LSU Health Sciences Center Discovers Breakthrough Therapy to Cure Deafness

LSU Health Sciences CenterUsher Syndrome is a relatively rare genetic disorder that is the leading cause of deafblindness. It affects one in every 6,000 babies, more so affecting Acadian people in Louisiana and in Canada. For over a decade, researchers at the LSU Health Sciences Center have been working towards a cure for the deafness.

In 1998, Professor of Genetics Bronya Keats, PhD, made headlines when she found a gene defect that caused this type of deafness. At the time, she expected it would take another ten or so years for a therapy to be produced that would fix the deafness.

Jennifer Lentz, PhD, Assistant Professor of Otorhinolaryngology & Biocommunications and a scientist who worked in Dr. Keats’ lab years prior, found a breakthrough therapy to treat deafness through a mouse study. The therapy allows the gene to do what it is supposed to do without the malfunction.

“A single dose of the therapy corrects deadness for at least six months,” Lentz said.

The mouse with Usher Syndrome had inner ear problems, causing imbalance and deafness. Once treated with the therapy, the mouse acted normal for months. Fourteen years after the gene defect was found, the study done by Dr. Lentz and her team at LSU was released in Nature Medicine, a prestigious biomedical journal. The video of the mouse pre and post therapy is available on the site.

“This is without any doubt a very significant breakthrough in medicine,” said the head of the Neuroscience Center, Dr. Nicolas Bazan.