Auditory neuropathy: Diagnostic and therapeutic challenge report of first series of four cases from India

Auditory neuropathy (AN), a recently described clinical entity, is a sensorineural disorder where the patient has hearing loss with impaired word discrimination out of proportion to pure tone loss in the presence of abnormal/absent auditory brain stem responses, and normal outer hair cell as measured by otoacoustic emissions and/cocklear microphonics. It is essential that the practicing ENT surgeon have a high degree of suspicion of AN in patients complaining of difficulty in understanding speech with hearing loss and audiological evidence of dissociation between pure-tone and speech audiometry. Appropriate newer diagnostic tests of ABR and OAE and/or CM for confirmation of AN is essential. We present a series of four patients with auditory neuropathy from a tertiary care teaching hospital. This is the first Series of 4 cases of this clinical entity from the Indian subcontinent.