Abnormal ABRs are characteristic of the auditory neuropathy observed in late-onset CMT1B patients harboring the P0Y145S mutation [17], patients with PMP22 mutations [18,19], and those with GJB1 (gap junction beta 1) mutations [18,20], suggesting that hearing impairment in those with the P0T124M mutation may be due in part to degeneration of the distal part of the auditory nerve. This evidence concerns the gene GJB1 and auditory neuropathy.