Keywords: auditory neuropathy, hearing disorder, neural disynchronization

Abstract: Auditory neuropathy consists of an exclusive aggression of the auditory nerve, that causes a desynchrony in the nervous conduction, probably related to myelinization alterations of these fibers. Clinically, there is hearing loss and abnormal vocal discrimination. Otoacoustic emissions are normal with non-responsive ABR. It may be associated with some diseases that happen with peripheral neuropathy. The authors reviewed the literature and present a case of a patient with auditory neuropathy associated with pigmentar retinosis, with discussion of possible etiologies.