Open-access Amusia and its electrophysiological correlates in neurofibromatosis type 1

Amusia e seus correlatos eletrofisiológicos na neurofibromatose tipo 1

ABSTRACT

Auditory processing deficits are common in people with neurofibromatosis type 1 (NF1) and they often report difficulties in musical performance.

Objective:  We investigated whether NF1 could be associated with amusia as well as with some impairment of primary auditory cortex activity.

Methods:  Eighteen people with NF1 and 22 healthy volunteers, matched for age, sex and educational level, were evaluated with the Montreal Battery Evaluation of Amusia – short version. The integrity of cortical primary auditory processing areas was evaluated by evoked potential mismatch negativity.

Results:  Amusia was correlated with NF1 (p = 0.001, odds ratio = 42.0, confidence interval 4.5–39.6). Patients with NF1 exhibited a greater prevalence of amusia than healthy controls (67% vs. 4.5%) and difficulties in both melodic and temporal music perception. Worse performance on the Montreal Battery Evaluation of Amusia was correlated with a greater mismatch negativity latency in NF1 group.

Conclusions:  Amusia is a common feature in NF1 and may result from impairment of activity in primary auditory processing areas.

Keywords: neurofibromatosis 1; music; evoked potentials; auditory perceptual disorders; pitch discrimination

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