Background: Cochlear implantation can be used when a patient's hearing cannot satisfactorily be improved after optimised hearing aid fitting. However, in patients with a cochlear nerve or brain disorder affecting hearing, the benefits of cochlear implants are not so straightforward.

Methods: This paper describes a 58-year-old patient suffering from multiple sclerosis and profound sensorineural hearing loss, rehabilitated with a cochlear implant. Literature concerning cochlear implantation in demyelinating conditions was systematically reviewed using PubMed/Medline and Web of Science databases.

Results: The patient's cochlear implantation was successful, with speech discrimination scores remaining above 90 per cent for eight years post-operatively. No previous cases of cochlear implantation with multiple sclerosis related hearing loss have been reported, despite the high incidence of hearing loss in multiple sclerosis patients.

Conclusion: This paper demonstrates that multiple sclerosis lesions should not be an exclusion criterion in an otherwise suitable candidate for cochlear implantation.