Auditory Neuropathy: Evaluation and Habilitation of a Hearing Disability

Auditory neuropathy (AN) is a type of hearing loss that can be present from birth or acquired postnatally. AN results in a loss of sensitivity for pure tones and very poor speech perception abilities, disproportionate to the degree of pure tone sensitivity impairment. A deficit in the timing of neural signals transmitted by the auditory nerve appears to underlie the significant speech perception disability. AN is diagnosed by considering the results of electrodiagnostic tests of auditory function in conjunction with pure tone and speech perception tests. This article will review the methods used to assess AN, the audiometric and speech perception findings for infants and children with AN, and the habilitation methods that are recommended. The etiologies and pathophysiologic processes of AN will also be discussed.