Kullar Peter, Park Ki Wan, Kaufman Adam, Santa Maria Peter L
Department of Otolaryngology-Head and Neck Surgery, Stanford Hospital, Palo Alto, USA.
Cureus. 2025 May 4;17(5):e83485. doi: 10.7759/cureus.83485. eCollection 2025 May.
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.
关于线粒体疾病所致综合征性听力损失患者的人工耳蜗植入，现有报道有限。在线粒体疾病中，线粒体脑肌病伴乳酸血症和卒中样发作（MELAS）就是一个显著例子。MELAS是一种以肌病、癫痫、糖尿病和局灶性神经功能缺损（包括感音神经性听力损失）为特征的疾病。必须仔细优化风险因素并考虑围手术期麻醉，以避免MELAS特有的并发症，包括代谢和心脏不稳定以及中风风险增加。在此，我们报告一例44岁双侧感音神经性听力损失病史的女性患者，其接受了常规的二期人工耳蜗植入，术中因严重颅内动脉粥样硬化并发缺血性中风。缺血性中风后，患者接受了基因检测，确诊为MELAS。
