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Usher Syndrome- How Gene Therapy Can Help Your Balance and Hearing

Monday, November 6, 2017 9:09:00 AM Australia/Melbourne

Usher Syndrome- How Gene Therapy Can Help Your Balance and HearingDid you know that hearing loss is the most common of any deficit associated with your senses? This means that more people around the world are impacted than those with sight loss and motor-skill issues. In fact, more than 280 million people worldwide currently suffer from a degree of hearing loss. Though it’s not the first side effect that may jump to mind, balance defects are increasing, but scientists are making leaps and bounds to find solutions.


Congenital hereditary hearing loss is normally caused by dysfunction within the inner ear. This is where the five balance organs and their sensory cells live, along with the cochlea—which is your hearing organ. Scientists have found more than 100 gene mutations that are associated with the defects of these hearing and balance organs.


One of the most common types of hereditary hearing loss is called the Usher Syndrome, type 1 USH1. In the United States alone, more than 16,000 are impacted. Usher Syndrome also impacts your sight, in fact that’s the primary side effect, and many affected are born with no hearing, but watch their sight decline quite quickly into blindness. Research has shown that the mutation is encoded in scaffold protein sans. This is absolutely essential for the hair bundle within the inner ear hair cells to maintain cohesive.


Back to the amazing progress being made. Audiologists have begun to fit those with hearing loss and balance dysfunction with auditory prostheses. They then potentially undergo balance rehabilitation therapy. The results have varied across the board. That’s why they’re looking into gene therapy. In a nutshell, the gene therapy process transfers a non-mutant copy of the gene that’s defective to help restore the missing protein that we just discussed.


A group of French scientists from a selection of institutions across the country are seeing increasing success, when testing on mice models. We’re talking about inner ear hair bundles that saw mass destruction even before the subjects were born. But with a single injection of this gene therapy, they’ve been able to help restore the impacted structure. They inject the gene into the ear using the AAV8 virus, which is innocuous. This is how they can very specifically target the hair cells that they’re looking for.


"We have just shown that it is possible to partially correct a specific form of hereditary hearing loss accompanied by balance problems using local gene therapy performed after the embryogenesis of the ear, which is primarily affected by the mutation responsible for the disorder. This suggests that the time window for effectively treating USH1 syndrome using gene therapy may be larger than initially thought."

- Saaïd Safieddine, CNRS Director of Research at the Institut Pasteur, and co-author of this particular study


What’s the bottom line? These patients are looking at least long-term partial recovery of their hearing, and by association, their balance. As you know, Hearlink is dedicated to innovations within the audiology industry. We feel that when we share the information available to us, we can help our valued patients and customers make the decisions that are best for their hearing.

Posted in Industry News By

Hearlink Admin