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Medical revolution: A child born deaf hears sounds for the first time in his life

2024-01-26 20:42:16Lifestyle SHKRUAR NGA REDAKSIA VOX
Medical revolution: A child born deaf hears sounds for the first time in his
A child born without hearing for the first time in his life hears sounds

His father's voice, the sound of cars passing by and scissors cutting his hair: these were the sounds an 11-year-old deaf boy heard for the first time in his life after receiving revolutionary gene therapy.

The Children's Hospital of Philadelphia (CHOP), which performed the treatment — the first of its kind in the United States — said Tuesday that the milestone offers hope for patients worldwide with hearing loss caused by genetic mutations.

Aissam Dam was born completely deaf due to a very rare abnormality in a gene.

"Gene therapy for hearing loss is something that we doctors and scientists in the world of hearing loss have been working toward for more than 20 years, and we've finally achieved it," said surgeon John Germiller, director of clinical research for Division of Otolaryngology CHOP.

"Although the gene therapy we performed on our patient was to correct an abnormality in a single, very rare gene, these studies may open the door to future 'fixes' for some of the more than 150 other genes that cause hearing loss in childhood", he said.

In patients like Aissam, the damaged gene prevents the production of otoferlin, a protein needed by the "hair cells" of the inner ear to convert sound vibrations into chemical signals that are sent to the brain. Defects in the otoferlin gene are very rare and cause 1-8 percent of hearing loss from birth.

On October 4, 2023, he underwent a surgical procedure that involved the partial removal of his eardrum and then the injection of a harmless virus, which had been modified to carry working copies of the otoferlin gene, into his cochlear fluid. As a result, the cells began to produce the missing protein and function properly.

Nearly four months since receiving treatment in one ear, Aissam's hearing has improved to the point where he only has mild to moderate hearing loss and is “literally hearing sounds for the first time in his life,” said the statement.

The New York Times reported that despite being able to hear, Aissam, who was born in Morocco and later moved to Spain, may never learn to speak, as the brain's window for acquiring speech closes around the age of five year.

The US Food and Drug Administration, which gave the green light to the study, wanted to start the research on older children first for safety reasons. The trial, sponsored by Akouos, Inc, a wholly owned subsidiary of Eli Lilly and Company, is one of several being conducted in the United States, Europe and China.

"As more patients of different ages are treated with this gene therapy, researchers will learn more about the rate at which hearing can improve and whether that level can be maintained for many years," Germiller said.


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