遗传性耳聋或将根治（完形填空）

中等

高中三年级

说明文

现代科技

这项由中国复旦大学主导的全球首个遗传性耳聋基因治疗临床试验研究，在国际上首次证明了其安全性和有效性，有望让更多耳聋患者回归正常生活。

A new gene therapy is bringing hope to children born with a specific type of genetic deafness. This year, researchers at China’s Fudan University and Harvard Medical School (HMS) announced that the groundbreaking treatment had _1 the hearing of five of the six test patients.

一种新的基因疗法为先天性遗传性耳聋儿童带来了希望。今年，中国复旦大学和哈佛医学院(HMS)的研究人员宣布，这项突破性的治疗已经恢复了6名测试患者中的5名的听力。

The study was __2 _ six children between the ages of one and seven. Of the six, four had cochlear implants (人工耳蜗). They allowed them __3 in one ear. When the implants were _4 _ , all participants were completely deaf. The two youngest _5 , ages one and two, had no implants.

这项研究是对6名年龄在1到7岁之间的儿童进行的。在这六人中，有四人植入了人工耳蜗。他们允许他们用一只耳朵听。当植入物被关闭时，所有参与者都完全失聪。两名年龄最小的参与者，一岁和两岁，没有植入。

The _6 children were born with a rare genetic disability. For the lack of the _7 OTOF gene, they cannot hear things. In the trial, the children were all injected in the cochlea (耳蜗) with two harmless viruses. __8 _ virus contained half of the normal gene. _9 the gene reached the target cells, it began to work. Within four to six weeks after the _10 , the researchers began testing for signs of hearing improvement.

这些儿童出生时患有罕见的遗传性残疾。由于缺乏正常的OTOF基因，他们听不到声音。在试验中，所有儿童都在耳蜗注射了两种无害的病毒。每种病毒都含有正常基因的一半。一旦基因到达目标细胞，它就开始起作用。在注射后的四到六周内，研究人员开始测试听力改善的迹象。

At the end of the 26 weeks, five of the six children showed substantial _11 in hearing. The three older children could _12 and respond to speech with their implants turned off. Two could _13 recognize speech in a noisy room and _14 a phone conversation. The youngest children also showed improvement in their __15 to recognize speech. HMS Associate Professor Zheng-Yi Chen, who co-led the study, said that the one-year-old began to respond to sounds and _16 simple first words like “mama.” The team is _17 why one of the participants showed no improvement in hearing. They suspect the child’s body may have 18 the viruses.

26周结束时，6个孩子中有5个在听力方面有了实质性的改善。三个大一点的孩子可以在关闭植入物的情况下理解并对语言做出反应。其中两个甚至可以在嘈杂的房间里识别语音，并参与电话交谈。最小的孩子在识别语言的能力上也有所提高。该研究的共同负责人、英国皇家医学学院副教授陈正毅说，一岁的婴儿开始对声音做出反应，并会说出像“妈妈”这样简单的第一个词。研究小组不确定为什么其中一名参与者的听力没有改善。他们怀疑孩子的身体可能已经排斥了病毒。

More _19 are needed before the treatment can be offered to everyone. But once ready, it will _20 the lives of the estimated 200,000 people worldwide.

在这种疗法能够普及到所有人之前，还需要进行更多的试验。但一旦准备就绪，它将改变全球约20万人的生活。

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