Profound hearing loss attributable to cochlear nerve disease: diagnosis with combination of otoacoustic emission and magnetic resonance imaging.

Abstract

To detect the causes of deafness based on the combined findings of auditory brainstem response (ABR), distortion product otoacoustic emissions (DPOAEs), and three-dimensional Fourier transformation-constructive interference in steady state (3DFT-CISS) magnetic resonance imaging (MRI). Retrospective review of the medical records of 13 patients with unilateral profound hearing loss since childhood. Subjects were tested with pure-tone audiometry, ABR, DPOAEs, and 3DFT-CISS imaging. No significant components of ABR were observable in any of the deaf ears. In 10 cases, the cochlear nerves of the deaf ears were found to be as normal as the healthy sides by 3DFT-CISS imaging, and no significant levels of DPOAEs were recorded. In the other three cases, no apparent cochlear nerves were identified by 3DFT-CISS imaging. Although no significant levels of DPOAEs were observable in two cases with cochlear nerves invisible by the MRI study, almost the same level of DPOAEs as that in the healthy side was recorded in the last case. In the last particular case, the cochlear nerve seemed to be mainly responsible for the profound deafness. 3DFT-CISS imaging in combination with preexisting audiological measures may provide direct evidence for the cochlear nerve disease. steady state, internal auditory canal, cochlear nerve disease.