A rare autoimmune disease that presents as nonsyphilitic interstitial keratitis combined with audiovestibular symptoms such as tinnitus, vertigo, and acute hearing loss (similar to Meniere disease). The cause is not well understood. In typical presentations, ocular and audiovestibular signs will appear within 2 years of each other. Atypical disease may or may not present with nonsyphilitic interstitial keratitis; it can also manifest as uveitis, episcleritis, scleritis, iritis, glaucoma, or superficial keratitis. In atypical presentations, there may be more than 2 years between the appearance of ocular and audiovestibular signs.

Both presentations commonly may involve large and small vessel systemic vasculitis as well as fever, headache, arthralgia, myalgia, and arthritis. Less commonly, the patient may present with neurological and cardiovascular manifestations as well as gastrointestinal complaints such as abdominal discomfort, nausea, melena, and diarrhea.

Treatment with steroids and other anti-inflammatory drugs are frequently used to alleviate the ocular, audiovestibular, and vascular signs. These treatments do not typically reverse hearing loss, which may be permanent.