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.