ArticleCoDAS2022
Audiological profile and cochlear functionality in Williams syndrome.
Article in CoDAS, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed, 6 citations in OpenAlex.
- Translation and cross-cultural adaptation of a questionnaire for assessing hyperacusis in Williams syndrome.Arquivos de neuro-psiquiatria · 2025Article
- Observational study to preliminarily characterize the audiological profile of chinese children with williams syndrome.Journal of otology · 2025Article
- Peripheral Auditory Pathway and ABR Characterization in Adults with Williams Syndrome.International archives of otorhinolaryngology · 2024Article
- Early Neurobehavioral Characterization of the CD Mouse Model of Williams-Beuren Syndrome.Cells · 2023Article
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeto evaluate cochlear functionality in Williams syndrome (WS) individuals.
methodsa study with 39 individuals, being 22 with WS aged between 7 and 17 years, 15 male and 7 female, and 17 individuals with typical development and normal hearing. All individuals were evaluated using pure tone audiometry, acoustic immittance measurements, and Transient Evoked Otoacoustic Emissions (TEOAE). The audiological profile in individuals with WS was analyzed, and TEOAE responses were compared between WS individuals without hearing loss and typical developmental individuals.
resultsThe hearing loss was observed in 50% of patients, being 78.95% sensorineural and 21.05% mixed. This hearing loss was predominantly mild to moderate, affecting mainly frequencies above 3 kHz. As for TEOAE, there was a higher incidence of absence and lower amplitude responses in individuals with WS.
conclusionWS individuals have hair cell dysfunction, mainly in the basal region of the cochlea. Thus, TEOAE analysis is an important clinical resource to be considered in the routine audiological evaluation.
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