Granulomatosis with polyangiitis (GPA) is a systematic autoimmune disorder characterized by necrotizing vasculitis of small to medium-sized-vessels and granuloma formation. GPA is commonly initially presented with respiratory and renal symptoms. Although involvement of the temporal bone is not unusual, an otologic disorder is rarely the presenting symptom. We present a case of a 36-year-old patient complaining of severe ear pain, hearing loss and unilateral facial palsy. After comprehensive clinical diagnostics and temporal bone and chest imaging, a multiorgan involving form of GPA was diagnosed. The patient was treated with cyclophosphamide and methylprednisolone which resulted in amelioration of ear pain, improvement of hearing loss, partial resolution of facial palsy and altogether clinical improvement of the patient. Although infrequently, systemic GPA can initially present with otologic symptoms and should not be overlooked as a possible cause of an otologic disorder resilient to usual pragmatic treatment
