Table 4.

Diagnostic criteria for otitis media with ANCA-associated vasculitis (OMAAV)

OMAAV classified if the following three criteria fulfilled

1. Disease onset with intractable otitis media (OM) resistant to conventional treatment

2. At least one of the following

Serum positive for MPO- or PR3-ANCA

Histopathology consistent with ANCA associated vasculitis (necrotizing vasculitis predominantly affecting small vessels with or without granulomatous inflammation)

At least one accompanying sign or symptom of ANCA associated vasculitis related organ or tissue involvement other than the ear (eye, nose, pharynx, larynx, lung, kidney, facial palsy, hypertrophic pachymeningitis)

3. Exclusion of other types of intractable OM and other autoimmune diseases with vasculitis

Bacterial otitis media, cholesterol granuloma, cholesteatoma, skull base osteomyelitis, tuberculous otitis, neoplasm, eosinophilic otitis media

Exclusion of other autoimmune diseases with vasculidities (ex: Cogan’s syndrome and polyarteritis nodosa

Adapted from Reference [43]