McCabe (1979) described 18 cases of sensorineural deafness having clinical and laboratory features suggestive of an auto-immune etiology. This condition was characterized by a prolonged period of pro-gressive deafness over weeks or months, rather than hours, days or years. It is usually bilateral, asymmetrical and the cochlear is always involved. Other features of this disorder are facial paralysis and vestibular dysfunction. Tissue destruction of the tympanic membrane and middle ear and mastoid may also occur. These responded well to a high dose of steroid therapy and cyclophosphamide. A total of 42 cases of this disorder were diagnosed in McCabe's series as of January, 1982 (McCabe and Harker, 1982). Two-thirds of the patients had vestibular symptoms of low grade without spells and the reduction in caloric responses paralleled the hearing loss.
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