[From sudden hearing loss to an inflammatory systemic disease]

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are a group of rare autoimmune disorders characterized by necrotizing inflammation of small- to medium-sized blood vessels. They typically involve the upper and lower respiratory tracts and frequently the kidneys. However, they may also present with otologic manifestations, which can occasionally occur as an early or even the sole clinical symptom. The following case report outlines the course of the disease in a patient in whom bilateral hearing loss ultimately emerged as the leading manifestation of ANCA-associated vasculitis.