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Severity of hyperacusis predicts individual differences in speech perception in Williams Syndrome
Author(s) -
Elsabbagh M.,
Cohen H.,
Cohen M.,
Rosen S.,
KarmiloffSmith A.
Publication year - 2011
Publication title -
journal of intellectual disability research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.941
H-Index - 104
eISSN - 1365-2788
pISSN - 0964-2633
DOI - 10.1111/j.1365-2788.2011.01411.x
Subject(s) - hyperacusis , psychology , audiology , williams syndrome , speech perception , perception , consonant , developmental psychology , hearing loss , cognition , medicine , neuroscience , speech recognition , vowel , computer science
Background Williams Syndrome (WS) is a neurodevelopmental disorder of genetic origin, characterised by relative proficiency in language in the face of serious impairment in several other domains. Individuals with WS display an unusual sensitivity to noise, known as hyperacusis. Methods In this study, we examined the extent to which hyperacusis interferes with the perception of speech in children and adults with WS. Participants were required to discriminate words which differed in one consonant of a cluster when these contrasts were embedded in a background of noise. Results Although the introduction of noise interfered with performance on a consonant cluster discrimination task equally in the WS and control groups, the severity of hyperacusis significantly predicted individual variability in speech perception within the WS group. Conclusions These results suggest that alterations in sensitivity to input mediate atypical pathways for language development in WS, where hyperacusis exerts an important influence together with other non‐auditory factors.