Echoes of Silence: Diagnosing Granulomatosis with Polyangiitis via Progressive Hearing Loss: A Case Report

Granulomatosis with polyangiitis (GPA) may initially present with otologic manifestations that mimic common ear pathology, delaying diagnosis and treatment. We report a 33-year-old woman whose refractory bilateral hearing loss, despite standard ENT management, was subsequently accompanied by systemic features leading to the identification of PR3-ANCA positivity, pulmonary nodules on imaging, and granulomatous endobronchial inflammation consistent with GPA. Induction therapy per EULAR recommendations began with high-dose corticosteroids and cyclophosphamide but was escalated to rituximab due to clinical deterioration. Multidisciplinary management involving ENT, pulmonology, nephrology, audiology, and Rheumatology achieved stabilization of systemic disease, although hearing loss persisted, requiring rehabilitation. This case underscores the importance of early consideration of GPA in atypical otologic presentations to preserve hearing and control systemic involvement.