showed that over 97% of newborns in the United States were screened for hearing loss,15 and yet less than a quarter of older adults had their hearing screened, even though half of those aged 70 and older have measurable hearing loss!16

The mounting evidence linking comorbidities of untreated hearing loss with cardiovascular disease and cognitive decline,17 18 as well as many other health conditions, may assist audiologists with establishing urgency for action. Additional research is necessary, however, to establish causation, as well as correlation, particularly for hearing loss and incident dementia, which in turn may increase market penetration of hearing aids beyond 30% of those with measureable hearing loss.13

Go to: Future Trends

Improved Accessibility and Affordability

In response to persistently low market penetration during the past 30 years, the Food and Drug Administration (FDA) issued a guidance document for industry and FDA staff related to hearing aid devices and personal sound amplification products (PSAPs) in February 2009.19 Subsequently, the National Institute on Deafness and Other Communication Disorders (NIDCD) established the Working Group on Accessible and Affordable Hearing Health Care for Adults With Mild to Moderate Hearing Loss in August, 2009.20 The goal of both of these initiatives was to increase hearing aid access and affordability for persons with mild to moderate degrees of hearing loss. The original guidance created the PSAP category for those individuals who did not have measureable hearing loss, but nonetheless had difficulty with distant or soft speech. Finally, the FDA issued draft guidance for regulatory requirements for PSAPs in 2013,21 which further clarified that PSAPs were designated for “non-hearing-impaired consumers.”

This federal activity led to considerable discussion and debate in the scientific community, leading to the President's Council of Advisors on Science and Technology (PCAST) to hold a series of meetings in 2015, culminating in a document that recommended that the FDA open the hearing aid market by withdrawing the 2013 FDA draft guidance and establish a new class of over the counter (OTC) hearing aids for mild to moderate age-related hearing loss that could be purchased without the need for a credentialed dispenser (i.e., audiologist or hearing instrument specialist).21 22 In addition, two more recommendations were made largely to provide portability of the hearing loss diagnostic testing results and suggested fitting results.

Finally, the Institute of Medicine (IOM), which eventually became known as the National Academies of Science, Engineering, and Medicine (NASEM), provided a series of consensus meetings and a report with 12 recommendations to improve access and affordability in June 2016.23 The report acknowledged that FDA regulation has not stifled innovation and hearing aid advancement, and also recommended regulation of OTC products, with distinction from regulations applied to hearing aids and PSAPs.

The “good news, bad news” of all of this activity is that it has raised awareness for the importance of hearing; at issue is how private practitioners withstand the harsh glare of the perception that hearing aids are too expensive, due in large part to the bundled model of technology and service that dominates the U.S. market. At issue is whether the creation of a separate category of OTC devices for persons with mild to moderate hearing loss would improve access and affordability without potentially harming the patient or compromising care. At the moment of this writing, no decision has been made by the FDA regarding expanding PSAPs or creating a new class of OTC devices.

The consumer electronics industry has introduced a flurry of products from Samsung, Apple, and smaller companies like Bragi that provide impressive technology for wireless connectivity, audio transparency, and even beam-forming microphone arrays previously found only on premium hearing instruments. If approved for use with mild to moderate hearing loss, without the need for a professional, it could dramatically impact the way hearing aids are distributed in the United States. Private practitioners will need to demonstrate their added value to this process in the face of unbundling of the device price from their professional services. To do this, they will need to demonstrate innovations beyond technology.

Innovations beyond Technology

Topics that have been addressed elsewhere in this issue, including the use of assistants and support personnel, key performance indicators to drive business, best (evidence-based) practice, and proper billing/coding techniques will all assist in improved delivery of hearing and balance services. The inexorable regulatory and technology changes that will impact the hearing aid industry, however, have the potential to alter the distribution process that has been in place for decades.

Clayton Christensen coined the term disruptive innovation as one that creates new market and distribution channels, eventually displacing existing market leading firms, products, and services.24 Disruptive innovations tend to be produced by outsiders and entrepreneurs, rather than from existing market leaders, because the products and services are initially seen as inferior or not profitable enough (at least at first). It is, however, possible for an industry or profession to self-disrupt if they are open to drastic change from the status quo.

The Inevitable

In his recent book, Kevin Kelly also addresses the rapid pace of innovation, in terms of a dozen technological forces that will shape the future of any discipline, due to digital technology.25 I have framed the predictions for the future of audiology private practice around Kelly's 12 forces of technology.25

Becoming

According to MarkeTrak 9,13 the average replacement cycle for hearing aids has increased to between 6 to 7 years, presumably due to cost and increased reliability. During the life span of the typical analog hearing aid, this meant that the primary emphasis was placed on maintenance to ensure that components were kept in proper working order and that changes in hearing loss were accounted for by the audiologist. The advent of first digitally programmable analog, and eventually, digital hearing aids in the early 1990s provided dramatic improvements in device flexibility to adjust hearing gain and output to meet patient changing needs. Today's devices, however, offer the opportunity for continual upgrades to feature sets including compression, noise management, feedback cancellation, and other sophisticated features as new algorithms become available. Patients from the traditionalist generation (born pre–World War II) may be less than enthusiastic about paying more for software or firmware upgrades to a device that they have already purchased, but it has become commonplace for baby boomers to pay for upgrades to software, apps, and other programs whose features change over time. Tomorrow's hearing aids will not necessarily last any longer, but upgradability will enable them to remain state-of-the-art over their life span. That said, not all patients will be comfortable with the continuous process of upgrades without guidance and support of their clinician. In a sense, the continuous evolution of the products due to products becoming better will provide a consistent role for the audiologist to help the patient overcome the feeling that they are a perpetual newbie. The momentum of technology, particularly as it appeals to the new generation of baby boomers who will soon become the dominant force in the hearing aid market, will provide the motivated professional with a way to prevent commoditization by serving as an interface between the hearing aid and the patient.

Cognifying

Artificial intelligence (AI) is ubiquitous these days; even a miniscule amount of useful intelligence embedded into an existing device promises increased effectiveness and efficiency. Or so we thought, over 20 years ago when hearing aids first incorporated data logging of wearing time, listening environment, and battery life. Hearing aids have used some form of AI without much fanfare for quite some time, and the more recent integration of “made for iPhone” and “made for Android” hearing aids have cognified them further. Using the smartphone's integrated geotagging capability and motion sensors permit hearing aids to know where the user is, whether they are moving or standing still, and whether they have been in that place before. Hearing aids already have moved well beyond the era of a stand- alone device that provides gain as a function of frequency, and the hearing aids of tomorrow will provide both the user and practitioner with a wealth of “always on” intelligence. Microelectromechanical sensors will provide information regarding whether the hearing aids are inserted in the proper ears, whether they have been exposed to high humidity, and even report biomedical information, such as heart rate, temperature, and blood sugar levels. They will be capable of providing appointment reminders (when to come in for appointments, recharge batteries, dehumidify), real-time translation, and health reminders (when to take medication, if they are at risk for a fall, or even if they are having a heart attack). Cognifying the world is a big deal, it is happening right now, and the ear will serve as an important interface. This transition will be overwhelming for many patients, and the role of the practitioner will be to assist with the interpretation of big data into bite-sized portions. The audiologist is uniquely qualified to serve in the capacity of counselor, coach, technical advisor, health practitioner, and data analyst.

Flowing

The Internet of things is a proposed development of the Internet in which everyday objects have network connectivity, allowing them to send and receive data seamlessly in real time. With regard to hearing aids, the most promising strategy relates to wireless connectivity. Imagine a hearing aid user walking into a theater, lecture hall, or movie and automatically being asked whether they want to pair with the sound system. Many televisions are already equipped with Bluetooth connectivity, which will eliminate the need for specialized, stand- alone devices for TV streaming. A recent paper by Strelcyk et al reported that although TV listening was important and relevant for the hearing aid users they surveyed, few understood or made program changes to specialized settings for use with television.26 Technology in the future will need to “flow” effortlessly to be adopted, and will require immediacy, personalization, interpretation, and accessibility. Audiologists can serve a vital role in all these areas, through use of demonstration, clear counseling, and follow-up to ensure understanding. Much has been made of the impact of consumer electronics to open up the hearing aid market. An alternative interpretation may be the reality of using professionally fit hearing aids to connect seamlessly to other consumer electronics, in turn providing the best of both worlds in ways that we do not accomplish today.

Screening

Rather than referring to examining for the presence of hearing loss, this force refers to digital “screens” in the form of display monitors, tablet screens, and smartphones. According to Kelly,25 we live in an increasingly visual world, with digital display manufacturers cranking out 3.8 billion new additional screens per year. Now that nearly every hearing aid dispensed in the United States in 2016 is digital, and with two-thirds of Americans owning a smartphone,27 it creates opportunities for audiologists to provide both face-to-face and telehealth support to their patients. Telehealth is central to the trifecta of health care: better population health, improved patient care, and lower costs. In a sense, professionally delivered telehealth services provide the opportunity to achieve the objectives set by the NIDCD, PCAST, and FDA while still preserving the role of the practitioner. By combining face time in the clinic with screen time in between, the private practitioner may improve access, efficiency, outcome, and satisfaction with a generation of patients accustomed to virtual reality. The baby boomers will demand the convenience of synchronous and asynchronous models of telehealth to meet their lifestyle needs, and the private practitioner is in an ideal position to pivot to the use of this modality. Furthermore, because the majority of hearing aids sold in the United States remain private pay, applications for concierge care that bundles different types of professional services into a single package are possible.

Accessing

Tom Goodwin recently observed that “Uber, the world's largest taxi company, owns no vehicles. Facebook, the world's most popular media owner, creates no content. Alibaba, the most valuable retailer, has no inventory. And Airbnb, the world's largest accommodation provider, owns no real estate. Something interesting is happening.” 28 In the emerging digital world, possession is not as important as it once was; accessing is more important than ever. How will the hearing aid market adapt? It is possible that, instead of a major purchase made every 5 to 7 years, leasing options will develop that provide for a monthly fee that covers product, battery, upgrades, and services. It is not inconceivable that in the future, today's hearable devices (e.g., Apple AirPods, Samsung Gear IconX) could be included in the phone purchase and paid for via a monthly service contract. Lower up-front costs to the consumer, in combination with several of the 12 forces, will provide the opportunity to forge in new directions that may commoditize the product, but not the professional service. In this way, it may be possible to build more loyalty to the practitioner than the product. The switch from “ownership that you purchase” to “access you subscribe to” overturns many conventions, and private practitioners can pivot more easily than large clinics to this type of approach.

Sharing

In addition to the change from ownership to subscription in the digital economy, another trend has been toward free through sharing or collaboration. The implications for private practice include the opportunities to share ideas, concepts, wearing tips with patients via open or private social media platforms, like Facebook, YouTube, Twitter, and Instagram. Furthermore, cooperation with current patients to assist in marketing to prospective patients is a high-impact, low-cost marketing tool. Organized collaboration and collectivism provides an opportunity to work with local businesses and charities to rate the ambience of dining establishments in terms of noise/reverberation levels, seating recommendations, and lighting for optimal communication. Finally, the opportunity to become a local, regional, or national expert on hearing loss prevention, ototoxicity, tinnitus management is enhanced by the sharing culture. Today's crowdsourcing was yesterday's networking for the practitioner.

Filtering

There has never been a better time to live for information junkies; a mountain of digital content is created each year. Kelly points out that every 12 months, 8 million new songs are produced, 2 million books are published, 16,000 films are made, and 30 million blog posts are published.24 If the Internet of things connects electronic devices, increasingly people are connected to the online library of everything. This seemingly infinite array of choices is overwhelming to many, and private practitioners have the opportunity to serve as professional filters to personalize and optimize choices for their patients. This is not possible without trust, engagement, and investment of time that exceeds patient expectation. Within audiology private practice, the specific opportunities for filtering include concierge care, as mentioned previously, in which the patient pays an annual fee or retainer in exchange for personalized/enhanced care by limiting patient loads to ensure adequate time and availability for each patient.

Another opportunity for filtering is via the use of technology, in the form of telehealth, Web-, app-, or text-based based approaches to customize, personalize, or even “gamify” the process of aural rehabilitation, tinnitus therapy, or other ongoing care that may use technology to assist patients with forming healthy habits using a combination of support mechanisms in an adaptive framework. Furthermore, the use of technology to provide momentary feedback in real time during the hearing aid trial period may assist professionals in addressing fitting/follow-up challenges early in the process, rather than the defined clinical visits (e.g., 2- week follow-up visit). This may, in turn, reduce return-for-credit rates and improve user satisfaction ratings.

Remixing

According to Brian Arthur,29 all new technologies derive from a combination of existing ones that have been rearranged and remixed in novel ways. Whether audiologists like it or not, the Apple AirPods, Samsung Gear IconX, or Bragi Dash may be repurposed with applications like Ear Machine (Bose Corporation, Framingham, MA) to create customized OTC devices if/when the FDA creates a new category of device. Entrepreneurial clinicians will decide whether they will choose to use these types of products and devices as lead generation tools for future patients, by providing technical support on a fee-for-service basis with these starter devices. Wu et al remixed a software/cloud recording-based device originally intended for use in infant children for their study of real-world speech listening environments in adults.30 31 Similar opportunities exist for combining hearing aid devices with third-party app developers to invent new research, clinical, and practice technologies that do not currently exist. During the next 30 years, the most important inventions and clinical tools will likely be the ones that have been remixed most successfully.

Interacting

Virtual reality (VR) is an artificial world that feels authentic. Currently, many VR approaches are heavily visually based, with audio as an afterthought. Similarly, the audio demonstrations used in many clinical settings do not replicate the real-world environment experienced by many patients when they wear their hearing aids. Two strategies for improving interactions with hearing aids are to focus more on externalization of sound with binaural hearing aids and also to move from practitioner-based to patient-based or patient-driven fittings.

Despite the fact that the United States has one of the highest binaural hearing aid fitting rates in the world,13 many clinicians pay little or no attention to whether the hearing aids selected preserve localization, minimize occlusion, and create the perception of externalized sound, rather than one that originates in the head. Increasingly, hearing aid technology will evolve from lo-fi to hi-fi devices that preserves (or restores) 3-D audio, at least for those with adequate residual auditory area to take advantage of high-frequency cues necessary to achieve this result.

Currently, many clinicians cling to the practitioner-based, real-ear measurement-based assumption that a match to prescriptive target ensures a successful outcome. Although this is a great starting point, technologies will continue to emerge in the future that will permit greater interaction/adjustment by the patient in real-world environments. Historically, this has often been perceived as a threat to the role of the professional, but the inevitable force of interacting will require a change in attitude and actually augment the role of the professional by facilitating greater patient control to assist with optimizing outcomes. The convergence of maximum interaction and maximum patient control will override professional insecurity and provide differentiation for the practitioners who are willing to cede control. In the coming years, anything that is not intensely interactive will not be considered state of the art.

Tracking

Scientific tracking and self-measurement via digital sensors has become far more convenient, less expensive, and widely available through the use of biometric tracking devices. These tiny recording devices have started to change our ideas of medicine, health, and human behavior. Currently, the majority of devices that track steps, sleep, and heart rate are in the form of wearable devices, typically worn on the wrist. The dirty secret of wearables, however, is that although 1 in 10 Americans over the age of 18 own an activity tracker, nearly half abandon their use after 12 months.32 It turns out that the ear serves as remarkably good real estate for tracking temperature, heart rate, steps, electroencephalogram, and respiration. The hearable device was created because many people want to track biometric activity while they are exercising,33 when they also want to stream audio from TV or a media device. Future applications will permit the next generation of data-logging instruments that provide acoustic information from various listening environments, as well as measures of social interaction subsequent to being fitted with hearing aids. In turn, this life-streaming information will provide clinicians with increasingly detailed longitudinal data regarding the impact of hearing technology. The personal archive of information may be used to detail comorbidity between hearing loss and cardiovascular disease or cognitive decline. Plus, more widespread use of wireless audio devices will help reduce stigma for hearing aids and provide opportunity for clinicians to employ customized solutions for hearable devices. The biggest obstacle remains the need for privacy; we are on our way to manufacturing 54 billion sensors every year by 2020.34 Certainly, this will require privacy solutions that we cannot yet anticipate.

Questioning

We have always had questions. Before Google or Wikipedia, the largest answering business was telephone directory assistance. The printed version was the Yellow Pages. Today, the number of reference databases to answer questions about general topics on hearing, where to get hearing aids, how satisfied patients are with clinic services, and a host of other questions are available online or through applications like Healthgrades, Yelp, RateMDs, AngiesList, Vitals, and a host of other Web sites. In the past, questions such as “Why are hearing aids so expensive?” or “Where should I get my hearing tested?” would be either more hierarchical or dependent on a physician, family member, or acquaintance. Society is increasingly moving away from a rigid order of hierarchy toward the fluidity of decentralization and search engine optimization. Questioning will be seen as the mechanism that generates new disciplines, new brands, and new possibilities.

Beginning

The final technological force of the future is, ironically, beginning. The future of technology will likely not be either utopian or dystopian. Rather, it will likely be protopian, with slight incremental improvements due to the interactions between all 12 forces, creating combinations that we cannot see from today.

Go to: Summary

The future of private practice audiology should first and foremost be focused on the needs of the patient and on hearing as an important health condition. By raising awareness for the importance of healthy hearing in the aging population, we will achieve the objectives of the PCAST, IOM/NASEM, NIDCD, and FDA to improve accessibility and affordability for the population with hearing loss.

The aging baby boomer generation will require different approaches than the ones used during the past 30 years. To be successful, practitioners will need to consider innovation strategies that employ combinations between technology and service, employing some/all of the 12 technological forces discussed in this article. Rather than waiting for disruptive innovation to commoditize the role of the clinician, self-disruptive innovation will provide solutions that are less dependent on the device alone, and more on the benefits of our professional service.

Go to: References

1. Careercast The best jobs of 2016: 4 Audiologist. 2016. Available at: http://www.careercast.com/slide/best-jobs-2016-4-audiologist. Accessed November 15, 2016 2. U.S. News and World Report Best healthcare jobs 2014. Available at: http://money.usnews.com/careers/best-jobs/audiologist. Accessed November 15, 2016 3. Rhodan M This is the best job in America Time. June 25, 2015. Available at: http://time.com/3935051/best-job-america/. Accessed November 15, 2016 4. Smriga D J For audiology, dentistry offers a good model for preserving independent private practice Hear J 200659936–38., 42, 44 5. Bureau of Labor Statistics Occupational employment and wages, May 2015 Available at: http://www.bls.gov/oes/current/oes291041.htm. Accessed November 15, 2016 6. American Dental Association Dental statistics 2015. Available at: http://www.ada.org/en/science-research/health-policy-institute/dental-statistics/workforce. Accessed November 15, 2016 7. Munson B Vujicic M Supply of dentists in the United States is likely to grow Health Policy Institute Research Brief. American Dental Association. October 2014. Available at: http://www.ada.org/∼/media/ADA/Science%20and%20Research/HPI/Files/HPIBrief_1014_ 1.ashx. Accessed November 15, 2016 8. Solomon E Dental workforce trends and the future of dental practice February 26, 2015. Available at: http://www.dentaleconomics.com/articles/print/volume-105/issue- 2/macroeconomics/dental-workforce-trends-and-the-future-of-dental-practices.html. Accessed November 15, 2016 9. Solomon E The future of dental practice: demographics Published April 10, 2015. Available at: http://www.dentaleconomics.com/articles/print/volume-105/issue- 4/macroeconomics/the-future-of-dental-practice-demographics-patients-professionals-and- procedures.html. Accessed November 15, 2016 10. American Academy of Audiology Member compensation and benefits survey 2005–2013 2016. Available at: http://www.audiology.org/about-us/membership/benefits/compensation- and-benefits-survey. Accessed November 15, 2016 11. Glaucoma Research Foundation Glaucoma facts and stats 2016. Available at: http://www.glaucoma.org/glaucoma/glaucoma-facts-and-stats.php. Accessed November 15, 2016 12. US Department of Health and Human Services, Centers for Disease Control and Prevention Health, United States: with special feature on racial and ethnic health disparities 2015. Available at: http://www.cdc.gov/nchs/data/hus/hus15.pdf#078. Accessed November 15, 2016 13. Abrams H B Kihm J An introduction to MarkeTrak IX: a new baseline for the hearing aid market Hearing Review. 201522(6):16. Available at: http://www.hearingreview.com/2015/05/introduction-marketrak-ix-new-baseline-hearing-aid- market/. Accessed November 15, 2016 14. Centers for Medicare and Medicaid Services Your “Welcome to Medicare” preventative visit 2016. Available at: https://www.medicare.gov/people-like-me/new-to- medicare/welcome-to-medicare-visit.html. Accessed November 15, 2016 15. Centers for Disease Control and Prevention Hearing loss in children 2013. Available at: http://www.cdc.gov/ncbddd/hearingloss/data.html. Accessed November 15, 2016 16. Lin F R, Niparko J K, Ferrucci L. Hearing loss prevalence in the United States. Arch Intern Med. 2011;171(20):1851–1852. [PMC free article] [PubMed] 17. Gates G A, Cobb J L, D'Agostino R B, Wolf P A. The relation of hearing in the elderly to the presence of cardiovascular disease and cardiovascular risk factors. Arch Otolaryngol Head Neck Surg. 1993;119(2):156–161. [PubMed] 18. Lin F R, Metter E J, O'Brien R J, Resnick S M, Zonderman A B, Ferrucci L. Hearing loss and incident dementia. Arch Neurol. 2011;68(2):214–220. [PMC free article] [PubMed] 19. US Department of Health and Human Services, Food and Drug Administration Guidance for industry and FDA staff: regulatory requirements for hearing aid devices and personal sound amplification products February 25, 2009. Available at: http://www.fda.gov/MedicalDevices/DeviceRegulationandGuidance/GuidanceDocuments/uc m127086.htm. Accessed November 15, 2016 20. U.S. Department of Health and Human Services NIDCD Working Group on Accessible and Affordable Hearing Health Care for Adults with Mild to Moderate Hearing Loss Workshop held August 25–27, 2009. Available at: https://www.nidcd.nih.gov/workshops/nidcd-workshop-accessible-and-affordable-hearing- health-care/2009/agenda. Accessed November 15, 2016 21. US Department of Health and Human Services, Food and Drug Administration Regulatory requirements for hearing aid devices and personal sound amplification products— draft guidance for industry and Food and Drug Administration staff November 7, 2013. Available at: http://www.fda.gov/MedicalDevices/DeviceRegulationandGuidance/GuidanceDocuments/uc m373461.htm. Accessed November 15, 2016 22. President's Council of Advisors on Science and Technology Aging America & hearing loss: imperative of improved hearing technologies September 2015. Available at: https://www.whitehouse.gov/sites/default/files/microsites/ostp/PCAST/Aging%20America% 20and%20Hearing%20Loss.pdf. Accessed November 15, 2016 23. National Academies of Science, Engineering, and medicine Hearing health care for adults: priorities for improving access and affordability June 2, 2016. Available at: http://www.nationalacademies.org/hmd/Reports/2016/Hearing-Health-Care-for-Adults.aspx. Accessed November 15, 2016 24. Christensen C. Boston, MA: Harvard Business School Press; 1997. The Innovators Dilemma: When New Technologies Cause Great Firms to Fail. 25. Kelly K. New York, NY: Penguin Press; 2016. The Inevitable: Understanding the 12 Technological Forces That Will Shape Our Future. 26. Strelcyk O Singh G Standaert L Rakita L Derlath P Launer S TV/Media listening and hearing aids Poster presented at: International Hearing Aid Conference; August 2016; Lake Tahoe, NV 27. Smith A U.S. smartphone us in 2015. Pew Research Center April 1, 2015. http://www.pewinternet.org/2015/04/01/us-smartphone-use-in-2015. Accessed November 15, 2016 28. Goodwin T The battle for the customer interfaceTech Crunch, March 3, 2015 29. Arthur W B. New York, NY: Free Press; 2009. The Nature of Technology: What It Is and How It Evolves. 30. Wu Y H Stangl E Welhaven A Oleson J Modeling real-world speech listening environments Paper presented at: International Hearing Aid Congress; August 10–14, 2016; Lake Tahoe, NV 31. Wikipedia LENA Research Foundation 2016. Available at: https://en.wikipedia.org/wiki/LENA_Foundation. Accessed November 15, 2016 32. Ledger D McCaffrey D Inside wearables: how the science of human behavior change offers the secret to long-term engagement. Endeavor Partners White Paper 2014. Available at: http://endeavourpartners.net/assets/Endeavour-Partners-Wearables-and-the-Science-of- Human-Behavior-Change-Part-1-January-20141.pdf. Accessed November 15, 2016 33. Ledger D The rocky path towards personalized, insightful wearables Endeavor Partners White Paper. 2016. Available at: http://endeavourpartners.net/assets/Endeavour-Partners- Inside-Wearables-Part-3-Jan-20162.pdf. Accessed November 15, 2016 34. Cisco 50 billion devices on the Internet by 2020 Connections Counter, Cisco, 2013. Available at: http://spectrum.ieee.org/tech-talk/telecom/internet/popular-internet-of-things- forecast-of-50-billion-devices-by-2020-is-outdated. Accessed November 15, 2016

International Journal of Audiology

ISSN: 1499-2027 (Print) 1708-8186 (Online) Journal homepage: http://www.tandfonline.com/loi/iija20

Adults with mild hearing impairment: Are we meeting the challenge?

Barbra H.B. Timmer, Louise Hickson & Stefan Launer

To cite this article: Barbra H.B. Timmer, Louise Hickson & Stefan Launer (2015) Adults with mild hearing impairment: Are we meeting the challenge?, International Journal of Audiology, 54:11, 786-795, DOI: 10.3109/14992027.2015.1046504

To link to this article: http://dx.doi.org/10.3109/14992027.2015.1046504

Published online: 08 Jun 2015.

Submit your article to this journal

Article views: 189

View related articles

View Crossmark data

Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=iija20

Download by: [UQ Library] Date: 22 November 2015, At: 14:59 International Journal of Audiology 2015; 54: 786–795

Original Article

Adults with mild hearing impairment: Are we meeting the challenge?

Barbra H.B. Timmer* , Louise Hickson* & Stefan Launer*,†

* School of Health and Rehabilitation Sciences, University of Queensland, Brisbane, Australia, and †Phonak AG, St ä fa, Switzerland

Abstract Objective: Acquired hearing impairment is recognized by the World Health Organization as the third leading cause of disability, with a mild impairment being the most prevalent. The aim of this study was to review research literature concerned with adults with acquired mild hearing impairment; the defi nitions and prevalence, the resulting activity limitations and participation restrictions, and hearing-aid interventions. Design: This study involved a systematized review of research literature identifi ed through searches in citation databases and through reference checking. Study sample: A total of 151 papers were identifi ed and of these, 33 papers were included in this review. Results: Prevalence rates are signifi cantly infl uenced by the defi nition used for mild hearing impairment, and range from 1 in 3 to 1 in 5 adults. The weak correlations between audiological assessments and self-reported diffi culties suggest that further assessment of individuals with mild hearing impairment is warranted. The most common intervention is the provision of hearing aids with varying rates of use, benefi t, and satisfaction. Conclusions: The development of appropriate audiological assessment in the clinic, and further evaluation of the real-world listening needs and performance of people with mild hearing impairment is required to provide a more effective pathway for this clinical population.

Key Words: Hearing loss; hearing impairment; hearing aids; mild; older adults; literature review

In 2004, the World Health Organization (WHO) reported that the Hearing function, and the effect a hearing impairment may have most common global cause of disability was adult-onset hearing on an individual’ s daily life, can be comprehensively described Downloaded by [UQ Library] at 14:59 22 November 2015 impairment (WHO, 2004). This is particularly noteworthy as the using the framework provided by the World Health Organization’ s WHO indicates the total number of adults over 65 years old will triple International Classifi cation of Functioning, Disability and Health to close to 1.5 billion people between 2010 and 2050 (WHO, 2011). (ICF). The ICF provides a “ components of health” classifi cation Combining these population statistics with the knowledge that mild and a common language to describe individuals’ functioning and hearing impairment is the most prevalent of all degrees of impairment disability associated with health conditions (WHO, 2001). The ICF (WHO, 2004), mild hearing impairment can be expected to increas- utilizes ‘ functioning ’ as a broad term to include all body structures ingly affect older adults in future years. Longitudinal studies of age- and functions, activities and participation, and ‘ disability ’ as the related decline in hearing, such as Kiely et al (2012) and Lee et al broad term for the negative consequences of changes to function; (2005) show this change to be gradual, at around 1 dB HL per year, these are impairments, activity limitations, and participation restric- with rates of change increasing with age. The results of studies of tions. Activity is defi ned as the execution of a task or action by other age-related changes in, for example, cognition (Lin et al, 2013) an individual, and participation is defi ned as involvement in a life and activities such as walking and preparing meals (Dalton et al, situation. When used in the context of hearing impairment, an activ- 2003) suggest that declines in these functions may be exacerbated if ity can be listening to the television or using the telephone, while hearing impairment is not addressed at an early stage. participation includes conversations with friends and family or

Correspondence: Barbra Timmer, School of Health and Rehabilitation Sciences, The University of Queensland, St Lucia, Brisbane, Queensland 4072, Australia. E-mail: [email protected]

( Received 11 May 2014; accepted 27 April 2015 ) ISSN 1499-2027 print/ISSN 1708-8186 online © 2015 British Society of Audiology, International Society of Audiology, and Nordic Audiological Society DOI: 10.3109/14992027.2015.1046504 Adults with mild hearing impairment 787

to warrant aid fi tting, or the clinicians involved may not perceive Abbreviations that individuals with mild impairment are candidates for hearing 3FAHL Three frequency average hearing loss aids. Alternatively, as Kochkin (2012) suggests, some adults with 4FAHL Four frequency average hearing loss mild hearing impairment may indeed not experience any hearing AI Articulation index diffi culties and therefore do not have suffi cient need for interven- APHAB Abbreviated profi le of hearing-aid benefi t tion. To further understand the nature of mild hearing impairment FAAF Four alternate auditory feature and the implications of it, the following questions were addressed HHIE Hearing handicap inventory for the elderly in this review: HHIE-S Hearing handicap inventory for the elderly - • How is mild hearing impairment defi ned, and how prevalent is it? Screening version • How does a mild hearing impairment impact an individual’ s HINT Hearing in noise test activities and participation? ICF International Classifi cation of Functioning, • Do hearing aids meet the needs of adults with mild hearing Disability and Health impairment? RCT Randomized controlled trial SF-36 Short-form health survey Methodology SIP Sickness impact profi le SPIN Speech-in-noise The primary objectives of this study were to review research litera- WHO World Health Organization ture concerned with adults with acquired mild hearing impairment and, specifi cally, the defi nitions of mild hearing impairment, the nature of the impairment and its impact, and common interventions with this population. To ensure a systematic approach to the litera- involvement in other social situations. The ICF framework recog- ture search, a systematized review methodology was chosen (Grant nizes that an individual ’ s functioning and disability is a ‘ dynamic & Booth, 2009). ʼ interaction between health conditions and contextual factors which To this end, systematized searches in PubMed, Medline, and include both environmental and personal contextual factors, (p. 8; SCOPUS databases, and through reference checking were carried WHO, 2001). out between December 2013 and May 2014. There is little consistency Applying the ICF framework to hearing, clinical audiometric in the usage of the terms hearing loss and hearing impairment, there- measures provide an insight into an individual’ s hearing impair- fore the database search ensured each term was included. In this article ment but they may not reveal the activity limitations and participa- the term hearing impairment is used throughout and refers to both tion restrictions that the individual experiences. In the case of mild ‘ hearing loss’ as measured using average pure tone thresholds, and hearing impairment, the negative effects can vary widely for indi- ‘ hearing impairment ’ as measured through other means, such as self- viduals with similar audiometric data, and for this reason, defi ning report. In each database search, the search string contained variations disability merely by assessing impairment reveals only a part of the of the phrase “ mild sensorineural hearing impairment” (“ mild hearing clinician ’ s challenge in meeting the client ’ s needs. In addition, the loss” OR “ minimal hearing loss” OR “ slight hearing loss” OR “ mild manner in which a health condition is experienced is also infl uenced hearing impairment” OR “ minimal hearing impairment” OR “ slight by contextual factors. For example, an individual with mild hearing hearing impairment ” ). To further defi ne the search, additional key- impairment may experience no or few activity limitations when at words were then added to the search query (see Table 1). home but experience severe activity limitations (e.g. problems in The following fi lter criteria were also used: conversation) and participation restrictions (e.g. avoidance of con- • Downloaded by [UQ Library] at 14:59 22 November 2015 versation) in noisy or social settings. Papers published in English only The ICF can be applied as a clinical tool to support clinicians in • Subject sample to include adults only (18 years and over) areas such as needs assessment, rehabilitation and measuring out- Unfortunately, despite the search query restrictions, many of comes (Hickson & Scarinci, 2007; Gagn é et al, 2009). For adult- the search results yielded studies that included participants with onset hearing impairment, common rehabilitation options are the provision of hearing aids (Sprinzl & Riechelmann, 2010; Humes & Krull, 2012; Laplante-L é vesque et al, 2012), auditory training (Sweetow & Sabes, 2006; Humes et al, 2009; Henshaw & Ferguson, Table 1. Search database, additional keywords, and articles 2013), aural rehabilitation or communication programs (Abrams identifi ed using the search string and additional search terms. et al, 2002; Hickson et al, 2007) or a combination of these. The Additional keywords Number of articles provision of hearing aids is the most common, possibly due to a Database used identifi ed lack of availability of other rehabilitation options in clinical settings (Laplante-Lévesque et al, 2011). There is evidence to suggest that PubMed/Medline hearing-aid fi tting, however, is much less common for those with Prevalence/epidemiology 44 mild hearing impairment. In a questionnaire returned by a random Defi nition 1 sample of 3975 hearing-aid non-adopters who self-reported a mild Outcomes 16 hearing impairment, 43% reported their audiologist had suggested Hearing aid 27 a ‘ wait and retest ’ approach (Kochkin, 2012). This large-scale con- Scopus sumer survey conducted in the USA does not however provide insight Prevalence/epidemiology 13 into the reasons for this approach. It could be because of differences Defi nition 8 Outcomes 19 in the help-seeking behaviour of adults with mild hearing impair- Hearing aid 22 ment, or the clients may not report suffi cient hearing diffi culties 788 B.H.B. Timmer et al.

audiometric hearing thresholds (3FAHL or 4FAHL) greater than 16 to 26 dB HL at the lower limit and 39 to 45 dB HL at the upper 40 dB HL and therefore with hearing impairments which would be limit. In summary, the lack of a universally accepted pure-tone audio- better defi ned as moderate rather than mild. These papers were then metric defi nition means a single measure of mild hearing impairment excluded except where the results were presented in such a manner does not exist, nor is it likely to exist in the short term. as to allow the separation of data for participants with mild hear- The lack of a universal defi nition, and the variations in defi nitions ing impairment. To ensure applicability to current hearing-aid tech- of mild hearing impairment have a resulting infl uence on prevalence nology, any studies related to hearing-aid characteristics published estimations. In general, however, all estimates indicate that mild before 2000 were excluded. The cut-off at 2000 was chosen to ensure hearing impairment is common in the general adult population. In the search included literature after the availability of digital hearing a 2012 review, the WHO suggested that 9% to 17% of the world’ s aids, launched from 1995 (Bentler & Wu, 2014), and still covered population over the age of 15 years has a hearing impairment of a substantial period of time (15 years) of available hearing-aid lit- between 26 and 40 dB HL averaged over 500, 1000, 2000, and erature. No date of publication limits were set for other searches. 4000 Hz (classifi ed as mild by WHO), with prevalence varying by All papers were screened by the fi rst author, with issues concerning geographic region (WHO, 2012). For example, the prevalence rate inclusion or exclusion discussed amongst all authors. of mild hearing impairment for adults over 15 years in South Asia The literature search identifi ed 151 papers, of which 33 met the is 17%, in the Middle East and North Africa it is 7% and, in high- inclusion criteria. Although the highest level of evidence can be income regions including North America and Western Europe, it is gained from randomized controlled trials (RCTs; Wong & Hickson, just over 10%. The systematic review conducted by the WHO Global 2012), only one reviewed paper followed this design. Therefore, Burden of Disease Hearing Loss Expert Group in 2013 reported that quasi-experimental (or non-randomized), and non-intervention stud- the global prevalence of mild hearing impairment, defi ned in that ies such as cohort studies, available on the topic of mild hearing study as between 20 and 34 dB HL (averaged over 500, 1000, 2000, impairment were also reviewed and included if suffi cient study and 4000 Hz), was 22.7% for men and 19.0% for women over the design and participant information was provided. Although these age of 15 years (Stevens et al, 2013). last study designs are lower on the evidence quality hierarchy, this The differences in prevalence between these two recent WHO review found a lack of higher quality evidence and therefore the best studies may be explained by the lower threshold range used to defi ne available evidence was included. The critical appraisal checklist for mild hearing impairment by the WHO Global Burden of Disease audiological intervention, proposed by Wong and Hickson (2012), Hearing Loss Expert Group in 2013. The infl uence of different was used as a guideline for inclusion for this review. defi nitions of ‘ mild ’ hearing impairment is also evident in two Euro- pean studies. In a sample of 1041 older adults over 55 years of age, Duijvestijn et al (1999) reported that changing the lower limit for How is mild hearing impairment defi ned? mild impairment from 35 to 30 dB HL resulted in a 10% prevalence Hearing impairment in adults is most commonly measured by means increase and changing from a three frequency average hearing loss of pure-tone audiometry. The earliest systematic classifi cation of the (3FAHL) to a four-frequency average (4FAHL) increased prevalence degree of hearing impairment was published in the 1930s (Guild, 1932) by 9%. Uimonen et al (1997) found similar trends when applying and improvements upon it were proposed in the mid-1940s (Carhart, two different mild hearing impairment defi nitions; the then-current 1945). The most commonly used descriptive terms for hearing impair- WHO (3FAHL from 26 to 40 dB HL) and European Union (4FAHL ment severity today are from Clark (1981) who proposed labels such from 21 to 39 dB HL) classifi cations. Their sample of 1233 Finnish as mild, moderate, severe and more based on the average hearing adults between the ages of 55 and 75 years, drawn from the gen- thresholds from a pure-tone audiogram (Clark, 1981). Although much eral population, had a mild hearing impairment prevalence of either of the nomenclature of Clark’ s proposal has survived, the threshold 10.1% (WHO defi nition) or 29.5% (European Union defi nition).

Downloaded by [UQ Library] at 14:59 22 November 2015 ranges associated with the terms vary widely. Table 2 shows a range In other geographic regions, a small number of population-based of deﬁ nitions associated with mild hearing impairment. studies have also provided insight into the prevalence of mild hear- It can be seen that a slight or mild degree of hearing impairment is ing impairment. Sindhusake et al (2001) found a prevalence rate of variously deﬁ ned as pure-tone threshold levels averaged across any 39.1% for mild hearing impairment in 2015 adults over 55 years in number of octave frequencies, between 250 and 4000 Hz, of between the Blue Mountains region in Australia. Cruickshanks et al (1998)

Table 2. Summary of descriptive classiﬁ cations of mild hearing impairment.

Frequencies used Organization/Authors Average thresholds to calculate average

American Speech-Language-Hearing Association (ASHA) Slight 16 – 25 dB HL 500, 1000, 2000 Hz (based on Clark, 1981) Mild 26 – 40 dB HL World Health Organization (WHO) 1991 Slight 25– 40 dB HL 500, 1000, 2000 Hz British Society of Audiology 2011 Mild 20 – 40 dB HL 250, 500, 1000, 2000, 4000 Hz EU, European Working Group on Genetics of Hearing, 1996 Mild 21 – 39 dB HL 500, 1000, 2000, 4000 Hz New Zealand Deafness Notiﬁ cation Database, 1996 Mild 26 – 40 dB HL 500, 1000, 2000, 4000 Hz World Health Organization (WHO) 1997 Slight 25– 40 dB HL 500, 1000, 2000, 4000 Hz Australian Hearing/NAL Mild 21 – 45 dB HL 500, 1000, 2000 Hz National Institute on Deafness and Other Communication Functional handicap 500, 1000, 2000, 3000 Hz Disorders (NIDCD) around 40 dB HL Adults with mild hearing impairment 789

reported that 26.7% of over 43 year olds in a sample of adults resid- communication situations in everyday life. In the context of the ICF ing in the Beaver Dam, Wisconsin region in the USA, showed a framework, speech perception tests are one means of assessing both mild hearing impairment, with prevalence rate increasing sharply for body function and activity (Saunders et al, 2005) and can provide adults over the age of 60 years. Whilst both studies used a 4FAHL an insight into an individual ’ s activity limitations associated with at 500, 1000, 2000, and 4000 Hz, and defi ned a mild impairment to their hearing impairment. Since both pure tone and speech audi- be between 25 and 40 dB HL, prevalence differences between the ometry measure functioning, such measures might be expected to studies could be expected due to differences in defi nitions; Sind- be strongly related. But, in the case of mild hearing impairment, husake et al (2001) defi ned a hearing impairment as being present can speech perception be predicted from an individual’ s pure tone using the individual ’ s better ear thresholds, whilst Cruickshanks thresholds? A number of studies have investigated the correlation et al (1998) used the thresholds in the worse ear to classify hearing between mild pure-tone threshold impairment and speech audiom- impairment. This difference in defi nition could have led to a higher etry results, with two conclusions in common: (1) the reported cor- prevalence of hearing impairment in the Beaver Dam study however relations are often signifi cant, but only mild or moderate in scale, the Sindhusake et al (2001) sample included adults over 55 years and (2) individual variability in speech scores is large amongst of age, whilst 34.4% of Cruickshanks et al ’ s (1998) sample group people with mild hearing impairment. These fi ndings were inde- were younger, that is, aged 43 to 54 years. In summary, defi nitions pendent of whether stimuli were speech in quiet (Duquesnoy, 1983; of hearing impairment and the age of participants in various study Dubno et al, 1984; Smoorenburg, 1992), speech in noise (Matthews samples infl uence reported prevalence. Nevertheless, research fi nd- et al, 1990; Smoorenburg, 1992; Helfer & Freyman, 2008) or speech ings from epidemiological studies suggests that between 1 in 3 and 1 in reverberation (Helfer & Wilber, 1990). Table 3 summarizes in 5 adults of the general population has a mild hearing impairment the test materials and conditions used in these studies and over- depending on age, gender and geographic region. all, it can be concluded that for individuals with mild hearing For the purposes of inclusion criteria for this systematized review, impairment, speech perception could not be predicted based on a mild hearing impairment was defi ned based on the pure-tone audio- pure-tone thresholds. metric averages listed in Table 2, however it is noted that hear- Smoorenburg (1992) used a sample of 200 adults (400 ears), with ing disability can be experienced by those with clinically normal noise-induced hearing impairment, all with a 3FAHL less than 40 dB audiometric confi gurations. Studies by Kujawa and Liberman (2009) HL and 75% with pure-tone thresholds up to 8000 Hz that were no have outlined the process of neurodegeneration and hair cell func- worse than 40 dB HL at any frequency. He found the highest correlation recovery following noise exposure, which could lead to normal tion between pure-tone thresholds and speech reception thresholds hearing thresholds yet signifi cant hearing diffi culty, particularly in in quiet was 0.73, and 0.72 for speech reception thresholds in noise. noisy environments. As the Plack et al (2014) review pointed out, Therefore, no more than 53% of variance in speech understanding both noise exposure and aging have been shown to reduce speech could be accounted for by pure-tone thresholds. Smoorenburg (1992) perception even in individuals with normal audiometric thresholds. concluded that speech perception in noise can be affected by an Future research should investigate if other clinical and real world impairment as mild as 15 dB HL at 4000 Hz. Smoorenburg ’ s sample measures can provide clinicians with better insight into clients ’ hear- did not include any adults over the age of 55 years but his fi ndings ing challenges in everyday life. are in line with the Duquesnoy (1983) study, which included 110 participants between the ages of 60 and 90 years. Duquesnoy (1983) found participants with similar speech scores in How does a mild hearing impairment impact an individual ’ s quiet showed large variability on speech in noise tests, and concluded activities and participation? one could not be predicted from the other. While he highlighted the The WHO defi nition of a ‘ disabling ’ hearing impairment as being a clinical relevance of testing both in quiet and in noise, Duquesnoy

Downloaded by [UQ Library] at 14:59 22 November 2015 4FAHL over 40 dB HL (WHO, 2012) suggests that adults with mild (1983) did not address any age effects which could be expected in a hearing impairment do not experience disability as a result of their sample of older adults. This was addressed by Dubno et al (1984), hearing impairment. However, Lutman and colleagues concluded who measured speech recognition in quiet and in noise, in younger there is little relationship between threshold and onset of disability, and older adults with either normal hearing or a mild hearing impair- and suggest even a 3FAHL of 15 dB HL is “ signifi cantly disabling ment. Regardless of age, participants with mild hearing impairment for everyday speech” (p. 56; Lutman et al, 1987). This may not showed signifi cant differences in speech understanding in quiet com- be the case for all individuals with mild hearing impairment. Both pared to normal-hearing participants, particularly so for sentences Salonen et al (2011) and Sindhusake et al (2001) reported a weaker with low predictability. For speech in noise, there were signifi cant relationship between mild hearing impairment and self-reported differences attributable to hearing status, age, speech material and hearing diffi culty than greater degrees of impairment, using the the level at which the speech material was presented. After conclud- popular screening version of the Hearing Handicap Inventory for ing that speech understanding could not be predicted by pure-tone the Elderly (HHIE-S; Lichtenstein et al, 1988). These results may thresholds, Dubno et al (1984) then investigated if the use of the not be surprising as Weinstein and Ventry (1983) concluded that Articulation Index (AI) could improve prediction of speech in noise audiometric thresholds account for less than 50% of the variance in results but found this not to be the case, as the AI model does not reported hearing handicap. incorporate the individual ’ s age. Helfer and Freyman (2008) also showed that age alone had an effect on speech understanding, using speech both as target and as masker. When speech signals are used Speech perception as the competing stimulus, it may overlap acoustically with, and The impact a mild hearing impairment has on an individual can reduce audibility of, the target (energetic masking) and/or may be be assessed using various outcome measures. Speech audiometry more diffi cult to differentiate from the target signal (informational is often used as a clinical tool to assess a client’ s speech percep- masking; Helfer & Freyman, 2008). Their results showed that older tion using materials and presentation levels that may represent adults, and particularly those with mild hearing impairment, may 790 B.H.B. Timmer et al.

Table 3. Test materials and conditions of cited speech perception studies.

Reported correlation between pure-tone Test Characteristics of Number of threshold and speech reception threshold Study material Condition competing noise participants Age of participants (SRT)

Dubno et al Sentences Earphones Multi-talker 72 Two groups; Ͻ 4 4 Not specifi ed, as correlations were “ not (1984) and babble years and Ͼ 65 years high enough to suggest that pure-tone spondaic thresholds could accurately predict words performance in quiet or in noise ” (p. 93). Duquesnoy Sentences Earphones Long-term 110 60 – 90 years; mean age Pure-tone thresholds and SRT in quiet (1983) average speech not reported correlation ϭ 0.92 spectrum Pure tone thresholds and SRT in noise correlation ϭ 0.61 Helfer & Sentences Free fi eld Two-talker and 24 Two groups; mean High frequency average pure-tone Freyman speech-shaped age 22.67 years and thresholds and SRT in noise correlations (2008) 71.5 years ranged from 0.37 to 0.76 depending on masking stimulus and spatial confi guration between target and masker Helfer & Nonsense Earphones Reverberation 32 Two groups; Ͻ 3 6 Pure-tone thresholds and SRT in quiet Wilber syllables and/or cafeteria years and Ͼ 60 years correlation ϭ 0.75 to 0.88 (1990) noise Pure-tone thresholds and SRT in noise correlation ϭ 0.71 to 0.88, depending on thresholds utilized in the calculations, cafeteria noise and/or reverberation Kramer et al Sentences Free fi eld Long-term 51 30 – 70 years; mean Pure-tone thresholds and SRT in quiet (1996) average speech 52 years correlation ϭ 0.61 spectrum Pure-tone thresholds and SRT in noise correlation ϭ 0.53 Smoorenburg Sentences Earphones Speech-shaped 200Ͻ 55 years Pure-tone thresholds and speech in quiet (1992) correlation ϭ 0.55 to 0.72 Pure-tone thresholds and speech in noise correlation ϭ 0.50 to 0.72, depending on thresholds utilized in the calculations

experience more energetic masking from undesired speech signals should be assessed using a different method, such as with self-report when compared to younger adults. measures. One reason for the variability found in speech perception scores

Downloaded by [UQ Library] at 14:59 22 November 2015 may be the complexity of the relationship between age and hearing S ELF-REPORT MEASURES status. As Divenyi and Haupt (1997) point out, overall speech under- Speech audiometry and self-report measures are ideally used as standing in babble noise, measured with tests such as the speech-in- complementary clinical tools as many speech audiometry results noise test (SPIN; Bilger et al, 1984), is more affected by peripheral show only a moderate correlation, at best, with self-reported hearing hearing impairment whereas tasks that involve spatial (source loca- diffi culties. This was illustrated by Matthews et al (1990) using the tion) or temporal processing (e.g. speech in reverberation) show a Hearing Handicap Inventory for the Elderly (HHIE) scale, which has stronger infl uence of age than hearing acuity. Helfer and Wilber been shown to be a robust and versatile inventory (Ventry & Wein- (1990) reported not only strong correlations between speech percep- stein, 1982; Mulrow et al, 1990). Using 64 participants between 52 tion and average high frequency pure-tone thresholds (at 2000, 3000, and 77 years, Matthews et al (1990) could explain only 15 – 22% of 4000, and 6000 Hz), but also a strong correlation between age and variance in HHIE scores using word recognition in quiet scores, and speech perception in reverberant conditions. However, with only 40% of the variance could be explained by either pure-tone thresh- eight participants in each hearing impairment / age group, the results olds at 1000 and 2000 Hz or SPIN results. These fi ndings may not be in this study should be interpreted with care. surprising as Weinstein and Ventry (1983), in validating the HHIE, Therefore, given that speech audiometry is deemed a clinical reported that only 14 – 20% of the variance in the self-report scores tool that refl ects more realistic listening conditions than pure-tone was accounted for by speech recognition in quiet results. It appears audiometry (Hall, 2008), yet results show large variability amongst that, at least in the case of the HHIE, pure-tone thresholds or SPIN individuals with mild hearing impairment, it seems that measures results, account for more variance than speech in quiet scores. other than standard pure-tone or speech audiometric testing are Kramer and her colleagues found 34% of variance on the intel- required to investigate the everyday hearing diffi culties experienced ligibility in quiet section of the Amsterdam Inventory for Auditory by an individual Indeed, as Granberg et al (2014) point out, while Disability and Handicap could be explained by speech reception activity limitations may be measured with speech perception tests, thresholds in quiet and 42% by speech reception thresholds in noise an individual ’ s participation restrictions are not, and participation (Kramer et al, 1996). A low 20% of the variance on the intelligibility Adults with mild hearing impairment 791

in noise section of the questionnaire could be explained by the speech in the other ear. The participants 3FAHL was less than 40 dB HL reception thresholds in noise, with speech reception thresholds in and the average better ear 3FAHL was 22.8 dB HL. While noting quiet not being signifi cantly correlated with that section. Kramer large individual variability, the study reported that participants with et al (1996) found pure-tone thresholds had lower correlations to mild bilateral hearing impairment reported the emotional impact of Amsterdam Inventory scores than any speech measure, contrary to their impairment to be irritability, feeling upset, and feeling left out Weinstein and Ventry (1983) fi ndings for the HHIE. of social groups. They experienced greater diffi culty than partici- In summary, current audiometric testing may be insuffi ciently pants with unilateral hearing impairment in social settings, such as sensitive and specifi c for those with a mild hearing impairment. restaurants and parties, and with the television and radio. Perhaps the answer lies in recognizing that common clinical tests The fi ndings reported by Espmark et al (2002) contrast markedly are appropriate in classifying or quantifying hearing function, but from those reported thus far in this section. Their study, conducted more complex auditory function tests are needed. Research in the in a city district in Sweden, measured quality of life using a heav- area of “ hidden hearing loss ” may also lead to diagnostic tests that ily modifi ed version of the Hearing Measurement Scale (Noble & are suffi ciently sensitive to assess neurodegeneration associated with Atherley, 1970) in a group of 154 adults between the age of 70 and common causes of acquired hearing impairment prior to any sign of 91. Participants were divided into three groups according to 4FAHL: threshold elevation or for those individuals with slight elevations (1) less than 30 dB HL (49%); (2) 30– 39 dB (22%), and (3) greater in thresholds but signifi cant hearing disability (Plack et al, 2014). than 40 dB HL (29%). Reported hearing diffi culties corresponded Combined with the use of self-report measures, such diagnostics with audiometric thresholds, although most participants reported no may lead to a clearer picture of an individual ’ s hearing function in or only mild quality of life impact due to their hearing. The differ- the real world. ence between the fi ndings of Espmark et al (2002) and those of, for example, Newman et al (1997) may be due to the average age of H EALTH-RELATED QUALITY OF LIFE the participants, being 78 and 48.7 years, respectively. Age effects Several studies have reported on the outcome of quality of life (i.e. decreasing hearing diffi culties with increasing age) have been measures with individuals with mild hearing impairment. Bess et al found in several studies investigating self-reported hearing disabil- (1991) investigated the activities and participation associated with ity, possibly a consequence of older adults having fewer communica- varying degrees of hearing impairment in a group of 152 adults over tion demands and being more accepting of their hearing impairment the age of 65 years. The authors used both the Sickness Impact Pro- (Gordon-Salant et al, 1994; Sindhusake et al, 2001). fi le (SIP; Bergner et al, 1981), a generic functional health measure, Similarly, Chia et al (2007) found their 478 participants with and the HHIE-S, a hearing-specifi c measure. Results from the SIP mild, bilateral hearing impairment to have no signifi cant difference showed the participants with a 3FAHL between 26 and 40 dB HL in in health-related quality of life compared to similar older adults with the better ear reported signifi cantly poorer physical and psychosocial no, or unilateral, hearing impairment. However, the measure used in functioning than those with average audiometric thresholds between this study was the 36-item Short-Form Health Survey (SF-36; Ware 0 and 16 dB HL. Participants with a 3FAHL of between 17 and 25 & Sherbourne, 1992), a generic measure of health functional status. dB HL reported signifi cant psychosocial, but little physical impact The SF-36 may be not be suffi ciently sensitive to hearing-related from their hearing impairment, compared to participants with aver- quality of life, particularly in the case of mild hearing impairment age thresholds between 0 and 16 dB HL. As the psychosocial scales or when using a small participant sample size (Hickson et al, 2008). of the SIP includes items about social interaction, communication, The use of an outcome measure specifi c to hearing and quality of and emotional behaviour, the conclusion can be reached that even life is likely to have shown greater effects of hearing impairment, with a slight hearing impairment of up to 25 dB HL, individuals a conclusion reached by Stark and Hickson (2004), who used both experience activity limitations and participation restrictions. the hearing-specifi c HHIE questionnaire and the SF-36 with their

Downloaded by [UQ Library] at 14:59 22 November 2015 In one of the few studies limited to participants with mild hearing sample of 93 older adults. impairment, Scherer and Frisina (1998) reported on two groups of In summarizing the research literature concerning the impact of adults between the ages of 60 and 81 years, representative of the gen- acquired mild hearing impairment, there is suffi cient evidence to eral population; one group of 20 with average audiometric thresholds indicate that while the impact on individuals varies, it can be con- between 5.3 to 16.3 dB HL over the range of 250 to 4000 Hz, and siderable, leading to noticeable activity limitations and participation another with acquired mild hearing impairment with audiometric restrictions. Self-report measures focusing on hearing and communi- thresholds ranging from 12.8 to 41.3 dB HL over the same frequency cation appear most sensitive to the experiences of individuals with range. The average hearing impairment at 1000, 2000, and 4000 Hz mild hearing impairment. As the most common audiological inter- of the fi rst group was 10.7 dB HL, and 25.4 dB HL for the second vention for acquired hearing impairment is the provision of hearing group. Participants were otherwise matched on health and cognitive aids, it is of interest to examine the evidence about whether or not status. Speech tests revealed the group with mild hearing impair- hearing aids mitigate the impact of mild hearing impairment. ment showed signifi cantly worse speech understanding in both quiet and noise. This group also reported more activity limitations and participation restrictions as a result of their hearing impairment on Do hearing aids meet the needs of adults with mild hearing The Communication Profi le for the Hearing Impaired (Demorest & impairment? Erdman, 1987), compared to the group of participants with normal In answering the question if hearing aids are meeting the needs of audiometric thresholds. those with mild hearing impairment, it is important to defi ne what is Newman et al (1997) utilized the Hearing Handicap Inventory for meant by ‘ meeting a need ’ . This review found a number of studies Adults (Newman et al, 1990) to assess 63 adults between the age using quality of life improvements to defi ne successful hearing-aid of 18 and 64 years, with a mild bilateral hearing impairment, or a outcomes (Mulrow et al, 1990; Chisolm et al, 2007); these will be mild unilateral hearing impairment with normal hearing thresholds discussed later in this section. Other relevant studies have measured 792 B.H.B. Timmer et al.

hearing-aid outcomes in terms of use, benefi t and/or satisfaction with Academy of Audiology (AAA) Task Force on the Health-Related devices. Although some studies have shown a relationship between Quality of Life Benefi ts of Amplifi cation in Adults found 16 relevant the three concepts (Dillon et al, 1999; Joore et al, 2002; Abdel- studies (Chisolm et al, 2007). In all studies, the participants had laoui & Huy, 2013), others reported no such relationship between an average age of over 60 years, and a mild to moderate degree of hearing-aid use, benefi t, and satisfaction (Hutton & Canahl, 1985) hearing impairment. Chisolm et al (2007) reported generic measures and some report a correlation between two of the three constructs. of quality of life, such as the SF-36, showed negligible effect, when For example, Kricos (1997) and Salomon et al (1988) found a strong comparing pre-fi tting and post-fi tting results, whereas measures spe- relationship between hearing-aid use and satisfaction, although it is cifi c to hearing, for example the HHIE, showed hearing aids could unclear which is cause and which is effect. In fact, the consensus is bring about signifi cant improvements in participants ’ activities and that use, benefi t, and satisfaction are separate constructs and should participation. Care should be taken in interpreting these results for be measured as such (Cox et al, 1991; Dillon et al, 1997). adults with mild hearing impairment, however, as the authors did Just as the relationship between hearing-aid use, benefi t, and sat- not report the intervention improvements specifi cally to degree of isfaction may not be clear, a further diffi cult aspect of reviewing the hearing impairment. available research literature in this context is that few studies have focussed on outcomes of hearing-aid fi tting for individuals with a H EARING HELP-SEEKING mild hearing impairment only. One of the limitations in reviewing the scientifi c literature assessing hearing-aid benefi t for participants with mild hearing impairment is D EGREE OF HEARING IMPAIRMENT that these studies include only those who sought help through ampli- Most of the studies reviewed for this paper included participants with fi cation; many with a mild degree of hearing impairment may not hearing impairment varying from mild to moderately severe and the seek help. This suggests that participants in these studies experienced majority report that there is no association between satisfaction or more activity limitations as a result of their hearing impairment than benefi t and degree of hearing impairment (Scherr et al, 1983; Dil- others with a similar mild impairment, and would therefore have the lon et al, 1997; Brooks & Hallam, 1998). For example, in a study potential to obtain greater benefi t from hearing aids. Rather than of hearing-aid outcomes of 98 adults with average 3FAHL between using self-report measures, Davis (2003) used objective speech tests 10 and 60 dB HL, Dillon et al (1997) concluded that “ the use of as indicators of benefi t. Defi ning benefi t as a difference in speech average hearing loss as a predictor of likely benefi t appears to be test scores, Davis (2003) used the Four Alternate Auditory Feature totally without support ” (p. 40). (FAAF; Foster & Haggard, 1987) test under headphones, simulat- This was also a conclusion reached by Mulrow et al (1990), who ing no amplifi cation and amplifi cation with 343 adults. Using these interviewed 188 older adults (average age of 72 years), 70% of results as the basis for extrapolation, he calculated that 20% of adults whom had a mild hearing impairment, defi ned as a 3FAHL (500, between 55 and 74 years with a 4FAHL in the worse ear between 25 1000, and 2000 Hz) between 0 and 40 dB HL in the better ear. Par- and 29 dB HL could benefi t from amplifi cation. The percentage of ticipants were randomly assigned to either a hearing-aid fi tting group people predicted to benefi t from amplifi cation increased to 60% of or to a waiting list and a variety of hearing-specifi c (e.g. HHIE) adults with a 4FAHL in the worse ear between 30 and 34 dB HL, and and generic outcome measures (e.g. Self-evaluation of Life Func- to 70% of adults with a 4FAHL between 35 and 39 dB HL. Interest- tion) were administered pre- and post-fi tting. The baseline measures ingly, the percentage of adults with hearing impairment of greater showed 63% of all participants reported severe activity limitations than 40 dB HL who could expect benefi t stayed at an average of 70% and participation restrictions due to hearing impairment and 20% as 4FAHL increased. Therefore, based on this predictive model, the reported mild to moderate effects. Communication diffi culties were proportion of adults with mild hearing impairment who could benefi t rated as moderate by 85% and depression was noted in 23%. Post- from amplifi cation was similar to individuals with greater degrees

Downloaded by [UQ Library] at 14:59 22 November 2015 fi tting, the hearing aid group showed signifi cant improvements in of impairment (Davis, 2003). self-reported activities and participation, as well as depression, com- It seems therefore that there is suffi cient evidence that hearing aids pared to the waiting list group. These changes were not correlated can meet some of the needs of individuals with mild hearing impair- with degree of hearing impairment, suggesting that those with mild ment, but is subjective benefi t correlated with the characteristics of impairment showed similar benefi ts to those with greater degrees the hearing aid, such as style or technology features? of impairment. Stark and Hickson (2004) did fi nd degree of hearing impairment H EARING-AID TECHNOLOGY to infl uence the degree of change in outcome scores pre- and post- Hickson et al (2014) found insertion gain, defi ned as the difference fi tting. The 93 participants with predominantly mild hearing impair- between real-ear unaided gain and real-ear aided gain, to be the only ment (67% with a 3FAHL in the better ear of up to 35 dB HL; 91% audiological factor signifi cantly correlated to successful hearing-aid with a 3FAHL up to 45 dB HL) reported signifi cant reductions in use, which was defi ned as self-reported benefi t and regular use. activity limitation and participation restrictions post-fi tting, as mea- A group of unsuccessful hearing-aid users (n ϭ 75) was compared sured by the HHIE, particularly for those with a 3FAHL of greater to a group of successful users (n ϭ 85) and the successful users had than 35 dB HL. The researchers suggest this could be attributed to insertion gain values closer to the NAL-NL1 target values. The style the better HHIE scores pre-fi tting for those with a milder degree of of the hearing aid did not predict hearing-aid outcome, whereas non- hearing impairment, and therefore less scope for further improve- audiological factors such as attitude to hearing aids, support from ment. HHIE scores also improved signifi cantly to a greater extent others, self-reported hearing handicap and hearing-aid self-effi cacy, for those who wore their hearing aids more than four hours per day, or an individual ’ s confi dence in their ability to use a hearing aid, compared to the participants who wore their hearing aids less than did. The relationships between specifi c hearing-aid features and out- 1one hour per day. comes were not investigated, although the authors noted that suc- In assessing the evidence of the impact of the provision of hear- cessful hearing-aid users reported greater self-effi cacy for advanced ing aids on quality of life, a systematic review by the American handling of the hearing aid than unsuccessful aid users. Adults with mild hearing impairment 793

Yueh et al (2001) reported a signifi cant relationship between microphones are benefi cial for speech understanding in noise, and hearing-aid technology and benefi t. In a randomized trial of differ- may be preferred in all listening situations. ent amplifi cation devices, the researchers assigned a total of 60 men As a whole, the research literature indicates that hearing aids between the age of 50 and 86 years to either a control group, or to have the potential to improve quality of life and provide satisfaction groups provided with assistive listening devices, nonprogrammable and benefi t for individuals with mild hearing impairment. There is omnidirectional microphone hearing aids or programmable direc- some evidence that more recent hearing-aid technologies can better tional microphone hearing aids. There was no signifi cant difference address the needs of those with mild hearing impairments, however in the pure-tone average hearing impairment between the groups, a few studies have shown deleterious hearing-aid effects in specifi c which ranged from 31.5 to 34.6 dB HL at 500, 1000, and 2000 Hz. listening situations, such as omnidirectional microphones in speech The two groups fi tted with hearing aids showed signifi cant improve- in noise conditions (Klemp & Dhar, 2008). Care should be taken that ment in HHIE scores compared to the control and assistive listening hearing aids address needs in both easy as well as diffi cult listen- device groups. These differences were also evident when partici- ing situations. Future research could explore the most appropriate pants rated their communication abilities using the Abbreviated Pro- hearing-aid characteristics for individuals with mild hearing impair- fi le of Hearing Aid Benefi t (APHAB; Cox & Alexander, 1995) and ment when in quiet situations, as well as in noisier environments. the revised Denver Scale of Communication Function (Tuley et al, 1990). Comparing the two hearing-aid groups, the participants with Conclusion programmable directional hearing aids reported greater improvement in HHIE scores than those with non-programmable omnidi- This review has highlighted that the research literature concerning rectional hearing aids. Although randomized to intervention groups, mild hearing impairment shows large individual variability in many the participants were not blinded to the type of hearing aid they were aspects. Although there is no universal pure-tone audiometric defi ni- provided, which may have introduced bias in their self-report scores. tion of mild hearing impairment, it is easy to apply a defi nition in In a double-blind study by Gnewikow et al (2009), directional micro- terms of frequency and threshold boundaries, but the evidence shows phones were also seen to provide benefi t using both subjective and that this does not go any way to revealing the true impact of mild objective measures. Their 32 participants with mild hearing impair- hearing impairment on an individual. Unfortunately other audiomet- ment showed signifi cantly better speech in noise scores, using the ric measures such as speech testing do not appear to add much more Hearing-in-Noise Test (HINT; Nilsson et al, 1994), in the directional information for clinicians who regularly see this population in their compared to the omnidirectional condition. clinics. It is therefore important to keep in mind that the best indi- While the Yueh et al (2001) and Gnewikow et al (2009) studies cators of mild hearing impairment are the daily experiences of the concluded hearing-aid features, specifi cally directional microphones, individuals, which also vary widely. Some clients will report few can improve speech in noise scores and thereby provide signifi cant activity limitations and participation restrictions while others experi- benefi t, these improvements may not be limited only to individu- ence severe impact on daily activity and participation, particularly in als with mild hearing impairment. Gnewikow et al (2009) showed social settings and other challenging listening environments. There the degree of benefi t obtained from directional microphones was not is a need for greater understanding of the diffi culties associated with related to the degree of hearing impairment, although a non-signifi cant mild hearing impairment as well as further investigation of the most trend for subjective preference for directional microphones in quiet appropriate measures of such diffi culties. Research should aim to situations was noted for the mild hearing impairment group. understand the context of the listening environments and tasks for A number of studies have suggested a limiting factor to success- this clinical population, with a view to developing guidelines for ful fi ttings for clients with mild hearing impairment is the fact that clinicians faced with determining intervention candidacy. they experience either occlusion or feedback in different listening The most common intervention, the provision of hearing aids, can potentially address the needs of individuals with mild hearing Downloaded by [UQ Library] at 14:59 22 November 2015 situations before benefi t can be achieved (Chung, 2004; Valente & Mispagel, 2008). More recently open-fi t hearing aids and advances impairment although this review found a distinct lack of higher level in feedback cancellation systems may mean that such limitations research evidence, such as randomized controlled trials, demonstrat- have been addressed and appropriate amplifi cation can be provided ing hearing-aid benefi t for this clinical population. It is hoped that without feedback or occlusion (Flynn, 2004; Kuk & Keenan, 2005; modern hearing-aid advancements can better address the needs of Klemp & Dhar, 2008; Valente & Mispagel, 2008). people with mild degrees of impairment. Future research could In assessing the benefi ts of open-fi t hearing aids, the Klemp and focus on appropriate amplifi cation, signal processing strategies and Dhar (2008) study of 16 older adults with a mean 4FAHL of 34 handling aspects designed specifi cally for people with mild hearing dB HL showed improved speech in noise understanding, using the impairments, ensuring benefi t in daily life and in as many listening HINT, when the hearing aids were in the directional mode compared situations as possible. to the omnidirectional mode. In fact, speech perception in the omni- While this review focused on the provision of hearing aids as it is directional mode was worse than unaided scores. Valente and Mispagel the most common intervention strategy for individuals with hearing (2008) used both speech in noise (HINT) and subjective benefi t mea- impairment, other rehabilitation strategies may be equally benefi cial sures (APHAB) with 26 new hearing-aid wearers with, on average, for those with mild hearing impairment. Options include telephone mild hearing impairment. Although speech in noise scores were not amplifi cation, personal sound amplifi cation products, auditory train- signifi cantly better with the open-fi t hearing aid in the omnidirec- ing, or educational programs. Indeed, the uptake of these alternative tional microphone mode compared to unaided, the speech scores in strategies may indicate a phase or continuum of intervention options the directional mode, as well as the scores on the APHAB, were for those with mild hearing impairment, with the adoption of hear- signifi cantly better than unaided. In summary, the evidence about ing aids being a later phase. In terms of candidacy and choosing hearing-aid feature benefi ts for people with mild hearing impairment appropriate intervention solutions, clinicians are likely to fi nd their is limited, with a small number of studies suggesting that directional patients ’ self-reported hearing disability to be a more effective guide 794 B.H.B. Timmer et al.

in decision making than any current audiometric testing (Laplante- Davis A . 2003 . Population study of the ability to benefi t from amplifi cation L é vesque et al, 2012). and the provision of a hearing aid in 55 – 74-year-old fi rst-time hearing-aid In all, the development of more appropriate audiological assess- users . Int J Audiol , 42 , 39 – 52 . ment in the clinic, and further evaluation of the real-world listen- Demorest M.E. & Erdman S.A . 1987 . Development of the communication ing needs and performance of people with mild hearing impairment profi le for the hearing impaired . J Speech Hear Disord , 52 , 129 – 143 . Dillon H. , Birtles G. & Lovegrove R . 1999 . Measuring outcomes of a is required in order to provide a more effective pathway for this national rehabilitation program: Normative data for the Client Oriented clinical population. Scale of Improvement (COSI) and the Hearing Aid User ʼ s Questionnaire (HAUQ) . J Am Acad Audiol , 10 , 67 – 79 . Acknowledgements Dillon H. , James A. & Ginis J . 1997 . The Client Oriented Scale of Improve- ment (COSI) and its relationship to several other measures of benefi t and This project was part of a PhD studentship for the fi rst author funded satisfaction provided by hearing aids . J Am Acad Audiol , 8 , 27 – 43 . by Phonak AG, St ä fa, Switzerland. Divenyi P.L. & Haupt K.M . 1997 . Audiological correlates of speech understanding defi cits in elderly listeners with mild-to-moderate hearing loss. I. Age and lateral asymmetry. Ear Hear , 18 , 42 – 61 . Declaration of interest: The authors report no confl ict of interest. Dubno J.R. , Dirks D.D. & Morgan D.E . 1984 . Effects of age and mild hearing loss on speech recognition in noise . J Acoust Soc Am , 76 , 87 – 96 . Duijvestijn J.A. , Anteunis L.J.C. , Hendriks J.J.T. & Manni J.J . 1999 . Defi ni- References tion of hearing impairment and its effect on prevalence fi gures: A survey Abdellaoui A. & Huy P.T.B . 2013 . Success and failure factors for hearing-aid among senior citizens . Acta Otolaryngol , 119 , 420 – 423 . prescription: Results of a French national survey . Eur Ann Otorhinolar- Duquesnoy A.J . 1983 . The intelligibility of sentences in quiet and in noise in yngol Head Neck Dis , 130 , 313 – 319 . aged listeners . J Acoust Soc Am , 74 , 1136 – 1144 . Abrams H. , Chisolm T.H. & McArdle R . 2002 . A cost-utility analysis of adult Espmark A.-K.K. , Rosenhall U. , Erlandsson S. & Steen B . 2002 . The two group audiologic rehabilitation: Are the benefi ts worth the cost? J Rehabil faces of presbyacusis: Hearing impairment and psychosocial conse- Res Dev , 39 , 549 – 558 . quences . Int J Audiol , 41 , 125 – 135 . Bentler R. & Wu Y.-H . 2014 . Hearing aid technology and the importance Flynn M . 2004 . Maintaining the directional advantage in open fi ttings . Hear of verifi cation . In: J.J. Montano & J.B. Spitzer (eds.) Adult Audiologic Rev , 11 , 32 – 36 . Rehabilitation . San Diego, USA: Plural Publishing , pp. 137 – 156 . Foster J.R. & Haggard M.P . 1987 . The Four Alternative Auditory Feature Bergner M. , Bobbitt R.A. , Carter W.B. & Gilson B.S . 1981 . The sickness test (FAAF): linguistic and psychometric properties of the material with impact profi le: Development and fi nal revision of a health status measure . normative data in noise . Br J Audiol , 21 , 165 – 174 . Med Care , 19 , 787 – 805 . Gagn é J.-P. , Jennings M.B. & Southall K . 2009 . The ICF: A classifi cation Bess F.H. , Lichtenstein M.J. & Logan S.A. 1991 . Making hearing impairment system and conceptual framework ideal for audiological rehabilitation. functionally relevant: Linkages with hearing disability and handicap . Perspect Aural Rehab Instr , 16 , 8 – 14 . Acta Otolaryngol , 111 , 226 – 231 . Gnewikow D. , Ricketts T. , Bratt G.W. & Mutchler L.C . 2009 . Real-world Bilger R.C. , Nuetzel J.M. , Rabinowitz W.M. & Rzeczkowski C . 1984 . Standard- benefi t from directional microphone hearing aids . J Rehabil Res Dev , ization of a test of speech perception in noise . J Speech Hear Res , 27 , 32 – 48 . 46 , 603 – 618 . Brooks D.N. & Hallam R.S . 1998 . Attitudes to hearing diffi culty and hearing Gordon-Salant S. , Lantz J. & Fitzgibbons P . 1994 . Age effects on measures aids and the outcome of audiological rehabilitation. Br J Audiol , 32 , of hearing disability . Ear Hear , 15 , 262 – 265 . 217 – 226 . Granberg S. , Dahlstr ö m J. , M ö ller C. , K ä h ä ri K. & Danermark B . 2014 . The Carhart R . 1945 . An improved method for classifying audiograms . Laryngo- ICF core sets for hearing loss: Researcher perspective. Part I: Systematic scope , 55 , 640 – 662 . review of outcome measures identifi ed in audiological research. Int J Chia E.M. , Wang J.J. , Rochtchina E. , Cumming R.R. , Newall P. et al . 2007 . Audiol , 53 , 65 – 76 . Hearing impairment and health-related quality of life: The Blue Moun- Grant M.J. & Booth A . 2009 . A typology of reviews: An analysis of 14

Downloaded by [UQ Library] at 14:59 22 November 2015 tains Hearing Study . Ear Hear , 28 , 187 – 195 . review types and associated methodologies. Health Information and Li- Chisolm T.H. , Johnson C.E. , Danhauer J.L. , Portz L.J. , Abrams H.B. et al . braries Journal , 26 , 91 – 108 . 2007 . A systematic review of health-related quality of life and hearing Guild S.R . 1932 . A method for classifying audiograms . Laryngoscope , 42 , aids: Final report of the American Academy of Audiology task force on 821 – 836 . the health-related quality of life benefi ts of amplifi cation in adults. J Am Hall J.W. III , 2008 . Diagnostic applications of speech audiometry . Seminars Acad Audiol , 18 , 151 – 183 . in Hearing , 4 , 179 – 203 . Chung K . 2004 . Challenges and recent developments in hearing aids . Helfer K.S. & Freyman R.L . 2008 . Aging and speech-on-speech masking . Part II. Feedback and occlusion effect reduction strategies, laser shell Ear Hear , 29 , 87 – 98 . manufacturing processes, and other signal processing technologies. Helfer K.S. & Wilber L.A . 1990 . Hearing loss, aging, and speech perception Trends Amplif , 8 , 125 – 164 . in reverberation and noise . J Speech Hear Res , 33 , 149 – 155 . Clark J.G . 1981 . Uses and abuses of hearing loss classifi cation . ASHA , 23 , Henshaw H. & Ferguson M.A . 2013 . Effi cacy of individual computer-based 493 – 500 . auditory training for people with hearing loss: A systematic review of the Cox R.M. & Alexander G.C . 1995 . The Abbreviated Profi le of Hearing Aid evidence . PLoS ONE , 8 , e62836 . Benefi t . Ear Hear , 16 , 176 – 186 . Hickson L. , Allen J. , Beswick R. , Fulton M. , Wolf D. et al . 2008 . Relation- Cox R.M. , Gilmore C. & Alexander G.C . 1991 . Comparison of two ques- ships between hearing disability, quality of life, and wellbeing in older tionnaires for patient-assessed hearing-aid benefi t . J Am Acad Audiol , community-based Australians . The Australian and New Zealand Journal 2 , 134 – 145 . of Audiology , 30 , 99 – 111 . Cruickshanks K.J. , Wiley T.L. , Tweed T.S. , Klein B.E. , Klein R. et al . 1998 . Hickson L. , Meyer C. , Lovelock K. , Lampert M. & Khan A . 2014 . An inves- Prevalence of hearing loss in older adults in Beaver Dam, Wisconsin. The tigation of factors that infl uence help-seeking for hearing impairment in Epidemiology of Hearing Loss Study. Am J Epidemiol , 148 , 879 – 886 . older adults Int J Audiol , pp. 1 – 15 . Dalton D.S. , Cruickshanks K.J. , Klein B.E. , Klein R. , Wiley T.L. et al . 2003 . Hickson L. & Scarinci N . 2007 . Older adults with acquired hearing The impact of hearing loss on quality of life in older adults. Gerontolo- impairment: Applying the ICF in rehabilitation . Semin Speech Lang , 28 , gist , 43 , 661 – 668 . 283 – 290 . Adults with mild hearing impairment 795

Hickson L. , Worrall L. & Scarinci N . 2007 . A randomized controlled trial Nilsson M. , Soli S.D. & Sullivan J.A . 1994 . Development of the hearing in evaluating the active communication education program for older people noise test for the measurement of speech reception thresholds in quiet with hearing impairment . Ear Hear , 28 , 212 – 230 . and in noise . J Acoust Soc Am , 95 , 1085 – 1099 . Humes L.E. , Burk M.H. , Strauser L.E. & Kinney D.L . 2009 . Development Noble W.G. & Atherley G.R.C . 1970 . The hearing measure scale: A question- and effi cacy of a frequent-word auditory training protocol for older naire for the assessment of auditory disability . J Aud Res , 10 , 229 – 250 . adults with impaired hearing . Ear Hear , 30 , 613 – 627 . Plack C.J. , Barker D. & Prendergast G . 2014 . Perceptual consequences of Humes L.E. & Krull V . 2012 . Hearing aids for adults . In: L. Wong & L. “ hidden ” hearing loss . Trends in Hearing , 18 , 1 – 11 . Hickson (eds.) , Evidence-based Practice in Audiology: Evaluating Salomon G. , Vesterager V. & Jagd M . 1988 . Age-related hearing diffi culties: Interventions for Children and Adults with Hearing Impairment . I. Hearing impairment, disability, and handicap: A controlled study. Int San Diego, USA: Plural Publishing , pp. 61 – 92 . J Audiol , 27 , 164 – 178 . Hutton C.L. & Canahl J.A . 1985 . Scaling patient reports of hearing aid Salonen J. , Johansson R. , Karjalainen S. , Vahlberg T. & Isoaho R . 2011 . benefi t . J Aud Res , 25 , 255 – 269 . Relationship between self-reported hearing and measured hearing im- Joore M.A. , Potjewijd J. , Timmerman A.A. & Anteunis L.J.C . 2002 . pairment in an elderly population in Finland . Int J Audiol , 50 , 297 – 302 . Response shift in the measurement of quality of life in hearing impaired Saunders G.H. , Chisolm T.H. & Abrams H.B . 2005 . Measuring hearing aid adults after hearing aid fi tting . Qual Life Res , 11 , 299 – 307 . outcomes: Not as easy as it seems . J Rehabil Res Dev , 42 , 157 – 168 . Kiely K.M. , Gopinath B. , Mitchell P. , Luszcz M. & Anstey K.J . 2012 . Cogni- Scherer M.J. & Frisina R . 1998 . Characteristics associated with marginal tive, health, and sociodemographic predictors of longitudinal decline in hearing loss and subjective well-being among a sample of older adults . hearing acuity among older adults . The Journals of Gerontology Series J Rehabil Res Dev , 35 , 420 – 426 . A: Biological Sciences and Medical Sciences , 67 , 997 – 1003 . Scherr C.K. , Schwartz D.M. & Montgomery A.A . 1983 . Follow-up survey of Klemp E.J. & Dhar S . 2008 . Speech perception in noise using directional mi- new hearing aid users . JARA , 16 , 202 – 209 . crophones in open-canal hearing aids . J Am Acad Audiol , 19 , 571 – 578 . Sindhusake D. , Mitchell P. , Smith W. , Golding M., Newall P. et al . 2001 . Kochkin S . 2012 . MarkeTrak VIII: The key infl uencing factors in hearing aid Validation of self-reported hearing loss: The Blue Mountains Hearing purchase intent . Hear Rev , 19 , 12 – 25 . Study . Int J Epidemiol , 30 , 1371 – 1378 . Kramer S.E. , Kapteyn T.S. , Festen J.M. & Tobi H . 1996 . The relationships Smoorenburg G.F . 1992 . Speech reception in quiet and in noisy conditions between self-reported hearing disability and measurements of auditory by individuals with noise-induced hearing loss in relation to their tone disability . Audiology , 35 , 277 – 287 . audiogram . J Acoust Soc Am , 91 , 421 – 437 . Kricos P.B . 1997 . Audiologic rehabilitation for the elderly: A collaborative Sprinzl G.M. & Riechelmann H . 2010 . Current trends in treating hearing loss approach . The Hearing Journal , 50 , 10 – 19 . in elderly people: A review of the technology and treatment options: A Kujawa S.G. & Liberman M.C . 2009 . Adding insult to injury: Cochlear nerve mini-review . Gerontology , 56 , 351 – 358 . degeneration after “ temporary ” noise - induced hearing loss . J Neurosci , Stark P. & Hickson L . 2004 . Outcomes of hearing-aid fi tting for older people with 29 , 14077 – 14085 . hearing impairment and their signifi cant others . Int J Audiol, 43 , 390 – 398 . Kuk F. & Keenan D . 2005 . Effi cacy of an open-fi tting hearing aid . Hear Rev , Stevens G. , Flaxman S. , Brunskill E. , Mascarenhas M. , Mathers C.D. et al . 12 , 26 – 32 . 2013 . Global and regional hearing impairment prevalence: An analysis Laplante-L é vesque A. , Hickson L. & Worrall L . 2012 . What makes adults of 42 studies in 29 countries . Eur J Public Health , 23 , 146 – 152 . with hearing impairment take up hearing aids or communication programs Sweetow R.W. & Sabes J.H . 2006 . The need for and development of an adap- and achieve successful outcomes? Ear Hear , 33 , 79 – 93 . tive listening and communication enhancement (LACE) program . J Am Laplante-Lé vesque A. , Hickson L. & Worrall L . 2011 . Predictors of rehabili- Acad Audiol , 17 , 538 – 558 . tation intervention decisions in adults with acquired hearing impairment . Tuley M.R. , Mulrow C.D. , Aguilar C. & Velez R . 1990 . A critical reevalu- J Speech Lang Hear Res , 54 , 1385 – 1399 . ation of the Quantifi ed Denver Scale of Communication Function . Ear Laplante-L é vesque A. , Knudsen L.V. , Preminger J.E. , Jones L. , Nielsen C. Hear , 11 , 56 – 61 . et al . 2012 . Hearing help-seeking and rehabilitation: Perspectives of Uimonen S. , M ä ki-Torkko E. , Jounio-Ervasti K. & Sorri M . 1997 . Hearing in 55 to adults with hearing impairment . Int J Audiol , 51 , 93 – 102 . 75 year old population in northern Finland. A comparision of two classifi ca- Lee F.-S. , Matthews L.J. , Dubno J.R. & Mills J.H . 2005 . Longitudinal study tions of hearing impairment. Acta Otolaryngol Suppl (Stockh) , 529 , 69 – 70 .

Downloaded by [UQ Library] at 14:59 22 November 2015 of pure-tone thresholds in older persons . Ear Hear , 26 , 1 – 11 . Valente M. & Mispagel K.M . 2008 . Unaided and aided performance with a Lichtenstein M.J. , Bess F.H. & Logan S.A . 1988 . Diagnostic performance directional open-fi t hearing aid . Int J Audiol , 47 , 329 – 336 . of the hearing handicap inventory for the elderly (screening version) Ventry I. & Weinstein B . 1982 . The Hearing Handicap Inventory for the El- against differing defi nitions of hearing loss . Ear Hear , 9 , 208 – 211 . derly: A new tool . Ear Hear , 3 , 128 – 134 . Lin F.R. , Yaffe K. , Xia J. , Xue Q.-L. , Harris T.B. et al . 2013 . Hearing loss Ware J.E. & Sherbourne C.D . 1992 . The MOS 36-item Short-Form Health and cognitive decline in older adults . JAMA Intern Med , 173 , 293 – 299 . Survey (SF-36): I. conceptual framework and item selection . Med Care , Lutman M. , Brown E.J. & Coles R.R.A . 1987 . Self-reported disability and 30 , 473 – 483 . handicap in the population in relation to pure-tone threshold, age, sex Weinstein B.E. & Ventry I.M . 1983 . Audiometric correlates of the hearing and type of hearing loss . Br J Audiol , 21 , 45 – 58 . handicap inventory for the elderly . J Speech Hear Disord , 48 , 379 – 384 . Matthews L.J. , Lee F.-S. , Mills J.H. & Schum D.J . 1990 . Audiometric and Wong L.L.N. & Hickson L. (eds.). 2012 . Evidence-based Practice in Audi- subjective assessment of hearing handicap. Arch Otolaryngol Head Neck ology: Evaluating Interventions for Children and Adults with Hearing Surg , 116 , 1325 – 1330 . Impairment . San Diego: Plural Publishing . Mulrow C.D. , Aguilar C. , Endicott J.E. , Tuley M.R., Velez R. et al . 1990 . World Health Organization 2001 . International Classifi cation of Functioning, Quality-of-life changes and hearing impairment. A randomized trial. Disability, and Health: ICF . Geneva: WHO . Ann Intern Med , 113 , 188 – 194 . World Health Organization . 2004 . The Global Burden of Disease . Geneva: Mulrow C.D. , Tuley M.R. & Aguilar C . 1990 . Discriminating and responsive- WHO . ness abilities of two hearing handicap scales . Ear Hear , 11 , 176 – 180 . World Health Organization . 2011 . Global Health and Aging . Geneva: WHO . Newman C.W. , Jacobson G.P. , Hug G.A. & Sandridge S.A . 1997 . Perceived World Health Organization. 2012 . WHO global estimates on prevalence of hearing handicap of patients with unilateral or mild hearing loss . Ann hearing loss: Mortality and burden of diseases and prevention of blind- Otol Rhinol Laryngol , 106 , 210 – 214 . ness and deafness . Geneva: WHO . Newman C.W. , Weinstein B.E. , Jacobson G.P. & Hug G.A . 1990. The Hearing Yueh B. , Souza P.E. , McDowell J.A. , Collins M.P. , Loovis C.F. et al . 2001 . Handicap Inventory for Adults: Psychometric adequacy and audiometric Randomized trial of amplifi cation strategies. Arch Otolaryngol Head correlates . Ear Hear , 11 , 430 – 433 . Neck Surg , 127 , 1197 . The Use of Listening Devices to Ameliorate Auditory Deficit in Children with Autism

Gary Rance, BEd, DipAud, MSc, PhD1, Kerryn Saunders, MBBS(Hons), RACP2, Peter Carew, BSc, MAud1, Marlin Johansson, BSc, MAud3, and Johanna Tan, BSc, MAud1

Objectives To evaluate both monaural and binaural processing skills in a group of children with autism spectrum disorder (ASD) and to determine the degree to which personal frequency modulation (radio transmission) (FM) listening systems could ameliorate their listening difﬁculties. Study design Auditory temporal processing (amplitude modulation detection), spatial listening (integration of binaural difference cues), and functional hearing (speech perception in background noise) were evaluated in 20 children with ASD. Ten of these subsequently underwent a 6-week device trial in which they wore the FM system for up to 7 hours per day. Results Auditory temporal processing and spatial listening ability were poorer in subjects with ASD than in matched controls (temporal: P = .014 [95% CI À6.4 to À0.8 dB], spatial: P = .003 [1.0 to 4.4 dB]), and performance on both of these basic processing measures was correlated with speech perception ability (temporal: r = À0.44, P = .022; spatial: r = À0.50, P = .015). The provision of FM listening systems resulted in improved discrimination of speech in noise (P < .001 [11.6% to 21.7%]). Furthermore, both participant and teacher questionnaire data revealed device-related beneﬁts across a range of evaluation categories including Effect of Background Noise (P = .036 [À60.7% to À2.8%]) and Ease of Communication (P = .019 [À40.1% to À5.0%]). Eight of the 10 participants who undertook the 6-week device trial remained consistent FM users at study completion. Conclusions Sustained use of FM listening devices can enhance speech perception in noise, aid social interaction, and improve educational outcomes in children with ASD. (J Pediatr 2014;164:352-7).

typical responses to sensory stimuli across a number of modalities including the visual, tactile, and auditory are frequently reported and are now a mandatory component of autism spectrum disorder (ASD) diagnosis as specified A 1 in the Diagnostic and Statistical Manual of Mental Disorders–Version 5. Aberrant reactions to auditory stimuli were noted in Kanner’s original article on autism2 and recent evidence has suggested that a high proportion of affected children suffer auditory processing deficits that particularly disrupt the ability to hear and understand speech in the presence of background noise.3-5 Identification and appropriate management of hearing problems in this population are especially important as auditory deficits can, in themselves, restrict social and communication development.6 There are 2 main approaches to reducing functional hearing deficit in children with listening-in-noise (figure/ground) problems. The first is to improve the quality of the signal reaching the child’s ear, and the second is to train him/her to make the best use of that signal. In the case of the latter, results of training programs for children with ASD have not been reported, but there is a growing amount of literature suggesting that figure/ground deficit may be ameliorated through targeted auditory habilitation.7-9 Optimization of the listening environment (particularly in the school setting) is important and can be achieved through structural modifications (fitting of ceiling tiles, carpets, etc) and sound field (loudspeaker) amplification of the teacher’s voice.10 Another option is the use of personal frequency modulation (radio transmission) (FM) listening systems. These devices transmit speech signals (detected by a lapel microphone worn by the speaker/teacher) via radio waves to ear-level receivers worn by the child. As such, they improve the signal-to-noise ratio as the speech is louder, relative to the background noise at the child’s ear. FM devices have been used effectively to minimize listening problems for children with cochlear hearing loss,11 central auditory processing deficit,12 and auditory neuropathy.13 Furthermore, a recent pilot investigation by Schafer et al14 has shown listening benefits for

ASD children wearing FM systems for short periods in “structured” educational From the 1Department of Audiology and Speech situations. The sustained use of these devices in mainstream classroom environ- Pathology, The University of Melbourne; 2School of Medicine, Nursing and Health Sciences, Monash ments has, however, has not yet been explored in children with ASD. University, Melbourne, Australia; and 3Department of Audiology, School of Medicine, Lund University, Lund, Sweden Funded by the Jack Brockhoff Foundation, the Collier Foundation, and the Deafness Foundation. PHONAK.org APHAB Abbreviated Profile of Hearing Aid Benefit donated the FM devices. The authors declare no conflicts of interest. ASD Autism spectrum disorder FM Frequency modulation (radio transmission) 0022-3476/$ - see front matter. Copyright ª 2014 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2013.09.041

352 Vol. 164, No. 2 February 2014

Table. Characteristics of the study participants with ASD Average hearing Other ASD Subject Sex Age at assessment, y level, dBHL CARS evaluations WISC-IV (FSIQ) ASD1 Male 15.4 13.75 31 - 106 ASD2 Male 15.2 10.00 39 - 104 ASD3 Male 15.1 5.00 35 - 94 ASD4 Male 14.8 8.75 34 - 92 ASD5 Male 14.2 3.75 36 - 94 ASD6 Male 13.5 5.00 36 - 86 ASD7 Female 13.4 13.75 30 - 91 ASD8 Male 13.0 10.00 33 - 83 ASD9 Male 12.6 13.75 35.5 - 71 ASD10 Male 12.5 8.75 37.5 - - ASD11 Male 12.0 6.25 - BASC II/ADOS 95 ASD12 Male 11.7 10.00 32 - 89 ASD13 Female 11.6 15.00 36 - 72 ASD14 Male 11.4 8.75 35.5 - 84 ASD15 Male 11.2 9.25 27 99 ASD16 Male 10.0 10.00 - BASC III/ASSQ/CBCL 89 ASD17 Male 9.1 6.25 33.5 - - ASD18 Male 9.1 11.25 36.5 - - ASD19 Male 9.0 6.25 31 - 106 ASD20 Female 8.0 3.75 <30 CBCL/ADI-R -

Average hearing level, 4-frequency average hearing level based on sound detection obtained to pure tones at 500-, 1000-, and 4000-Hz test frequencies; HL, hearing level; CARS, Childhood Autism Rating Scale; WISC-IV (FSIQ), Wechsler Intelligence Scale for Children IV (Full-Scale IQ); BASC II/III, Behavioral Assessment System for Children II/III; ADOS, Autism Diagnostic Observation Schedule; ASSQ, Autism Spectrum Screening Questionairre; CBCL, Child Behavior Checklist; ADI-R, Autism Diagnostic Interview-Revised.

We conducted the present study to explore the hypothesis Profile of Hearing Aid Benefit (APHAB). This metric ex- that provision of FM listening devices would enhance speech plores 4 aspects of auditory function: communication diffi- perception in noise, aid communication, and improve culty, effect of background noise, effect of reverberation, educational outcomes for children with ASD. and aversiveness to sound. Participants then underwent a battery of auditory assessments designed to evaluate aspects Methods of basic auditory processing and functional hearing. Auditory temporal processing ability was established psy- Twenty children with ASD participated. The disorder was chophysically using the techniques described by Alcantara confirmed in each case via a multidisciplinary clinical assess- et al.4 Thresholds for detection of sinusoidal amplitude mod- ment using a range of instruments including the Autism Diag- ulation in a broadband noise were established for a range of nostic Interview15,16 and Childhood Autism Rating Scale17 modulation rates and plotted as a temporal modulation (Table). Only children with no known coexisting disabilities transfer function.19 Overall, sensitivity to modulation was es- and Full-Scale IQ, Wechsler Intelligence Scale for Children18 tablished by calculating a y-intercept value (reflecting the values >70 were referred for the study. Sound detection height of the function), and the temporal constant (t) was ability was normal in each case. All participants could speak derived from À3-dB knee point in the function. and understand/follow verbal instructions and all attended Binaural processing, the ability to effectively combine in- their local (mainstream) school. None of the children were puts from the left and right ears, was evaluated using the receiving academic in-class support at the time of the study. Listening in Spatialized Noise test.20 This test measured the Participant age at assessment ranged from 8.0 to 15.4 years listener’s capacity to use subtle interaural difference cues to with a mean Æ SD of 12.0 Æ 2.3 years. The 10 younger chil- improve sentence perception in background noise.21 dren (ASD11-20) were enrolled in primary-school settings, Open-set speech perception ability was evaluated using the and the 10 older participants (ASD1-10) were secondary- Consonant-Nucleus-Consonant-Word test.22 Testing was school students. carried out in the free field with the subject seated between A cohort of age- and sex-matched control participants was 2 loudspeakers. The front speaker presented a series of mono- also evaluated. Age at data collection was within 12 months of syllabic words calibrated to reach the child at 65 dB sound the ASD partner, and each control was screened to ensure pressure level, while a rear speaker provided noise (4-talker normal cognitive profile and to confirm that they were not babble) at the same level. The subject imitated the stimulus on the autism spectrum (Childhood Autism Rating Scale words, and a percentage of discriminated phonemes (speech score <20). sounds) was calculated by the examiner. Following this “un- The research protocol was approved by the Ethics Com- aided” assessment, the child was fitted with a Phonak Inspiro mittee of the Royal Victorian Eye and Ear Hospital, and writ- FM transmitter paired with iSense receivers (Phonak Org, ten informed consent was obtained for each participant. Each Murten, Switzerland). The FM receivers are small devices subject began by completing (alone or with parental assis- that sit behind each pinna and are held in place by a soft, tance) a hearing disability questionnaire—the Abbreviated vented rubber earpiece inserted into the ear canal. Once the

353 THE JOURNAL OF PEDIATRICS www.jpeds.com Vol. 164, No. 2

60 -20 Control (N = 20) ASD (N = 20)

50 -18 Control 40 -16 ASD

30 -14

-12 20 Mod. Depth 20logM (dB)

Perceived DifficultyPerceived (%) -10 10 1 10 100 1000

0 Modulation Rate (Hz) Communication* Noise* Reverberation* Aversiveness Global* Figure 2. Mean amplitude modulation detection thresholds Figure 1. Mean APHAB hearing disability survey results for (dB) plotted as a function of modulation rate (Hz) for the ASD 20 children with ASD and 20 controls. Error bars represent 1 group and controls. Error bars denote Æ1 SEM. SD from the mean. Asterisks show the metrics in which there was a significant difference between groups (P < .001). Mean Global APHAB score for the ASD group was 36.3% Æ 17.2%, and that for the control group was 7.6% Æ 6.6% (95% CI age and hearing level can influence listening ability, these fac- 19.3%-38.1%; P < .001). tors were also included in the analysis. No relation was found between Global APHAB score and either age (P = .060) or hearing level (P = .799), but subject group was a significant child was comfortable with the fitting, speech testing was factor (P < .001). repeated in the “FM-aided” condition with the transmitter microphone suspended 20 cm in front of the “speech” loud- Auditory Temporal Processing speaker to replicate device distance (from the teacher’s Fourteen children with ASD were able to reliably complete 13 mouth) in everyday use. the psychophysical temporal processing task. Detection of Day-to-day listening ability was investigated in the amplitude modulation in these subjects was significantly primary-school ASD group (n = 10) across a 6-week, take- impaired relative to matched controls. Figure 2 shows home device trial. A balanced design (ABBA) was used, mean modulation-depth thresholds for ASD and control involving 2-week periods of device use (B) and 2-week pe- groups. For children in both cohorts, the temporal riods of nonuse (A) to allow for learning/experience effects. modulation transfer function showed the typical low-pass During “device-on” phases, the children (and teachers/par- filter pattern (indicating that high-rate modulation was ents) were encouraged to wear the system throughout the more difficult to perceive than low-rate modulation), and day. Four to 6 hours of sustained use per day was typical. mean t values were equivalent (ASD 1.1 Æ 0.3 ms, control Subjects (with parent support as required) completed the 1.4 Æ 0.3 ms, 95% CI À2.8 to 122 ms; P = .061). Children APHAB questionnaire after each 2-week phase. At the with ASD were, however, consistently less sensitive to conclusion of the trial, the child’s teacher provided an evalu- amplitude variations over time, showing higher detection ation of classroom/educational benefit by completing the thresholds for each modulation rate. Mean y-intercept 23 Listening Inventory For Education questionnaire. value for the ASD group was À16.3 Æ 2.5 dB and that for The data were analyzed by the first author (G.R.) using the control group was À19.9 Æ 3.7 dB (95% CI À6.4 to paired t tests and multiple regression analyses unless other- À0.8 dB; P = .014). wise stated. Regression analysis found no relation between y-intercept value and hearing level (P = .200), but age at assessment (P = Results .006) and subject group (P = .001) were significant factors reflecting poorer sensitivity to temporal changes in the younger Hearing Disability Survey participants and those with ASD. The children with ASD in this study considered their everyday listening ability and conversational understanding Spatial Processing to be impaired in a wide range of circumstances. Mean Binaural processing was also impaired in the children with APHAB scores (representing the proportion of situations in ASD. Spatial advantage (Listening in Spatialized Noise test) which the individual perceived a difficulty) were significantly is the speech-in-noise improvement (in dB) obtained when higher in this group than in controls across a range of percep- the origin of a target signal is spatially separated from that tual and communication subscales (P < .001) (Figure 1). of a background noise.24 Mean spatial advantage for the Multiple regression analysis was used to look for associa- ASD group was 9.2 Æ 3.2 dB and that for the control group tions between APHAB result and subject group. As subject was 11.9 Æ 1.4 dB (95% CI 1.0-4.4 dB; P = .003). Regression

354 Rance et al February 2014 ORIGINAL ARTICLES

85.6% Æ 5.1% with a mean aided improvement of 16.9 Æ

unaided FM-aided 10.6% (95% CI 11.6%-21.7%; P < .001) (Figure 3, A). 100 Mean FM-aided score for the control group was 89.1% Æ )

% 4.0% and mean improvement was 10.1% Æ 7.0% (95% CI

( 80 e

r 6.9%-13.4%; P < .001). Multiple regression showed no o c

S relation between aided improvement and either age (P =

e 60 m

e .539) or hearing level (P = .425) but a signiﬁcant n o

h 40 correlation with subject group (P = .039), indicating that P

C participants with ASD had greater device beneﬁt than N

C 20 controls.

0 FM Device Trial 1 2 3 4 5 6 7 8 9 10 11 14 15 16 17 19 20 12 13 18 Eight of the 10 children with ASD who started the 6-week de- Mean Subject # vice trial were still consistent users at the end of the evalua- 60 tion period. The 2 who failed to complete (ASD13 and Unaided FM-aided ASD16) became nonusers in the ﬁrst 2-week phase and pro- 50 vided no data beyond the initial ﬁtting session.

40 Hearing Disability. For the children who completed the trial, FM device use afforded significant listening and 30 communication benefits. APHAB Global scores across the 4 data collection points were Unaided1: 38.6% Æ 18.4%, 20 Aided1: 17.0% Æ 5.0%, Aided2: 13.8% Æ 8.4%, and Un- aided2: 41.6% Æ 23.4%. ANOVA showed a significant point Perceived Difficulty (%) 10 difference (F3,28 = 6.73, P = .001). Post-hoc analysis found that both aided data points were significantly lower than 0 the unaided (P < .05). As no difference was obtained between Communication* Noise* Reverberation* Aversiveness Global* the 2 aided and 2 unaided points, the data were collapsed. Figure 3. A, Unaided and FM-aided Consonant-Nucleus- Figure 3, B shows mean unaided and FM-aided scores Consonant-Word scores for each of the children with ASD. across the 4 listening categories. Mean phoneme score for the unaided condition was 68.9% Æ 11.7%, and that for the aided condition was 85.6% Æ 5.1% Classroom Listening. The Listening Inventory For Edu- (95% CI À21.7% to À11.6%; P < .001). B, APHAB hearing cation questionnaire was completed by each teacher at the disability questionnaire results for unaided and aided condi- trial conclusion. This metric generates a Total Appraisal tions. Error bars represent 1 SEM. Asterisks represent the Score between À35 and +35, on which a negative value indi- metrics in which there was a significant difference between cates that classroom performance was adversely affected by P CNC conditions ( < .001). , Consonant-Nucleus-Consonant. the intervention and a positive value denotes a device- related improvement. The Total Appraisal Score for each analysis showed no relation between spatial advantage and child was in the positive range, reflecting a perceived either subject age (P = .192) or hearing level (P = .484) but improvement in listening/comprehension, classroom did reveal a significant correlation with subject group (P = behavior, and general attentiveness. Mean score for the group .001), indicating poorer binaural integration in participants was 24.2 Æ 8.2, indicating that the teachers considered the with ASD than in controls. FM device fitting to be “highly beneficial” for each of the children who completed the study. Open-Set Speech Discrimination Speech perception in noise was poorer in participants with Discussion ASD than in matched controls (P = .009). Results for each child with ASD are shown in Figure 3, A. Regression Children with ASD suffer auditory figure/ground problems analysis showed no relation between phoneme score and severe enough to exacerbate the communication deficits cen- either subject age (P = .722) or hearing level (P = .074) but tral to the disorder and to delay academic progress. The most did reveal a correlation with subject group (P = .008), significant predictor of educational performance in children indicating poorer functional hearing in participants with ASD. with ASD is their ability to understand speech and maintain concentration in the presence of background noise.25 The Open-Set Speech Perception (FM Aided). Children in mechanisms underlying figure/ground deficit in ASD are both subject groups showed significant speech perception yet to be fully determined. Coexisting disabilities, such as improvement when fit with the FM listening system. Mean attention deficit, language disorder, and intellectual impair- phoneme score for the children with ASD increased to ment (all relatively common in ASD populations), are likely

The Use of Listening Devices to Ameliorate Auditory Deficit in Children with Autism 355 THE JOURNAL OF PEDIATRICS www.jpeds.com Vol. 164, No. 2 to exhert an influence.3,26 Another limiting factor is the rep- was newly diagnosed at the time of the study and lacked fam- resentation of acoustic stimuli in the central auditory path- ily support. ways. Electrophysiologic investigation has shown neural FM device use afforded clear listening and communication conduction abnormalities extending from brainstem to cor- benefits for each of the trial participants. All reported fewer tex in ASD listeners,27-30 and recent psychophysical studies background noise problems and greater ease of social interac- have revealed specific processing deficits affecting the perception during aided phases of the protocol. Furthermore, it was tion of timing cues.3,4 Temporal processing was impaired in the opinion of the teachers that each ASD child showed the present study with most individuals showing reduced consistent improvement in comprehension, attentiveness, sensitivity to signal-envelope changes over time consistent and classroom behavior when wearing the device. with abnormality at the level of the inferior colliculus and/ This study represents an attempt to implement consistent or increased levels of neural noise.4 FM device use in a mainstream classroom context. There The link between auditory temporal deficit and figure/ were several limitations that can be addressed in future ground problems is well established.4,13 In the current study, research, First, while effect sizes were large and consistent temporal processing ability was strongly correlated with across participants, the trial involved a small number of sub- speech perception in noise, with those children showing the jects (N = 10). Second, the use of questionnaire data involves poorest sensitivity to temporal modulation also presenting the possibility of participant bias. We used a balanced trial with the poorest phoneme scores (r = À0.440, P = .022). design and found that survey results in the final weeks of Impaired temporal sensitivity can influence figure/ground the trial were similar to those in the initial phases, suggesting perception in a number of ways. Most obviously, it affects that “halo” effects were not an issue. Still, it is possible that “gap-listening,” the ability to use the brief, relatively quiet pe- subjective bias may have been a factor in the results. riods that occur in environmental noise to access the speech The psychophysical and subjective test methodologies signal.3 Furthermore, temporal abnormality can impair the involved in this study required that only “high-functioning” individual’s capacity to use spatial cues when listening in ASD children could participate. The efficacy of FM fitting in noise. In everyday listening, integration of subtle interaural children lower on the spectrum is not established. It is likely timing, level, and spectral differences allows the individual that ASD children with severe behavioral issues or coexisting to perceive a 3-dimensional auditory environment and to pre- disabilities also suffer auditory processing deficits, but the cisely judge sound direction. Sound localization cues may also practical difficulties associated with fitting a head-worn de- be used to improve speech perception in noise when the target vice in such cases may be prohibitive. Auditory intervention, signal and the background noise arise from different loca- as with all aspects of ASD management, needs to be consid- tions.19,31 In the current study, ASD children were signifi- ered on a case-by-case basis. cantly impaired in their ability to use these cues and, again, The findings of the present study confirm that hearing dif- degree of binaural disruption (ie, spatial advantage) was corre- ficulties are common in ASD. Children in this study were not lated with temporal processing ability (r = À0.500, P = .015). selected based on hearing history, yet 12 of 20 (60%) met the Despite clear evidence that FM listening systems can clinical definition of central auditory processing disorder, improve listening and educational outcomes in other scoring >2 SDs poorer than age-related means on temporal hearing-impaired populations, these systems have not yet processing and/or speech-in-noise tasks. The use of FM been explored as an intervention option for children with listening devices ameliorated these auditory processing prob- ASD, perhaps reflecting concerns about device tolerance in lems, resulting in listening, attention, and social interaction cases with intellectual impairment and/or tactile defensive- improvements, and appear to be a viable auditory manage- ness. Schafer et al14 did carry out a limited trial that showed ment option for some children with ASD. n improvements in brief (45-minute), structured listening sessions. Our study evaluated sustained FM use in the main- We gratefully acknowledge the contribution of all of the children, fam- stream school classroom. ilies, and teachers who participated in this study, in particular Ms Na- The signal-to-noise ratio advantage provided by the FM talie Rule and the staff at Hampton Park Secondary College. Thanks also to Dr Jose Alcantara and Professor Colette McKay who contributed listening device resulted in clear perceptual benefits. While to the study design/analysis. unaided speech perception for many children with ASD was severely depressed (with 10/20 scoring outside age-related Submitted for publication Jun 24, 2013; last revision received Aug 14, 2013; 32 norms on the Consonant-Nucleus-Consonant-Word test ), accepted Sep 18, 2013. each participant improved when FM-aided and all showed Reprint requests: Prof Gary Rance, BEd, DipAud, MSc, PhD, Department of speech scores equivalent to, or better than, the (unaided) level Audiology and Speech Pathology, The University of Melbourne, 550 Swanston St, Parkville, Australia 3010. E-mail: [email protected] of their matched control, even without any training or device experience. Eight of the 10 participants who undertook the device trial were content to wear the FM system for prolonged periods in References a range of listening conditions. One child who failed to adjust 1. American Psychiatric Association (APA). Diagnostic and statistical had overriding tactile sensitivity issues and could not tolerate manual of mental disorders (DSM V). 5th edition. Washington, DC: the FM receiver for more than a few minutes. The other child APA; 2013, www.dsm5.org/Documents/AutismSpectrumDisorder.

356 Rance et al February 2014 ORIGINAL ARTICLES

2. Kanner L. Autistic disturbances of affective contact. Nerv Child 1943;2: possible pervasive developmental disorders. J Autism Dev Disord 217-50. 1994;24:659-85. 3. Alcantara JI, Weisblatt EJ, Moore BCJ, Bolton PF. Speech perception in 17. Magyar CI, Pandolfi V. Factor structure evaluation of the Childhood high-functioning participants with autism or Asperger’s syndrome. J Autism Rating Scale. J Autism Dev Disord 2007;37:1787-94. Child Psychol Psychiatry 2004;45:1107-14. 18. Needelman H, Schnoes CJ, Ellis CR. The new WISC-IV. J Dev Pediatr 4. Alcantara JI, Cope TE, Cope W, Weisblatt EJ. Auditory temporal- 2006;27:127-8. envelope processing in high-functioning children with autism spectrum 19. Viemeister NF. Temporal modulation transfer functions based on mod- disorder. Neuropsychologia 2012;50:1235-51. ulation threshold. J Acoust Soc Am 1979;66:1364-80. 5. Groen WB, van Orzo L, tar Horne N, Winkles S, van der Gaga R-J, 20. Cameron S, Dillon H. Development of the listening in Spatialized Noise- Butler JK, et al. Intact spectral but abnormal temporal processing in Sentences Test (LiSN-S). Ear Hear 2007;28:196-211. autism. J Aut Dev Disord 2009;39:742-50. 21. Rance G, Ryan MM, Carew P, Corben LA, Yiu E, Tan J, et al. Binaural 6. Beck DL, Bellis TJ. (Central) auditory processing disorders: overview speech processing in individuals with auditory neuropathy. Neurosci- and amplification issues. Hearing J 2007;60:44-7. ence 2012;226:227-35. 7. Cameron S, Glyde H, Dillon H. Efficacy of the LiSN & Learn auditory 22. Petersen GE, Lehiste I. Revised CNC lists for auditory tests. J Speech training software: randomized blinded control study. Audiol Res 2012; Hear Disord 1962;27:62-70. 2e15:86-93. 23. Anderson K, Smaldino J. Listening Inventories for Education: a class- 8. Song J, Skoe E, Banai K, Kraus N. Training to improve hearing speech in room measurement tool. Hearing J 1999;52:74-5. noise: biological mechanisms. Cereb Cortex 2012;22:1180-90. 24. Cameron S, Dillon H, Newall P. Development and evaluation of the 9. Anderson S, White-Schwoch T, Parbery-Clark A, Kraus N. Reversal of Listening in Spatialized Noise Test. Ear Hear 2006;27:30-42. age-related neural timing delays with training. Proc Natl Acad Sci 25. Ashburner J, Rodger S, Ziviani J. Sensory processing and classroom USA 2013;110:4357-62. emotional, behavioural, and educational outcomes in children with 10. Crandell CC, Smaldino JJ. Classroom acoustics for children with normal autism spectrum disorder. Am J Occ Ther 2008;62:564-73. hearing and with hearing impairment. Lang Speech Hear Serv Schools 26. Corbett B, Constantine L. Autism and attention deficit hyperactivity dis- 2000;4:362-70. order: assessing attention and response control with the integrated visual 11. Hawkins D. Comparisons of speech recognition in noise by mildly- to and auditory continuous performance test. Child Neuropsychol 2006;12: moderately-hearing-impaired children using hearing aids and FM sys- 335-48. tems. J Speech Hear Disord 1984;49:409-18. 27. Klin A. Auditory brainstem responses in autism: brainstem dysfunction 12. Johnstone KN, John AB, Hall JW, Crandell CC. Multiple benefits of per- or peripheral hearing loss? J Aut Dev Disord 1993;23:15-35. sonal FM system use by children with auditory processing disorder 28. Rosenhall U, Nordin V, Brantberg K, Gillberg C. Autism and auditory (APD). Int J Audiol 2009;48:371-83. brainstem responses. Ear Hear 2003;24:206-14. 13. Rance G, Corben LA, DuBourg E, King A, Delatycki MB. Successful 29. Russo N, Nicol T, Trommer B, Zecker S, Kraus N. Brainstem transcrip- treatment of auditory perceptual disorder in individuals with Friedreich tion of speech is disrupted in children with autism spectrum disorders. ataxia. Neuroscience 2010;171:552-5. Dev Sci 2009;12:557-67. 14. Schafer EC, Mathews L, Mehta S, Hill M, Munoz A, Bishop R, et al. Per- 30. Russo N, Zecker S, Trommer B, Chen J, Kraus N. Effects of background sonal FM systems for children with autism spectrum disorders (ASD) noise on cortical encoding of speech in autism spectrum disorders. J and/or attention-deficit hyperactivity disorder (ADHD): an initial inves- Autism Dev Disord 2009;39:1185-96. tigation. J Commun Disord 2013;46:30-52. 31. Micheyl C, Carlyon RP, Gutschalk A, Melcher JR, Oxenham AJ, 15. LeCouteur A, Rutter M, Lord C, Rios P. Autism Diagnostic Interview: a Rauschecker JP, et al. The role of auditory cortex in the formation of standardized investigator-based instrument. J Autism Dev Disord 1989; auditory streams. Hear Res 2007;229:116-31. 19:363-87. 32. Rance G, Ryan MM, Bayliss K, Gill K, O’Sullivan C, Whitechurch M. 16. Lord C, Rutter M, LeCouteur A. Autism Diagnostic Interview–Revised: a Auditory function in children with Charcot-Marie-Tooth disease. Brain revision of a diagnostic interview for caregivers of individuals with 2012;135:1412-22.

The Use of Listening Devices to Ameliorate Auditory Deﬁcit in Children with Autism 357 See discussions, stats, and author profiles for this publication at: https://www.researchgate.net/publication/256192356

Syndromic Hearing Loss: an update

Article · August 2013 DOI: 10.3109/21695717.2013.820514

CITATIONS READS 5 293

3 authors:

Alessandro Castiglione Micol Busi University of Padova University of Ferrara

28 PUBLICATIONS 127 CITATIONS 10 PUBLICATIONS 68 CITATIONS

SEE PROFILE SEE PROFILE

Alessandro Martini University of Padova

409 PUBLICATIONS 3,827 CITATIONS

SEE PROFILE

Some of the authors of this publication are also working on these related projects:

Temporomandibular disorders management View project

Molecular markers involved in the biology of temporal bone carcinoma that have a potential prognostic and therapeutic role View project

All content following this page was uploaded by Alessandro Castiglione on 06 October 2017.

The user has requested enhancement of the downloaded file. Hearing, Balance and Communication, 2013; Early Online: 1–14

ORIGINAL ARTICLE

Syndromic hearing loss: An update

ALESSANDRO CASTIGLIONE 1 , MICOL BUSI2 & ALESSANDRO MARTINI1

1 Department of Neurosciences, C.O.U. of Otorhinolaryngology and Otosurgery, University Hospital of Padua, and 2 Department of Medical & Surgical Disciplines of Communication and Behavior, University of Ferrara, Italy

Abstract Hearing impairment is one of the commonest clinical conditions. It has been estimated that approximately 1 in 10 persons has hearing concerns. Further epidemiological studies have found that the percentage of the general population with hearing loss greater than 45 dB HL and 65 dB HL is 1.3% and 0.3%, respectively, between 30 and 50 years of age; and 2.3% and 7.4% between 60 and 70 years of age. The prevalence of childhood and adolescent hearing loss is around 3%. At birth, between one and two out of 1000 newborns are affected by hearing loss of such a degree as to require treatment (auditory training and rehabilitation, hearing aids or cochlear implantation). To summarize, hearing impairment affects up to 30% of the international community and estimates indicate that 70 million persons are deaf. The causes of hearing loss differ and they can vary in severity and physiopathology. In many cases it is not possible to defi ne a defi nite aetiology. Neverthe- less, it is known that most are due to a genetic cause and among these the majority appear in a non-syndromic form. The aetiology of hearing loss in children is unknown in 40% of cases, genetic non-syndromic in 30%, and genetic syndromic in 3 – 5%. The two most common genes involved in hearing loss are GJB2 and SLC26A4 . Mutations in these genes can be responsible for syndromic hearing loss, as keratitis ichthyosis deafness (KID) and Pendred syndromes, respectively, or non- syndromic hearing loss (as DFNB1 and DFNB4, respectively). DFNB1 with GJB2 mutations is the most common non- syndromic form and Pendred syndrome is the most common syndromic form. Neither of these last two is usually characterized by congenital macroscopic dysmorphic features, and affected children can be generally considered as well For personal use only. babies. Nonetheless, 2– 4% of live births have congenital malformations, most commonly caused by multifactorial defects, followed by chromosomal disorders, single gene mutations and teratogens (alcohol, drugs). Some of these conditions could directly affect the auditory system and be responsible for sensorineural, conductive or mixed hearing loss. The London Dysmorphology Database lists approximately 400 syndromes that include hearing loss among the clinical features. Other conditions such as cystic fi brosis are not usually responsible for hearing loss but they can indirectly affect the auditory system as a consequence of the management of the disease. Other systemic disorders can lead to hearing impairment when the disease involves a part of the auditory system from the external ear to the auditory cortex. From this standpoint there are a huge number of syndromes or conditions that can directly or indirectly cause hearing impairment. They can be responsible for congenital or prelingual, progressive and post-lingual hearing loss, with sensorineural, mixed or conductive defi cits. In this updating research we have focused on syndromic forms that are known to be associated with hearing loss or that directly affect the auditory system. Some conditions of particular interest, or with high incidence, are also included.

Keywords: hearing impairment , syndrome , syndromic hearing loss , non-syndromic hearing loss Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13

Introduction conditions caused by well-known pathophysiological The term ‘ syndrome ’ , from the Greek σ υ ν δ ρ μ η´ events or characterized by signs or symptoms affect- (course along with, together), defi nes an association ing the same organ: for example, some authors call or concurrence of clinically recognizable signs EVA syndrome the association between hearing loss (objectives, that can be observed by someone and enlarged vestibular aqueduct. This is accepted, other than the patient) and symptoms (subjectives, even if improper use of the meaning of ‘ syndrome ’ reported by the patient) with unknown, unclear or can generate some misunderstanding and certainly various aetiology, that involves at least two different makes the defi nition of syndromic hearing loss dif- organs, apparatus or systems. We also call ‘ syndrome ’ fi cult. Consequently, fi ltering out the prevalence of

Correspondence: A. Castiglione, Department of Neurosciences Operative Unit of Otolaryngology and Otosurgery, 2 Giustiniani – Padua 35100, Italy. Fax: 39 049 821 1994. E-mail: [email protected]

(Accepted 25 June 2013) ISSN 2169-5717 print/ISSN 2169-5725 online © 2013 Informa Healthcare DOI: 10.3109/21695717.2013.820514 2 A. Castiglione et al. syndromic hearing loss among non-syndromic and physical fi ndings that may help determine the diagno- non-hereditary hearing loss is an imperfect task, sis and aetiology of a patient’s hearing impairment given also phenotypic variability, complicating med- (Figures 1– 4). As most hearing loss is recognized as ical risk factors, and incomplete family histories. In having a genetic cause, patients generally benefi t from addition, epidemiological studies are complicated a genetic evaluation, particularly if they present syn- when the same condition can lead at different times dromic features. To date, more than 90 genes have (depending on symptoms and signs onset) to non- been identifi ed as being potentially or defi nitely related syndromic or syndromic forms. For example, to hearing loss (4,5); this is an increasing number DFNB4 could become Pendred syndrome when the and it could be doubled in the coming years (http:// disease involves the thyroid gland with clinical man- hereditaryhearingloss.org/). Approximately 400 syn- ifestations. This ‘ instability ’ in defi nitions makes dromes (of around 7000 described rare syndromes or comprehensive research on syndromic hearing loss diseases of which approximately 1500 are known to quite diffi cult. An update could be that syndromic have a genetic aetiology) include hearing impairment hearing loss includes every condition, disorder, dis- as a potential feature of the clinical picture. It could ease or syndrome that shows hearing impairment therefore be deduced that around 6% of rare diseases associated with one other sign or symptom. affect hearing. Even if this is an approximation, it indi- A correct defi nition and classifi cation could help cates that hearing is a hot spot for genetic syndromes in managing and organizing particular events with a or rare diseases and disorders, probably due to its copious number of elements that are clinically diffi - specifi c function, anatomical structure and topo- cult to consider or treat together. From this point of graphic position; additionally, auditory cells have a view, a multidisciplinary approach in managing syn- lifespan different from that of cells of other sensory dromic hearing loss is mandatory. Conversely, the organs. Gustatory cells survive for 14– 21 days and term syndromic hearing loss could be strictly used olfactory cells survive for 30– 90 days. The ciliated to name conditions that almost constantly affect cells of the ear have an average life of 70– 100 years, hearing and at least one other organ or system with but their number is fewer than 20,000, compared to clinical manifestations, without a clear aetiology. 137 million cells in the retina. These aspects increase Other conditions could be called diseases or disor- the probability of the ear being involved in a lifetime ders, even if accepting this will probably not change disease. In summary, it could be concluded that every the indications for treatment or therapy of the hear- systemic condition, including genetic disorders, ing loss that essentially remains based on the avail- requires an audiological investigation. Reviewing the For personal use only. able audiological data. most common systemic disorders, such as auto- In waiting for a better defi nition of the fi eld of immune and metabolic disorders, diabetes and syndromic hearing loss, the present study adopted hypertension, reveals that they could be strongly the following criteria for selection of conditions that associated with hearing loss (5– 8). can be related to hearing loss: independently from Recognizing a syndrome depends on many fac- names, aetiology and entity of hearing loss, disorders tors, and is essentially based on a multidisciplinary were selected based on their estimated prevalence, in approach. Nevertheless, it is often thought among decreasing order and divided into different types, in specialists that the diagnosis of a syndrome depends order to illustrate a few examples of various condi- on a kind of intuitive reaction when seeing a patient. tions. Generally, among these syndromes there are It could be noted that this preconception is quite syndromes with a high prevalence and no constant wrong and it could lead to misdiagnosis (1). Intuition hearing concerns, as well as very rare syndromes that and inspection are very important, but a correct Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13 could be characterized by a strong association with approach to syndromes requires a specifi c, clear and severe to profound hearing loss. It should be noted methodical approach. The physical examination that not all syndromes are recognized as having a should incorporate an in-depth ear, nose, throat, genetic cause, e.g. cytomegalovirus and foetal-alcoholic head, and neck evaluation, along with an overall syndromes. Cases of particular interest are also des- assessment of general physical and neurological sta- cribed and discussed herein, and reported in decreas- tus. Children with syndromic features associated ing order of estimated prevalence, if not otherwise with hearing loss should be screened early and rou- specifi ed (1– 6). tinely for hearing loss. Valid and reliable techniques are available to determine the presence, degree, and nature of hearing impairment and such tech- Clinical aspects niques include the following: auditory brainstem Because abnormalities of virtually every organ sys- response, speech and tonal audiometry, tympa- tem have been associated with hearing loss, physi- nometry and acoustic refl ex threshold measurement cians must become familiar with the constellation of and otoacoustic emissions. Syndromic hearing loss 3

Congenital/Prelingual hearing loss 1-2:500-1000 newborns

Genetic 35-40% Non Genetic 25-30% Unknown/Idiopathic 35-40%

Non syndromic hearing loss 70% Syndromic hearing loss 30%

15-2% AD 15-30% AD DFN A Treacher Collins Waardenburg BOR 65-85% ar DFN B 30-60% ar Pendred 1-2% X-linked Usher DFN X Jervell & Lange-Nielsen Mendelian inheritance 0-1% Y-linked 5-10% X-linked DFN Y Albinism (ar) Alport (AD) 0-1% Auditory Neuropathies Norrie (ar) AUN For personal use only. 1-3% Imprinting/uniparental disomy Angelman/Prader Willi Syndromes (?) 0-1 % Modifier Genes DFN M 5-15% Multifactorial disorders Goldenhar (non genetic?) CHARGE (?) 0-1 % Mitochondrial DNA Non mendelian inheritance DFN MT 2-5% mt DNA MIDD, MELAS, MERRF

Figure 1. Aetiology and epidemiology of congenital hearing loss. AD: autosomal dominant; ar: autosomal recessive.

Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13 Early identifi cation of hearing loss and appropri- varies with the severity of involvement; however, it ate intervention provides the best opportunity for is a signifi cant problem even for the most mildly counselling, habilitation, and development. Addi- involved. Bilateral profound hearing loss has a great tionally, early enrolment in services for a child with potential for morbidity. Many studies support that hearing impairment reduces health care, special deafness signifi cantly affects quality of life; social, education, and other service costs. Even if initial educational, and earning potential are diminished. screening examinations indicate normal hearing, the Patients with unilateral hearing loss have diffi culty child remains at risk. During infancy and early hearing in background noise and diffi culty localizing childhood, parents should be aware of, and ques- sound. Furthermore, some syndromes, such as tioned about, the child’s hearing and language mile- Usher syndrome, which accounts for a large per- stones. Some syndromes, such as Pendred, Alport, centage of the aetiology of deaf-blindness, could Refsum, neurofi bromatosis type II, Usher, and affect more than one sensory system. The dual sen- osteopetrosis, may place the patient at risk for pro- sory impairment has huge implications for commu- gressive hearing loss. The morbidity of hearing loss nication and education. 4 A. Castiglione et al.

Hearing loss in the general population 1-3:10

Congenital/Prelingual Hearing Loss Non-Congenital/Peri/Post-lingual Hearing Loss 1-2/500-1000 newborns

Genetic GJB2 mutations (35-55%) OTOF mutations Wolfram Usher 1 Usher 2 Usher 3

Waardenburg Neurofibromatosis type 2 Acoustic Schwannoma Treacher-Collins Norrie BOR Alport DFNB4 Pendred Stickler/Marshall Mitochondrial DNA mutations Otosclerosis/Menière Drugs Noise Presbiacusis Acquired (20-45%) Infections Prematurity

Pre-natal and peri-natal Iatrogenic suffering Trauma

Idiopathic/Unknown 0 within 1 year of life 18 65 (10-25%) Age in months Age in years

Figure 2. Aetiology and epidemiology of hearing loss in the general population with age of symptom onset.

Upon identifi cation of hearing loss, a complete (ESR), antinuclear antibody (ANA), rheumatoid For personal use only. history should include gestational, perinatal, postna- factor (RF), ENA and circulating immune complexes tal, and family histories. Other conditions could be (http://emedicine.medscape.com/). characterized by comorbidities or mortality. Glom- CT scanning offers very high-resolution images erulonephritis associated with Alport syndrome can with 1 mm slices, allowing good visualization of end in kidney failure and necessitate kidney trans- the anatomy of the bones, ossicles, and inner ear. plantation. Children with Jervell and Lange-Nielsen CT scanning may be used to identify potential surgi- syndromes are at risk for syncope, arrhythmias, and cally reparable causes of SNHL and may also be sudden death (1– 8). used to identify the less dysplastic, and presumably Even if clinical testing for many genes or param- better hearing, ear, when considering auditory habil- eters associated with hearing loss is available, a rou- itation. CT abnormalities are found in up to 30% of tine series of laboratory tests is not recommended in individuals with hearing loss and thus are an impor- the evaluation of patients with hearing impairment. tant component of the evaluation of a patient. For Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13 A rational assessment of the cost-benefi t ratio and example, an enlarged vestibular aqueduct and Mon- the clinician’s index of suspicion dictate the selection dini malformation are common fi ndings in Pendred of laboratory studies to be performed for each indi- syndrome. New techniques such as the CONE vidual patient. Studies may include the following: BEAM TC allow better quality images with a reduced genetic testing, including GJB2 , GJB6 and SCL26A4 ray exposure. Magnetic resonance imaging com- (in the presence of dilated vestibular aqueduct) gene pletes the neuroradiological investigations. High mutation testing, blood routine testing with CBC soft-tissue contrast makes MRI ideal for evaluation count with differential, glycaemic levels, BUN/ of the inner ear, internal auditory canal, and cerebel- creatinine and urinalysis (to investigate renal func- lopontine angle. In addition it could be considered tion), thyroid function studies, fl uorescent trepone- with thyroid and renal ultrasonography when abnormal antibody absorption (FTA-ABS) and specifi c malities are suspected. Other radiological investiga- immunoglobulin M (IgM) assays for toxoplasmosis, tions can help in the diagnosis of achondroplasia and rubella, cytomegalovirus, herpes virus, and autoim- Goldenhar syndrome (http://emedicine.medscape. mune panel, e.g. erythrocyte sedimentation rate com/) (7– 11). Syndromic hearing loss 5 identifi cation of vestibular disorders that could be 100% associated with hearing loss, in particular condi- Jervell Lange-Nielsen Pendred TreacherCollins tions such as Pendred, Usher, CHARGE and Kartagener Achodroplasia Alagille syndromes (1– 8). Usher BOR Goldenhar Down Turner Waardenburg II Patau (?) Crouzon Airport Edwards (?) Hearing evaluation in most common genetic CHARGE disorders

Waardenburg I Down syndrome or trisomy 21 (1:600 newborns) Waardenburg III Noonan Trisomy 21, or Down syndrome, is the most com- Sickle Cell Disease mon chromosomal disorder characterized by dys- Norrie Turner morphic features, macroglossia, abnormal teeth, Klinefelter hearing loss % of patients who develop Martin Bell Noonan epicanthal folds, broad short trunk, muscular hypo- Waardenburg IV 0% tonia, congenital heart disease, thyroid dysfunction, Prader Willi transverse palmar crease and neurological abnor- Angelman malities including mental retardation of a variable

Type of hearing loss degree. Hearing loss occurs in up to 80% of cases and it can be conductive (the majority), secondary Sensorineural Mixed Conductive to external ear stenosis, chronic middle ear diseases (otitis media with effusion or cholesteatoma) or Figure 3. Types of hearing loss and percentage of hearing impaired ossicular chain anomalies, sensorineural or mixed. patients in different genetic conditions. The risk of this syndrome in the offspring increases with maternal age at pregnancy (1,2,6– 8,10). Speciﬁ c tests could be performed in certain conditions when an appropriate degree of clinical Fragile X or Martin-Bell syndrome (1:750 newborns) suspicion is present. ECG reveals cardiac conduction anomalies and echocardiography allows the Fragile X syndrome is associated with the most com- identiﬁ cation of heart defects. Electro-oculography mon types of inherited developmental delay and men-

For personal use only. can identify retinitis pigmentosa earlier than a tal retardation. The delay and cognitive difﬁ culties physical examination. Vestibular testing allows the can range from very mild to severe and are sometimes Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13

Figure 4. Schematic representation of the entity and distribution in the frequency range of the hearing loss in different genetic conditions. 6 A. Castiglione et al. associated with autism. Even if there are reported due to an accelerated loss of oocytes in the 45,X cases of hearing loss in patients with fragile X syn- ovary, leaving few follicles in a fi brous strike by birth. drome, they do not have an increased incidence of Nevertheless, approximately one-third of girls with hearing loss. Nevertheless, 85% of patients develop TS may undergo spontaneous puberty, particularly in recurrent episodes of otitis media, which could lead milder phenotypes (12). Although in TS there is not to transient or permanent hearing impairment (1,2). a clear genotype/phenotype correlation, girls with mosaic TS who have a normal cell line, or an extra X chromosome, exhibit a better prognosis (1– 3). Klinefelter syndrome (1:500 newborn males) Up to 80% of cases suffer from conductive hear- Klinefelter syndrome (KS) is the most common sex ing loss probably due to more horizontally oriented chromosomal aberration (47, XXY) in males. It is Eustachian tubes. In addition, or as a consequence characterized by neurodevelopmental problems of of chronic otitis media, they could show progressive variable degree, tall stature with abnormal body pro- sensorineural hearing loss in the high frequencies. In portions, infertility, hypergonadotropic hypogonadism approximately 10% of patients, hearing loss requires with increased urinary excretion of FSH, azoospermia hearing aid amplifi cation before 20 years of age. and gynaecomastia. While the association between KS and infertility has been well documented, hearing loss Sickle cell disease (15:100,000) has been poorly investigated in these patients. The external and middle ear are not generally affected and Sickle cell disease (SCD) is a hereditary haemoglobin they usually appear normal; consequently no conduc- disorder characterized by intermittent episodes of tive hearing loss is reported among these patients. vascular occlusion and end-organ damage. Mutations Sensorineural hearing loss was found in approxi- in the HBB gene cause sickle cell disease. Reviewing mately 20% of cases; nevertheless, further studies are the literature revealed a signifi cant incidence (up to needed to confi rm these data (9). 40%) of sensorineural hearing loss (more severe at the higher frequencies) in SCD patients (13).

Noonan syndrome (40:100,000) Cystic fi brosis (12.6:100,000) Noonan syndrome (NS) is a common autosomal dominant condition characterized by short stature, Cystic fi brosis (CF) is an autosomal recessive genetic congenital heart defects, developmental delay, cryp- disorder caused by mutations in the CFTR gene. CF For personal use only. torchidism, broad or webbed neck and chest deformi- is the second most common life-shortening, inherited ties. The incidence of NS is estimated to be 1– 2 cases disorder occurring in childhood in the United States, per 2500 live births. Mutations in PTPN11 , SOS1 , after sickle cell anaemia. The incidence of cystic fi bro- RAF1 and KRAS are known to be responsible for NS. sis is estimated to be 1– 2 cases per 2500 live births. Even if NS is usually included among syndromes with Patients with CF do not seem to have a higher associated hearing impairment, this is not a constant incidence of hearing impairment than healthy people. feature and its degree and type are extremely variable. Nevertheless, recurrent treatment with aminoglyco- The percentage of affected individuals that develop sides increases the risk of sensorineural hearing loss. hearing problems is approximately 50%; the degree of hearing loss is mild to moderate and secondary to serous otitis media, therefore of conductive type. Sen- Prader Willi (10.7:100,000) and Angelman syndrome (7.5:100,000)

Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13 sorineural hearing loss is less common and is generally mild (10%). Severe to profound sensorineural hearing These two different conditions derive from a ‘ specu- loss is uncommon (3– 5%) (1,2). lar ’ genomic or imprinting disorder: Prader-Willi syndrome is due to the lack of paternal contribution in a critical region on 15q; in contrast, Angelman Turner syndrome (20:100,000; 1:2000 newborn syndrome is due to the lack of maternal contribution females) in the same region. Such conditions are primarily Described for the fi rst time in 1938 by Henry Turner, related to microdeletions of the critical region 15q this syndrome is caused by monosomy (45, X) or or to uniparental disomy of chromosome 15. Less structural abnormalities of the X chromosome. Turner than 5% of cases could be related to the involvement syndrome (TS) is characterized by short stature, lym- of genes located on the proximal region of the long phoedema, gonadal insuffi ciency, cardiac defects and arm of chromosome 15 (15q11). learning disabilities. Absent pubertal development Interestingly, patients affected by Angelman syn- and primary amenorrhoea occur in most individuals drome generally have normal vision and hearing; Syndromic hearing loss 7 even if there is mild to moderate hearing loss it is identifi ed in eight genes, reported in decreasing order rarely reported. Despite their hearing level, most of involvement as follows: DNAH5 , DNAI1 , RSPH4A , show severe language retardation (none or few DNAI2 , DNAH11 , RSPH9 , KTU , TXNDC3 . KS is words). In Prader Willi syndrome, hearing is gener- characterized by malfunction of the cilia (primary ally normal and language development is delayed; ciliary dyskinesia) within organs, such as the nose, nevertheless, verbal skills are not markedly affected ears and lungs, resulting in ineffective clearance of even if speech is poorly articulated. Both syndromes mucus and bacteria. This condition leads to recur- are characterized by neurological defects, mental rent infections of the lungs, ears and nose which, if retardation and cognitive impairment that infl uence left untreated, results in long-term organ damage. It audiological assessment and rehabilitation (2). also affects the fl agella of sperm with consequent sterility. It should be noted that the outer and inner hair cells are not affected by Kartagener syndrome Edwards syndrome or trisomy 18 (1:5000 newborns) because the cilia are different in structure. Karta- Edwards syndrome is named after John Hilton gener syndrome specifi cally affects microtubules Edwards, who fi rst described the syndrome in 1960. constituted of alpha and beta tubulins. In contrast, Clinical features include malformed pinna, micro- stereocilia of the inner and outer cells are made of gnathia, prominent occiput, intestinal defects, heart actin. Other ciliopathologies that are reported to be abnormalities, kidney malformations and mental responsible for retinopathy and sensorineural retardation. It is the second most common autosomal hearing loss, affect cilia constituted mainly of actin. trisomy, after Down syndrome and around 80% of Kartagener syndrome can cause chronic glue ear, those affected are female. Most affected infants do rhinitis and sinusitis, with the prevalence of conduc- not survive past their third month of life, although tive hearing loss (14– 19). up to 13% live past the age of one year. The risk of this syndrome increases with maternal age at pregnancy. Temporal bone studies have revealed severe Genetic syndromes with hearing loss as a abnormalities in the middle and inner ear; therefore characteristic feature it is thought that babies with Edwards syndrome are Autosomal recessive syndromes (60– 70% of congenital deaf or at least severely hearing impaired. and genetic hearing loss) Pendred syndrome (5.5:100,000). First described in For personal use only. Patau syndrome or trisomy 13 (1:6000) 1896 by Vaughan Pendred, the Pendred syndrome is responsible for approximately 1– 8% of congenital Trisomy 13 occurs in approximately 1 per 6000 hearing loss and mutations in the SLC26A4 gene are births. Congenital malformations are so severe that the second commonest cause of genetic hearing loss most affected infants do not survive beyond the fi rst (after GJB2 mutations). There are two clinical forms year of life. Clinical features include microcephaly, related to these mutations: syndromic and non- cleft lip and palate, polydactyly, rocker-bottom feet, syndromic deafness; the former is called Pendred low-set malformed pinna, cardiac dextroposition, syndrome (PS) when deafness is associated with a scalp defects, and mental retardation. Temporal bone thyroid goitre; the latter is called DFNB4, when no analysis reveals cystic changes within the stria vascu- other symptoms are present. PS and DFNB4 can be laris, a shortened cochlea, saccular degeneration, and associated with inner ear malformations. In most anomalies of the semicircular canals. The risk of this cases (around 80%), these consist of an enlarged ves-

Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13 syndrome in the offspring increases with maternal tibular aqueduct (EVA). PS and DFNB4 are trans- age at pregnancy. Temporal bone fi ndings in these mitted as an autosomal recessive trait, but simple patients suggest that they are likely to be deaf or at heterozygotes can develop both forms of deafness, least severely hearing impaired. which is probably due to mutations in unidentifi ed genes. The FOXI1 (5q34) gene encodes a transcriptional activator that is required in order to develop Kartagener syndrome (1– 2:32,000 newborns) normal hearing, sense of balance and kidney func- In 1930, a physician from Switzerland, Kartagener, tion. This activator allows the transcription of the described a familial form of bronchiectasis, situs SLC26A4 gene. Furthermore, mutations in the inversus and rhino-sinusitis with nasal polyps. inwardly rectifying K( ) channel gene KCNJ10 Kartagener syndrome is a genetically heterogenous (1q23.2) are also associated with non-syndromic disorder that prevalently shows an autosomal reces- hearing loss in carriers of SLC26A4 mutations with sive inheritance pattern. To date mutations have been an EVA/PS phenotype. It is thought that Pendred 8 A. Castiglione et al. syndrome occurs when both alleles of SLC26A4 gene identifi ed including MYO7A , USH1C , CDH23 , are mutated; DFNB4 (or non-syndromic EVA) seems PCDH15, SANS, USH2A, VLGR1, WHRN, USH3A, to be due to monoallelic mutations. PDZD7 . Some patients with hearing loss and EVA do not have mutations in the SLC26A4 gene. This condition Jervell and Lange-Nielsen syndrome (0.3:100,000). is known as ‘ hearing loss associated with EVA’ or Although prolongation of the QT interval has the ‘ EVA syndrome ’ . EVA can also be associated with higher prevalence, this condition is only called Jervell other syndromes, such as BOR syndrome or and Lange-Nielsen syndrome when it is accompa- Waardenburg syndrome, but in these syndromes nied by hearing loss; thus, by defi nition, 100% of other genes are considered to be involved. EVA is patients have severe to profound hearing loss. Jervell diagnosed by neuroimaging (CT and MRI): the ves- and Lange-Nielsen syndrome is thought to be the tibular aqueduct is defi ned as enlarged if its diameter third most common cause of autosomal syndromic is greater than 1.2– 1.5 mm at the midpoint. Hearing hearing loss and accounts for 1% of all cases of reces- loss is sensorineural or mixed with a conductive sive hearing loss. This disorder is characterized by component in the low frequencies. Generally, hear- electrocardiographic changes of a prolonged QT ing impairment is congenital or prelingual, but pro- interval, Stokes-Adams attacks, congenital bilateral gressive and post-lingual worsening is also possible severe hearing loss, and sudden death. Syncopal (10,11). attacks begin in early childhood, with sudden death often occurring in later years. The genetic basis of Usher syndrome (3.5:100,000). Children with Usher Jervell and Lange-Nielsen syndrome is thought to be syndrome develop hearing loss, vestibular and visual mutations in the KCNQ1 , and less commonly, the impairment. It has been estimated that Usher syn- KCNE1 gene, which are responsible for coding pro- drome accounts for approximately 3– 5% of hearing teins that form potassium transport channels. These loss among children. The disorder is responsible for channels are critical in the function of the inner ear up to 8% of cases of congenital deafness and it is and heart muscle. inherited in an autosomal recessive pattern. Usher syndrome is characterized by progressive blindness due to retinitis pigmentosa, along with moderate to Alstr ö m syndrome (0.14:100,000). Alströ m syndrome severe sensorineural hearing loss, and accounts for is an autosomal recessive disorder caused by muta- about 50% of the deaf-blind in the United States. tions in the ALMS1 gene. Alströ m syndrome is char-

For personal use only. Funduscopic examination before the age of 10 years acterized by features such as retinitis pigmentosa, is limited. Electroretinography can reveal early retinal diabetes mellitus, cardiomyopathy, short stature, abnormalities in young children but is not routinely obesity, and progressive hearing loss. It is important available. Night blindness and visual fi eld defi cits to distinguish this condition from Usher syndrome may mark developing retinitis pigmentosa. Loss of because of a marked difference in prognosis. Alströ m vision is progressive and 50% of individuals develop syndrome can lead to liver failure, kidney failure and complete blindness before the age of 50 years. pulmonary problems, although the presentation is Hearing loss is generally present at birth, and variable. Hearing loss, generally of the sensorineural 85% of affected individuals eventually become totally variety, typically occurs by age 10 years. deaf. Usher syndrome is classifi ed in three types, as follows: Autosomal dominant syndromes (20– 30% of •

Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13 Type I is characterized by bilateral congenital congenital and genetic hearing loss) severe to profound hearing loss and poor or These are less frequent causes of hearing loss than absent vestibular function with retinitis pigmen- autosomal recessive disorders. Examples include tosa diagnosed by age 10 years. Stickler syndrome, achondroplasia, Waardenburg, • Type II is characterized by mild to moderate neurofi bromatosis, Crouzon, and branchio-oto-renal hearing loss at birth and normal vestibular func- syndromes. tion with onset of retinitis pigmentosa before the age of 20 years. Stickler syndrome (13.5:100,000). Stickler syn- • Type III Usher syndrome is characterized by pro- drome, also named hereditary progressive arthro- gressive hearing loss and vestibular dysfunction ophthalmopathy, is a genetically Heterogeneous with a variable degree of retinitis pigmentosa. condition with involvement of the connective tissue The genetic basis of Usher syndrome is complex and showing an autosomal dominant pattern. It is with mutations at 13 loci (USH1 previously called characterized by high phenotypic variability essen- USH1A does not in fact exist) and 10 genes tially based on disorders of the ocular, auditory, Syndromic hearing loss 9 orofacial and musculoskeletal systems. There are in the EYA1 gene. To date it is uncertain that BOR, three types of Stickler syndrome: type I is caused by BO and OFC syndromes are different entities; over- mutations of the gene COL2A1 (12q13.11-q13.2); lapping features have suggested that they could be type II by mutations of the gene COL11A1 (1p21) considered as three different clinical pictures of the and type III (non-ocular type) by mutations of the same disorder. BOR and BO syndromes are so sim- gene COL11A2 (6p21.3). Also recorded are muta- ilar that researchers often consider them together. In tions in the gene COL9A1 causing autosomal reces- contrast, Oto-Facio-Cervical (OFC) syndrome is sive inheritance. Marshall and Stickler syndromes still not universally accepted as an allelic variant, are similar conditions and it is not yet clear whether because of additional features (hypoplasia of the cer- Marshall syndrome should be considered a variant of vical musculature, pronounced sloping shoulders Stickler syndrome (type II) or rather an entity in and short stature) that are usually not present in BO/ itself. The hearing loss is of mixed type or sensorineu- BOR syndrome. Branchio-Oculo-Facial (BOF) syn- ral, and more serious in Marshall syndrome and drome presents overlapping features with BO/BOR/ types II (progressive hearing loss) and III (non pro- OFC disorders, with or without kidney anomalies. It gressive hearing loss). is now clear that it is caused by mutations in the TFAP2A gene and BOF syndrome is generally Treacher Collins or Franceschetti syndrome (6:100,000). considered a different entity. To date, BOR, BOS, Treacher Collins or Franceschetti syndrome is a mal- OFC and even Branchio-oto-ureteral (BOU) syn- formation condition with high penetrance and vari- dromes should be considered in the light of molecu- able expressivity that is caused by mutations in the lar genetics: affected persons in families segregating gene TCOF1 , located in the region 5q31.3 . The EYA1 mutations have clinical fi ndings consistent TCOF1 gene encodes a protein (treacle) involved in with the diagnosis of BOR, BOS, OFC or BOU syn- nucleolar function. Around 60% of cases are ‘ de drome. These syndromes are best considered as novo ’ . The typical clinical picture is due to faults in branchio-oto-renal spectrum disorders or EYA1- the development of the fi rst two branchial arches, related disorders. These similar conditions require a bilateral and symmetrical oblique eyelid sloping multidisciplinary approach in diagnosis, management downwards and laterally, lower eyelid coloboma, and treatment: an otorhinolaryngologist, audiologist, hypoplasia cheekbones, micrognatia, mouth wide neuroradiologist, nephrologist, ophthalmologist and and thin, dental faults. There may be cleft palate and geneticist are involved in a comprehensive evaluation. nasal spread with nostrils narrow, and sometimes Branchio-oto-renal (BOR) syndrome (MIM#

For personal use only. choanal atresia. Faults of the roof are constant (ano- 113650), also known as Melnick-Fraser syndrome tia, hypoplasia, cup), which is associated with the (because fi rst described by Melnick and Fraser in presence of fi stulae or pre-auricular appendages, fre- 1972), is an autosomal dominant disorder that quent atresia or stenosis of the external auditory consists of external, middle and inner ear malforma- meatus. In the middle ear, hypoplasia of the mastoid tions, branchial cleft sinuses, cervical fi stulae, mixed bone, frequent anomalies of the ossicular chain or conductive hearing loss and renal anomalies. (undescended testes of the incus, malformation or The prevalence of BOR syndrome is approximately ankylosis or absence of the bracket, lack of the oval 1:40,000 newborns, and it has been reported to window) are common. Impairment of the vestibular occur in about 2% of deaf children (1 – 4). Branchio- apparatus (dysplasia of the semicircular canal) is fre- otic (BO) and Oto-Facio-Cervical (OFC) syndromes quent, while the cochlea is normal. The hearing loss are much less common disorders that show clinically is generally conductive or mixed, rarely sensorineu- overlapping features of BOR syndrome without kid-

Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13 ral, and is bilateral and symmetrical in 55% of cases ney involvement (5,6). Branchio-oto-ureteral (BOU) (1,2,10). syndrome defi nes similar conditions characterized by constant duplication of the ureters. BOR syndrome and other EYA1-related disorders (2.5: The EYA1 (eyes absent homologue 1) gene is the 100,000). Branchio-oto-renal (BOR), Branchiootic human homologue of Drosophila eyes absent gene, (BO) and Oto-Facio-Cervical (OFC) syndromes are which is essential for eye development in that species. dominant disorders characterized by variable hearing In humans, it consists of 16 coding exons and has impairment (HI) and branchial defects. BOR syn- been localized to chromosome 8q13.3 (1– 4). To date, drome includes additional kidney malformations. BOR/ at least four different isoforms are known ( EYA1A , BO/OFC syndromes are genetically Heterogeneous EYA1B , EYA1C , EYA1D ), respectively, constituted and caused by mutations in EYA1 , SIX1 , and SIX5 by 559 amino acids (AA), 592 AA, 592 AA and 557 genes. Nevertheless, mutations in the same gene can AA; furthermore, many transcriptional variants or lead to different syndromes. About 40% of people polymorphisms have been reported. Mutations in the with BO, BOR or OFC syndromes have a mutation EYA1 gene are associated with both sporadic and 10 A. Castiglione et al. familial cases. There are over 160 reported mutations discrimination. The hearing loss can be bilateral and in EYA1 to date (http://www.healthcare.uiowa.edu/ asymmetrical in case of bilateral schwannomas, lead- labs/pendredandbor/), even if in some cases muta- ing to deafness within 5– 10 years. tions in different isoforms are deduced by bioinfor- matics analysis. EYA1 mutations were found in CHARGE association (0.14:100,000). CHARGE (iris 31% of families fi tting established clinical criteria for coloboma, heart defects, choanal atresia, growth and BOR and 7% of families with questionable BOR development delay and retardation, genitourinary phenotype. SIX1 (1– 2%) and SIX5 (approximately hypoplasia, ear abnormalities and deafness) associa- 2 – 5%) mutations are much less common. EYA1 tion is a recognizable (genetic) pattern of birth defects mutations produce a wide range of clinical manifes- due to mutations in the CHD7 gene in approximately tations even within the same family, from mild to 60 – 70% of cases. Up to 90% of patients present with severe clinical phenotypes associated with hearing sensorineural hearing loss. Generally, there is no his- and renal anomalies (penetrance appears to be tory of CHARGE association or any other similar around 100% although expressivity is highly variable). condition in the family. Babies with CHARGE syndrome are often born with life-threatening birth Waardenburg syndrome (2.4:100,000). Waardenburg defects, including complex heart defects and breath- syndrome (WS) is one of the most common causes ing problems. of autosomal dominant syndromic hearing loss and Hypoplastic cranial nerves and external or inner it is estimated to account for 2– 5% of all cases of ear malformations are usually present and these can congenital hearing loss. Mutations in the PAX3 gene be responsible for facial paresis or hearing loss with cause WS type I and WS type III with an autosomal consequent diffi culties in cochlear implantation dominant inheritance pattern. WS type II is caused (20,21). by mutations in the MITF or SNAI2 (also called SLUG ) genes. WS type IV has been linked to muta- FGFR2-related disorders and craniosynostosis (1:3000): tions in EDNRB , EDN3 and SOX10 . Phenotypes are Crouzon (2:100,000), Apert (1.25:100,000), and inherited with a recessive pattern when the genes Pfeiffer syndromes (1:100,000). Mutations in the SNAI2 , EDNRB and EDN3 are involved. WS type I FGFR2 gene can be responsible for Crouzon, Apert is characterized by: 1) dystopia canthorum (lateral and Pfeiffer syndromes, the most common cranio- displacement of the medial canthi); 2) hyperplastic, synostosis syndromes. They follow an autosomal high and large nasal root; 3) hyperplasia of the medial dominant pattern, even if sporadic cases are quite

For personal use only. portion of the eyebrows that can be confl uent; 4) skin common. The FGFR1 gene can also be involved in or iris pigmentary anomalies (iris heterochromia); Pfeiffer syndrome. Craniosynostosis is the premature 5) circumscribed albinism of the frontal head hair or fusion of the cranial sutures and Crouzon, Apert and white forelock; 6) sensorineural deafness (20– 25% of Pfeiffer syndromes are bi-coronal craniosynostosis. cases), unilateral or bilateral. Type I and type II are All these syndromes can be associated with a hyp- distinguished on the basis of the presence of the dys- oplastic middle ear and conductive hearing loss topia canthorum, which is absent in type II. The pres- (1,2,7– 10,22). ence of abnormalities of the upper limb identifi es type III, which is also called Klein-Waardenburg syndrome. Finally, the association of autosomal recessive X-linked recessive syndromes: Norrie disease inheritance and Hirshprung disease is defi ned as type Norrie disease or syndrome is caused by mutations IV or Waardenburg-Shah syndrome (1,2,8– 10). in the NDP gene (Xp11.3) that encodes a protein Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13 (norrin) of 133 amino acids. The norrin protein is Neurofi bromatosis type 2 (0.5:100,000). Neurofi bro- essential to the development of blood vessels and matosis 2 is caused by mutations in the NF2 gene retinal cells; furthermore, it seems to be involved in and is characterized by the development of multiple inter- and intra-cellular communications. tumours, including vestibular schwannomas (bilateral The clinical picture is characterized by a degen- in 95% of cases), meningiomas, gliomas, and erative process causing blindness in childhood. Dur- ependymomas that could manifest as early as 8– 12 ing the fi rst days of life the retinal dysplasia manifests years of age. The hearing loss caused by a vestibular with sickle creases of the retina and progressively schwannoma in neurofi bromatosis 2 is approximately results in retinal detachment. At birth the corneas the same as a single-sided acoustic neuroma. About are usually clear but during the pre-school years half of patients present with sudden onset of unilateral become opaque and also develop a secondary cata- sensorineural hearing loss, that is characterized by ract. About 35% of subjects have a sensorineural slow pure-tone change (more marked at high hearing loss, progressive in the high frequencies after frequencies) and progressive impairment in speech 10 years of age. Approximately 35% of patients show Syndromic hearing loss 11 progressive mental retardation and 25% of cases are OTOF gene, which are currently considered as affected by psychosis. responsible for an auditory neuropathy, are classifi ed as DFNB9 (non-syndromic recessive hearing loss). OPA1 mutations have been associated with optic X-linked dominant syndromes: Alport syndrome atrophy type 1, which is a dominantly inherited optic Alport syndrome is characterized by progressive sen- neuropathy resulting in progressive loss of visual sorineural hearing loss in addition to renal disorders acuity, leading in many cases to legal blindness. It (such as renal insuffi ciency, glomerulonephritis or could be accompanied by involvement of different haematuria) and ocular anomalies. Mutations in the cranial nerves such as the eighth nerve, with conse- COL4A5 gene are responsible for the dominant quent hearing impairment (25– 29). inheritance of the syndrome and they account for approximately 80% of all affected patients (23). The Biotinidase defi ciency (1 – 2:100,000). Biotinidase autosomal forms are caused by mutations in the defi ciency is inherited as an autosomal recessive trait; COL4A3 and COL4A4 genes. as expected, the vast majority of mutations are Sensorineural hearing loss develops in 80– 90% of homozygous or compound heterozygous. Biotinidase males with Alport syndrome by the age of 40 years. defi ciency usually manifests in children from one Generally, hearing loss is not congenital. In its early week of age to adolescence, with most exhibiting stages the hearing defi cit is detectable only by audi- symptoms between three and six months of age. If ometry, with bilateral reduction in sensitivity in the untreated, children with profound biotinidase defi - high frequencies. In affected males, the hearing loss ciency usually develop one or more of the following: is progressive and eventually extends to other fre- neurological disorders (myoclonic seizures, hypoto- quencies, including those of conversational speech. nia, ataxia), developmental delay, visual problems, Hearing loss is frequently identifi able by formal hearing loss and dermatological disorders (alopecia, assessment of hearing in late childhood, but in some eczema, and/or candidiasis). In a few cases, individu- families is not detectable until relatively late in life als with profound biotinidase defi ciency may remain (http://www.ncbi.nlm.nih.gov/books). asymptomatic or develop symptoms after adolescence. All symptomatic-affected individuals improve with oral pharmacological doses of the vitamin, bio- Y-linked syndromes tin. Biotin treatment prevents the development of To date, there are no recognized syndromic forms of symptoms in affected children identifi ed before they For personal use only. hearing loss related to anomalies on chromosome Y. have clinical fi ndings or are identifi ed by newborn Nevertheless, a review of the literature suggests a screening. Biotin therapy is lifelong (30). potential role for chromosome Y in syndromic hearing loss (24). Modifi er genes Modifi er genes can modify the expression of other Auditory neuropathies genes. Mutations in these genes can be responsible Hearing loss due to auditory neuropathy is currently for non-syndromic hearing loss or infl uence the divided in two main groups: the fi rst, called Auditory clinical picture in the syndromic forms. These condi- Neuropathy Spectrum Disorders (ANSD), includes tions do not appear to follow traditional Mendelian all diseases or conditions or syndromes characterized expression patterns. For example, cadherin 23 pro- by neuropathy that can affect the auditory system. duces a spectrum of phenotypic traits, including Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13 The second group is strictly related to specifi c genes, presbycusis, non-syndromic prelingual hearing loss which, if mutated, can lead to hearing loss due to (DFNB12), and syndromic hearing loss as part of auditory neuropathy. It should be noted that some Usher syndrome (Usher 1D). Missense mutations in authors prefer to consider the second group as part CDH23 have been associated with presbycusis and of the fi rst. The acronym AUN could be used exclu- DFNB12, whereas null alleles cause the majority of sively to indicate conditions mainly characterized by Usher 1D. Modifi er gene products that interact with auditory neuropathy (non-syndromic) and caused by cadherin 23 also affect the phenotypic spectrum. In mutations in specifi c genes for which loci are defi ned addition, the phenotypic spectrum of Wolfram syn- as AUNA in the case of autosomal dominant inheri- drome is also hypothesized to be infl uenced by mod- tance, followed by identifi cation numbers in chrono- ifi er gene products. Further studies are needed to logical order. To date, there is only one recognized provide more evidence for the importance of modi- gene responsible for non-syndromic auditory neu- fi er genes. Characterizing modifi er genes may result ropathy, i.e. DIAPH3 (AUNA1). Mutations in the in better treatment options for patients with hearing 12 A. Castiglione et al. loss and defi ne new diagnostic and therapeutic tar- in children with craniofacial anomalies such as cleft gets (31,32). lip/palate (70%) and Down syndrome (62%). A small percentage of FAS children have also been shown to display mild to moderate sensorineural Mitochondrial diseases: MELAS syndrome hearing loss (33– 38). (16:100,000), MERRF syndrome (1:100,000) and MIDD (0.1:100,000) Mitochondrial encephalopathy, lactic acidosis and Cytomegalovirus syndrome (40:100,000) stroke-like episodes (MELAS) is a childhood disor- Cytomegalovirus (CMV) is one of the most frequently der characterized by intermittent vomiting, proximal transmitted intrauterine infections, detectable in limb weakness and recurrent cerebral insults resem- an estimated 0.64– 0.70% of live births worldwide. bling strokes and causing hemiparesis and cortical Sensorineural hearing loss is the most common blindness. MELAS is frequently associated with sequel following congenital CMV infection. CMV is short stature. The wide range of clinical signs and estimated to be the leading environmental cause symptoms include hearing loss in approximately of childhood hearing loss, accounting for approxi- 30% of affected persons. mately 15– 21% of all hearing loss at birth in the Myoclonic epilepsy and ragged red fi bres United States. In addition, CMV-related hearing (MERRF) is characterized by myoclonus, epilepsy losses may also be progressive or late onset, requiring and ataxia, although dementia, optic atrophy and more frequent audiological monitoring of infants and deafness frequently occur. The degree of hearing loss young children who have been diagnosed with con- is variable. genital CMV infection (39– 46). In maternally inherited diabetes and deafness (MIDD), several families have been described with diabetes mellitus and sensorineural hearing loss, in Autoimmune disorders which mitochondrial mutations have been found. The most frequently found mutation is the 3243A- G Cogan syndrome. Cogan syndrome is a rare disorder mutation, also found in MELAS. In population stud- characterized by recurrent infl ammation of the front of ies of diabetics, the 3243A- G mutation has been the eye (the cornea) and often fever, fatigue, and weight found in a small percentage of patients (1,2,8,10). loss, episodes of dizziness, and hearing loss. It can lead to deafness or blindness if untreated. The classic form of the disease was fi rst described by Cogan in For personal use only. Non-genetic syndromes 1945. In 60% of patients with Cogan syndrome, cochlear implantation remains the only treatment Foetal alcohol syndrome (1:1000 newborns) option for the auditory rehabilitation. Literature data Foetal alcohol syndrome (FAS) is induced by exces- agree that once the electrode array is properly inserted, sive maternal alcohol intake during pregnancy. FAS functional outcomes are very good. Nevertheless, is characterized by: facial dysmorphisms such as results may deteriorate due to progressive cochlear smooth philtrum, thin vermillion border and small ossifi cation (47,48). palpebral fi ssures (at or below the 10th percentile), central nervous system abnormalities, growth prob- Goodpasture syndrome. Goodpasture syndrome, a lems and maternal alcohol exposure. Diagnostic cri- rare human autoimmune disorder, is characterized teria for FAS are all three of the following fi ndings: by the presence of pathogenic autoantibodies that

Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13 1) all three facial abnormalities; 2) growth defi cits; react with the components of the glomerular base- and 3) central nervous system abnormalities. Growth ment membrane. The clinical condition of Good- defi cits are diagnosed when prenatal or postnatal pasture syndrome is characterized by an acute height or weight, or both, are at or below the 10th necrotizing glomerulonephritis, often with accom- percentile, documented at any one point in time panying pulmonary haemorrhage. Notably, the (adjusted for age, gender, gestational age, and race Goodpasture antigen has been localized to the non- or ethnicity). Central nervous system anomalies collagenous domain of the α 3 chain of type IV col- include structural, neurological and functional lagen. Goodpasture syndrome is an uncommon abnormalities. In multiple studies, children with FAS disease, having an incidence of 0.5 to 1 cases per have been reported to show much higher rates of million of the population with a slight preponder- intermittent hearing loss due to recurrent middle ear ance of males to females (49). Type IV collagen, the infections (75– 93%) compared to the general paedi- major structural component of basement mem- atric population (12%). The FAS fi ndings are more branes, is a multimeric protein composed of three consistent with middle ear disorder prevalence rates alpha subunits. These subunits are encoded by six Syndromic hearing loss 13 different genes, alpha 1 through alpha 6, each of (Branchio-otic) syndrome and OFC (Oto-Facio- which can form a triple helix structure with two Cervical) syndrome. Nevertheless, it should be other subunits to form type IV collagen. This gene noted that the aetiology remains unknown in most encodes alpha 3 (COL4A3 on 2q36-q37). In Good- cases and an indiscriminate use of testing does pasture syndrome, autoantibodies bind to the col- not always change the prognosis or therapy. In lagen molecules in the basement membranes of addition, it should be remembered that not all syn- alveoli and glomeruli. The epitopes that elicit these dromes have a genetic cause and there are several autoantibodies are localized largely to the non- heterogeneous conditions for which the aetiology collagenous C-terminal domain of the protein. A and pathophysiology is still unclear (50,51). specifi c kinase phosphorylates amino acids in this Non-syndromic and syndromic hearing losses same C-terminal region and the expression of this demand early evaluation and rehabilitation to reduce kinase is up-regulated during pathogenesis. This gene the auditory deprivation that could affect perceptual, is also linked to an autosomal recessive form of verbal and linguistic skills. To date, hearing aids and Alport syndrome. The mutations contributing to this cochlear implantation are the most reliable and effec- syndrome are also located within the exons that tive interventions for auditory rehabilitation. Although encode this C-terminal region. Like other members further studies are necessary, the results of the pres- of the type IV collagen gene family, this gene is orga- ent work have confi rmed the need to carry out diag- nized in a head-to-head conformation with another nostic, therapeutic and rehabilitation processes in type IV collagen gene so that each gene pair shares specialized centres with extensive and proven a common promoter (http://www.genecards.org). experience.

Declaration of interest: The authors have no Discussion and Conclusions confl icts of interest to disclose. The defi nition of syndromic hearing loss still remains an open fi eld that requires constant updating. Clas- sifi cation, aetiology, clinical and epidemiological References data undergo continuous change, therefore it is 1. Toriello HV , Reardon W , Gorlin R . Hereditary hearing loss quite diffi cult to obtain a comprehensive assessment and its syndromes. 2nd edn. Oxford University Press, 2004 . on the prevalence and management of complex and 2. Cassidy SB , Allanson J . Management of Genetic Syndromes. 3rd edn . Hoboken New Jersey: Wiley-Blackwell; 2010 . For personal use only. heterogeneous conditions. The multidisciplinary approach is certainly the most correct and continu- 3. Calvo F , Karras BT , Phillips R , Kimball AM , Wolf F . Diag- noses, syndromes, and diseases: a knowledge representation ous monitoring is essential. The diagnosis of syndro- problem . AMIA Annu Symp Proc. 2003: 802 . mic hearing loss requires a careful history and 4. Mahboubi H , Dwabe S , Fradkin M , Kimonis V, Djalilian HR . objective examination, MRI and CT scanning and Genetics of hearing loss: where are we standing now? Eur laboratory testing (including genetic testing), tests Arch Otorhinolaryngol. 2012; 269 : 1733– 45 . that are ever more sophisticated. The role of genet- 5. Diaz-Horta O, Duman D, Foster J 2nd, Sirmaci A , Gonzalez M , Mahdieh N , et al . Whole-exome sequencing ics is constantly growing and giving us important effi ciently detects rare mutations in autosomal recessive information on diagnosis and treatment. The most non-syndromic hearing loss. PLoS One. 2012; 7 : e50628. common mutations that are responsible for hearing 6. Bovo R , Ciorba A , Martini A . Environmental and genetic loss involve the GJB2 gene; SLC26A4 mutations are factors in age-related hearing impairment. Aging Clin Exp the second most common cause of genetic hearing Res. 2011; 23 : 3 – 10 . 7. Newton VE . Paediatric Audiological Medicine. 2nd edn. Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13 loss and the fi rst among syndromic deafness. The Chichester: John Wiley & Sons ; 2009 . SLC26A4 (PDS) gene mutations result in abnor- 8. Martini A , Stephens D, Read AP . Genes, Hearing and malities of the endolymphatic system, which lead to Deafness, 1st edn. Informa Healthcare, UK, 2007 . the dilation of the vestibular aqueduct as seen in 9. Vignozzi L , Corona G , Forti G , Jannini EA , Maggi M . Pendred syndrome. Nonetheless, other genes can be Clinical and therapeutic aspects of Klinefelter’s syndrome: sexual function. Mol Hum Reprod. 2010; 16 : 418 – 24 . involved. Normal expression of the PAX2 and PAX3 10. Martini A , Prosser S . Audiologia e foniatria. 1st edn. Omega genes is necessary for the normal development of Edizioni , 2011 . the cochlea. The FGF3 gene appears to be necessary 11. Busi M , Castiglione A , Taddei Masieri M , Ravani A , for differentiation within the otic vesicle (22). The Guaran V, Astolfi L, et al . Novel mutations in the SLC26A4 EYA1 gene has an important role in encoding gene . Int J Pediatr Otorhinolaryngol. 2012; 76 : 1249– 54 . 12. Improda N , Rezzuto M , Alfano S , Parenti G , Vajro P , transcription factors and mutations of EYA1 Pignata C , et al . Precocious puberty in Turner syndrome: are responsible for EYA1 -related disorders that report of a case and review of the literature. Ital J Pediatr. include BOR (branchio-oto-renal) syndrome, BO 2012; 38 : 54 . 14 A. Castiglione et al.

13. Al Okbi MH , Alkindi S , Al Abri RK , Mathew J , Nagwa AA , 32. Hilgert N , Huentelman MJ , Thorburn AQ , Fransen E , Pathare AV . Sensorineural hearing loss in sickle cell disease: Dieltjens N , Mueller-Malesinska M , et al . Phenotypic varia- a prospective study from Oman. Laryngoscope. 2011; 121 : bility of patients homozygous for the GJB2 mutation 35delG 392– 6 . cannot be explained by the inﬂ uence of one major modiﬁ er 14. Leigh MW , Pittman JE , Carson JL , Ferkol TW , Dell SD , gene . Eur J Hum Genet. 2009; 17 : 517 – 24 . Davis SD, et al. Clinical and genetic aspects of primary ciliary 33. Carlo WA . Foetal alcohol syndrome. In: Kliegman RM, dyskinesia/Kartagener syndrome. Genet Med. 2009 ; 11 : Behrman RE, Jenson HB, Stanton BF, editors. Nelson 473– 87 . Textbook of Pediatrics. 18th edn. Philadelphia, Pa: Saunders 15. Shu X , Black GC , Rice JM , Hart-Holden N , Jones A , Elsevier; 2007 . O’Grady A , et al . RPGR mutation analysis and disease: 34. Cunningham FG , Leveno KJ , Bloom SL , Hauth J , an update . Hum Mutat. 2007; 28 : 322 – 8 . Rouse D , Spong C , et al . Teratology and medications that 16. Zito I , Downes SM , Patel RJ , Cheetham ME , Ebenezer ND , affect the foetus. In: Cunningham FG, Leveno KJ, Bloom Jenkins SA , et al . RPGR mutation associated with retinitis SL, Hauth J, Rouse D, Spong C et al, editors. Williams pigmentosa, impaired hearing, and sinorespiratory infections. Obstetrics. 23rd edn. New York, NY: McGraw-Hill; 2010 . J Med Genet. 2003; 40 : 609 – 15 . Chapter 14. 17. Chaitin MH , Schneider BG , Hall MO , Papermaster DS . 35. Ornoy A, Ergaz Z . Alcohol abuse in pregnant females: effects Actin in the photoreceptor connecting cilium: immuno- on the foetus and newborn, mode of action and maternal cytochemical localization to the site of outer segment disk treatment. Int J Environ Res Public Health. 2010 ; 7 : 364 – 79 . formation. J Cell Biol. 1984; 99 : 239 – 47 . 36. Evrard SG . Diagnostic criteria for foetal alcohol syndrome 18. Gakovic M , Shu X , Kasioulis I , Carpanini S , Moraga I , and foetal alcohol spectrum disorders. Arch Argent Pediatr. Wright AF . The role of RPGR in cilia formation and actin 2010; 108 : 61 – 7 . stability. Hum Mol Genet. 2011; 20 : 4840– 50 . 37. Medina AE . Foetal alcohol spectrum disorders and abnormal 19. Wright AF , Chakarova CF , Abd El-Aziz MM , neuronal plasticity. Neuroscientist. 2011; 17 : 274 – 87 . Bhattacharya SS . Photoreceptor degeneration: genetic and 38. Nayak RB , Murthy P . Foetal alcohol spectrum disorder. mechanistic dissection of a complex trait. Nat Rev Genet. Indian Pediatr. 2008; 45 : 977 – 83 . 2010; 11 : 273 – 84 . 39. Alarc ó n Allen A , Baquero-Artigao F . Review and guidelines 20. Sanlaville D, Verloes A . CHARGE syndrome: an update . Eur on the prevention, diagnosis and treatment of post-natal J Hum Genet. 2007; 15 : 389 – 99 . cytomegalovirus infection. An Pediatr (Barc). 2011; 74 : 52 . 21. Blake KD , Prasad C . CHARGE syndrome. Orphanet J Rare 40. Pinillos-Pisó n R , Llorente-Cereza MT , L ó pez-Pisó n J , Dis. 2006; 1 : 34 . P é rez-Delgado R , Lafuente-Hidalgo M , Mart í nez-Sapiñ á A , 22. Sensi A , Ceruti S , Trevisi P , Gualandi F , Busi M , Donati I , et al . Congenital infection by cytomegalovirus. A review of et al . LAMM syndrome with middle ear dysplasia associated our 18 years ’ experience of diagnoses . Rev Neurol . 2009; with compound heterozygosity for FGF3 mutations. Am J 48 : 349 – 53 . Med Genet A. 2011; 155 : 1096– 101 . 41. Swanson EC , Schleiss MR . Congenital cytomegalovirus 23. Bekheirnia MR , Reed B , Gregory MC , McFann K , infection: new prospects for prevention and therapy. Pediatr Shamshirsaz AA , Masoumi A , et al . Genotype-phenotype cor- Clin North Am. 2013 ; 60 : 335 – 49 .

For personal use only. relation in X-linked Alport syndrome. J Am Soc Nephrol. 42. Parmar H , Ibrahim M . Paediatric intracranial infections. 2010; 21 : 876 – 83 . Neuroimaging Clin N Am. 2012; 22 : 707 – 25 24. Wang Q , Xue Y , Zhang Y , Long Q , Asan , Yang F , et al . Genetic 43. Buonsenso D, Serranti D, Gargiullo L , Ceccarelli M , basis of Y-linked hearing impairment. Am J Hum Genet. Ranno O, Valentini P . Congenital cytomegalovirus infection: 2013; 92 : 301 – 6 . current strategies and future perspectives. Eur Rev Med 25. Santarelli R , Starr A , del Castillo I , Huang T , Scimemi P , Pharmacol Sci. 2012; 16 : 919 – 35 . Cama E , et al . Presynaptic and postsynaptic mechanisms 44. Plosa EJ , Esbenshade JC , Fuller MP , Weitkamp underlying auditory neuropathy in patients with mutations in JH . Cytomegalovirus infection. Pediatr Rev. 2012; 33 : the OTOF or OPA1 gene. Audiol Med. 2011; 9 : 59 – 66 . 156 – 63 . 26. Santarelli R , del Castillo I , Rodr í guez-Ballesteros M , Scimemi 45. del Pizzo J . Focus on diagnosis: congenital infections (TORCH). P , Cama E , Arslan E , et al . Abnormal cochlear potentials from Pediatr Rev. 2011; 32 : 537 – 42 . deaf patients with mutations in the otoferlin gene. J Assoc Res 46. Lazzarotto T , Guerra B , Gabrielli L , Lanari M , Landini MP . Otolaryngol. 2009; 10 : 545 – 56 . Update on the prevention, diagnosis and management of 27. Huang T , Santarelli R , Starr A . Mutation of OPA1 gene cytomegalovirus infection during pregnancy. Clin Microbiol

Hearing, Balance and Communication Downloaded from informahealthcare.com by 217.148.121.9 on 08/19/13 causes deafness by affecting function of auditory nerve Infect. 2011; 17 : 1285– 93 . terminals . Brain Res. 2009; 1300: 97 – 104 . 47. Bovo R , Ciorba A , Martini A . The diagnosis of autoimmune 28. Santarelli R, Scimemi P, dal Monte E, Genovese E, inner ear disease: evidence and critical pitfalls . Eur Arch Arslan E . Auditory neuropathy in systemic sclerosis: a speech Otorhinolaryngol. 2009; 266 : 37 – 40 . perception and evoked potential study before and after 48. Bovo R , Ciorba A , Trevisi P , Aimoni C , Cappiello L , cochlear implantation. Eur Arch Oto-Rhino-Laryngol. 2006; Castiglione A , et al . Cochlear implant in Cogan syndrome. 263: 809 – 15 . Acta Otolaryngol. 2011; 131 : 494 – 7 . 29. Zadro C , Ciorba A , Fabris A , Morgutti M , Trevisi P , 49. Alenzi FQ , Salem ML , Alenazi FA , Wyse RK . Cellular and Gasparini P , et al . Five new OTOF gene mutations and auditory molecular aspects of Goodpasture syndrome. Iran J Kidney neuropathy. Int J Pediatr Otorhinolaryngol. 2010; 74 : 494 – 8 . Dis. 2012; 6 : 1 – 8 . 30. K ü ry S , Ramaekers V , B ézieau S , Wolf B . Clinical utility gene 50. Ottaviano G , Calzolari F , Martini A . Goldenhar syndrome in card for biotinidase deﬁ ciency. Eur J Hum Genet. 2012 ; 20: association with agenesia of the internal carotid artery. Int J doi: 10.1038/ejhg.2012.28. Epub 2012 Feb 29. Pediatr Otorhinolaryngol. 2007; 71 : 509 – 12 . 31. McHugh RK , Friedman RA . Genetics of hearing loss: allel- 51. Martini A , Calzolari F , Sensi A . Genetic syndromes involving ism and modiﬁ er genes produce a phenotypic continuum. hearing . Int J Pediatr Otorhinolaryngol. 2009 ; 73(Suppl 1): Anat Rec A Discov Mol Cell Evol Biol. 2006; 288 : 370 – 81 . 2 – 12 .

View publication stats ORIGINAL RESEARCH

A Systematic Review of Telehealth Applications in Audiology

De Wet Swanepoel, Ph.D.,1,2 and James W. Hall, III, Ph.D.3,1 reliability and effectiveness of telehealth applications compared to conventional methods. The limited information on patient percep- 1Department of Communication Pathology, University of Pretoria, tions reveal mixed findings and require more specific investigations, Pretoria, South Africa. especially post facto surveys of patient experiences. Tele-audiology 2Callier Center for Communication Disorders, School for holds significant promise in extending services to the underserved Behavioral and Brain Sciences, University of Texas at communities but require considerable empirical research to inform Dallas, Texas. future implementation. 3 Department of Communicative Disorders, University of Florida, Gainesville, Florida. Introduction he field of audiology encompasses prevention, assessment, and rehabilitation of hearing, auditory function, balance, Abstract and other related systems.1,2 With an estimated 642 million Hearing loss is a pervasive global healthcare concern with an esti- T people in the world affected to a mild or greater degree, and mated 10% of the global population affected to a mild or greater 278 million to a moderate and greater degree, hearing loss is clearly a degree. In the absence of appropriate diagnosis and intervention it significant global healthcare concern3 with pervasive and far– can become a lifelong disability with serious consequences on the reaching consequences. If not identified and treated early, children quality of life and societal integration and participation of the af- with hearing loss may suffer lifelong disability due to developmental fected persons. Unfortunately, there is a major dearth of hearing delays in language, literacy, academic achievement, and social well- healthcare services globally, which highlights the possible role of being.4,5 Hearing loss in adults tends to isolate and stigmatize them, telehealth in penetrating the underserved communities. This study leading to poor social participation and severely restricting voca- systematically reviews peer-reviewed publications on audiology- tional opportunities, as evidenced by significantly higher under- and related telehealth services and patient=clinician perceptions unemployment.6 Hearing loss is therefore reported as one of the most regarding their use. Several databases were sourced (Medline, significant contributors to the global burden of disease.7 SCOPUS, and CHINAL) using different search strategies for optimal Audiological diagnosis and intervention for children and adults coverage. Though the number of studies in this field are limited with hearing loss offer the possibility of excellent outcomes as op- available reports span audiological services such as screening, di- posed to the negative consequences of undetected and undiagnosed agnosis, and intervention. Several screening applications for popu- hearing loss without intervention services.8,9 The problem in pro- lations consisting of infants, children, and adults have demonstrated viding the necessary services, however, is the shortage of audiological the feasibility and reliability of telehealth using both synchronous professionals and services in the majority of regions in the world.10,11 and asynchronous models. The diagnostic procedures reported, in- Even in developed countries like the United States and Australia, rural cluding audiometry, video-otoscopy, oto-acoustic emissions, and and remote communities may not be able to access the necessary auditory brainstem response, confirm clinically equivalent results for hearing healthcare services. Telehealth applications in audiology may remote telehealth-enabled tests and conventional face-to-face ver- offer some solutions to the mismatch in the apparent need for services sions. Intervention studies, including hearing aid verification, and the limited capacity to deliver services.12 Using information and counseling, and Internet-based treatment for tinnitus, demonstrate communication technology in healthcare, as implied in telehealth,

DOI: 10.1089=tmj.2009.0111 ª MARY ANN LIEBERT, INC. . VOL. 16 NO. 2 . MARCH 2010 TELEMEDICINE and e-HEALTH 181 SWANEPOEL AND HALL

holds significant promise in improving healthcare access, quality A varied search strategy was employed to extract relevant peer- of service delivery, and the effectiveness and efficiency of ser- reviewed reports in English from several databases as illustrated in vices. Employing different models of telehealth service delivery in Appendix I. The Medline database was searched using three distinct audiological practice, such as synchronous (real-time), asynchronous strategies: (1) using MeSH terms to search for reports related to au- (store-and-forward), and hybrid models, may improve the reach of diology and telemedicine, (2) searching for audiology-related reports audiological services to underserved communities globally.13 in telemedicine journals, (3) searching for telemedicine-related re- Professional bodies in audiology have proposed tele-audiology as ports in audiology-related journals. The SCOPUS database, which a valid means of delivering services, but more studies are necessary to also covers Medline, was searched using a combination of terms ensure these services are comparable to face-to-face service provi- related to audiology and telemedicine occurring in the same report. sion.14,15 The aim of this study was to review the current body The third database searched was CINAHL, for which main subject of peer-reviewed publications on available empirical studies of words relating to audiology and telemedicine were used as identifi- audiology-related telehealth services and patient=clinician percep- ers. This multipronged approach covering multiple databases with tions regarding its use. variations in search strategy was employed to maximize the coverage and to cross-check results. Reference lists in the reports finally se- Materials and Methods lected for review were subsequently surveyed to identify any addi- To perform a systematic review of tele-audiology, a search was tional report applying to the scope of the study that was not obtained conducted for articles in peer-reviewed journals reporting empirical through the database searches. investigations related to audiological services with a telehealth The reports selected for review were carefully studied and subse- component or patient=clinician perceptions of telehealth for audio- quently categorized according to four criteria specifying their scope logical services. There was overlap between audiological and otolo- of relevance: (1) audiological screening, (2) audiological diagnosis, gical practices in the area of otoscopic examinations. Any report (3) audiological intervention, and (4) patient=clinician perceptions. within the scope of the review, whether related to otology or audiology, was therefore considered for inclusion. The exceptions in- Results cluded cases where a microscope=endoscope was used at the remote Table 1 describes the search results according to the procedural site, because audiologists typically do not use these devices; studies steps applied. We reviewed the abstracts of 261 reports to determine if specifically related to medical diagnoses of ear disease; and reports they were in any way relevant to the scope of the study. Sixty-three providing comparison between devices. All relevant reports pub- reports indicated some relevance and these were subsequently re- lished until May 31, 2009, were included. viewed. A total of 25 articles were identified to be directly within the

Table 1. Description of Search Results Identifying Reports for Inclusion PROCEDURAL STEPS NUMBER OF REPORTS DESCRIPTION (a) Database search results 386 3 databases (Medline, SCOPUS, and CINAHL); 5 search strategies

(b) Database search results—duplicates omitted 261 125 duplicates from the 5 searches were omitted

(d) Database reports within scope of review 25 39 reports were not directly relevant to scope of review.

(e) Additional reports within scope of review 1 Reports included from survey of reference lists; only those reports not contained in database search

(f ) Final reports for review 26 Articles constituting the systematic review

182 TELEMEDICINE and e-HEALTH MARCH 2010 TELEHEALTH APPLICATIONS IN AUDIOLOGY

scope of the systematic review. A survey of the reference lists in these colleagues16 reported on the development and validation of a speech- articles revealed a single additional report not identified by the da- in-noise screening procedure using triple digits and an adaptive tabase searches, which brought the number of reports for final review procedure that can be used reliably over the telephone and computer to 26. These reports, which date from 1997 to 2009, are briefly headsets. They subsequently reported on a national self-screening summarized in Appendix II according to authors and year of publi- program in the Netherlands using this test to screen large numbers of cation, journal, category, study type, connection=model, subjects, adolescents and adults using the telephone (n ¼ 6,351) or Internet procedures, and conclusions. (n ¼ 30,260).17 The participation in this program was high, but the The reports were divided into four categories as illustrated in elderly population used the telephone-based test in preference to Table 2. The majority of reports were concerned with diagnosis, Internet-based screening. The compliance of the Internet-based test while two exclusively considered patient perceptions related to tele- was compromised because few people (31%) used headphones, which audiology. Three of the reports on intervention also included a are necessary for a more reliable and valid screening. section on patient perceptions. A variety of audiological procedures A self-test, Internet-based, pure tone audiometry screening pro- or techniques were used across the categories of screening, diag- cedure was reported by Bexelius and colleagues.18 This proof-of- nosis, and intervention in a combination of synchronous, asyn- concept study screened patients by determining threshold frequen- chronous, and hybrid models. cies between 500 and 8,000 Hz as against a more conventional screening criterion that assesses hearing at a preset intensity across a Discussion limited range of frequencies. This study tested the members of a

AUDIOLOGICAL SCREENING hunting organization and reported poor participation in the self-test, Five reports on audiological screening using telehealth configu- but demonstrated that Internet-based hearing screening tests can be rations were identified. Screening procedures included pure tone performed. Self-test, Internet-based screening is, however, con- audiometry, tympanometry, oto-acoustic emissions (OAE), and au- founded by the lack of control over environmental variables at the tomated auditory brainstem response (AABR) used in populations remote test site, such as noise levels and transducer type, which varying from infants to adults. Three reports described self-test makes these procedures no better than a preliminary screening. Va- screening procedures; two of these used speech-in-noise screening lidated procedures such as the triplet speech-in-noise test used in the and one described pure tone audiometric screening.16–18 Smits and Netherlands may be more useful. All these procedures may ultimately

Table 2. Summary of Tele-Audiology Reports According to Category, Populations, and Models CATEGORIES NO. OF REPORTS STUDY POPULATIONS PROCEDURES=TECHNIQUES TELEHEALTH MODELS Screening 5 Infants, children, and adults Video-otoscopy, immittance, OAE, AABR, Synchronous, asynchronous, audiometry, speech-in-noise hybrid, and self-test

Diagnosis 12 Children and adults Video-otoscopy, audiometry Synchronous and (AC and BC), HINT, ABR, asynchronous intraoperative monitoring, balance testing

Intervention 7 Adults HA fitting and verification, CI Synchronous and programming, tinnitus therapy, asynchronous HA counseling

Patient perceptions 2(3a) Adult clinic patients, tinnitus Questionnaires Synchronous and patients, cochlear implant asynchronous mapping patients aReports of audiological intervention also including patient perceptions. AABR, automated auditory brainstem response; ABR, auditory brainstem response; AC, air conduction; BC, bone conduction; CI, cochlear implant; HA, hearing aid; HINT, Hearing-in-Noise-Test; OAE, oto-acoustic emissions.

ª MARY ANN LIEBERT, INC. . VOL. 16 NO. 2 . MARCH 2010 TELEMEDICINE and e-HEALTH 183 SWANEPOEL AND HALL

improve public awareness regarding the risks of hearing loss and the handheld device (unspecified distance and bandwidth). Audiologists importance of hearing healthcare. were blind to results from the remote or face-to-face settings, and the The other two reports on audiological screening compared face- testing order was counterbalanced. Mean differences between to-face screening with remote screening of infants using AABR and thresholds obtained with the two methods varied by no more than OAE19 and of young children using otoscopy, pure tone audiometry, 1.3 dB for air and 1.2 dB for bone conduction, and Pearson correla- and tympanometry.20 A synchronous setup using videoconferencing tion coefficients across frequencies varied between 0.82 and 0.97. and application sharing was used to screen infants remotely. The on- Statistically there was no significant difference between test results site audiologist prepared the tests and conducted two face-to-face from the remote versus face-to-face methods.22 assessments, while the remote audiologist conducted one test. The In a similar experimental setup, Choi and colleagues23 compared tests were randomized and testers were blind to the results. Telehealth face-to-face audiometry using a PC-based audiometer with remote screening provided exactly the same results as face-to-face screen- testing (1 km distance) over the Internet (broadband, unspecified ing, and comparison of distortion product OAE (DPOAE) amplitudes bandwidth) on 12 adult subjects with normal hearing capabilities. showed these were equivalent within typical test-retest reliability Threshold comparisons revealed a difference of more than 5 dB in limits. The second report compared telehealth hearing screening with only 10.7% of cases (18=168) and none differed by more than 15 dB. on-site screening of 32 children in a rural elementary school. Oto- Comparisons for this same sample between face-to-face audiometry scopic examination and pure tone testing were conducted synchro- on the PC-based system and on a conventional audiometer revealed a nously, while tympanometry was interpreted asynchronously in a smaller percentage (3.7%) of differences exceeding 5 dB. The fourth store-and-forward model. The testing was counterbalanced to avoid study also used a PC-based audiometer remotely controlled via ap- an order effect, and examiners were blinded to each other’s results. plication sharing software with interactive videoconferencing for The interpretation of otoscopy and tympanometry were identical, communication to test 30 adult subjects.24 Audiologists were blind to and screening responses on pure tone audiometry were perfectly results in the face-to-face and remote test methods. The order of tests correlated in 188 of 193 frequencies tested. These differences trans- were rotated to avoid an order effect, and remote testing was con- lated to four false-positive and one false-negative screen results ducted from a distance of 1,100 km. No statistically significant dif- using telehealth. However, in the context of the large number of ference was noted between the two methods, and the thresholds frequencies tested, this did not constitute a statistically significant corresponded within 5 dB of each other in 97% of cases. A comparison difference. The authors note that although similarly high test sensi- of face-to-face threshold values yielded 99% correspondence.24 tivity values were obtained for face-to-face and remote screening, The only speech audiometric procedure reported with relevance to the test specificity for pure tone audiometric screening may be telehealth has been the Hearing-in-Noise-Test (HINT). A comparison slightly less for a telehealth setup. of face-to-face evaluations to remote testing through the same local area network and a different, more remote Internet connection was AUDIOLOGICAL DIAGNOSIS reported for a group of 20 adults.25 The means and standard deviation Four of the 12 reports on audiological diagnosis investigated for each test condition from both test sites were within the normative hearing evaluations using pure tone audiometry in a sound booth or data reported for HINT, except for one instance where the difference sound-treated room from remote locations.21–24 All studies were in means between tests sites was less than 1 dB, indicating the reli- performed on adult subjects using air conduction audiometry (250– ability of performing HINT via a telemedicine configuration. 8,000 Hz; octave frequencies), and one also used bone conduction Three studies of video-otoscopy facilitated through telemedicine audiometry (250–4,000 Hz; octave frequencies). Two of the four applications were included in the review, even though all examina- studies reported on the same data set. One of these was a preliminary tions were conducted by physicians as opposed to audiologists.26–28 report, and therefore only the second report was considered.21,22 This The first study compared the interpretation of face-to-face micro- study by Givens and Elangovan22 compared air (n ¼ 45) and bone scopic examinations of the ear canal and tympanic membrane to conduction (n ¼ 25) pure tone thresholds determined using conven- video-otoscopic still images of 40 subjects, including adults and tional face-to-face audiometry with thresholds determined through children.26 The still images were reviewed at 6 and 12 weeks post remote synchronous audiometry. The remote audiologist controlled face-to-face examination, and findings were compared between the the conventional audiometer through a control unit, which was ac- test conditions and between two independent examiners. Observa- cessed through the Internet from a remote personal computer (PC) or tions on video-otoscopic still images and microscopy were compa-

184 TELEMEDICINE and e-HEALTH MARCH 2010 TELEHEALTH APPLICATIONS IN AUDIOLOGY

rable (88% concordance), which corresponded to the concordance monitoring sessions followed by four sequential on-site monitoring between independent face-to-face examinations (84%). A follow-up sessions. Remote testing was easily performed and lasted 9 min on study found the concordance between video-otoscopic images of the average compared to 93 min required for on-site testing. tympanic membrane taken in remote clinics and in-person micro- Other reports include a remote consultation for a balance disorder scopic examinations for follow-up care in children aged 1–16 years and the use of online forms for tinnitus evaluations. Only one report (70 ears) following tympanostomy tube placement.27 Two otolaryn- was sourced in regard to balance assessment through telemedicine.31 gologists conducted the face-to-face examinations and also exam- This single case study demonstrated the feasibility and success of a ined the images at 8 and 14 weeks postexamination. Image quality remote consultation using a two-way digital video and audio network was rated adequate or better in 79% of cases, and the majority of for assessing a patient with benign positional vertigo. The use of poor-quality images (50%) were of 2-year-olds, who accounted for cameras allowed for viewing the patient’s eye movements, which were 26% of the total number of cases. Analyses revealed a high level of essential to the diagnosis. A report on the use of an online evaluation concordance between face-to-face microscopic examinations and form for anxiety and depression related to tinnitus was included as part corresponding image reviews. The authors concluded that video- of a diagnostic tinnitus assessment to measure the self-perceived effect otoscopy image reviews of the tympanic membrane are comparable of tinnitus on life activities and functioning.32 Online forms completed to an in-person examination for assessment and treatment in follow- by 157 adult patients were compared to questionnaires completed on up care for tympanostomy tubes.27 A similar study on 66 children paper and with pencil by other patients, revealing that online forms (127 ears) compared face-to-face otoscopy to digital images inter- provide meaningful and valid data. The Internet group data was mostly preted 1 month later, which revealed significant agreement equivalent although slightly higher, and no statistically significant ( p < 0.05) between clinically important observations. The agreement results were obtained. The authors suggest that the differences may be between otological recommendations from images and face-to-face due to less inhibition given the anonymity of an online form. examinations was also statistically significant ( p < 0.01), although the rates of referrals were 4–16% higher.28 A significant correlation AUDIOLOGICAL INTERVENTION was also reported between image quality and age of the subject, with A sequence of four reports on Internet-based cognitive behavioral better quality images generally reported for older children. self-help treatment for tinnitus was presented by the same research Reports of tele-audiology using objective measures of auditory group in Sweden.33–36 The treatment program was a self-help manual functioning have included DPOAE, ABR, and intraoperative moni- constructed following cognitive behavioral principles and included toring.24,29,30 A study investigating the correspondence between 10 components presented in six modules on a weekly basis for 6 DPOAE measures recorded remotely (through desktop sharing soft- weeks. This self-help program was presented on Web pages, and ware and interactive video) and face-to-face assessments in 30 adult weekly diaries were submitted to follow progress and give feedback. subjects demonstrated that there were essentially no differences be- Outcome measures included several questionnaires and ratings of tween the findings.24 An overall agreement of 97–99% was reported tinnitus-related handicap, reaction, anxiety, depression, and in- across frequencies (2,000, 2,500, 3,000, 4,000, and 5,100 Hz) and was somnia (e.g., Tinnitus Reaction Questionnaire and Tinnitus Handicap comparable to the agreement between face-to-face assessments, Inventory) conducted before treatment, after treatment, and at 1-year which was 97% on average. In a comparison between remote ABR follow-up. The first report compared a randomized controlled trial of recordings using desktop sharing software and face-to-face record- Internet-based cognitive behavior therapy to conventional cognitive ings in a group of 15 adults, comparable wave latencies within the behavioral therapy in a waiting-list control group for distress asso- clinically allowable range of variability were obtained.29 Recordings ciated with tinnitus.33 Participants receiving treatment via the In- included ABRs elicited with toneburst (500 and 3,000 Hz) and ternet improved to a significantly greater extent than the control broadband click stimuli presented at 55 and 75 dB. No significant group, with 29% demonstrating an improvement of at least 50% on effect as a result of different test sites was reported, and the results the Tinnitus Reaction Questionnaire as opposed to 4% in the control suggested that remote test was as reliable as face-to-face testing. group. However, dropout rate in the treatment group was much Remote intraoperative evaluation of the cochlear implant device higher, almost 51% compared to almost none in the control group. A and responses to electrical stimulation was recently reported as a single case report subsequently illustrated the process of Internet- time-saving, practical, and cost-efficient option.30 Desktop sharing based cognitive behavioral therapy.34 A follow-up nonrandomized software was used to conduct and time four sequential remote clinical study reported on the efficacy of Internet-based treatment in

ª MARY ANN LIEBERT, INC. . VOL. 16 NO. 2 . MARCH 2010 TELEMEDICINE and e-HEALTH 185 SWANEPOEL AND HALL

a sample of consecutive tinnitus patients.35 Significant reductions in Other rehabilitation components of audiological intervention fa- distress associated with tinnitus were evident, and at 3-month cilitated by telehealth include counseling and cochlear implant map- follow-up the patients had remained improved. The dropout rate was ping. A qualitative multiple case study described an Internet-based 30% and primarily attributed to time constraints. counseling program for new hearing aid users through daily e-mail Based on the feedback and clinical experience obtained from these interchanges for 1 month provided by an audiologist.40 The data were studies, the authors redeveloped the program to improve retention acquired from interviews, analyses of e-mail interchanges, and from and treatment outcomes and subsequently published a follow-up audiological files. Results indicated that this was a powerful com- controlled trial incorporating these changes.36 Main changes in- munication medium for observing changes in behavior and percep- cluded expanding the self-help text; having participants define their tion of new hearing aid users. The immediacy of e-mail enabled timely own treatment goals and set priorities for free time required for response to concerns. A randomized study recently compared on-site treatment before commencement; encouraging participants to plan cochlear implant programming to remote cochlear implant mapping homework assignments on the Web site; providing more detailed and in a group of five adults.41 Twelve remote cochlear implant mapping personalized instructions and registration sheets for printing; con- sessions and 12 face-to-face sessions were completed at four intervals. siderably expanding the Internet diaries for reporting homework Each interval was separated by 3 months in a randomized order with assignments; allowing participants to choose if, and when, to start performance evaluations after each of the initial 3-month intervals (all with some of the less general treatment tools; and ensuring that the subjects did not participate in the first level). Authors report that Web site was informative regarding expectations. Both treatment remote programming through application sharing proceeded without groups (Internet-based vs. group cognitive therapy) yielded signifi- incident and that no significant differences were evident between cant positive results with no significant differences in main outcome the programs established for each subject on each programming day measures. The results were relatively stable at 1-year follow-up. The (M-1, M-8, and M-16 values were used for comparison; M ¼ the most attrition rate was lower than for previous Internet-based treatments comfortable level; 1, 8, and 16 denote the electrode number). In for tinnitus,35 and the method was 1.7 times as cost-effective as addition, remote and standard recorded threshold neural response conventional group treatment. imaging values were very similar (not tested statistically). The per- The only peer-reviewed empirical report on hearing aid fitting and formance of subjects on either a standard or a remote program after verification was recently published by Ferrari and Bernardez- 3 months also showed no statistically significant difference in free- Braga.37 The authors compared probe microphone measurements field threshold values (0.25, 0.5, 1, 2, and 4 kHz) or in disyllabic open conducted remotely to verify hearing aid performance to face-to- word test scores. Therefore, no significant differences between remote face measurements in a group of 60 adults. This was facilitated by a and face-to-face cochlear implant programming were evident. telehealth setup that included application sharing software, interactive desktop videoconferencing, and a facilitator at the remote site PATIENT AND CLINICIAN PERCEPTIONS to place the probe and make necessary adjustments. The remote Five reports contained some mention of patient perceptions, of measures significantly correlated with face-to-face measures at all which only two exclusively surveyed patient perceptions and atti- frequencies and the differences varied by only 0–2.2 dB, which cor- tudes toward audiological practices related to telehealth.35,36,41–43 responds to clinically accepted between-measure variability on probe The first surveyed 116 adult patients attending four audiological microphone verification. Some previous reports have, however, clinics in Australia regarding their attitudes toward telemedicine and discussed the remote fitting and verification of hearing aids through willingness to make use of tele-audiological services.42 Although telehealth, but these were either not published in a peer-reviewed 45% of respondents had used the Internet for health-related matters, journal or did not describe an empirical study.38,39 Wesendahl38 only 25% had been aware of telemedicine previously. Overall, 32% described the possibility of initial fitting, fine tuning, and follow-up were willing to use telemedicine, 10% would sometimes be willing, for programmable hearing aids through telehealth applications using 28% were unsure, and 30% were not willing. These findings indicate a special GSM handheld device (combination of a mobile phone and a that tele-audiology is still a foreign concept to many patients espe- hearing programmer) in real acoustic environments. Subsequently, cially in this sample, where more than 46% of respondents were 65 Ferrari39 reported on the successful remote fitting of hearing aids years and older.42 The limitation of the study was that respondents through application sharing software and interactive desktop vid- had not experienced tele-audiology and were therefore only com- eoconferencing in a group of adults. menting on their perceived notions of a telemedicine consultation.

186 TELEMEDICINE and e-HEALTH MARCH 2010 TELEHEALTH APPLICATIONS IN AUDIOLOGY

In another study, 202 adult respondents with hearing loss from the and intervention. Several screening applications for populations United Kingdom, Germany, and the Netherlands indicated their pref- consisting of infants, children, and adults have demonstrated the erence for a self-test screening via a questionnaire, telephone, or the feasibility and reliability of screening facilitated through telehealth Internet.43 The respondents were generally enthusiastic about the using both synchronous and asynchronous models. The diagnostic prospect of self-screening but generally preferred a questionnaire to procedures reported, including audiometry, video-otoscopy, OAE, the Internet, which was preferred to the telephone. The majority of and ABR, confirm clinically equivalent results for remote, telehealth- subjects were older than 65 years and were also less likely to be positive enabled tests compared to conventional face-to-face versions. Fur- about Internet-based screening for hearing. Interestingly, respondents ther, the few reported intervention studies using telehealth, such as reported trusting results from a questionnaire-based screening more than those from an objective screening procedure although there was Table 3. Research and Development Priorities sufficiently high trust in objective procedures to fulfill the intention of for Tele-Audiology a screening test—to seek medical assistance. Responses differed among Validation of tele-audiology diagnostic procedures (particularly for the three countries, but the vast majority of respondents found the pediatric populations) prospect of having their hearing screened from home acceptable.43 Video-otoscopy by audiologists Other reports were primarily intervention-related with a component concerning patient perceptions included. In a report on remote Audiometry (pure tone and speech) cochlear implant programming, subjects indicated the same satis- Immittance (tympanometry and acoustic reflexes) faction on the remote and face-to-face sessions, but one in three Oto-acoustic emissions remote sessions lasted too long as opposed to face-to-face sessions.41 Also, in 2 of the 12 remote sessions subjects reported some discomfort Auditory evoked potentials and requested the stimulation to be stopped as opposed to the face- Intraoperative monitoring to-face sessions. Case history information In a randomized clinical trial of a self-help, Internet-based treatment program for tinnitus based on cognitive behavioral therapy Validation of tele-audiology intervention services (particularly principles, patients were surveyed before treatment commenced on for pediatric populations) their beliefs about whether the treatment will help them or not Counseling and follow-up (treatment credibility).35 Surprisingly, no differences were found in Hearing aid fitting, verification, and troubleshooting patient preferences or credibility ratings between traditional (face- to-face) and self-help Internet treatments. In a follow-up clinical trial Cochlear implant mapping and troubleshooting with some adjustments made to the Internet-based program, the Rehabilitation programs credibility rating for the Internet treatment was significantly lower Establishing best practice protocols and service delivery models than for the conventional group-based cognitive behavioral treat- employing synchronous and asynchronous models ment.36 This was attributed to the timing of the questionnaire ad- Integration of automated test procedures for store-and-forward ministration, which was collected before randomization when applications in tele-audiology participants had less knowledge about the treatment they were to receive (as opposed to the previous clinical trial). In addition, par- Development of novel tele-audiology specific devices (e.g., monitoring of environmental noise remotely) ticipants were asked to rate the credibility of both treatments instead of rating only the assigned treatment. Further, the authors propose Determining patient and clinician perceptions and experiences with that the actual importance of these findings may be questionable tele-audiology because treatment credibility and preference did not affect outcome. Audiological training and mentoring through telehealth

Establishing minimum equipment, bandwidth, and personnel Conclusions requirements for synchronous and asynchronous audiological procedures Peer-reviewed empirical studies on tele-audiology are limited in Cost-effectiveness studies comparing conventional and tele-audiology services number, but the scope of utilization of this technology spans various areas of audiological service delivery including screening, diagnosis,

ª MARY ANN LIEBERT, INC. . VOL. 16 NO. 2 . MARCH 2010 TELEMEDICINE and e-HEALTH 187 SWANEPOEL AND HALL

hearing aid verification and Internet-based treatment for tinnitus, 2. American Speech-Language-Hearing Association. 2004. Scope of practice in demonstrate reliable and effective applications of telehealth com- audiology [scope of practice]. Available at www.asha.org=docs=pdf=SP2004- 00192.pdf (last accessed June 24, 2009). pared to conventional face-to-face methods. The very limited in- 3. WHO. 2006. Primary ear and hearing care training manuals. Geneva. Available formation on patient perceptions reveal mixed findings and require at www.who.int=pbd=deafness=activities=hearing_care=en=index.html (last more specific investigations, especially post facto surveys of patient accessed June 11, 2009). experiences. To date, no reports describe audiology clinicians’ per- 4. Yoshinaga-Itano C, Sedey A, Coulter D, Mehl A. Language of early- and later- ceptions of tele-audiology services. identified children with hearing loss. Pediatrics 1998;102:1161–1171. Although initial findings are promising, significant research on 5. Moeller MP, Tomblin JB, Yoshinaga-Itano C, Connor CM, Jerger S. Current state audiological practice and education facilitated through telehealth is of knowledge: Language and literacy of children with hearing impairment. Ear Hear 2007;28:740–753. required as highlighted by the limitations in the depth and breadth of current reports. The majority of these studies on audiological diagnosis 6. Olusanya BO, Ruben RJ, Parving A. Reducing the burden of communication disorders in the developing world: An opportunity for the millennium and intervention were conducted on adults, and many audiological development project. JAMA 2006;296:441–444. areas of practice have not been applied through telehealth means. No 7. WHO. 2008. The global burden of disease: 2004 update. Geneva. Available at protocols and service delivery models are currently specified for spe- www.who.int=healthinfo=global_burden_disease=GBD_report_2004 cific populations, and the current understanding of patient and clini- update_full.pdf (last accessed May 14, 2009). cian perceptions is poor and incomplete. Further, important issues such 8. Yoshinaga-Itano C. Levels of evidence: Universal newborn hearing screening as financial costs and resources for tele-audiology within existing (UNHS) and early hearing detection and intervention systems (EHDI). J Commun Disord 2004;37:451–465. healthcare infrastructures and models remain to be addressed by 9. Olusanya BO. Self-reported outcomes of aural rehabilitation in a developing systematic investigations and cost-analysis studies. Current reports are country. Int J Audiol 2004;43:563–571. almost exclusively from research-funded studies and not from existing 10. Goulios H, Patuzzi RB. Audiology education and practice from an international service delivery mechanisms where healthcare funding models are perspective. Int J Audiol 2008;47:647–664. employed. Although initial evidence suggests that significant cost 11. Fagan JJ, Jacobs M. Survey of ENT services in Africa: Need for a comprehensive savings are possible across the scope of audiological services, these intervention. Global Health Action 2009;doi:10.3402=gha.v2i0.1932. must be quantified and potential funding sources=models identified. In 12. Wootton R. The future use of telehealth in the developing world. In: Wootton R, developing countries, where medical resources are scarce and tele- Patil NG, Scott RE, Ho K, eds. Telehealth in the developing world. London: Royal health promises cost-efficient access, such studies are particularly Society of Medicine Press Ltd., 2009:299–308. important, along with models of funding these services.44 Table 3 13. Swanepoel D, Clark JL, Koekemoer D, Hall III JW, Krumm M, Ferrari DV, McPherson B, Olusanya BO, Mars M, Russo I, Barajas JJ. Telehealth in summarizes the priority areas for future research and development in audiology—The need and potential to reach underserved communities. Int J tele-audiology to address some of these limitations. Audiol (in press). As a field in its infancy much work remains to be done to develop 14. American Academy of Audiology. 2008. The use of telehealth=telemedicine and validate tele-audiology as a means of delivering services and for to provide audiology services. Available at www.audiology.org=advocacy= providing training and education. The global absence of hearing publicpolicyresolutions=Documents=TelehealthResolution200806.pdf (last accessed May 21, 2009). healthcare for the vast majority of people with hearing loss raises a 15. American Speech-Language-Hearing Association. 2005. Audiologists providing moral obligation to pursue ways of penetrating the underserved clinical services via telepractice: Technical report [technical report]. Available at communities with audiological services. Tele-audiology holds the www.asha.org=policy (last accessed May 21, 2009). unique promise of bridging this gap by delivering services through 16. Smits C, Kapteyn T, Houtgast T. Development and validation of an automatic the expanding reach of global connectivity. speech-in-noise screening test by telephone. Int J Audiol 2004;43:15–28. 17. Smits C, Merkus P, Houtgast T. How we do it: The Dutch functional hearing Disclosure Statement screening tests by telephone and Internet. Clin Otolaryngol 2006;31: 436–440. No competing financial interests exist. 18. Bexelius C, Honeth L, Ekman A, Eriksson M, Sandin S, Bagger-Sjo¨ba¨ck D, Litton JE. Evaluation of an Internet-based hearing test—comparison with REFERENCES established methods for detection of hearing loss. J Med Internet Res 2008;10:e32. 1. American Academy of Audiology. 2004. Scope of practice. Available at www.audiology.org=resources=documentlibrary=Pages=ScopeofPractice.aspx 19. Krumm M, Huffman T, Dick K, Klich R. Telemedicine for audiology screening of (last accessed August 5, 2009). infants. J Telemed Telecare 2008;14:102–104.

188 TELEMEDICINE and e-HEALTH MARCH 2010 TELEHEALTH APPLICATIONS IN AUDIOLOGY

20. Lancaster P, Krumm M, Ribera J, Klich R. Remote hearing screenings via 36. Kaldo V, Levin S, Widarsson J, Buhrman M, Larsen HC, Andersson G. telehealth in a rural elementary school. Am J Audiol 2008;17:114–122. Internet versus group cognitive-behavioral treatment of distress associated with tinnitus: A randomized control trial. Behav Ther 2008; 21. Givens GD, Blanarovich A, Murphy T, Simmons S, Blach D, Elangovan S. 39:348–359. Internet-based tele-audiometry system for the assessment of hearing: A pilot study. Telemed J E Health 2003;9:375–378. 37. Ferrari DV, Bernardez-Braga GR. Remote probe microphone measurement to verify hearing aid performance. J Telemed Telecare 2009;15: 22. Givens G, Elangovan S. Internet application to tele-audiology—‘‘Nothin’ but 122–124. net.’’ Am J Audiol 2003;12:50–65. 38. Wesendahl T. Hearing aid fitting: Application of telemedicine in audiology. 23. Choi J, Lee H, Park C, Oh S, Park K. PC-based tele-audiometry. Telemed J E Int Tinnitus J 2003;9:56–58. Health 2007;13:501–508. 39. Ferrari DV. Remote programming and verification as a mean to improve 24. Krumm M, Ribera J, Klich R. Providing basic hearing tests using remote quality of hearing aid fitting. In: Rasmussen AN, Paulsen T, Andersen T, computing technology. J Telemed Telecare 2007;13:406–410. Larsen CB, eds. Hearing aid fitting. Proceedings of the 21st Danavox 25. Ribera J. Interjudge reliability and validation of telehealth applications of the Symposium Centertryk, 2006:531–544. Hearing in Noise Test. Semin Hear 2005;26:13–18. 40. Laplante-Le´vesque A, Kathleen Pichora-Fuller M, Gagne J-P. Providing an 26. Patricoski C, Kokesh J, Ferguson AS, Koller K, Zwack G, Provost E, Holck P. A Internet-based audiological counselling programme to new hearing aid users: comparison of in-person examination and video otoscope imaging for A qualitative study. Int J Audiol 2006;45:697–706. tympanostomy tube follow-up. Telemed J E Health 2003;9:331–344. 41. Ramos A, Rodriguez C, Martinez-Beneyto P, Perez D, Gault A, Falcon JC, Boyle P. 27. Kokesh J, Ferguson AS, Patricoski C, Koller K, Zwack G, Provost E, Holck P. Digital Use of telemedicine in the remote programming of cochlear implants. Acta images for postsurgical follow-up of tympanostomy tubes in remote Alaska. Otolaryngol 2009;129:533–540. Otolaryngol Head Neck Surg 2008;139:87–93. 42. Eikelboom RH, Atlas MD. Attitude to telemedicine, and willingness to use it, in 28. Eikelboom RH, Mbao MN, Coates HL, Atlas MD, Gallop MA. Validation of tele- audiology patients. J Telemed Telecare 2005;11:S22–S25. otology to diagnose ear disease in children. Int J Pediatr Otorhinolaryngol 43. Koopman J, Davey E, Thomas N, Wittkop T, Verschuure H. How should hearing 2005;69:739–744. screening tests be offered? Int J Audiol 2008;47:230–237. 29. Towers AD, Pisa J, Froelich TM, Krumm M. The reliability of click-evoked and 44. Swanepoel DW, Olusanya BO, Mars M. Hearing healthcare delivery in frequency-specific auditory brainstem response testing using telehealth sub-Saharan Africa—Any role for tele-audiology. J Telemed Telecare 2009; technology. Semin Hear 2005;26:19–25. doi:jtt.2009.009003. 30. Shapiro WH, Huang T, Shaw T, Roland JT, Lalwani AK. Remote intraoperative monitoring during cochlear implant surgery is feasible and efficient. Otol Neurotol 2008;29:495–498. 31. Virre E, Warner D, Balch D, Nelson JR. Remote medical consultation for Address correspondence to: vestibular disorders: Technological solutions and case report. Telemed J De Wet Swanepoel, Ph.D. 1997;3:53–58. Department of Communication Pathology 32. Andersson G, Kaldo-Sandstro¨m V, Stro¨m L, Stro¨mgren T. Internet administration University of Pretoria of the hospital anxiety and depression scale in a sample of tinnitus patients. J Psychosom Res 2003;55:259–262. C=O Lynnwood & Roper Pretoria 0002 33. Andersson G, Stro¨mgren T, Stro¨m L, Lyttkens L. Randomized controlled trial of Internet-based cognitive behavior therapy for distress associated with tinnitus. South Africa Psychosom Med 2002;64:810–816. E-mail: [email protected] 34. Andersson G, Kaldo V. Internet-based cognitive behavioral therapy for tinnitus. J Clin Psychol 2004;60:171–178. Received: August 5, 2009 35. Kaldo-Sandstro¨m V, Larsen HC, Andersson G. Internet-based cognitive- behavioral self-help treatment of tinnitus: Clinical effectiveness and predictors Revised: September 3, 2009 of outcome. Am J Audiol 2004;13:185–192. Accepted: September 3, 2009

ª MARY ANN LIEBERT, INC. . VOL. 16 NO. 2 . MARCH 2010 TELEMEDICINE and e-HEALTH 189 SWANEPOEL AND HALL

APPENDIX I: Databases and Search Strategy Details DATABASE SEARCH STRATEGY IDENTIFIERS RESULTS LIMITERS Medline MeSH terms related to telehealth and Telehealth MeSH terms: 107 English audiology for the same article ‘‘telemedicine’’ OR ‘‘computer communication networks’’ Audiology MeSH terms: ‘‘Diagnostic Techniques, Otological’’ OR ‘‘audiology’’ OR ‘‘hearing disorders’’ OR ‘‘sensory aids’’ OR ‘‘rehabilitation of hearing impaired’’ Medline Audiology-related terms occurring in Telehealth-related journals: 33 English all fields of telemedicine-related journals Any journal with the syllable ‘‘tele’’ in the title (8 journals) Audiology-related terms: ‘‘audiolog’’a OR ‘‘audiometr’’a OR ‘‘hearing’’ OR ‘‘otoscopy’’ OR ‘‘auditory’’ OR ‘‘vestibular’’ OR ‘‘cochlear’’ OR ‘‘ear’’ OR ‘‘tympanometry’’ OR ‘‘immittance’’ OR ‘‘otoacoustic’’ OR ‘‘tinnitus’’ OR ‘‘hyperacusis’’ Medline Telehealth-related terms occurring in all Audiology-related journals: 128 English a

fields of audiology-related journal articles Any journal containing ‘‘oto’’ OR ‘‘audiolog’’ OR ‘‘ear’’ (45 journals) OR ‘‘hearing’’ OR ‘‘communication disorders’’ in the title Telehealth-related terms: ‘‘tele-audiology’’ OR ‘‘telehearing’’ OR ‘‘telehealth’’ OR ‘‘telemedicine’’ OR ‘‘e-health’’ OR ‘‘telepractice’’ OR ‘‘Internet’’ SCOPUS Telehealth- and audiology-related terms Telehealth-related terms: 101 English; occurring in the title, abstract, ‘‘tele-audiology’’ OR ‘‘telehearing’’ OR ‘‘telehealth’’ exclude re- or keywords of articles OR ‘‘telemedicine’’ OR ‘‘e-health’’ OR ‘‘telepractice’’ views and Audiology-related terms: editorials ‘‘audiolog’’a OR ‘‘audiometr’’a OR ‘‘hearing’’ OR ‘‘otoscopy’’ OR ‘‘auditory’’ OR ‘‘vestibular’’ OR ‘‘cochlear’’ OR ‘‘ear’’ OR ‘‘tympanometry’’ OR ‘‘immittance’’ OR ‘‘otoacoustic’’ OR ‘‘tinnitus’’ OR ‘‘hyperacusis’’ CINAHL Telehealth- and audiology-related terms Telehealth-related terms: 29 English; peer occurring in main subject words of ‘‘tele-audiology’’ OR ‘‘telehearing’’ OR ‘‘telehealth’’ reviewed; re- articles OR ‘‘telemedicine’’ OR ‘‘e-health’’ OR ‘‘telepractice’’ search article Audiology-related terms: ‘‘audiolog’’a OR ‘‘audiometr’’a OR ‘‘hearing’’ OR ‘‘otoscopy’’ OR ‘‘auditory’’ OR ‘‘vestibular’’ OR ‘‘cochlear’’ OR ‘‘ear’’ OR ‘‘tympanometry’’ OR ‘‘immittance’’ OR ‘‘otoacoustic’’ OR ‘‘tinnitus’’ OR ‘‘hyperacusis’’ aAny word starting with the specified part of a word, e.g., ‘‘audiologic’’ will include terms such as ‘‘audiological’’ and ‘‘audiology.’’

190 TELEMEDICINE and e-HEALTH MARCH 2010 TELEHEALTH APPLICATIONS IN AUDIOLOGY

APPENDIX II. Summary of Studies Included in the Review MODEL= CONNECTION= AUTHORS YEAR JOURNAL CATEGORY STUDY TYPE DISTANCE SUBJECTS PROCEDURES CONCLUSIONS Virre et al.31 1997 Telemedicine Diagnostic Proof-of- Asynchronous; Single-case Cameras allow- Effective con- Journal concept study. T1 connection adult report ing patient sultation of Case report on and distance eye move- balance disor- remote bal- unspecified ments to be ders and ance disorder recorded analysis of consultation nystagmus remotely Andersson 2002 Psychosomatic Intervention Randomized Asynchronous Adult subjects 117 adult sub- High dropout et al.33 Medicine controlled self-help; with history jects as- rate for In- trial of In- Internet- of at least 6 signed to the ternet-based ternet-based based months of two groups treatment. cognitive tinnitus. 117 But results behavioral adults on indicate In- therapy for the Internet- ternet-based

tinnitus-re- based treat- treatment lated distress ment can decrease the annoyance associated with tinnitus Andersson 2003 Journal of Psy- Diagnostic Proof-of-con- Asynchronous Adults with Questionnaires The Internet et al.32 chosomatic cept study. Internet- tinnitus completed yielded com- Research One group based completing online and parable and completing question- with pencil valid data an anxiety naire online and paper and depres- (n ¼ 157). sion scale for Adults with tinnitus on tinnitus the Internet completing and a second question- group com- naire on pa- pleting on per with paper with pencil pen

continued "

ª MARY ANN LIEBERT, INC. . VOL. 16 NO. 2 . MARCH 2010 TELEMEDICINE and e-HEALTH 191 SWANEPOEL AND HALL

Appendix II. Summary of Studies Included in the Review continued MODEL= CONNECTION= AUTHORS YEAR JOURNAL CATEGORY STUDY TYPE DISTANCE SUBJECTS PROCEDURES CONCLUSIONS Givens et al.21 2003 Telemedicine Diagnostic Experimental Synchronous; PT AC audiome- Sound-treated PT AC audiome- Journal design com- unspecified try (31 adults) room. Two try was and e-Health paring face- connection independent equivalent to-face audi- and distance audiologists between re- ometry with tested syn- mote and on- remote test- chronous PC- site testing ing based audiometry Givens and 2003 American Jour- Diagnostic Experimental Synchronous; PT AC audiom- Sound-treated PT AC and BC Elangovan22 nal of design com- unspecified etry (45 room. Two audiometry Audiology paring face- connection adults). PT independent were equiva- to-face audi- and distance BC audiome- audiologists lent between ometry with try (25 tested syn- remote and remote test- adults) chronous on-site testing PC-based ing audiometry Patricoski 2003 Telemedicine Diagnostic Experimental Asynchronous Video-oto- Two physicians Review of et al.26 Journal and design com- store-and- scopic still examined video- e-Health paring face- forward images (40 ear in face- otoscope to-face mi- children and to-face ses- images is croscopic ex- adults aged sions. Still comparable amination between 1 images were to in-person with video- and 21 years; taken. microscopic otoscope still 80 ears). Images re- examination images Face-to-face viewed at 6 microscope and 12 ear examina- weeks by the tion same two physicians Andersson and 2004 Journal of Intervention Proof-of-con- Asynchronous Single-case Six modules to Anxiety and Kaldo34 Clinical Psy- cept study. self-help; In- adult report be completed depression chology Case report ternet-based in 6–10 levels were study on In- weeks with e- reduced ternet-based mail corre- self-test spondence. treatment Pretreatment, program ac- posttreat- cessed re- ment follow- motely up measures

continued "

192 TELEMEDICINE and e-HEALTH MARCH 2010 TELEHEALTH APPLICATIONS IN AUDIOLOGY

Appendix II. Summary of Studies Included in the Review continued MODEL= CONNECTION= AUTHORS YEAR JOURNAL CATEGORY STUDY TYPE DISTANCE SUBJECTS PROCEDURES CONCLUSIONS Kaldo- 2004 American Jour- Intervention, Nonrandomized Asynchronous Internet-based Six modules to Valid procedure Sandstro¨m nal of patient per- clinical effec- self-help; In- self-test be completed indicating et al.35 Audiology ceptions tiveness ternet-based treatment in 6–10 weeks. positive find- study program ac- Pretreatment, ings but cessed re- posttreatment, dropout rates motely by and 3 month are problem- patients (77 follow-up atic adults) measures Smits et al.16 2004 International Screening Proof-of-con- Asynchronous Telephone- Compared Reliable Journal of cept study. self-test; In- based self- screening in screening Audiology Development ternet-based test (n ¼ 38 laboratory test. Tele- and compari- subjects; 22 setup using phone and son of normal ears, headphones headphone speech-in- 54 ears with and tele- screening noise screen- hearing loss) phones to was efficient ing test over telephone telephone use from and head- home; com- phones pared results with diagnostic HINT Eikelboom 2005 International Diagnostics Proof-of-con- Asynchronous Video- Same physician Digital images et al.28 Journal of cept study. store-and- otoscopic still conducted were of good Pediatric Comparing forward images com- in-person quality al- Otorhinolar- in-person pared to in- assessment though yngology otoscopic ex- person oto- and 1 month poorer with amination to scopic exam- later evalu- younger- digital im- inations (66 ated digital aged children ages of the children; 127 images ear canal and ears) tympanic membrane

continued "

ª MARY ANN LIEBERT, INC. . VOL. 16 NO. 2 . MARCH 2010 TELEMEDICINE and e-HEALTH 193 SWANEPOEL AND HALL

Appendix II. Summary of Studies Included in the Review continued MODEL= CONNECTION= AUTHORS YEAR JOURNAL CATEGORY STUDY TYPE DISTANCE SUBJECTS PROCEDURES CONCLUSIONS Eikelboom and 2005 Journal of Tele- Patient percep- Descriptive sur- N=A Survey of pa- Questionnaire 30% of patients Atlas42 medicine and tions vey of patient tient atti- completed by were unwill- Telecare attitudes tudes to patients at- ing to receive telemedicine tending four audiological and willing- audiology services ness to use it clinics through tele- (n ¼ 116 medicine, adult pa- 32% were tients; 46% willing, 10% older than 65) would be willing sometimes, and 28% were unsure

Ribera25 2005 Seminars in Diagnostic Experimental Synchronous HINT (20 Two setups. HINT can be Hearing design com- high-speed adults) One at two administered paring face- LAN separate lo- remotely to-face eval- cations on with equiva- uation with the same lent results HINT and re- LAN. Second mote testing included a remote site Towers et al.29 2005 Seminars in Diagnostic Experimental Synchronous; ABR (500, Synchronous Comparable re- Hearing design com- T1 connec- 3,000 Hz to- PC-based sults be- paring face- tion; unspec- neburst and testing at 55 tween sites to-face ABR ified distance click stimuli) and 75 dB. with values testing with (15 adults) Two tests within clini- remote test- on-site and a cally ing third test accepted remotely. variability Evaluated latency

194 TELEMEDICINE and e-HEALTH MARCH 2010 TELEHEALTH APPLICATIONS IN AUDIOLOGY

Appendix II. Summary of Studies Included in the Review continued MODEL= CONNECTION= AUTHORS YEAR JOURNAL CATEGORY STUDY TYPE DISTANCE SUBJECTS PROCEDURES CONCLUSIONS Smits et al.17 2006 Clinical Otolar- Screening Proof-of-con- Asynchronous Speech-in-noise Self-test. Sub- Screening is yngology cept study. self-test; In- screening via jects either possible over Observational ternet-based telephone call in for the the telephone cross-sec- (n ¼ 6,351 automated and Internet. tional design adults and hearing Calibration is adolescents) screening or an issue and via connect to Internet the Internet (n ¼ 30,260 site adults and adolescents) Laplante- 2006 International Intervention Qualitative Asynchronous New hearing Internet-based Powerful com- Le´vesque Journal of multiple Internet- aid users (3 counseling munication et al.40 Audiology case study based adults) program medium for design through daily observing e-mails for changes in the first behavior and month from perceptions audiologist. of new Data included hearing aid interviews users. Imme- with partici- diacy of e- pants, e-mail mail provides interchanges, possibility and audio- for timely logical files response to concerns

continued "

ª MARY ANN LIEBERT, INC. . VOL. 16 NO. 2 . MARCH 2010 TELEMEDICINE and e-HEALTH 195 SWANEPOEL AND HALL

Appendix II. Summary of Studies Included in the Review continued MODEL= CONNECTION= AUTHORS YEAR JOURNAL CATEGORY STUDY TYPE DISTANCE SUBJECTS PROCEDURES CONCLUSIONS Choi et al.23 2007 Telemedicine Diagnostic Experimental Synchronous; PT AC audiome- Sound booth. Comparable and e-Health design com- broadband try (12 nor- Synchronous thresholds paring face- wired net- mal) PC-based au- although to-face audi- work; 1 km diometry slightly high- ometry with distance er variation remote test- between re- ing mote and face-to-face thresholds compared to face-to-face comparison on PC-based vs. conven-

tional audiometer Krumm et al.24 2007 Journal of Tel- Diagnostic Experimental Synchronous; PT AC audiom- Synchronous Equivalent re- emedicine design com- broadband etry and PC-based sults from and Telecare paring face- LAN; DPOAE (30 audiometry remote loca- to-face audi- 1,100 km adult sub- and DPOAE tion ometry and distance jects) in sound DPOAE eval- booth uations with remote testing Bexelius et al.18 2008 Journal of Screening Proof-of-con- Asynchronous Subjects com- Description of Hearing Medical In- cept study self-test; In- pleting an results screening ternet Re- with obser- ternet-based Internet- can be con- search vational based hear- ducted over cross-sec- ing screen- the Internet. tional design ing test Calibration is (n ¼ 88) an issue

continued "

196 TELEMEDICINE and e-HEALTH MARCH 2010 TELEHEALTH APPLICATIONS IN AUDIOLOGY

Appendix II. Summary of Studies Included in the Review continued MODEL= CONNECTION= AUTHORS YEAR JOURNAL CATEGORY STUDY TYPE DISTANCE SUBJECTS PROCEDURES CONCLUSIONS Kaldo et al.36 2008 Behavior Ther- Intervention, Randomized Asynchronous Standard group- Comparison of Internet treat- apy patient per- controlled self-help; In- based therapy Internet- ment was ceptions trial for cog- ternet-based (n ¼ 25 based and comparable, nitive behav- adults). Inter- standard statistically ior therapy net-based group-based and clinically, for tinnitus therapy (n cognitive to conven- delivered as 26 adults) therapy for tional therapy Internet- tinnitus. Self- based and report inven- standard tories mea- group-based suring tinnitus distress immediately after treat-

ment and 1 year later Koopman 2008 International Patient percep- Survey of pref- N=A Survey of pref- Questionnaires Enthusiastic et al.43 Journal of tions erences for erence for mailed about pros- Audiology hearing hearing pect of self- screening screening via screening. delivery question- Question- methods naire, tele- naire gener- phone, or ally preferred Internet to Internet, (n ¼ 202 re- which was spondents; preferred to majority over telephone 65 years) Krumm et al.19 2008 Journal of Tel- Screening Experimental Synchronous; DPOAE and Synchronous Identical find- emedicine design com- broadband AABR DPOAE and ings for re- and Telecare paring face- connection; screening AABR testing mote and on- to-face 200 km dis- (30 infants) site screen- screening tance ings. DPOAE with remote amplitudes testing equivalent across frequencies between sites

continued "

ª MARY ANN LIEBERT, INC. . VOL. 16 NO. 2 . MARCH 2010 TELEMEDICINE and e-HEALTH 197 SWANEPOEL AND HALL

Appendix II. Summary of Studies Included in the Review continued MODEL= CONNECTION= AUTHORS YEAR JOURNAL CATEGORY STUDY TYPE DISTANCE SUBJECTS PROCEDURES CONCLUSIONS Shapiro et al.30 2008 Otology and Diagnostic Prospective de- Synchronous; Cochlear im- Operating the- Remote testing Neurotology sign to deter- unspecified plant and ater. On-site of the co- mine feasibil- connection; patient re- audiological chlear im- ity and time same neigh- sponse to monitoring plant device efficiency borhood electrical and off-site and patient’s stimulation synchronous response to (4 devices monitoring electrical tested on- through PC- stimulation is site and based appli- technically 4 tested cation shar- feasible, remotely) ing time-saving, practical, and cost-efficient

Lancaster 2008 American Jour- Screening Experimental Synchronous Otoscopy, PT Synchronous No statistically et al.20 nal of design com- and asyn- AC audiome- (otoscopy significant Audiology paring face- chronous; try, tympa- and PT AC differences to-face 200 kb Inter- nometry (32 audiometry), between screening net connec- children) asynchro- screen re- with remote tion; 30-mile nous (tym- sults. Oto- testing distance panometry) scopy and tympanometry gave same results. Pure tone screen results differed in 5 cases (n 32)—only 5 of 193 frequencies tested

continued "

198 TELEMEDICINE and e-HEALTH MARCH 2010 TELEHEALTH APPLICATIONS IN AUDIOLOGY

APPENDIX II. Summary of Studies Included in the Review continued MODEL= CONNECTION= AUTHORS YEAR JOURNAL CATEGORY STUDY TYPE DISTANCE SUBJECTS PROCEDURES CONCLUSIONS Kokesh et al.27 2008 Otolaryngolo- Diagnostic Experimental Asynchronous Children be- Video-otoscopy Video-otoscopy gy—Head and design. Diag- store-and- tween 1 and still images still images Neck Surgery nosis from forward 16 years of compared to are compara- video-oto- age (n ¼ 70 on-site ble to in- scopic still ears) for fol- examination person images of low-up care by two examination. tympanic following independent Store-and- membrane tympanost- ENTs forward compared to omy tube acceptable face-to-face placement method microscopic examination Ramos et al.41 2009 Acta-Otolaryn- Intervention, Randomized Synchronous; Cochlear im- 12 remote and Remote pro- gologica patient per- study high-speed plant map- 12 standard CI gramming ceptions comparing connection; ping (5 adult mapping ses- without inci- on-site CI 300 m subjects) sions (4 pro- dents. No program- gramming significant ming to days sepa- differences remote CI rated by 3 between program- months) com- groups. Per- ming pared pro- formance in gram parame- groups 3 ters, auditory months post- progress, per- programming ceptions of indicated no sessions, difference. technical as- Subjects indi- pects, risks, cated satis- and difficul- faction with ties both methods

continued "

ª MARY ANN LIEBERT, INC. . VOL. 16 NO. 2 . MARCH 2010 TELEMEDICINE and e-HEALTH 199 SWANEPOEL AND HALL

Appendix II. Summary of Studies Included in the Review continued MODEL= CONNECTION= AUTHORS YEAR JOURNAL CATEGORY STUDY TYPE DISTANCE SUBJECTS PROCEDURES CONCLUSIONS Ferrari and Ber- 2009 Journal of Tele- Intervention Experimental Synchronous; Probe micro- Synchronous Comparable re- nardez- medicine and design com- 384 kb LAN; phone mea- measure- sults between Braga37 Telecare paring face- distance not surements ments sites with to-face verifi- specified (REUR, REAR, values within cation of and REIG). 60 clinically ac- hearing aid adult hearing cepted vari- performance aid users (105 ability with remote ears) verification Diagnostic Intervention Screening Patient perceptions ABR, auditory brainstem response; AC, air conduction; BC, bone conduction; CI, cochlear implant; DPOAE, distortion product oto-acoustic emissions; HINT, Hearing-in-Noise-Test; LAN, local area network; N=A, not applicable; PC, personal computer; PT, pure tone. Note: Light blue rows indicate diagnostic, dark blue rows indicate intervention, white rows indicate screening, and gray rows indicate patient perceptions.

200 TELEMEDICINE and e-HEALTH MARCH 2010