Immune-mediated inner-ear disorders in neuro-otology

Immune-mediated inner-ear disorders may present to different medical disciplines and new research findings emerge rapidly. The purpose of this review is to draw the different strands together to produce an overview describing the clinical presentation of immune-mediated inner-ear disorders and to discuss useful diagnostic criteria with a focus on tissue-specific and tissue-non-specific antibodies.

The importance of diagnosing an immune-mediated inner-ear disorder is highlighted in the context of it being one of few forms of treatable inner-ear disorder with a good response to immunosuppressive therapy. Due to a lack of reliable tests, the criteria upon which the diagnosis of immune-mediated inner-ear disease is based are often arbitrary. Previous and current research focuses mainly on the investigation of the aetiology of immune-mediated disorders, studying the presence of autoantibodies and the antigens responsible for their production. The prognostic and therapeutic values of inner-ear-specific antibodies are still unclear. Various antigens have been suggested. However, most antigens identified have been ubiquitous proteins not specific to the inner ear and therefore lack logical association with localized inner-ear pathology.

Early diagnosis of immune-mediated inner-ear disorders with prompt treatment may prevent irreversible damage to inner-ear structures. Accordingly, it is important to include immune-mediated inner-ear disorders in the differential diagnosis of patients presenting with 'idiopathic' audiovestibular dysfunction. To enable early diagnosis, we recommend that the current available routine immunological laboratory tests (antinuclear, antineutrophil cytoplasmic, antiendothelial cell, antiphospholipid/anticardiolipin and antithyroid antibodies) are used when assessing a patient with suspected immune-mediated inner-ear disorder.