Isolated otologic presentation of ANCA-associated vasculitis as an initial manifestation: clinical features, treatment response, and long-term outcomes

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) may rarely present as isolated, intractable otitis media (OM) without initial systemic involvement, resulting in delayed diagnosis.

To characterize the clinical presentation, diagnostic clues, treatment response, and long-term outcomes of patients with refractory OM as the initial manifestation of AAV.

We retrospectively reviewed patients at a tertiary referral center who presented with intractable OM and were diagnosed with AAV based on serologic testing and otologic findings.

Ten patients were identified (median age 63 years; 80% female), nine with bilateral disease. Myeloperoxidase-ANCA was positive in eight patients and proteinase 3-ANCA in two. All exhibited persistent effusion or otorrhea unresponsive to conventional therapy. Prominent hypervascular and dilated tympanic membrane capillaries were frequently observed and provided an important diagnostic clue. Facial palsy occurred in six patients. Mean pure-tone average improved from 75.7 dB to 42.8 dB after systemic corticosteroids combined with immunosuppressants. Sustained disease control was achieved without relapse during a mean follow-up of 4 years 11 months, with only mild delayed systemic involvement in a subset.

Consistent otologic features and favorable long-term outcomes highlight the importance of considering ANCA-associated OM in refractory bilateral disease or facial palsy to enable timely immunosuppressive treatment.