The central nervous system (CNS) is often a preferential target of Behçet's disease and diverse neurological manifestations have been described. Isolated hearing impairment or disequilibrium is also known to occur, but the simultaneous bilateral impairment of both vestibular and cochlear functions has been only rarely documented in Behçet's disease. A 52-year-old Japanese woman with neuro-Behçet's disease had meningoencephalitis, a profound thrombocytopenia, and bilateral vestibulocochlear impairment during the acute exacerbation. Although the CNS involvement was apparent, the clinical history and neuro-otological findings pointed to bilateral inner ear involvement rather than the brainstem or the 8th nerve lesions. She has made an excellent clinical response to pulse-dose methylprednisolone therapy with improvement in her neurological symptoms and in arresting the acute deterioration of hearing loss, but repeated audiograms failed to maintain the improvement with a modest dose of oral prednisolone. Since the bilateral vestibulocochleopathy can be as incapacitating as oculopathy in Behçet's disease, an early detection and prompt instillation of adequate corticosteroid therapy may be mandatory, although not curative.