Polyarteritis nodosa is a systemic disease which affects the small to medium-sized muscular arteries. Sudden or progressive, bilateral hearing loss is a presenting otologic manifestation. To date, no case of cochlear implantation in patients with polyarteritis nodosa has been reported. The authors present a case of polyarteritis nodosa (confirmed by biopsy) in a 71-year-old man with progressive, bilateral sensorineural hearing loss who underwent cochlear implantation. A successful full insertion of the Nucleus 3G electrode array was achieved without surgical or post-operative complications. The patient immediately showed a positive subjective response and, at three month post-operative evaluation, had gained useful open-set speech perception. A review of five temporal bone cases with hearing loss and polyarteritis nodosa revealed the possibility of fibrosis and ossification in the basal turn of the cochlea, of which the surgeon should be aware prior to cochlear implantation.