If the lesion is mainly the former, then we can expect good CI results, similar to the ANSD associated with OTOF and OPA1. In accordance with this expectation, one (SH191-430) of our two ANSD subjects carrying p.E818K (c.2452 G > A) of ATP1A3 underwent CI and showed excellent performance at 3 and 6 months after CI, although longer observation is needed (Fig. 3). The gene discussed is OPA1; the disease is auditory neuropathy.