A Retrospective Observational Study of Otitis Media with Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis

Objective This study aimed to clarify the clinical features and effective treatments for localized otitis media-associated vasculitis (OMAAV). Methods We conducted a single-center retrospective study, classifying the patients into localized OMAAV and systemic OMAAV groups, based on the OMAAV diagnostic criteria, and compared their clinical features, laboratory findings, clinical course, treatment, and hearing prognosis. We also assessed any tympanic membrane findings suggestive of vasculitis using the scoring system of the OMAAV tympanic membrane (SCOT). Results We collected data from 20 OMAAV patients between January 2013 and July 2023. Seventy percent of the patients visited the otolaryngology department as their first consultation, with a median period of 2.5 months from the onset of ear symptoms to diagnosis. Compared to systemic OMAAV, localized OMAAV had fewer systemic symptoms, such as high fever, and the markers of inflammation were also lower. The positivity rate of ANCA was similar between the groups, and 90% of the cases with localized OMAAV were positive for MPO-ANCA. The combination rate of glucocorticoids (GC) and immunosuppressants (IS) in localized OMAAV was high (80%), and the initial dosage of GC was similar to that in systemic OMAAV. Regarding the treatment outcomes, the rate of hearing improvement was high at 75%, and no patients experienced a relapse of localized OMAAV. Conclusion An early diagnosis of localized OMAAV requires the recognition of tympanic membrane findings suggestive of vasculitis and measurement of the ANCA levels. Early aggressive treatment with GC and IS for localized OMAAV can lead to a favorable hearing prognosis.