Abstract
Antineutrophil cytoplasmic antibody (ANCA)-mediated otitis media is rare, posing challenges for diagnosis and treatment. This study aimed to facilitate the diagnosis and understanding of the clinical features of intractable otitis media as the initial manifestation of ANCA-associated vasculitis (AAV) and provide a compelling basis for refining treatment guidelines specific to this rare condition. We retrospectively analyzed the medical records of patients exhibiting intractable chronic otitis media as their initial AAV. The most common initial symptoms were intractable chronic otitis media with effusion or otorrhea, unresponsive to standard treatments, leading to progressive hearing loss and facial palsy. Patients exhibited characteristic tympanic membrane findings, including typical vasodilated hypervascularity. Facial palsy was a common accompanying symptom, affecting 60% of the patients. Following a treatment regimen involving a combination of corticosteroids and immunosuppressants, otitis media subsided, restoring hearing. After initiating treatment with corticosteroids and immunosuppressants, all patients experienced significant improvement, with no reported relapses. Intractable chronic otitis media, as the initial presentation of AAV, originated from the middle ear before progressing to other organs, potentially causing severe otologic complications such as facial palsy and hearing loss. However, timely diagnosis of ANCA-associated otitis media and initiation of treatment should be considered to improve clinical outcomes.