We report on a patient with Lyme disease who presented with chronic bilateral otitis media and cranial neuropathy with rapid progressive hearing loss. After ceftriaxone and high-dose intravenous immunoglobulins, the disease was controlled only with methylprednisolone and cyclophosphamide. The relationship between Lyme disease and granulomatous vasculitis is discussed. Lyme disease should be kept in mind in the differential diagnosis of various otolaryngological and neurological presentations.