Auditory Disability Study

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Approachability Task (Figure 4), administered to 26 adolescents and adults with WBS, age and gender-matched to 26 controls, asked them to rate pictures of adult faces based on how much they would like to approach and have a conversation with them. WBS subjects were significantly more likely than controls to rate both negative and positive faces as more approachable. In tasks not structured to measure social behavior, WBS children often appeared far more interested in the researcher than the task at hand, often to the detriment of their performance. Consistent with this observation, it has been demonstrated that children with WBS will often attempt to engage the researcher or distract them instead of attempting the task at hand (Järvinen-Pasley…show more content…
Language abilities, long thought to be intact in WBS, have recently been shown to be deficient relative to normal controls, although still a strength relative to the overall cognitive profile. Future studies should analyze various aspects of language abilities in WBS relative not only to children with other disorders with mental disabilities, but also to typically developing children. Auditory rote memory has long been characterized as a relative strength in WBS. However, it is under debate whether this is related to a potential increased aptitude for and interest in musical ability. Research in this area has been conflicting, and there is a need for well-controlled and replicable studies investigating whether these individuals are truly musically talented. This proves challenging as WBS subjects are eager to please and tend to show increased pleasure in all types of social interactions, potentially including listening to music. This leads to another difficulty in a variety of tasks used to evaluate aspects of the cognitive and behavioral phenotype of WBS. Due to their hypersociability, these individuals often neglect assigned tasks in favor of social interaction. It will be important to consider the effect of human presence in the administration of tasks evaluating the performance of WBS subjects. The genetic aspect of WBS is one that is incredibly interesting and has many implications for the future of our knowledge of gene-phenotype linkage. Unfortunately, individuals with abnormal and microdeletions in WBS are rare. However, identification of such subjects suggests exciting possibilities for the investigation of how specific genes in the WBS region contribute to specific phenotypes and particular aspects of its complex cognitive and behavioral
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