Clinical features and treatment outcomes of otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV): A retrospective analysis of 235 patients from a nationwide survey in Japan

Abstract

Since OMAAV has novel clinical features, the disease may be categorized as a subentity for the classification of AAV.

Anti-neutrophil cytoplasmic antibody-associated hypertrophic cranial pachymeningitis and otitis media: a review of literature

“…Patients with PR3-ANCA positive OM have granulomatous formation or effusion in the middle ear, whereas MPO–ANCA positive OM predominantly presents as OM with effusion [7, 8, 27]. Most patients with MPO–ANCA positive HP have the CNS-limited form and a less severe phenotype compared with patients with PR3-ANCA positive HP [27, 36, 37].…”

“…Prednisolone and methotrexate can be used for remission and induction of patients with AAV with non-organ threatening or non-life threatening disease [40]. Corticosteroid and cyclophosphamide are more effective than corticosteroid alone, offering long-term remission, better hearing outcomes, and survival for patients with ANCA-positive HP or OMAAV [7, 8, 27, 30, 41]. Therefore, initial treatment with corticosteroid and an immunosuppressant such as cyclophosphamide should be recommended for ANCA-positive HP and OM, moreover, it should be also used in the event of a recurrence [5, 42, 43].…”

Do you know otitis media with ANCA‐Associated Vasculitis (OMAAV)?

“…OMAAV was diagnosed based on three findings: first, otitis media with effusion or granulation that was resistant to antibiotics and insertion of tympanic ventilation tubes; second, serum and/or histopathological and/or clinical signs/symptoms of AAV; third, other types of otitis media were excluded . Our case fulfilled these criteria, but eardrum biopsy was not performed.…”

Cochlear implantation in patients with bilateral deafness caused by otitis media with ANCA-associated vasculitis (OMAAV): A report of four cases

“…Although AAV is commonly accompanied by systemic symptoms particularly in the upper respiratory tract, lungs, and kidneys, AAV without systemic symptoms but with initial symptoms related to ear, such as hearing loss, otalgia, and dizziness, has recently been reported. [1][2][3] We have categorized this condition as otitis media with AAV (OMAAV), and have recently proposed its diagnostic criteria. 1) Definition of OMAAV is as follows, if the following three criteria (A, B, C) are fulfilled: (A) disease onset with initial signs/symptoms due to intractable otitis media with effusion or granulation, which is resistant to antibiotics and insertion of tympanic ventilation tubes; (B) at least one of the following three findings: (1) positivity for serum MPO-or PR3-ANCA; (2) histopathology consistent with AAV; and (3) at least one accompanying sign/symptom of AAV-related involvement other than the ear; and (C) exclusion of the other types of intractable otitis media.…”

“…1) Definition of OMAAV is as follows, if the following three criteria (A, B, C) are fulfilled: (A) disease onset with initial signs/symptoms due to intractable otitis media with effusion or granulation, which is resistant to antibiotics and insertion of tympanic ventilation tubes; (B) at least one of the following three findings: (1) positivity for serum MPO-or PR3-ANCA; (2) histopathology consistent with AAV; and (3) at least one accompanying sign/symptom of AAV-related involvement other than the ear; and (C) exclusion of the other types of intractable otitis media. 1) OMAAV is an intractable otitis media, which requires a considerable amount of time to be diagnosed as well as to start a specific therapy, including medication, and has a poor prognosis in terms of hearing. 4) Recently, owing to early detection and intervention using therapies such as steroid or immunosuppressive agents, remarkable recovery has been observed in approximately 40% of such patients, and another 30% patients have shown moderate recovery.…”