Extreme sensitivity to sound mediates pathways for language development in Williams syndrome


Hyperacusis is an extreme sensitivity to sound, which may have a psychological or organic basis. People with Williams Syndrome, a neurodevelopmental disorder, often have this sensitivity to noise.

The researchers in this study looked at the extent to which such sensitivity might interfere with perception of speech in children and adults with the syndrome. All the participants had to discriminate words differing from each other in one consonant of a cluster, but they were asked to do this when the differences were embedded in background noise.

The authors found that the noise interfered with the performance of both the Williams syndrome and the control group equally when differentiating consonant clusters. However they also found that the severity of hyperacusis was able to significantly predict individual variability in speech perception within the Williams syndrome group.

They conclude that their results suggest alterations in sensitivity to input mediate atypical pathways for language development in Williams syndrome, where hyperacusis is important influence along with other non-auditory factors.

Severity of hyperacusis predicts individual differences in speech perception in Williams Syndrome, Elsabbagh M et al., in Journal of Intellectual Disability Research, 55: 563–571.

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John Northfield

After qualifying as a social worker, John worked in community learning disability teams before getting involved in a number of long-stay hospital closure programmes, working to develop individual plans for people moving into their own homes. He worked for BILD, helping to develop the Quality Network and was editorial lead for the NHS electronic library learning disabilities specialist collection. This led him to found the Learning Disabilities Elf site with Andre Tomlin as a way of making the evidence accessible to practitioners in health and social care. Most recently he has worked as part of Mencap's national quality team and also been involved in a number of national website developments, including the General Medical Council's learning disabilities site.

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