Purpose: The evaluation of long-term results of cochlear implantation in patients with typical Cogan syndrome. Procedures: The medical records of approximately 3,000 patients who underwent cochlear implantation in a tertiary centre within the period 1992–2007 were retrospectively examined. Results: Four cochlear implant recipients with Cogan syndrome (6 implantations) were identified. One cochlea was found to be partially obliterated; the electrode could, however, be completely inserted. Wound healing disorders and infections in a patient with systemic symptoms were the only postoperative complications. Hearing outcome was favorable, with average HSM and monosyllabic scores of 96.7 and 82.5%, respectively, in an average follow-up time of 9.25 years. Conclusions: Cochlear implantation is the appropriate hearing rehabilitation method in Cogan syndrome patients. Although the basic illness does not affect the long-term hearing outcome, skin-atrophy-related complications in cases with systemic symptoms may occur. Finally, the cochlear implant surgeon should be aware of the possible cochlea obliteration.