Tuberculosis of the middle ear is currently a rare disease in developed countries but one that still occurs and typically causes significant morbidity manifested by profound and permanent hearing loss. Diagnosis is difficult since most physicians are unfamiliar with the typical presenting features; in addition, special cultural and pathologic studies are required for diagnosis. A case report and review of the literature are presented to delineate typical clinical, epidemiologic, and laboratory features of tuberculous otitis media and associated tuberculous disease. A diagnostic and therapeutic plan is outlined that would allow early therapy and preservation of hearing.