The potential role of the cABR in assessment of normal hearing adults with inherited neuropathies.

Objectives: The aim of this study was to investigate 1) the auditory pathway function and 2) the potential role of the complex auditory brainstem response (cABR) in a group of normal hearing (NH) adults with inherited neuropathies. Material and methods: Thirty-five NH adults with Charcot- Marie-Tooth (CMT) disease and 48 heathy NH listeners participated. We assessed speech in noise perception and recorded ABRs to three speech syllables. We calculated FO amplitude and average phase-shift values. Results: CMT patients showed significantly higher SNR thresholds than control group. Speech in noise perception was significantly correlated with phase difference between /DA/ and /BA/ after controlling age, sex, and pure tone thresholds. Conclusions: While CMT patients had normal pure tone thresholds, they were difficult to understand in noisy background. Speech perception in noise ability was significantly correlated with subcortical auditory processing of speech [ABSTRACT FROM AUTHOR]