In general, patients affected by ANSD forms involving the distal portion of auditory fibers, such as the OPA1 disease, benefit from cochlear implantation in restoring speech perception [23], whereas patients with ANSD involving the proximal portion of the auditory nerve or the entire auditory fibers show a poor outcome after cochlear implantation with little or no improvement in perceptive abilities. The gene discussed is OPA1; the disease is auditory neuropathy.