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Psychoacoustics and neurophysiological auditory processing in patients with Charcot-Marie-Tooth disease types 1A and 2A.

BACKGROUND AND PURPOSE: Hidden hearing loss has been reported in patients with Charcot-Marie-Tooth (CMT) disease, however, the auditory processing deficits have not been widely explored. We investigated the psychoacoustical and neurophysiological aspects of auditory processing in patients with CMT type 1A (CMT1A) and CMT type 2A (CMT2A).

METHODS: Forty-three patients with CMT1A and 15 patients with CMT2A were prospectively enrolled. All CMT patients had a normal sound detection ability by using pure-tone audiometry. Spectral ripple discrimination, temporal modulation detection, and auditory frequency-following response (FFR) were compared between CMT1A, CMT2A, and control groups.

RESULTS: Although all participants had normal audiogram, CMT patients had difficulty understanding speech in noise. The psychoacoustic auditory processing was somewhat different depending on the underlying pathophysiology of CMT disease. CMT1A patients had degraded auditory temporal and spectral processing. CMT2A patients had no reduced spectral resolution, but they showed further reduced temporal resolution than did the CMT1A patients. The amplitudes of the FFR were reduced in CMT1A and CMT2A patients, but the neural timing remained relatively intact.

CONCLUSIONS: When we first assessed the neural representation to speech at the brainstem level, the grand average brainstem responses were reduced in both CMT1A and CMT2A patients compared to healthy controls. Since psychoacoustic aspect of auditory dysfunctions in CMT1A and CMT2A disease were somewhat different, it is necessary to consider future auditory rehabilitation methods based on their pathophysiology.

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