Centre for Brain and Cognitive Development, Birkbeck, University of London, London, UK.
J Intellect Disabil Res. 2011 Jun;55(6):563-71. doi: 10.1111/j.1365-2788.2011.01411.x. Epub 2011 Mar 15.
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.
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.
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.
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.
威廉姆斯综合征（WS）是一种遗传性神经发育障碍，其特征是在其他几个领域严重受损的情况下相对熟练的语言能力。WS 患者对噪声表现出异常的敏感性，称为听觉过敏。
在这项研究中，我们研究了听觉过敏对 WS 儿童和成人言语感知的干扰程度。参与者需要在噪声背景下辨别一个辅音簇中只有一个辅音不同的单词。
尽管在 WS 组和对照组中，噪声的引入同样干扰了辅音簇辨别任务的表现，但听觉过敏的严重程度显著预测了 WS 组中言语感知的个体差异。
这些结果表明，对输入的敏感性改变介导了 WS 中语言发展的异常途径，其中听觉过敏与其他非听觉因素一起产生重要影响。
