Sensory Modulation in Children with Williams Syndrome

• ¹ University of Louisville, Department of Psychological and Brain Sciences, United States

Background and Purpose: Recently there has been interest in understanding the similarities and differences between the phenotypes associated with autism and Williams syndrome (WS). Although the popular press and some researchers have argued that the phenotypes associated with these developmental disorders are opposites of one another, careful examination of the WS phenotype has yielded several similarities (e.g., difficulties with pragmatics and with socio-communicative behaviors). However there are many areas yet to be explored. For example, although sensory issues are often found in children with autism, to date, little is known about the nature of sensory issues in children with WS. Klein-Tasman et al. (2007) examined children with WS between 30 and 63 months of age in a structured interaction (ADOS-G) and found that 28% demonstrated definite unusual responses to sensory aspects of toys or surroundings. The purpose of the present study was to further investigate the nature of sensory abnormalities in children with WS and examine their relation to cognitive ability and adaptive functioning.

Methods: Participants were 72 children with WS aged 4.00 - 10.95 years for whom three types of data were available: parental report data for the Short Sensory Profile (SSP; Dunn, 1999) designed to examine sensory modulation, cognitive ability, and adaptive functioning. Mean IQ on the Kaufman Brief Intelligence Test, 2nd edition was 70.26 (SD = 17.32). Mean overall adaptive functioning as measured by the Scales of Independent Behavior – Revised was 49.06 (SD = 20.10). Nine children had comorbid diagnoses on the autism spectrum (ASD) based on clinical judgment following administration of the ADOS-G and ADI-R.

Results: Based on caregiver report, most children were classified as having definite sensory modulation issues (Table 1). To assess the relations between SSP total score, overall intellectual ability, and adaptive functioning, a series of correlations was computed. Overall Sensory score was not significantly related to cognitive ability (r = .21) but was related to adaptive functioning (r = .46, p < .001). This correlation remained significant even when the children with ASD were excluded. Interestingly, the children with ASD were not clustered at the low (severe problem) end of the distribution of Sensory Profile Total Score. Most children with and without ASD demonstrated impairments on Auditory Filtering, Energy, Under-responsive/Sensation Seeking, and Visual/Auditory Sensitivity.

Discussion: In summary, based on parental report most children with WS demonstrate sensory modulation problems. Most children with WS both with and without comorbid diagnoses of ASD demonstrated definite abnormalities in the ability to use and screen out sounds, the ability to use muscles to move, noticing sensory events, and responding to sounds and sights. Overall sensory score was moderately correlated with overall adaptive functioning but was not significantly related to cognitive ability. Theoretical implications will be discussed.