年龄与线粒体DNA A1555G突变所致的听力损失关系的研究

目的：分析线粒体DNA A1555G突变致聋的患者其发病年龄及病程长短与听力损失是否有相关性，为临床上预测其病情发展提供依据。方法：将154例线粒体DNA A1555G突变致聋的患者，按其发病年龄分为三组（〈1岁，〉1岁且〈3岁，〉3岁），分别对各组耳聋患者的听力损失程度进行统计学分析；将156例患者按其病程长短分为三组（〈10年，〉10年且〈20年，〉20年），分别对各组耳聋患者的听力损失程度进行统计学分析。结果：发病年龄〈1岁和〉1岁且〈3岁的患者，其听力损失为极重度的所占比例较高（分别为85．7篇和72．2％）（P=0．000）；病程长短为〉10年且〈20年及〉20年的患者，其听力损失为极重度的所占比例较高（分别为74．1％和70．3％）（P=0．000）。结论：线柱体DNA A1555G突变致聋的患者，其听力损失与发病年龄、病程长短有关，发病越早，病程越长，其听力损失越重，发病越晚，病程越短，其听力损失越轻。

Study on the relationship between age and hearing loss induced by mtDNA A1555G mutation

Objective： To analyze the relevance among patient＇s age of onset, duration of disease and theirhearing loss whose deafness was caused by the mitochondrial DNA A1555G mutation, to provide a basis for predicting the progression of their disease. Methods： One hundred and fifty-four patients were divided into three groups according to their age of onset （ 〈 1, 〉1 and 〈 3, 〉3）, to analyze the degree of their hearing loss with statistical separately;and 156 patients were divided into three groups according to their duration of disease （ 〈 10, 〉10 and 〈 20, 〉20）, to analyze the degree of their hearing loss with statistical separately too. Results： The patients whose age of onset was under age 1 and between 1 and 3 years old had severe hearing loss in a high proportion （respectively 85.7% and 72.2%） （P=0.000）;.the patients, duration of disease was between 10 and 20 years and more than 20 years had severe hearing loss in a high proportion （respectively 74.1% and 70.3%） （P=0.000）. Conclusion： The patient＇s age of onset, duration of disease and their hearing loss whose deafness is caused by the mitochondrial DNA A1555G mutation are relevant, the earlier onset and longer duration, the more severe hearing loss, on the contrary, the less.