OBJECTIVE: To investigate the findings of peripheral and central auditory assessment of 2 subjects from the same family with auditory neuropathy spectrum disorder. CASE REPORT: The first case is that of a 35-year-old woman who presented with complaints of difficulty in hearing with no oral lips and tinnitus in the right ear. Pure tone audiometry revealed mild neurosensorial hearing loss in the left ear and moderate neurosensorial hearing loss in the right, both with rising configuration. The second case is that of a 17-year-old woman who presented with complaints of difficulty in hearing, tinnitus, and vertigo. She had difficulty undergoing pure tone audiometry and performing actions such as raising her hand in the silence. The result was mild neurosensorial hearing loss in both ears. In the 2 cases, the speech recognition index was incompatible with the degree of hearing. Type A tympanometry was observed and ipsilateral and contralateral acoustic reflexes were absent. Transient and distortion product otoacoustic emissions were present and brainstem auditory-evoked potential was absent. However, the evoked auditory event-related potential was normal in both cases. CONCLUSION: These results demonstrate that auditory neuropathy spectrum disorder causes peripheral disorder with normal cochlear outer hair cell function and abnormal auditory nerve function and normal central auditory function.
