Two children who were born deaf can now listen and dance to music after undergoing pioneering gene therapy that restored their hearing. They are among a small group who were the first in the world to receive ground-breaking treatment. Given as an infusion into the ear, it works by replacing the faulty DNA which causes a type of inherited deafness known as DFNB9.
Five children, two girls and three boys, gained hearing abilities in both ears, and were able to hear and speak words and locate where a sound was coming from. But two of them showed more advanced improvements and were able to respond to music, which has more complex sound signals, the researchers said. Zheng-Yi Chen, an associate scientist in the Eaton-Peabody Laboratories at Mass Eye and Ear in the US, said: “The results from these studies are astounding.
“We continue to see the hearing ability of treated children dramatically progress and the new study shows added benefits of the gene therapy when administrated to both ears, including the ability for sound source localisation and improvements in speech recognition in noisy environments.” They said treating children in both ears gives them the ability to hear sound in three dimensions, which is important for communication and common daily tasks such as driving. The researchers said their work, published in the journal Nature Medicine, shows this type of treatment “holds great promise and warrants larger international trials”.
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