Bilateral Sensorineural Hearing Loss as the Sentinel Presentation of Granulomatosis With Polyangiitis

Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatous vasculitis classically involving the upper and lower respiratory tracts and kidneys.Timely diagnosis requires a high index of suspicion for the presenting symptoms.Otolaryngologic manifestations are commonly reported but most often involve the nasal and paranasal sinuses.Sensorineural hearing loss (SNHL) as the sole initial manifestation of GPA is uncommon and may contribute to diagnostic delay.We report a case of a 67-year-old male who presented with rapidly progressive bilateral SNHL that preceded pulmonary and renal manifestations by several months, during which the patient was treated for otitis externa and Eustachian tube dysfunction without improvement.Development of pulmonary symptoms prompted additional workup, which demonstrated markedly elevated cytoplasmic anti-neutrophil cytoplasmic antibody (c-ANCA) and proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), raising concern for GPA.Histopathology then confirmed the diagnosis.The patient was treated with high-dose corticosteroids and rituximab with serologic remission; however, his hearing loss was largely irreversible.This case highlights bilateral SNHL as an uncommon sentinel manifestation of GPA and emphasizes the importance of considering GPA in patients with unexplained rapidly progressive bilateral SNHL.Early recognition and prompt treatment may reduce irreversible injury.