ANCA vasculitis presenting as isolated treatment refractory otitis media

A man in his 60s presents with 3 months of progressive hearing loss, bilateral ear discharge and nasal congestion. He was treated for acute suppurative otitis media with multiple courses of topical, oral and intravenous antibiotics with minimal effect. Audiometry demonstrated severe mixed hearing loss, left worse than right. PR-3 antineutrophil cytoplasmic antibodies (ANCA) was strongly positive on an autoimmune screen, and he was diagnosed with ANCA vasculitis with isolated otologic and nasal involvement. The ANCA-associated vasculitides (AAV) are a group of small to medium vessel vasculitides with multi-system involvement characterised by blood vessel inflammation and subsequent tissue damage. This case illustrates the importance of considering alternative diagnoses when patients present with persistent treatment refractory otitis media. Otitis media with associated ANCA vasculitis (OMAAV) is increasingly recognised as an entity which may represent an earlier stage of ANCA vasculitis prior to multi-organ involvement. This may be a window of opportunity for prompt treatment.