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  • Updated 05.14.2024
  • Released 04.26.1996
  • Expires For CME 05.14.2027

Autoimmune sensorineural hearing loss



Autoimmune hearing loss is a potentially reversible form of hearing loss defined by rapidly progressive sensorineural loss and a characteristic recovery with immunosuppressive therapy. The disorder’s pathogenesis is uncertain, but damage to inner ear structures is thought to occur through an immune-mediated process. In this article, the authors review the current understanding of the disorder. The authors also provide an update on treatments for autoimmune hearing loss.

Key points

• Autoimmune sensorineural hearing loss is a well-described clinical entity with an incompletely understood pathogenesis.

• Cogan syndrome is characterized by audiovestibular dysfunction and interstitial keratitis.

• Treatment focuses on suppressing a likely, though presumed, abnormal immune response to inner-ear antigens.

• Classically, autoimmune sensorineural hearing loss improves with initiation of immunosuppressive therapy, deteriorates with discontinuation of therapy, and recovers with reinstitution of treatment.

Historical note and terminology

Autoimmune sensorineural hearing loss is a well-described clinical entity with an uncertain pathogenesis. Known also as "immune-mediated sensorineural hearing loss," "autoimmune inner-ear disease," and "steroid-responsive sensorineural hearing loss," this disorder is one of only a few forms of sensorineural hearing loss that can be treated with the expectation of significant improvement in auditory acuity.

A relationship between autoimmune diseases and hearing loss is well recognized, with descriptions of sensorineural hearing loss seen in such systemic autoimmune diseases as systemic lupus erythematosus, polyarteritis nodosa, granulomatous polyangiitis (formerly called Wegener granulomatosis), and rheumatoid arthritis (40). In addition, reports have also described associations with celiac disease (124; 55), primary antiphospholipid syndrome (14; 125), Behcet disease (24; 25), Sweet syndrome (16), ankylosing spondylitis (01), Sjögren's syndrome (88; 90); systemic sclerosis (67), sclero-uveitis in the setting of HLA-B27 positivity (97), Cogan syndrome (79), Susac syndrome (22; 50; 06; 09; 101; 111; 112; 86; 96; 102; 127), sarcoidosis (128), and type 1 autoimmune hepatitis (138). See Table 1 in the “Etiology” section for a complete listing of associated diseases.

An immune response directed against the inner ear resulting in sensorineural hearing loss was first described in 1979 (75). McCabe described sensorineural hearing loss in 18 patients with a variable presentation of progressive bilateral asymmetrical sensorineural hearing loss accompanied by vestibular symptoms and a positive serologic response to inner-ear antigens. Treatment with dexamethasone and cyclophosphamide substantially improved hearing. The distinctive clinical course, laboratory findings, and clinical response to immune therapy suggested that this entity is autoimmune-mediated. Subsequent literature described a characteristic clinical presentation to include rapid onset (usually unilateral) and a more slowly progressing form of sensorineural hearing loss (76).

A condition referred to as "steroid-responsive bilateral sensorineural hearing loss" appeared in the literature in the 1980s (54). Several patients diagnosed with idiopathic sensorineural hearing loss showed improvement in audiologic measurements following steroid therapy and subsequent deterioration of auditory sensitivity on discontinuation of treatment. These patients also revealed elevated serum levels of immune complexes that responded to steroid treatment (53). Also described was an immune complex-mediated disorder with features of vasculitis that targeted the inner ear and improved with glucocorticoids (122).

Although diagnostic criteria for autoimmune sensorineural hearing loss have not been established, the hallmark features of progressive sensorineural hearing loss and steroid responsiveness are widely recognized features.

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