Progressive neural hearing impairment: case report

This report describes a patient whose ability to understand speech had so deteriorated over 20 years time that she was being considered for a cochlear implant, despite the fact that pure-tone sensitivity loss had not deteriorated proportionately. An unusual combination of otoacoustic emissions (OAEs) and auditory evoked potential (AEP) results are described. Click-evoked and distortion product emissions were present and normal-appearing. Auditory brainstem and middle latency responses were totally absent but the N1-P2 complex of the long-latency response was present. The case illustrates the contribution of otoacoustic emissions to site of lesion testing. It also illustrates that the manifestations of pathology can appear in certain epochs of the family of AEPs without affecting the others.