Photos from the Children’s Hospital of Philadelphia show 11-year-old Aissam Dam completing a hearing test, raising his hand for every sound he hears.
It is Chinese researchers who, since 2022, have tested the treatment on six children who have a type of congenital deafness that is due to a mutation in a certain gene.
Aissam Dam has had a defect in the so-called OTOF gene since birth. The gene codes for a protein needed to transport sound signals from the ear to the brain.
– I did this with my fingers by his ear when I met him today, says John Germiller, a researcher at the Children’s Hospital of Philadelphia, and rubs his fingers together.
– He could hear it, a very vague sound. He can hear people’s voices, he can hear when cars are coming.
With the help of gene therapy, the mutated gene is replaced so that the protein can be formed. The results from the six trials are now published in the scientific journal Lancet seems promising.
Opening to process more
According to the study, the children’s hearing improved 26 weeks after the treatment. The children should also have improved their speech about six months after they started hearing. With the help of a harmless virus, the new gene was operated into the children, who then began to produce the protein.
– There are roughly 150 genes that cause hearing problems at birth, and this is only the first one that we have managed to treat, says John Germiller.
In total, around two to eight percent of all congenital deafness and hearing loss are due to this particular mutation. Based on the results and the fact that no serious long-term side effects were discovered, the researchers are now planning for larger studies on more children.
– It is only this gene that we can treat now, no other. But we may be able to treat more within five to ten years, says John Germiller.