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.

Keywords::

• Speech recognition
• mitochondrial disease
• hearing level

Acknowledgments

This work has been supported by grants from the Hi-Tech Research and Development Program of China (863) (#2007AA02Z150), National Natural Science Foundation of China (NSFC) (#30871398, 30730040, 30571017), and National Eleventh Scientific Program (2008BAI50B08, 2007BAI18B12, 2007BAI18B14) to S.M.Y. We thank the patient and her family for their cooperation. The patient has reviewed all materials including text and pictures to be published. She has signed the consent form and gave her consent for this report to appear in Acta Oto-Laryngologica and associated publications.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.