Korean J Otolaryngol-Head Neck Surg.  2001 Sep;44(9):982-985.

A Case of the Audiotory Neuropathy

• Woo HY
• Kang MW
• Choi JH
• Jung SW

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.

Keyword

Auditory neuropathy; Auditory brain stem evoked potential; Otoacoustic emissisons

MeSH Terms

Cochlear Nerve
Discrimination (Psychology)
Ear, Inner
Evoked Potentials, Auditory, Brain Stem
Hair
Hearing
Hearing Disorders
Hearing Loss
Humans
Peripheral Nervous System Diseases