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线粒体细胞病是许多神经综合征的病因,且可引起听力下降。但这种伴有线粒体细胞病的家族性聋在耳聋的病因学分类中的类型尚不清楚,它可能是一种全身性神经性疾病或者是一种综合征,可能由于线粒体对各种能量物质摄入的改变、三羧酸循环中氧化磷酸化能力的变化或呼吸链中某些复合物的缺乏等原因而造成有氧代谢的改变,以致形成伴有明显的神经性聋的综合征。在患肌病者的肌肉标本中显示了所谓的“粗红纤维”及线粒体堆积。除了肌病外,可表现小脑、锥体外系、前庭、耳蜗或视网膜病变,因脑部症状在某些综合征中是
Mitochondrial disease is a cause of many neurological syndromes and can cause hearing loss. However, the type of familial deafness associated with mitochondrial cytopathy is not clearly defined in the etiological classification of deafness. It may be a generalized neurological disease or a syndrome that may be due to mitochondrial effects on various energy substances Changes in ingestion, changes in oxidative phosphorylation in the tricarboxylic acid cycle, or lack of certain complexes in the respiratory chain and other causes of aerobic metabolism changes, resulting in the formation of syndromes with significant deafness. Muscle specimens from patients with muscular dystrophy show so-called “reddish fibers” and mitochondria buildup. In addition to myopathy, can show the cerebellum, extrapyramidal, vestibular, cochlear or retinopathy, due to brain symptoms in some syndromes