Audiologic findings in Charcot-Marie-Tooth disease

An audiologic study of a 54-year-old patient with Charcot-Marie-Tooth disease whose initial symptom was hearing difficulty is presented. While findings from basic audiologic tests were not consistent with the degree of auditory difficulty reported by the patient, special testing did indicate a notable deficit. Although the auditory periphery was probably mildly involved, it seemed that the major auditory deficit was central.