Ischemic Stroke After Cochlear Implantation in a Patient With an Undiagnosed Mitochondrial Disorder

Limited reports exist on cochlear implantation in patients with syndromic hearing loss due to mitochondrial disorders. Among mitochondrial disorders, mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a notable example. MELAS is a disorder characterized by myopathy, seizures, diabetes, and focal neurological deficits, including sensorineural hearing loss. Careful optimization of risk factors and perioperative anesthetic considerations must be taken to avoid complications unique to MELAS, including metabolic and cardiac instability and an increased risk of stroke. Herein, we present the case of a 44-year-old woman with a history of bilateral sensorineural hearing loss who underwent a routine second side cochlear implantation which was complicated by ischemic stroke in the setting of severe intracranial atherosclerosis. Following the ischemic stroke, the patient underwent genetic testing, which confirmed a diagnosis of MELAS.