Sensory modulation impairments in children with Williams syndrome

Abstract

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; , 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, problems behaviors, and more difficult temperaments than children in the low impairment group.

Brief Report: Exploring the Relationship Between Sensory Processing and Repetitive Behaviours in Williams Syndrome

“…visual, auditory, vestibular, or proprioceptive stimuli is managed in the cerebral cortex and brainstem for the purpose of enabling adaptive responses to the environment" (Baker, Lane, Angley, & Young, 2008: 867). Critical to the current investigation, sensory processing abnormalities have been identified in up to 90% of children with WS (John & Mervis, 2010). Such problems may relate to impairments of visual, auditory, and tactile perception (e.g.…”

“…Such problems may relate to impairments of visual, auditory, and tactile perception (e.g. Semel & Rosner, 2003) and / or sensory modulation difficulties (including auditory, vestibular, and proprioceptive hyper-and hypo-sensitivity; John & Mervis, 2010).…”

“…Individuals with WS may engage in obsessive-compulsive behaviours, such as the compulsive need to identify the source of sudden noises or compulsive greeting behaviours (Semel & Rosner, 2003). Although John and Mervis (2010) found evidence of a relationship between sensory processing abnormalities, problem behaviours, and adaptive functioning in children with WS, there are no studies to date that have looked specifically at the relationship between sensory processing and repetitive behaviours in WS. The aim of this preliminary study was therefore to explore sensory processing abnormalities and repetitive behaviours for the first time in children with WS.…”

Heterogeneity of social approach behaviour in Williams syndrome: The role of response inhibition

“…Barton et al (2004). It has been used RUNNING HEAD: Social Approach in Williams syndrome 12 previously to investigate sensory abnormalities in children with WS (John et al, 2010). The variables used for cluster analysis here were selected on the basis of predictions from theories of SAB; emotion recognition and response inhibition.…”

“…Cluster analysis techniques are increasingly used with neurodevelopmental research in order to capture heterogeneity and can be used with comparable small sample sizes to the one reported here e.g. Barton et al (2004) and John et al (2010). In order to avoid a type one error, variables used for cluster analysis were selected on the basis of predictions from underlying theories of SAB.…”