PMP22 and auditory neuropathy: 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.