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Effects of Aural Atresia on Speech Development and Learning Retrospective Analysis from a Multidisciplinary Craniofacial Clinic

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Effects of Aural Atresia on Speech Development and Learning Retrospective Analysis from a Multidisciplinary Craniofacial Clinic Research Original Investigation Effects of Aural Atresia on Speech Development and Learning Retrospective Analysis From a Multidisciplinary Craniofacial Clinic Daniel R. Jensen, MD; Lynn M. Grames, MA, CCC-SLP; Judith E. C. Lieu, MD, MSPH IMPORTANCE Aural atresia (AA) is associated with maximal conductive hearing loss in affected ears, and children with bilateral AA require amplification. Some recent research has suggested an increased risk for speech and learning problems among children with unilateral sensorineural hearing loss. OBJECTIVE To investigate whether increased risk for speech and learning problems exists among children with AA. DESIGN Retrospective medical record review. SETTING Multidisciplinary craniofacial clinic. PARTICIPANTS Children with unilateral or bilateral AA. INTERVENTIONS Records review, including evaluations by audiologists, speech pathologists, and psychologists. MAIN OUTCOME MEASURES Rates of speech and/or language delay, prevalence of speech therapy and educational interventions, and parental report of psychosocial problems. RESULTS A total of 74 patients were identified who met inclusion and exclusion criteria: 48 with right-sided AA, 19 with left-sided AA, and 7 with bilateral AA. Children with AA demonstrated high rates of speech therapy (86% among bilateral, 43% among unilateral). Reports of school problems were more common among children with right-sided AA (31%) than those with left-sided AA (11%) or bilateral AA (0%) (P = .06). Educational interventions were common in all groups (33% right, 21% left, 43% bilateral). In the case of bilateral AA, all children who received additional interventions were enrolled in schools for the hearing impaired, without any identified learning deficiencies. CONCLUSIONS AND RELEVANCE Children with unilateral AA may be at greater risk of speech and learning difficulties than previously appreciated, similar to children with unilateral sensorineural hearing loss. Whether amplification may alleviate this risk is unclear and warrants further study. Author Affiliations: Department of Otolaryngology–Head and Neck Surgery, Washington University School of Medicine, St Louis, Missouri (Jensen, Lieu); Center for Communication Disorders, St Louis Children’s Hospital, St Louis, Missouri (Grames). Corresponding Author: Judith E. C. Lieu, MD, MSPH, Department of Otolaryngology–Head and Neck Surgery, Washington University School of Medicine, 660 S Euclid Ave, JAMA Otolaryngol Head Neck Surg. 2013;139(8):797-802. doi:10.1001/jamaoto.2013.3859 Campus Box 8115, St Louis, MO 63110 Published online July 18, 2013. ([email protected]). 797 Downloaded From: http://archotol.jamanetwork.com/ by Hernan Goldsztein on 08/15/2013 Research Original Investigation AA in Speech Development and Learning ural atresia (AA) is a congenital absence or stenosis of the external auditory canal with variable middle ear Methods A anomalies. It is almost always accompanied by a mal- formed (microtia) or absent (anotia) external ear.1 Atresia and A waiver of informed consent was obtained from the institu- microtia are common congenital malformations, with the in- tional review board of Washington University in St Louis, Mis- cidence of microtia reported to be 0.5 to 3 per 10 000 live births, souri. We performed a retrospective medical chart review of and AA reported in 55% to 93% among individuals with patients with AA, both unilateral and bilateral, treated in the microtia.1-3 Microtia is unilateral in most patients, with a right- pediatric craniofacial multidisciplinary clinic at the St Louis sided and male predominance.1 Children with bilateral hear- Children’s Hospital between 1975 and 2010. We attempted to ing loss are known to be at increased risk of speech and lan- collect data for children with microtia without AA (ie, normal guage delays as well as poor performance in school.4,5 Children hearing) as a control group, but there were too few. Data were with bilateral AA typically demonstrate bilateral maximal con- collected from the evaluations performed by audiologists, ductive (ie, moderately severe) hearing loss and are generally speech pathologists, and psychologists. assumed to have similar speech-language and school prob- lems, though this has not been specifically studied. In con- Inclusion Criteria trast, the clinical significance of unilateral hearing loss (UHL) We searched the craniofacial clinic patient database using search is not universally recognized.6 Traditionally, children with UHL terms aural atresia and microtia. Children aged 2 to 12 years car- were thought to develop normally without any major se- rying a diagnosis of unilateral or bilateral AA were included. quelae, and most have not received amplification. Several small studies of children with UHL have shown delays in word ac- Exclusion Criteria quisition in younger children, increased grade failure rates, and Subjects with any of the following characteristics were ex- a higher prevalence of special educational interventions; how- cluded from the study: ever, these results have been inconsistently demonstrated.7-14 1. Younger than 2 years or older than 12 years at the time of Some studies have demonstrated poor hearing aid compli- the clinic visit (except for purposes of calculations regard- ance among children with UHL, though others have shown ing clinic attendance); greater than 80% acceptance.12,15 Lieu et al16 conducted a pro- 2. Lacked audiogram or speech pathologist examination data; spective case-control study comparing scores on the Oral and 3. Carried other potentially confounding diagnoses, includ- Written Language Scales (OWLS) test in children with UHL with ing developmental delay, cleft lip or palate, or other syn- normal-hearing siblings. This study demonstrated signifi- drome, with the exception of hemifacial microsomia; cant deficits among children with UHL in language compre- 4. Had 40-dB or less hearing level (HL) in an atretic ear, or 20-dB hension, oral expression, and oral composite score. These chil- or greater HL in unaffected ears in children with unilateral dren were also more likely to have received speech or language AA; and/or therapy and to have an individual education plan (IEP) at 5. History of recurrent or chronic otitis media requiring tym- school. Longitudinally, a subgroup of these children with UHL panostomy tube placement in the unaffected ear. were found to improve their language scores, whereas their aca- demic and behavioral problems persisted.17 While these find- Outcome Variables ings are significant, it is unclear if they can be extrapolated to Demographic variables included age and sex. To understand children with unilateral AA because most of the children in clinic usage patterns for these patients, we collected data re- those studies had profound sensorineural loss (SNHL). It is also flecting patient age at first and last visits to the clinic as well unclear whether the side of the AA might impact develop- as total number of visits. ment of speech and language. Audiogram values included thresholds in dB HL for both The purpose of the present study was to use assessments air and bone conduction and pure tone average. Amplifica- by speech therapists and psychologists during visits to a cra- tion data included type of amplification and age at initiation. niofacial clinic to investigate the potential impact of AA on Otologic history included history of recurrent or chronic oti- school performance and successful acquisition of speech and tis media and prior atresiaplasty. language. We hypothesized that children with AA would dem- Data collected from speech pathologist evaluations in- onstrate delays in language acquisition and require speech cluded presence of articulation or language errors, voice or therapy at a rate greater than that of the general population and resonance abnormalities, and history of speech therapy with that these delays would adversely affect school performance, duration and age at initiation. as reported by their parents. We further hypothesized that chil- Parental report of school performance was reviewed, es- dren with unilateral AA would experience fewer problems than pecially problems with learning, discipline, or attention. Uti- those with bilateral AA but more than the general population. lization of additional educational resources was recorded, in- Finally, one of the goals of any multidisciplinary craniofacial cluding brief trial interventions to see if the patient’s clinic is to track the developmental progress of its patients. Little performance would catch up (response to intervention), for- is known, however, about the actual attendance patterns of chil- mal structured instruction (tutoring, individual education plan), dren with AA at craniofacial clinics. We collected data to re- or enrollment in special education outside the mainstream flect the duration and frequency of clinic visits and the age range classroom. Psychologic data included history of any psychi- of the patients included in the study. atric diagnosis or treatment. 798 JAMA Otolaryngology–Head & Neck Surgery August 2013 Volume 139, Number 8 jamaotolaryngology.com Downloaded From: http://archotol.jamanetwork.com/ by Hernan Goldsztein on 08/15/2013 AA in Speech Development and Learning Original Investigation Research Table 1. Demographic and Audiologic Profile of Patients with AA Right AA Left AA Bilateral AA Total Characteristic (n = 48) (n = 19) (n=7) (n = 74) Sex, No. (%) Male 30 (63) 9 (47) 2 (29) 41 (55) Female 18 (38) 10 (52) 5 (71) 33 (45) Race/ethnicity, No. (%) African
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