We report a patient with definite multiple sclerosis (MS) who developed unilateral sudden hearing loss coincident with exacerbation of central nervous system symptoms. Involvement of the peripheral cochlear nerve, suggested by auditory findings including auditory brainstem response, was confirmed by magnetic resonance imaging. The clinical, electrophysiological and neuroradiological abnormalities disappeared in response to steroid pulse therapy. These findings suggest that acute inflammatory demyelinative lesion of the peripheral cochlear nerve can occur in MS, manifesting sudden hearing loss.