Leftward Lateralization of Auditory Cortex Underlies Holistic Sound Perception in Williams Syndrome

• Published in: PloS one Vol. 5; no. 8; p. e12326
• Main Authors: Wengenroth, Martina, Blatow, Maria, Bendszus, Martin, Schneider, Peter
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 23.08.2010; Public Library of Science (PLoS)
• Subjects: Acoustics; Adolescent; Adult; Asymmetry; Auditory Cortex - pathology; Auditory Cortex - physiopathology; Auditory Perception - physiology; Auditory system; Child; Comparative analysis; Cortex (auditory); Dyslexia; Evoked Potentials, Auditory; Female; Functional Laterality; Genetic disorders; Genetic factors; Humans; Information processing; Magnetic fields; Magnetic resonance; Magnetic resonance imaging; Magnetoencephalography; Male; Medical schools; Music; Musical instruments; Nervous system; Neuroimaging; Neurological Disorders/Developmental and Pediatric Neurology; Neurological Disorders/Neuroimaging; Neuroscience/Cognitive Neuroscience; Neuroscience/Neurodevelopment; Neuroscience/Sensory Systems; Neurosciences; NMR; Nuclear magnetic resonance; Organ Size; Perception; Psychoacoustics; Radiology and Medical Imaging/Magnetic Resonance Imaging; Structure-function relationships; Studies; Substantia grisea; Training; Transcription factors; Williams syndrome; Williams Syndrome - pathology; Williams Syndrome - physiopathology; Young Adult; Germany
• DOI: 10.1371/journal.pone.0012326
• ISSN: 1932-6203

Individuals with the rare genetic disorder Williams-Beuren syndrome (WS) are known for their characteristic auditory phenotype including strong affinity to music and sounds. In this work we attempted to pinpoint a neural substrate for the characteristic musicality in WS individuals by studying the structure-function relationship of their auditory cortex. Since WS subjects had only minor musical training due to psychomotor constraints we hypothesized that any changes compared to the control group would reflect the contribution of genetic factors to auditory processing and musicality. Using psychoacoustics, magnetoencephalography and magnetic resonance imaging, we show that WS individuals exhibit extreme and almost exclusive holistic sound perception, which stands in marked contrast to the even distribution of this trait in the general population. Functionally, this was reflected by increased amplitudes of left auditory evoked fields. On the structural level, volume of the left auditory cortex was 2.2-fold increased in WS subjects as compared to control subjects. Equivalent volumes of the auditory cortex have been previously reported for professional musicians. There has been an ongoing debate in the neuroscience community as to whether increased gray matter of the auditory cortex in musicians is attributable to the amount of training or innate disposition. In this study musical education of WS subjects was negligible and control subjects were carefully matched for this parameter. Therefore our results not only unravel the neural substrate for this particular auditory phenotype, but in addition propose WS as a unique genetic model for training-independent auditory system properties.