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Audiovestibular disease exists in conjunction with several autoimmune disorders. Ankylosing spondylitis, Cogan disease, rheumatoid arthritis, systemic lupus erythematosus, Sjögren syndrome, Wegener granulomatosis, ulcerative colitis, relapsing polychondritis, Behçet disease and scleroderma are associated with autoimmune inner ear disease. We present a patient with HLA-B27-associated sclerouveitis and sensorineural hearing loss, and postulate an association between atypical Cogan syndrome and HLA-B27 autoimmunity.
A 52-year-old HLA-B27 positive Caucasian woman with a 3-year history of intermittent sclerouveitis, presented with a 2-month history of bilateral redness and discomfort. She reported a concurrent 3-month history of left-sided hearing deficit and tinnitus. The corticosteroids prescribed for her ocular symptoms also improved her auditory deficit. Past medical history was significant for vitiligo and Raynaud phenomenon. Family history was non-contributory.
Review of systems revealed a prodrome of fever, chills, myalgia, arthralgia and night sweats. Intraocular inflammation was quiescent. An audiogram demonstrated a left-sided sensorineural deficit. Brainstem auditory evoked potentials were …
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