Abstract

Williams syndrome (WS), a genetic, neurodevelopmental disorder, is of keen interest to music cognition researchers because of its characteristic auditory sensitivities and emotional responsiveness to music. However, actual musical perception and production abilities are more variable. We examined musicality in WS through the lens of amusia and explored how their musical perception abilities related to their auditory sensitivities, musical production skills, and emotional responsiveness to music. In our sample of 73 adolescents and adults with WS, 11% met criteria for amusia, which is higher than the 4% prevalence rate reported in the typically developing (TD) population. Amusia was not related to auditory sensitivities but was related to musical training. Performance on the amusia measure strongly predicted musical skill but not emotional responsiveness to music, which was better predicted by general auditory sensitivities. This study represents the first time amusia has been examined in a population with a known neurodevelopmental genetic disorder with a range of cognitive abilities. Results have implications for the relationships across different levels of auditory processing, musical skill development, and emotional responsiveness to music, as well as the understanding of gene-brain-behavior relationships in individuals with WS and TD individuals with and without amusia.

Highlights

  • Williams syndrome (WS) is a neurodevelopmental disorder caused by the deletion of 26–28 genes on chromosome seven and has an estimated prevalence of one in 7,500 births (Strømme et al, 2002)

  • RATES OF AMUSIA Figure 1 displays the distribution of scores on the Distorted Tunes Test (DTT) in our WS sample

  • (A)musicality in Williams syndrome prevalence rate in the typically developing (TD) population based on an exact binomial test (p = 0.009)

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Summary

Introduction

Williams syndrome (WS) is a neurodevelopmental disorder caused by the deletion of 26–28 genes on chromosome seven and has an estimated prevalence of one in 7,500 births (Strømme et al, 2002). Research into WS has become increasingly popular because its known genetic etiology and unique cognitive-behavioral profile allows for study of gene-brain-behavior links. IQ appears to be relatively stable with age (Howlin et al, 1998; Porter and Dodd, 2011; Mervis et al, 2012) with greater verbal than spatial abilities (Bellugi et al, 1994). Receptive language abilities appear to be a relative strength in WS while other aspects of language are consistent with their cognitive profile (Brock, 2007). WS is further characterized by anxiety (Dykens, 2003), attention problems (Rhodes et al, 2010), and hypersociability and empathic behavior (Zitzer-Comfort et al, 2007)

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