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Am J Med Genet C Semin Med Genet. 2010 May 15;154C(2):266-76. doi: 10.1002/ajmg.c.30260.

Sensory modulation impairments in children with Williams syndrome.

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Department of Psychological and Brain Sciences, University of Louisville, Louisville, KY 40292, USA.


The ability to organize information detected by our senses ("sensory modulation") allows us to act or respond effectively to situations encountered, facilitating learning, social behavior, and day-to-day functioning. We hypothesized that children with Williams syndrome (WS) would demonstrate symptoms of poor sensory modulation and that these sensory modulation abnormalities contribute to the phenotype. Participants were 78 children with WS aged 4.00-10.95 years. Based on parent ratings on the Short Sensory Profile [SSP; Dunn, 1999], most children were classified as having definite sensory modulation issues. Cluster analysis identified the presence of two clusters varying in level of sensory modulation impairment. Children in the high impairment group demonstrated poorer adaptive functioning, executive functioning, more problem behaviors, and more difficult temperaments than children in the low impairment group.

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