It is now evident that the inner ear is not an "immunologically privileged" site and may mount an immune response against both foreign and self-antigens. This chapter focuses on sensorineural hearing loss (SNHL). This has been described and termed in different ways: autoimmune SNHL, immunemediated inner ear disease (IMIED), idiopathic progressive bilateral SNHL, sudden SNHL, and idiopathic SNHL, bilateral immune-mediated Menière's disease, autoimmune vestibulo-cochlear disorders, generating a great confusion in the identification of patients and in the evaluation of different studies. The association of IMIED with systemic autoimmune diseases provides evidence that autoimmunity can damage the inner ear, but it does not address organ-specific disease. Antibodies directed against different inner ear antigens have been identified in some patients. However, they are neither diagnostic nor correlate with disease state. In the future, the major goals for research in this field can be the identification of pathogenetically relevant autoantigens, development of a highly specific diagnostic test, and a better knowledge of the immunopathologic mechanisms in an organ as inaccessible as the inner ear.
ASJC Scopus subject areas
- Immunology and Microbiology(all)