Do Children with Williams Syndrome Really Have Good Vocabulary Knowledge? Jon Brock, Christopher Jarrold, Emily K. Farran, Glynis Laws, & Deborah M. Riby.

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Do Children with Williams Syndrome Really Have Good Vocabulary Knowledge? Jon Brock, Christopher Jarrold, Emily K. Farran, Glynis Laws, & Deborah M. Riby Comparison of age-equivalent scores for the BPVS-II and RCPM among individuals with Williams syndrome (WS) and typically developing (TD) children. WS n524TD n528 Age (years; months) 12;0 (3;1) 8;6 (0;4) RCPM (years; months) 7;5 (1;5) 8;9 (1;3) BPVS-II (years; months) 8;3 (2;2)8;1 (1;3) Introduction: Williams syndrome is a genetic disorder caused by a deletion of around 25 genes in the 7q11.23 region of chromosome 7. Most individuals with Williams syndrome are categorized as having mild to moderate intellectual disability but the cognitive profile is uneven with notable difficulties faced on tasks that involve visual-spatial construction. This study attempted to address the issues by pooling and re-analyzing data collected from a number of studies in which children with Williams syndrome had been tested on the British Picture Vocabulary Scale, the British equivalent of the Peabody Picture Vocabulary Test. Participants were also tested on the Ravens Colored Progressive Matrices—a measure of non-verbal reasoning ability and fluid intelligence in which participants are required to complete geometric patterns by selecting the correct piece. Abstract: The comparison of cognitive and linguistic skills in individuals with developmental disorders is fraught with methodological and psychometric difficulties. This study, illustrates some of these issues by comparing the receptive vocabulary knowledge and non-verbal reasoning abilities of 41 children with Williams syndrome, a genetic disorder in which language abilities are often claimed to be relatively strong. Data from this group were compared with data from typically developing children, children with Down syndrome, and children with non-specific learning difficulties using a number of approaches including comparison of age-equivalent scores, matching, analysis of covariance, and regression-based standardization. Standardized residuals for BPVS-II scores (controlling for age and RCPM scores) for children with Down syndrome (DS) and children with Williams syndrome (WS). References: Clinical Linguistics & Phonetics, September 2007; 21(9): Presented by Cecelia Mueller Conclusion: It has been widely argued that individuals with Williams syndrome have good vocabulary knowledge specifically as a consequence of relatively short-term memory skills. It is also worth pointing out that phonological short-term memory is thought to have the greatest influence during the earliest stages of vocabulary development, whereas the vocabulary advantage appears to be greatest in older children. The receptive vocabularies of individuals with Williams syndrome remains, therefore, something of mystery and certainly warrants further consideration. It is perhaps reassuring to note that all the analyses led to the same conclusion- that children with Williams syndrome have relatively good receptive vocabulary knowledge. Methods: Children with medically confirmed diagnoses of Williams syndrome were tested on the British Picture Vocabulary Scale and the Ravens Colored Progressive Matrices. The results were then recorded and compared to the results of children with Down syndrome and “typically developing” children to determine whether or not Williams syndrome would cause a difference in vocabulary knowledge. Discussion: This study used the example of receptive vocabulary knowledge in Williams syndrome to illustrate some of the methodological and psychometric challenges facing researchers in developmental disorders and to demonstrate the advantages and limitations of various analytical techniques. This study highlighted the fact that many of the approaches adopted in current studies in this area are prone to significant biases that can seriously distort the results. For example, the use of age-equivalent scores is problematic for a number of reasons, to the extent that even typically developing children evidenced a discrepancy between their vocabulary knowledge and non-verbal intelligence. For this reason, researchers are strongly advised against simply assuming that children’s mental ages are the same as their chronological ages when recruiting matched control groups.