Autoimmune inner ear disease: A treatment update

Abstract

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.

Systematic Review of Treatments for Autoimmune Inner Ear Disease

“…Case reports have described a moderate recovery in a subject who was myeloperoxidase-antineutrophil cytoplasmic antibody positive and had failed steroid treatment (19), one pediatric subject who had stabilization of hearing on cyclophosphamide after failing prednisone and methotrexate (20), one subject with Cogan's syndrome that progressed with steroids, temporarily improved with cyclophosphamide before progressing again (21), a subject with hearing loss and Wegener's granulomatosis who improved with cyclophosphamide (22), and two other subjects showing improvement (23).…”

“…Additionally, case reports of the use of plasmapheresis/plasma exchange in AIED include: improvement in a patient with systemic lupus erythematous and secondary AIED (38), one subject who failed azathioprine with temporary improvement with plasma exchange (39), three subjects with immune-complex associated hearing loss (4), and one others (23).…”