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首页> 外文期刊>Genetics research international >Mitochondrial Sensorineural Hearing Loss: A Retrospective Study and a Description of Cochlear Implantation in a MELAS Patient
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Mitochondrial Sensorineural Hearing Loss: A Retrospective Study and a Description of Cochlear Implantation in a MELAS Patient

机译：线粒体感觉神经性听力损失：MELAS患者的回顾性研究和人工耳蜗的描述。

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Hearing impairment is common in patients with mitochondrial disorders, affecting over half of all cases at some time in the course of the disease. In some patients, deafness is only part of a multisystem disorder. By contrast, there are also a number of “pure” mitochondrial deafness disorders, the most common probably being maternally inherited. We retrospectively analyzed the last 60 genetically confirmed mitochondrial disorders diagnosed in our Department: 28 had bilateral sensorineural hearing loss, whereas 32 didn't present ear's abnormalities, without difference about sex and age of onset between each single group of diseases. We reported also a case of MELAS patient with sensorineural hearing loss, in which cochlear implantation greatly contributed to the patient's quality of life. Our study suggests that sensorineural hearing loss is an important feature in mitochondrial disorders and indicated that cochlear implantation can be recommended for patients with MELAS syndrome and others mitochondrial disorders.
机译：线粒体疾病患者常见听力障碍，在疾病过程中的某个时候影响所有病例的一半以上。在某些患者中，耳聋只是多系统疾病的一部分。相比之下，还有许多“纯粹的”线粒体性耳聋疾病，最常见的可能是母体遗传。我们回顾性分析了在本部门诊断出的最近60种经遗传学证实的线粒体疾病：28例患有双侧感觉神经性听力丧失，而32例未表现出耳朵的异常，每组疾病的性别和发病年龄没有差异。我们还报道了一例MELAS患者的感音神经性听力损失，其中耳蜗植入极大地提高了患者的生活质量。我们的研究表明，感音神经性听力丧失是线粒体疾病的重要特征，并建议对MELAS综合征和其他线粒体疾病的患者推荐人工耳蜗植入。

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