Although children with Williams syndrome (WS) have relatively good structural language and concrete vocabulary abilities, they have difficulty with pragmatic aspects of language. To investigate the impact of pragmatic difficulties on listener-role referential communication, we administered a picture placement task designed to measure ability to verbalize message inadequacy to a speaker separated by a barrier. 57 children with WS aged 6 -12 years participated. When messages were inadequate, children verbalized that a problem was encountered less than half the time. The likelihoods that children would indicate a message was insufficient and that children who verbalized message inadequacy also would effectively communicate the problem varied as a function of type of problem encountered, theory of mind knowledge, receptive vocabulary, and CA.
KeywordsWilliams-Beuren syndrome; referential communication; conversational inadequacy; social communication; pragmatics; intellectual disability Williams syndrome (WS) is a neurodevelopmental disorder resulting from a hemideletion of 25 genes on chromosome 7q11.23 (Ewart et al., 1993;Morris, 2006), with a prevalence of 1 in 7500 live births (Strømme, Bjørnstad, & Ramstad, 2002); boys and girls are equally likely to be affected (American Academy of Pediatrics Committee on Genetics, 2001). WS is characterized by mild to moderate intellectual disability or learning difficulties, dysmorphic facial features, and heart disease, especially supravalvar aortic stenosis (Morris, 2006). WS has drawn considerable attention from researchers due to its unusual cognitive and personality profiles. The WS cognitive profile is characterized by relative strengths in verbal short term memory and the structural and concrete vocabulary components of language accompanied by severe weakness in visuospatial construction (Mervis et al., 2000;Mervis & Morris, 2007;Udwin & Yule, 1991).The WS personality profile involves high levels of sociability and empathy, over-eagerness to interact with others, and high levels of tension and sensitivity (Klein-Tasman & Mervis, 2003). Children with WS have been described as very sociable (Dilts et al., 1990), outgoing and talkative (Jones & Smith, 1975), approachable (Tomc et al., 1990), charming (Fryns et al., 1991), and unusually friendly (Preus, 1984). Despite these seemingly positive characteristics, individuals with WS demonstrate difficulty with peer relationships (e.g., Sullivan et al., 2003) with one study (Davies et al., 1998) reporting that 96% of parents and caregivers of adults with WS described problems with establishing friendships. Address Correspondence to: Angela E. John, Dept of Psychological and Brain Sciences, University of Louisville, Louisville, KY 40292. . Email: E-mail: aejohn11@gwise.louisville.edu.
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Author ManuscriptAm J Intellect Dev Disabil. Author manuscript; available in PMC 2010 March 1.
NIH-PA Author ManuscriptThe combination of a relative strength in the structural and concrete vocabulary aspects of language a...