Are there any specific diagnostic tests for IMIED?
Antibodies to a 68-kDa inner ear antigen, which is a heat shock protein (HSP-70), may be seen in up to 70% of patients with IMIED. Due to low sensitivity, this test is unreliable to exclude a diagnosis of IMIED. Patients with Cogan’s syndrome and other autoimmune diseases associated with SNHL can also have these antibodies (<50%), although the specificity compared with normal control populations may be as high as 90%. Some studies suggest that their presence may have a prognostic role, predicting both a more aggressive course and a more favorable response to steroids. Data on antibody specificity and association with steroid response is controversial, however, with conflicting data in the literature.