Cochlear implantation is now a routine clinical procedure for deaf patients in many countries (Gibson, 1987). It replaces the function of damaged cochlear hair cells and, therefore, detects sound and by electrical stimulation produces an appropriate signal in the remaining cochlear nerve fibres (House and Berliner, 1991). Wegener's granulomatosis is an uncommon auto-immune disease. It has a peak incidence at the fifth decade with slight male predominance. Nasal problems are the predominant presentation with otological manifestations presenting rarely. We report a case of Wegener's granulomatosis presenting with total hearing loss and after right cochlear implantation a free field threshold of 40 dB and 20 per cent Bamford-Kowal-Bench (BKB) speech test.We conclude that Wegener's granulomatosis is not an absolute contraindication for cochlear implantation.