The Journal of International
Advanced Otology
Case Report

Cochlear Implantation in Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes Syndrome: Case Presentation

1.

Emmeline Centre, Cambridge University Hospitals, Cambridge, UK

2.

Department of Clinical Neurosciences, University of Cambridge Addenbrooke’s Health Campus, Cambridge, UK

J Int Adv Otol 2022; 18: 71-73
DOI: 10.5152/iao.2022.21316
Read: 432 Downloads: 126 Published: 01 January 2022

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome is a multisystem, progressive neurodegenerative condition, and the most common mitochondrial cytopathy. While not a primary characteristic, sensorineural hearing loss is a common additional symptom reported in up to 78% of cases. This article presents 2 cases of cochlear implantation in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome. Both cases demonstrated significantly improved speech recognition, with results significantly better than previous case reports. Cochlear implants are an appropriate treatment for severe-profound hearing loss in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome. While anesthetic risks and cognitive skills need to be taken into consideration, routine programming and rehabilitation pathways may be appropriate for this cohort.

Cite this article as: Crundwell G, Kullar P, Bance M. Cochlear implantation in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome: Case presentation. J Int Adv Otol. 2022;18(1):71-73.

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