Successful cochlear implantation in a patient with MNGIE syndrome.

Abstract A 28-year-old woman with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE syndrome) undergoing evaluation for multichannel cochlear implantation is described. The case history, diagnosis of mitochondrial disease, and assessment of the benefits of cochlear implantation are documented. The hearing level with cochlear implant and speech recognition were improved significantly for this patient. MNGIE syndrome is a rare congenital disorder of mitochondrial DNA (mt-DNA). It is crucial for the otolaryngologist to have awareness of MNGIE syndrome and other mitochondrial encephalomyopathies when patients present with sensorineural hearing loss (SNHL). Cochlear implantation can be recommended to patients with MNGIE syndrome and satisfactory results can be achieved.