Li Jia-Nan, Han Dong-Yi, Ji Fei, Chen Ai-Ting, Wu Nan, Xi Xin, Shen Wei-Dong, Yang Shi-Ming
Department of Otolaryngology Head and Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital, Beijing, PR China.
Acta Otolaryngol. 2011 Sep;131(9):1012-6. doi: 10.3109/00016489.2011.579623. Epub 2011 May 12.
Abstract A 28-year-old woman with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE syndrome) undergoing evaluation for multichannel cochlear implantation is described. The case history, diagnosis of mitochondrial disease, and assessment of the benefits of cochlear implantation are documented. The hearing level with cochlear implant and speech recognition were improved significantly for this patient. MNGIE syndrome is a rare congenital disorder of mitochondrial DNA (mt-DNA). It is crucial for the otolaryngologist to have awareness of MNGIE syndrome and other mitochondrial encephalomyopathies when patients present with sensorineural hearing loss (SNHL). Cochlear implantation can be recommended to patients with MNGIE syndrome and satisfactory results can be achieved.
摘要 本文描述了一名28岁患有线粒体神经胃肠性脑肌病（MNGIE综合征）的女性患者，她正在接受多通道人工耳蜗植入评估。记录了该病例的病史、线粒体疾病的诊断以及人工耳蜗植入益处的评估。该患者植入人工耳蜗后的听力水平和言语识别能力得到了显著改善。MNGIE综合征是一种罕见的线粒体DNA（mt-DNA）先天性疾病。当患者出现感音神经性听力损失（SNHL）时，耳鼻喉科医生了解MNGIE综合征和其他线粒体脑肌病至关重要。对于MNGIE综合征患者可以推荐进行人工耳蜗植入，并且能够取得满意的效果。
