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Brain Sci. 2014, 4(4), 594-612;

Neural Correlates of Amusia in Williams Syndrome

Vanderbilt Kennedy Center, Vanderbilt University, Nashville, TN 37203, USA
Psychology and Human Development, Vanderbilt University, Nashville, TN 37203, USA
Emory University School of Medicine, Marcus Autism Center, Children's Healthcare of Atlanta, Atlanta, GA 30329, USA
Department of Radiological Sciences, St. Jude Children's Research Hospital, Memphis, TN 38105, USA
Vanderbilt University Institute of Imaging Science, Vanderbilt University, Nashville, TN 37203, USA
Center for Human Genetics Research, Vanderbilt University, Nashville, TN 37203, USA
Author to whom correspondence should be addressed.
Received: 8 August 2014 / Revised: 29 October 2014 / Accepted: 5 November 2014 / Published: 21 November 2014
(This article belongs to the Special Issue Music and Neural Plasticity)
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Congenital amusia is defined by marked deficits in pitch perception and production. Though historically examined only in otherwise typically developing (TD) populations, amusia has recently been documented in Williams syndrome (WS), a genetic, neurodevelopmental disorder with a unique auditory phenotype including auditory sensitivities and increased emotional responsiveness to music but variable musical skill. The current study used structural T1-weighted magnetic resonance imaging and diffusion tensor imaging to examine neural correlates of amusia in 17 individuals with WS (4 of whom met criteria for amusia). Consistent with findings from TD amusics, amusia in WS was associated with decreased fractional anisotropy (FA) in the right superior longitudinal fasciculus (SLF). The relationship between amusia and FA in the inferior component of the SLF was particularly robust, withstanding corrections for cognitive functioning, auditory sensitivities, or musical training. Though the number of individuals with amusia in the study is small, results add to evidence for the role of fronto-temporal disconnectivity in congenital amusia and suggest that novel populations with developmental differences can provide a window into understanding gene-brain-behavior relationships that underlie musical behaviors. View Full-Text
Keywords: Williams syndrome; amusia; music; DTI; MRI; superior longitudinal fasciculus; pars orbitalis Williams syndrome; amusia; music; DTI; MRI; superior longitudinal fasciculus; pars orbitalis

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Lense, M.D.; Dankner, N.; Pryweller, J.R.; Thornton-Wells, T.A.; Dykens, E.M. Neural Correlates of Amusia in Williams Syndrome. Brain Sci. 2014, 4, 594-612.

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