科学家将揭示儿童期耳聋的病因

Deafness is the most common disorder¹ of the senses. Tragically², it commonly strikes in early childhood, severely³ damaging an affected⁴ child's ability to learn speech and language. In many cases, children gradually lose their hearing to become profoundly（深深地，极度地） deaf over a long period of months to years, but scientists know very little about how this progressive loss happens, making prospects⁵ for prevention and cure very slim. Over half the cases of childhood deafness are estimated to be due to defects in just one gene⁶ passed from either the mother or father, and many of these deafness genes⁷ have been identified. However, as the way we hear is so complicated, it has been really difficult to work out exactly how these genes cause such wholesale⁸（大规模的，批发的） effects. Dr John Oghalai, of Baylor College of Medicine in Texas, has been wrestling with（全力对付，努力克服） this problem for his whole career. His work as a clinician, directing a busy team performing cochlear implants⁹（人工耳蜗） and corrective surgery on the ear and cranium（颅骨，头骨） , has armed him with crucial clinical insights which inform his laboratory's research into the causes and treatment of deafness. Together with a team spearheaded（带头，先锋） by postdoctoral fellow Anping Xia, he has now created mice which carry a mutation¹⁰ in one of the genes, called alpha tectorin（覆膜蛋白） , known to cause progressive childhood deafness. Remarkably¹¹, the mice develop the same hearing problems as children. Oghalai's team are now able to look in detail at exactly what is going wrong in the development of the inner ears of their mice. New insights have already been gained into how and why the mutation affects processing of soundwaves entering the ear, and, in an ironic¹² twist to the story, there are clues that the inner ear's attempts to compensate¹³（补偿，赔偿） for the hearing loss may cause ongoing¹⁴ damage leading eventually to profound deafness. One day, Oghalai hopes that the mice will help in developing new therapies to slow or even halt hearing loss, not only in children, but also in elderly people, tackling a major cause of isolation¹⁵（隔离，孤立） and depression.