Abstract
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.
Highlights
As a complex auditory stimulus, music engages multiple brain areas and networks and can be a window into understanding neural connectivity (e.g., [1])
The zero-order correlations of the volumetric data revealed that Distorted Tunes Test (DTT) score was significantly correlated with white matter volume in the right pars orbitalis (r = 0.507, p = 0.038; see Figure 3A)
There was a significant relationship between DTT score and left (r = 0.560, p = 0.024) and right (r = 0.543, p = 0.03) superior temporal gyrus gray matter volume only when controlling for sensitivity to specific sounds but not when controlling for other potential confounds
Summary
As a complex auditory stimulus, music engages multiple brain areas and networks and can be a window into understanding neural connectivity (e.g., [1]). Similar to studies in amusic adults [15,16], in children with amusia, event-related potentials (ERPs) to a tone series indicated intact initial auditory processing via the N100, mismatch negativity (MMN) and P200 components, but impaired conscious detection of deviant tones via the P300 [13]. Even at this earlier developmental time point, support was found for the idea that amusia is due to disconnectivity along the fronto-temporal cortical pathway rather than isolated impairments in primary auditory cortex [7,8,9,10,13]
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