Autoimmune inner ear disease (AIED): A diagnostic challenge.

Authors: Ciorba A, Corazzi V, Bianchini C, Aimoni C, Pelucchi S, Skarżyński PH, Hatzopoulos S Abstract Autoimmune inner ear disease (AIED) has been defined as a condition of bilateral sensorineural hearing loss (SNHL), caused by an 'uncontrolled' immune system response. The inner ear can be the direct target of the immune response, but it can be additionally damaged by a deposition of circulating immune complexes or by systemic immune-mediated diseases. The clinical expression of immune-mediated inner ear disease shows a progressive bilateral and asymmetric SNHL profile, which typically benefits from a steroid and immunosuppressive therapy. The onset of AIED is between 3 and 90 days. Cochlear symptoms can be associated with vestibular disorders and in 15%-30% of cases, AIED occ...

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