Abstract Patients affected by mitochondrial disorders (MD) commonly present with sensorineural hearing loss (SNHL). As such, patients with MD may be candidates for cochlear implantation (CI). Given the nature of presenting symptoms associated with MD, accurate preoperative diagnosis is challenging. Although special surgical and medical considerations for this patient population must be made to ensure safety and treatment success, surgical complications of CI in these patients are limited in the literature. We report a 44-year-old female with bilateral SNHL who underwent CI. She experienced intraoperative complications, including ischemic thrombosis in the setting of severe intracranial atherosclerosis, refractory metabolic acidosis, and an infarct requiring thrombectomy. Her perioperative course was then complicated by renal failure and shock. Further medical work-up, laboratory findings, and postoperative genetic testing confirmed the diagnosis of mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). Since CI is an important treatment for MELAS patients, it is important to understand how to manage pre- and perioperative risk factors for patients with MD undergoing CI, which will improve patient outcomes.
Tong et al. (Thu,) studied this question.