A Case of the Audiotory Neuropathy / 대한이비인후과학회지

ABSTRACT

Auditory neuropathy is a recently described hearing disorder in which patients demonstrate hearing loss for pure tones, impaired word discrimination out of proportion to pure tone loss, absent or abnormal auditory brainstem responses (ABR), but normal outer hair cell function as measured by otoacoustic emissions and cochlear microphonics. Auditory neuropathy affects the normal synchronous activity in the auditory nerve, without affecting the amplification function in the inner ear. There is impairment in their temporal processing in the auditory nerve. So patients can hear sounds, but can not understand speech. Auditory neuropathy occurs either in isolation or as a part of a generalized neuropathy. We have identified one pediatric patient who demonstrated hearing defects and developed signs for peripheral neuropathy. We report it with a review of literatures.