A CASE OF UNILATERAL AUDITORY NEUROPATHY (AUDITORY NERVE DISEASE)

Abstract

Auditory neuropathy is a sensorineural disorder characterized by absent or abnormal auditory brainstem evoked potentials and normal cochlear outer hair cell function. A variety of processes is thought to be involved in its pathophysiology and their influence on hearing may be different. We present here the management of a case of unilateral auditory neuropathy with reference to a review of the literature. The diagnosis was made through a neonatal hearing screening programme based on automated auditory brainstem response testing. The auditory brainstem response was normal for the left ear and absent for the right ear. OAEs were present for both ears. Audiometric testing indicated normal pure-tone thresholds across the frequency range for the left ear and a profound loss for the right ear at the age of six years. These findings were consistent with normal outer hair cell function bilaterally and presence of auditory neuropathy in the right ear.