Auditory neuropathy/dys-synchrony disorder affects neural responses, either directly or indirectly. Patients may demonstrate good ability to detect sound, but have significant difficulty listening in noise. Clinical auditory physiologic measures are used to characterize cochlear, eighth nerve, and brainstem function, and are needed to accurately identify this disorder. Cochlear implants provide benefit to many patients, and some patients derive benefit from amplification. This disorder can be identified and managed in infants, may have later onset, may be a part of a syndrome, and may include fluctuation in hearing ability.