Otitis Media in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Case Series and Review

Otitis media associated with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis represents an underrecognized manifestation of systemic vasculitic diseases, including eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis (GPA). Eosinophilic otitis media shares several clinical and immunological features with localized forms of EGPA, which complicates differential diagnosis. This study presents two cases that illustrate diagnostic and therapeutic challenges and highlight the clinical overlap and heterogeneity of these conditions.

A 30-year-old woman with severe asthma, nasal polyposis, and ANCAnegative eosinophilic otitis media reported ear fullness, hearing loss, and otorrhea. She met the diagnostic criteria for EGPA, with peripheral eosinophilia and pulmonary infiltrates. These findings may have been related to dupilumab therapy. Treatment with systemic glucocorticoids and benralizumab led to clinical stabilization. However, symptoms of otitis media with a predominant neutrophilic inflammatory pattern recurred during glucocorticoid tapering. A 65-yearold woman with ANCA-positive GPA presented with systemic manifestations, including weight loss, episcleritis, and pulmonary and renal involvement, as well as otitis media. She achieved clinical improvement following treatment with rituximab and glucocorticoids.

Eosinophilic otitis media may represent a local manifestation of eosinophilic granulomatosis with polyangiitis, in which overlapping type 1 and type 2 inflammatory processes complicate classification. The limited number of observations currently precludes definitive conclusions, underscoring the need for further research. In contrast, granulomatosis with polyangiitis is marked by severe systemic involvement. Myeloperoxidase-DNA complexes in middle ear effusion show promise as biomarkers, pending further study. To advance understanding, multicenter studies are essential to refine nosological classification and optimize therapeutic strategies for eosinophilic middle ear disease.