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Intervention Strategies for Air‐ and Bone‐ Conduction Unilateral Hearing Loss (UHL)

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Intervention Strategies for Air‐ and Bone‐ Conduction Unilateral Hearing Loss (UHL) 2020-09-29 Intervention strategies for air‐ and bone‐ conduction unilateral hearing loss (UHL) Susan A. Small, PhD Associate Professor Hamber Professor of Clinical Audiology University of British Columbia Virtual Speech & Hearing BC Conference 2020 October 23, 2020, 10:30 am ‐ 12:00 pm Disclosure statement . NSERC Discovery Grant . BC Early Hearing Program (consultant): receive funds & equipment that contribute to my research program . Hamber Chair position: small contribution to research program . Interacoustics: equipment on loan Other funding . UBC Faculty of Medicine: general funds for research . Eric W. Hamber Professorship: partial salary award TOPIC AREAS TO BE ADDRESSED Prevalence of UHL Loss of binaural hearing benefits with UHL‐‐ effects on spatial hearing + other consequences Review of current data re: intervention for UHL across the lifespan‐‐ infants to adults Bone‐conduction hearing loss: Unilateral & binaural 1 2020-09-29 BC EHP Definitions Unilateral hearing loss (UHL) Normal hearing in one ear (considered to be the majority of thresholds ≤ 20 dB HL) Majority of air‐conduction (AC) thresholds >20 dB HL in the ear with permanent hearing loss Historically, audiologists most concerned with effects of severe‐to‐profound hearing loss on speech & language development Rubella outbreak in US Severe/profound clinical in 1964/5: 12,000 babies focus for years after born deaf UHL (& minimal bilateral hearing loss) might have Studies in negative effects on academic early 1980’s progress & psychosocial skills of school‐aged children ** Renewed research focus in last 3-5 years Unilateral hearing loss Focus of recent international conferences ‐ BCEHP Workshop 2017, Oct 17, 2017, Vancouver: R. McCreery, J. Lieu & S.A. Small ‐ Phonak Unilateral Hearing Loss in Children Conference 2017, Oct 22‐24, 2017, Philadelphia ‐‐ lack of clarity re: best practice for UHL ‐‐ recent changes for infant UHL followed (e.g., BC & ON) Today: overview of past & more recent research available to guide clinicians... some data to guide us but more needed, particularly for infants & young children! 2 2020-09-29 mild conductive NH mixed sensory/neural profound Prevalence of UHL • with age • Newborns: ~ 0.6‐0.7 in 1000 (2013‐14 est. from CDC) ‐ ~1/3 of children with congenital hearing loss (progresses to bilateral in 7.5‐11.5%) (Paul et al., 2017; Haffey et al., 2013) • School age: 2.5‐3.0 % > 25 dB HL ‐ 19.5% with hearing thresholds >15 dB HL (Shargorodsky et al., 2010) mild conductive NH mixed sensory/neural profound Prevalence of UHL cont’d • May also help to follow infants at risk for progressive‐late‐onset ‐ >50% Enlarged Vestibular Aqueduct (EVA)/Mondini dysplasia ‐ + acquired CMV, genetic causes, NICU stay, in utero infections, craniofacial anomalies & postnatal infections/syndromes (reviewed in Bagatto et al., 2019) Audiologist should recommend complete otological evaluation with imaging Main concern for UHL: loss of hearing benefits associated with binaural hearing – effects on spatial hearing + other effects ‐ localization ‐ speech in noise ‐ early auditory behaviours/pre‐verbal vocalizations (Kishon‐Robin et al., 2015) ‐ speech/language development (Ead et al., 2013; Lieu, 2013) ‐ academic success (Bess & Tharpe, 1986; Lieu, 2004, 2013) ‐ balance (Wolter et al., 2016) Still do not have clinically feasible approach to assess spatial hearing ‐ some recommend survey/questionnaires (ABEL, SSQ) ‐ others: anechoic chamber/sound booth with speaker arrays (much harder to do clinically) (reviewed in Bagatto et al., 2019) 3 2020-09-29 Still early days re: evidence of effectiveness of intervention strategies available to ameliorate deficits for UHL ‐‐ what are the challenges? ‐‐ what do we know/not know? UHL & device use One challenge re: best practices for device recommendations for UHL in infants/children – no consensus but some suggestions…. Limited high‐level evidence of benefit (or harm) for different ages, types of losses & devices e.g., recent systematic review identified only 12‐13 studies with functional/objective auditory outcomes; 7 with bone‐ anchored hearing systems (BAHS) (Appachi et al. ,2017; Anne et al., 2019) BUT…. # studies investigating UHL starting to increase: ‐ Clinical outcomes: hearing aids (HA), CIs, BAHS, CROS & FM ‐ Cortical plasticity and binaural hearing in infants/children/adults with normal hearing (NH) & UHL‐‐ although mostly post‐lingual populations– more on this… ‐ Measures to assess binaural benefit with devices Some clinical questions yet to be answered fully… but now more clinical data available! Will devices improve long‐term effects of UHL on speech/language development/academic success/psychosocial function, or only for some children? How should we manage conductive vs sensory/neural UHLs? Air‐ conduction HAs versus BAHS (softband/implanted)? CI? CROS? FM? How should we plan intervention for mild‐moderate vs severe‐ profound UHL? Should intervention strategies for infants, children & adults differ? Are there any detrimental/limited benefits depending on type of UHL, type of device, or age of child? 4 2020-09-29 Reaching consensus on best practices for intervention for UHL in infants and young children difficult for several reasons: ‐ For many years, conflicting findings & low‐level evidence regarding long‐term effects on academic success & psychosocial function ‐ Limited data available supporting intervention with hearing aids, CIs, FM systems &/or communication strategies results in better speech & language outcomes More data in last 4‐5 years: more aggressive hearing‐aid fitting approaches & implantation of CIs (for review: McKay et al. 2008; Lieu 2015) Other questions re: UHL Do devices actually restore binaural hearing? How do we explain variability in binaural benefit with devices? Concepts that relate to binaural hearing: • head shadow, binaural summation, & binaural squelch • with UHL, typically lose benefits related to these phenomena & additionally experience negative effects relating to head shadow, if speech directed toward ear with UHL (shadows speech signal to NH ear) Infants & preschoolers Mixed early findings re: effects of UHL on language development ‐ some showed effects, some did not! Ex. 1 (Kiese‐Himmel (2002) Retrospective study over 4 yrs; 1 mos to 10 yrs (NH vs UHL) ‐UHL: no delay in production of first word & no difference on standardized linguistic tasks vs NH infants ‐ BUT infants with UHL produced first 2‐word combinations 5 mos later than NH infant (but not outside of normal range) Ex.2 (Sedey et al. [results reported in Lieu (2004)] ‐ 4/15 of 12‐mos‐old infants with UHL showed language delays in the absence of any known disabilities Renewed research focus on UHL & infants auditory development in last 4‐5 years 5 2020-09-29 Infants & preschoolers More infant UHL data to support intervention Ex. 1: (Lieu et al., 2010) ‐UHL: delay in 1st 2‐word phrase by parent recall vs NH Ex. 2: (Kishon‐Rabin et al., 2015; 34 UHL, 331 NH; ~9 month‐old infants) ‐ auditory behavior (IT‐MAIS) delayed in 21% UHL vs 4% NH ‐ preverbal vocalization (PRISE) delayed in 41% UHL vs 4% NH Ex. 3: (Fitzpatrick et al., 2015; 31 UHL; 45 NH (12, 24, 36, & 48 mos) ‐ Assessment tools: PEACH/ELF/CHILD/MacArthur‐ Bates/MLU/CDI ‐ CHILD scores worse at age 36 & 48 mos Important question: if device used for UHL, does bilateral auditory stimulation automatically result in restored spatial hearing ??? One of the early studies (only 2017!) looked at bilateral hearing loss and bilateral CIs‐‐ interesting findings: cortical representation of interaural time differences (ITDs) (Easwar et al., 2017) • NH vs children with simultaneous bilateral CI implantation (<3.5 y; 2 y auditory experience) (Young children‐ not infants) NH CIs L leading --R lateralization ITDs: ITDs: no difference in preference R leading-- more bilateral activation for R & L leading - Clear behavioural responses to ITD cues - Poor behavioural responses to ITD cues - Lower than NH in non-auditory areas ‐‐ Bilateral simultaneous electrical stimulation essential to protect bilateral auditory pathways from unilateral‐driven re‐organization BUT not sufficient for normal development of ITD coding Possible reasons for bilateral CI findings? Effect of early auditory deprivation? Type of hearing experience? Reliable ITDs unavailable from CIs functioning independently? CIs change ITDs of signal fine structure? Greater neural synchrony from electrical pulses affects timing cues? CI input affects brainstem coding? Less efficient compensatory strategies being used instead of ITD cues? More in later section about CIs and UHL 6 2020-09-29 Effects of UHL across the lifespan: Infants Toddlers School‐age children Adults School‐age children number of studies investigated effects of UHL on school‐age children (6‐18 years) ‐ showed poorer localization skills compared to NH children: ↓ performance with ↑ severity loss (Bess et al., 1996) ‐ consistently showed poorer performance in school ‐ 18‐35% repeated at least one grade in school (3.5% failure rate in general population) ‐ 12‐60% required a resource teacher (Bess & Tharpe, 1986; Oyler et al.,1988; Bovo et al., 1988; Jensen et al.,1989; Lieu 2004, 2010, 2012) School‐age children Early research showed varied results for speech/language/cognition IQ findings ‐ slight decrement in verbal IQ relative to performance IQ (Davis et al., 1981) ‐ no difference NH vs UHL children for 2 different IQ tests BUT children with more severe UHL had significantly lower overall IQ scores vs children with less severe UHLs
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