2020 Volume 30 Issue 2 Pages 97-103
Otitis media with anti-neutrophil cytoplasm (ANCA)-associated vasculitis (OMAAV) is a disease that comprehensively involves middle ear and inner ear lesions due to ANCA-associated vasculitis (AAV). We reviewed the clinical course of 16 ears in 9 patients with OMAAV. Of the 9 cases, seven suffered bilaterally, and 6 had the eardrums matched bilaterally, a characteristic suggestive of a systemic disease. On the other hand, unilateral cases and different onset of bilateral ears were also included. MPO-ANCA was positive in 5 cases and PR3-ANCA in 2 cases; no case was positive for both. For AAV, 5 cases were diagnosed as granulomatosis with polyangiitis, and 3 cases were diagnosed with microscopic polyangiitis. A corticosteroid was applied in all cases, and an immunosuppressant or a molecular targeting agent was added depending on the case. Complete or marked recovery of hearing was achieved in 5 ears. Four eardrums were classified as typical OMAAV (normal type), which had a better hearing outcome than the effusion or granulation type. Although no relationship between hearing outcome and duration from onset to treatment was established, hearing loss in the patients who needed a long period to begin treatment either did not change or worsened. Facial nerve palsy was observed in one patient, multiple cranial nerve palsy due to hypertrophic pachymeningitis in another, and hearing loss of the affected side in both ears did not improve. Early diagnosis and prompt treatment are required.
