Auditory hypersensitivity in Williams syndrome

Abstract

Objectives: The objective of this study was to investigate auditory hypersensitivity in WS and to evaluate hyperacusis through standardized protocols, checking if it can be associated with the absence of acoustic reflexes in people with WS.

Method: The study was performed in 17 individuals with WS, aged between seven and 17 years old (10 males and seven females), and 17 individuals with typical development age- and gender-matched to individuals with WS. Statistical tests were used to analyze the responses collected with the Loudness Discomfort Level (LDL) test as well as ipsilateral and contralateral reflex responses.

Results: Auditory hypersensitivity was commonly found. Individuals with WS had phonophobia and were less tolerant to high sound intensity, presenting a reduced discomfort threshold compared to those with typical development. However, hyperacusis was found in 35.29% of individuals with WS and was mild in 50% of cases. There was an association between hyperacusis and acoustic reflex responses, and individuals with absence of the contralateral acoustic reflex were more likely to have hyperacusis.

Conclusions: Individuals with WS have a high prevalence of auditory hypersensitivity, with the presence of phonophobia; however, hyperacusis was not as prevalent and may be associated with the absence of contralateral acoustic reflexes.

Keywords: Audiology; Cochlear; Hearing loss; Hyperacusis; Williams syndrome.