title: Successful cochlear implantation in a patient with MNGIE syndrome.
abstract: 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.
authors.affiliation: Department of Otolaryngology Head and Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital, Beijing, PR China.
authors.forename: Jia-Nan
authors.initials: JN
authors.lastname: Li
authors.forename: Dong-Yi
authors.initials: DY
authors.lastname: Han
authors.forename: Fei
authors.initials: F
authors.lastname: Ji
authors.forename: Ai-Ting
authors.initials: AT
authors.lastname: Chen
authors.forename: Nan
authors.initials: N
authors.lastname: Wu
authors.forename: Xin
authors.initials: X
authors.lastname: Xi
authors.forename: Wei-Dong
authors.initials: WD
authors.lastname: Shen
authors.forename: Shi-Ming
authors.initials: SM
authors.lastname: Yang
mesh_terms: D000328:Adult; D001301:Audiometry, Pure-Tone; D001921:Brain / Q000473:pathology; D019929:Cochlear Implantation; D003056:Cochlear Nerve / Q000473:pathology; D003638:Deafness / Q000534:rehabilitation*; D005072:Evoked Potentials, Auditory / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000534:rehabilitation*; D006801:Humans; D007418:Intestinal Pseudo-Obstruction / Q000175:diagnosis / Q000534:rehabilitation*; D008279:Magnetic Resonance Imaging; D017237:Mitochondrial Encephalomyopathies / Q000175:diagnosis / Q000534:rehabilitation*; D039141:Muscular Dystrophy, Oculopharyngeal; D009886:Ophthalmoplegia / Q000151:congenital; D013063:Speech Discrimination Tests; D013069:Speech Reception Threshold Test; D014057:Tomography, X-Ray Computed; D028361:Mitochondrial Diseases