A patient with myeloperoxidase antineutrophil cytoplasmic antibody-positive polyangiitis who developed sensorineural hearing loss and scleritis.

A 70-year-old woman was admitted to our hospital because of sudden hearing loss. She was treated with intratympanic dexamethasone, but her hearing impairment progressed. After admission, she developed scleritis of her left eye. Laboratory findings included elevated white blood cell count and C-reactive protein level, microhematuria, and proteinuria. Serology was positive for myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA), but negative for proteinase 3 (PR3)-ANCA. Renal biopsy revealed a single glomerulus with extensive glomerular tuft necrosis, indicating necrotizing vasculitis. She was diagnosed with MPO-ANCA-associated polyangiitis. ANCA-related polyangiitis should be considered in the differential diagnosis of sudden deafness or scleritis.