Neural Correlates of Auditory Perception in Williams Syndrome: An fMRI Study

Authors: Levitin D.J.¹; Menon V.^{2, 4}; Schmitt J.E.²; Eliez S.²; White C.D.²; Glover G.H.⁵; Kadis J.³; Korenberg J.R.⁶; Bellugi U.⁷; Reiss A.L.^{2, 4}

Source: NeuroImage, Volume 18, Number 1, January 2003 , pp. 74-82(9)

Publisher: Academic Press

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Abstract:

Williams syndrome (WS), a neurogenetic developmental disorder, is characterized by a rare fractionation of higher cortical functioning: selective preservation of certain complex faculties (language, music, face processing, and sociability) in contrast to marked and severe deficits in nearly every other cognitive domain (reasoning, spatial ability, motor coordination, arithmetic, problem solving). WS people are also known to suffer from hyperacusis and to experience heightened emotional reactions to music and certain classes of noise. We used functional magnetic resonance imaging to examine the neural basis of auditory processing of music and noise in WS patients and age-matched controls and found strikingly different patterns of neural organization between the groups. Those regions supporting music and noise processing in normal subjects were found not to be consistently activated in the WS participants (e.g., superior temporal and middle temporal gyri). Instead, the WS participants showed significantly reduced activation in the temporal lobes coupled with significantly greater activation in the right amygdala. In addition, WS participants (but not controls) showed a widely distributed network of activation in cortical and subcortical structures, including the brain stem, during music processing. Taken together with previous ERP and cytoarchitectonic studies, this first published report of WS using fMRI provides additional evidence of a different neurofunctional organization in WS people than normal people, which may help to explain their atypical reactions to sound. These results constitute an important first step in drawing out the links between genes, brain, cognition, and behavior in Williams syndrome. © 2002 Elsevier Science (USA)

Keywords: Williams syndrome; auditory cortex; amygdala; acoustic stimulation; music; noise; hyperacusis

Language: English

Document Type: Research article

DOI: 10.1006/nimg.2002.1297

Affiliations: 1: Department of Psychology, McGill University, Montreal, Quebec, H3A 1B1, Canada 2: Department of Psychiatry and Behavioral Sciences 3: Department of Psychiatry and Behavioral Sciences, Center for Computer Research in Music and Acoustics 4: Department of Psychiatry and Behavioral Sciences, Center for Computer Research in Music and Acoustics, Stanford Brain Research Center 5: Department of Psychiatry and Behavioral Sciences, Center for Computer Research in Music and Acoustics, Stanford Brain Research Center, Department of Radiology, Stanford University School of Medicine, Stanford, California, 94305 6: Human Genetics, UCLA, Los Angeles, California, 90048 7: Salk Institute for Biological Studies, La Jolla, California, 92037

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