Auditory Children and Function Adults and With Hearing Williams Loss Syndrome: in Cochlear Normal Hearing Impairment in Individuals With Otherwise

Hearing loss is common in school-age individuals with Williams syndrome (WS) and extensive in adults. Prior studies with relatively small sample sizes suggest that hearing loss in WS has an early onset and may be progressive, yet the auditory phenotype and the scope of the hearing loss have not been adequately characterized. We used standard audiometric tools: Otoscopy, tympanometry, air-conduction (bone conduction when available) behavioral testing, and distortion product otoacoustic emissions (DPOAEs) to measure hearing sensitivity and outer hair cell function. We tested 81 individuals with WS aged 5.33–59.50 years. Sixty-three percent of the school-age and 92% of the adult participants had mild to moderately-severe hearing loss. The hearing loss in at least 50% was sensorineural. DPOAE testing corroborated behavioral results. Strikingly, 12 of 14 participants with hearing within normal limits bilaterally had 4,000-Hz DPOAE input/output (DPOAE IO) functions indicative of outer hair cell damage and impaired cochlear compression. Our results indicate that hearing loss is very common in WS. Furthermore, individuals with WS who have ''normal'' hearing as defined by behavioral thresholds may actually have sub-clinical impairments or undetected cochlear pathology. Our findings suggest outer hair cell dysfunction in otherwise normal hearing individuals. The DPOAE IO in this same group revealed growth functions typically seen in groups with noise-induced damage. Given this pattern of findings, individuals with WS may be at increased risk of noise-induced hearing loss. Recommendations regarding audiological testing for individuals with WS and accommodations for these individuals in both academic and nonacademic settings are provided. College of Medicine. His primary research focus is hearing, auditory processing and phonological development in children with Williams syndrome, duplication of the Williams syndrome region, and nonsyndromic supravalvar aortic stenosis. He also conducts electrophysiological research involving children with developmental disabilities and learning disorders. is an Assistant Professor and doctoral candidate in Communication Sciences and Disorders at James Madison University. Her primary research focus includes investigating the impact of hearing loss on phonological memory and language learning in special populations, such as Williams syndrome. Her primary research focus is on the language, cognitive, social-emotional, and behavioral development of children with Williams syndrome, duplication of the Williams syndrome region, and Down syndrome. She also conducts research on neuroimaging and genotype/phenotype correlations involving the Williams syndrome region. Her primary research focus is on the development of auditory processing skills and spatial hearing in children with normal hearing and hearing disorders. His primary research interest …