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1 in 1,000 children is born deaf.
This treatment can change that
A breakthrough at Tel Aviv University could change the lives of children with hearing loss.
Researchers have injected mice with a normal gene, which replaces a defective gene in the cells responsible for hearing.
The deaf mice treated in this way evolved with normal hearing
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Deafness
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health
Tuesday, 29 December 2020, 07:27
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Innovative and effective method A doctor examines a girl's ear (Photo: shutterstock)
Deafness is a very common disability.
According to the World Health Organization there are about half a billion hearing impaired today, and that number is expected to double in the coming decades.
One in 200 children is born with a hearing impairment, and one in 1,000 children is born deaf.
In half of these cases, the cause of deafness is a genetic mutation.
Currently, about 100 genes are known in the medical literature that mutations in them can cause hearing loss.
Now a new study from Tel Aviv University offers an innovative treatment for such deafness, which is based on inserting a genetic charge into the inner ear cells.
As part of the treatment, the genetic load is inserted into the defective cell in such a way that it actually "corrects" the genetic defect and allows the cell to continue to function properly.
According to the researchers, the treatment may lead to a breakthrough in the treatment of children born with a variety of mutations that cause hearing loss.
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In the study, published in the journal EMBO Molecular Medicine, the researchers were able to prevent the hearing impairment of mice with gradual deafness.
"In this study, we focused on genetic deafness resulting from a mutation in the SYNE4 gene - a rare deafness that was discovered in our laboratory in two families in Israel a few years ago, and has since been diagnosed in Turkey and England," explained Prof. Keren Avraham of the Sackler School of Human Genetics and Biochemistry. And from the Purple School of Neuroscience at Tel Aviv University.
According to Prof. Avraham, "Children who inherit the defective gene from both parents are born with normal hearing, but their hearing deteriorates during childhood.
"This is because the mutation causes a change in the location of the nucleus and the death of the hair cells in the cochlea in the inner ear, which are responsible for absorbing sound waves."
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Avraham and her research team worked with us on innovative technology in the field of genetic therapies.
"We created an artificial virus that does not cause the disease, and inserted a genetic material into it - a normal version of the gene that harms families and mice. We injected the virus into the mice's inner ear so that the hair cells became infected with the virus and absorbed the genetic material we injected into it. The damaged cell allows the hair cells to continue to function properly, "explained Shahar Tiber, a doctoral student in the combined track of medicine and research.
This treatment was given near birth, and then the researchers examined the mice's hearing using physiological and behavioral tests.
The treated mice developed normal hearing, almost identical to that of normal mice without a mutation.
In light of the success of the study, researchers are now developing similar treatments for additional mutations that cause deafness.
"Mental Breakthrough, 'Breaking Equality' in Deaf Treatment."
Prof. Keren Avraham and Shachar Tiber (Photo: Tel Aviv University Spokeswoman)
"Unlike conventional treatments for hearing improvement today, such as hearing aids and cochlear implants, which are given after the damage has already occurred and achieve only partial improvement, Prof. Avraham presents an innovative and effective method for treating hearing impairments even before the damage is caused. The therapeutic approach presented in the study is "Equality in the treatment of deafness," said Prof. Benny Negris, director of the Department of Otolaryngology at Meir Medical Center and head of the Department of Otolaryngology at the Faculty of Medicine at Tel Aviv University, who was not involved in the project.
The research is supported by the Israel-USA Bi-National Science Foundation (BSF), the National Institutes of Health (NIH), the European Research Council (ERC), and the National Science Foundation's Research Program in Personalized Medicine.
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