Granulomatosis with polyangitis is a rare granuloma forming necrotizing vasculitis, which involves mainly the respiratory tract and renal system. Otologic involvement may occur primarily as chronic serous otitis media and chronic silent mastoiditis with conductive hearing loss and may rarely lead to sensorineural hearing loss requiring cochlear implantation. This case describes a patient with granulomatous poylangitis with profound sensorineural hearing loss who underwent subtotal petrosectomy with cochlear implantation.