Preserved otoacoustic emission in a child with a profound unilateral sensorineural hearing loss

We report a case of profound unilateral sensorineural hearing loss with goo d response of otoacoustic emission. The patient was a 5-year-old boy, who w as diagnosed to have unilateral hearing impairment on pure tone audiometry at the first visit. The affected ear showed the absence of auditory brainst em response; however, its transiently evoked otoacoutic emission and distor tion product otoacoustic emission were considered to be normal. These findi ngs indicated that the outer hair cell of cochlea was not impaired and that the impairment should be localized between inner hair cells, primary affer ent fiber or its synapses, spiral ganglion of the cochlea and acoustic fibe r, or at a combination of these areas. That is, evaluation of otoacoustic e mission was useful in determining the region of impairment in sensorineural hearing loss. Further follow-up will be necessary to differentiate the pre sent case from auditory neuropathy. (C) 2001 Elsevier Science Ireland Ltd. All rights reserved.