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Updated: Oct 6, 2025

Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
Audiological profile and cochlear functionality in Williams syndrome
Liliane Aparecida Fagundes Silva1, Rachel Sayuri Honjo Kawahira2, Chong Ae Kim2
1Departamento de Fisioterapia, Fonoaudiologia e Terapia Ocupacional, Faculdade de Medicina - FM, Universidade de São Paulo - USP - São Paulo (SP), Brasil.
Purpose:
to evaluate cochlear functionality in Williams syndrome (WS) individuals.
Methods:
a 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.
Results:
The 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.
Conclusion:
WS 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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