[Auditory neuropathy].

OBJECTIVE

To study the clinical feature and audiological and electrophysiological characteristics of auditory neuropathy.

METHODS

The history, audiometry, stapedial reflex, electrocochleogram, auditory brainstem response, promontory stimulation tests, otoacoustic emissions (OAE) and its contralateral supression tests were studied in 17 cases of auditory neuropathy.

RESULTS

The patients were predominently female teenagers with a history of gradual onset of bilateral hearing loss, pure tone auditometry revealed symmetric ascending audiograms with moderate hearing loss at frequencies below 1 kHz, but relatively normal hearing at 2 kHz and above. Speech reception thresholds were between 40 to 85 dB HL, discrimination score was from 29% to 68%. Stapedial reflex, auditory brainstem response and compound action potentials were absent or threshold elevated disproportionately to the pure tone threshold. All of the patients had normal cochlear microphonics and robust spontaneously and transiently evoked otoacoustic emissions (SOAE and TEOAE), the TEOAE showed no contralateral suppression effects as seen in normal subjects.

CONCLUSION

The diagnosis of the disease depends on the discrepancies between subjective and objective hearing and pre-neural and neural responses. The crossed olivocochlear bundle function testing in terms of contralateral acoustic stimulation induced otoacoustic emission reduction is specific for differential diagnosis. There is a need for more awareness of the disease among otolaryngologists and neurologists.