Alex Hamdar, Reda Chelly, Liza Charlier, Ngongang Ouandji A David, Clément Lelong
Granulomatosis with polyangiitis (GPA) is a rare antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis that predominantly affects the upper and lower respiratory tracts and the kidneys. Although otolaryngological manifestations are common, isolated otologic symptoms as the initial presentation are rare and may delay diagnosis. We report the case of a 65-year-old man who initially presented with refractory bilateral otitis media associated with progressive mixed hearing loss, vestibular dysfunction, and subsequent peripheral facial nerve palsy. Initial temporal bone imaging was unremarkable, showing only middle ear and mastoid effusion. Further investigations revealed ANCA positivity and bilateral necrotizing pulmonary opacities on chest CT, leading to the diagnosis of GPA. Treatment with rituximab and corticosteroids resulted in marked improvement of hearing, vestibular symptoms, and facial nerve function. This case highlights the importance of considering GPA in patients with refractory otologic disease, particularly when associated with severe otalgia, sensorineural hearing loss, vertigo, or facial nerve palsy. Early recognition of these atypical manifestations and prompt multidisciplinary management are essential to prevent irreversible organ damage and improve clinical outcomes.