Abstract

BackgroundWilliams syndrome (WS) is a neurodevelopmental disorder characterized by hypersociability, heightened auditory sensitivities, attention deficits, and strong musical interests despite differences in musical skills. Behavioral studies have reported that individuals with WS exhibit variable beat and rhythm perception skills. MethodsWe sought to investigate the neural basis of beat tracking in individuals with WS using electroencephalography. Twenty-seven adults with WS and 16 age-matched, typically developing control subjects passively listened to musical rhythms with accents on either the first or second tone of the repeating pattern, leading to distinct beat percepts. ResultsConsistent with the role of beta and gamma oscillations in rhythm processing, individuals with WS and typically developing control subjects showed strong evoked neural activity in both the beta (13–30 Hz) and gamma (31–55 Hz) frequency bands in response to beat onsets. This neural response was somewhat more distributed across the scalp for individuals with WS. Compared with typically developing control subjects, individuals with WS exhibited significantly greater amplitude of auditory evoked potentials (P1-N1-P2 complex) and modulations in evoked alpha (8–12 Hz) activity, reflective of sensory and attentional processes. Individuals with WS also exhibited markedly stable neural responses over the course of the experiment, and these responses were significantly more stable than those of control subjects. ConclusionsThese results provide neurophysiological evidence for dynamic beat tracking in WS and coincide with the atypical auditory phenotype and attentional difficulties seen in this population.

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