Characteristics of auditory evaluation in Williams syndrome: a systematic review

Identify the characteristics of the clinical audiological evaluation of individuals with Williams syndrome by means of a systematic literature review.

The following research question was initially determined: "What are the characteristics of clinical auditory assessment in individuals with Williams syndrome?". From this, a bibliographic search was conducted in four databases using the descriptors: Williams syndrome, Hearing loss, and Audiology.

Only full articles with evidence levels 1 or 2, published in Brazilian Portuguese or English, were selected.

Results obtained in the auditory tests used in the clinical routine, namely: immittance test, pure-tone audiometry, otoacoustic emissions, and brainstem auditory evoked potential were analyzed.

Two hundred nine studies were found, but only 12 met the inclusion criteria for the study. It was possible to observe prevalence of type A tympanometry curve, which may occur with absence of acoustic reflexes, mild to moderate sensorineural hearing loss, affecting mainly the high frequencies, absent or less amplified otoacoustic emissions, and brainstem auditory evoked potential without retrocochlear alteration.

Cochlear impairment is common in individuals with Williams syndrome and the main disorders found in the hearing assessment in this population are absence of otoacoustic emissions and acoustic reflexes, as well as presence of mild to moderate sensorineural hearing loss, mainly in the high-frequency range, observed by audiometry.