Genetic patch stops deafness in mice who have Usher's syndrome

Genetic Patch ‘Stops Deafness’ In Baby Mice, Research Finds

A genetic patch has been found to stop deafness in baby mice. The tiny patch can be used to prevent a form of deafness that runs in families, according to animal tests.

The form of deafness is caused by Usher syndrome, a genetic abnormality that can cause problems with hearing, sight, and balance. The study, published in Nature Medicine, revealed that the same defects could be corrected in mice.

Experts called the study an “encouraging” start in preventing hearing loss in people with the syndrome.

While there are many types of Usher syndrome tied to different errors in a patient’s DNA, one of those mutations runs in families descended from French settlers in North America. When these people try to build the protein called hormonin — needed to form the tiny hairs in the ear that detect sound — their bodies do not complete the task. Thus, they are left with hearing loss at birth.

The syndrome has a similar effect on the eye and can cause a gradual loss of vision. Scientists at the Rosalind Franklin University of Medicine and Science in Chicago, Illinois designed a small strip of genetic material which attaches to the mutation.

The patch causes the body to keep building the protein.

When newborn mice with Usher syndrome were injected with the “genetic patch,” they grew up able to hear and had no balance problems. But by the time they reached six months of age, their hearing began to deteriorate.

One of the researchers Michelle Hastings, assistant professor at Rosalind Franklin University, told theBBC, “It was a surprising result that we could treat mice right after they are born and have such a profound effect.”

The treatment had to be given to the mice within the first 10 to 13 days of life, which poses a unique problem for humans. Since the gestational period of mice is much shorter than that of humans, researchers speculate that the patch would have to be injected in utero in order to work.

The researchers do not know if this is because the patch needs to be in place during early development in order to make a difference in hearing or if the patch struggles to make it into the inner ear beyond a certain point.

Dr Ralph Holme, head of biomedical research at Action on Hearing Loss, said, “It is encouraging that researchers have been able to rescue hearing using an approach which targeted a specific type of inherited deafness.

“More research is now needed to understand how this new therapy could be used to treat this particular type of Usher Syndrome in humans and discover whether vision can also be rescued.”

The genetic patch could be the first step to stopping deafness in humans with Usher’s syndrome.

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