wwltv.com
Posted on February 4, 2013 at 6:06 PM
Updated today at 6:22 PM
Meg Farris / Eyewitness News Email: mfarris@wwltv.com | Twitter: @megfarriswwl
NEW ORLEANS -- Usher Syndrome is the leading cause of combined deafness and blindness in the world, affecting one of every 6,000 babies born. The genetic defect is even higher in Acadian people here in Louisiana and Canada.
Now a local research team has taken a major first step to curing this deafness by successfully treating it in a mouse model.
In 1998 Dr. Bronya Keats made research headlines when her team at LSU Health Sciences Center found a genetic defect that caused a type of deafness. She said then that a therapy to fix it was a decade or so away.
Now 14 years later, a young scientist who worked in her lab has done just that, by treating Usher Syndrome in mice that had the human gene for Usher put in them.
"The study reported today, shows that a single dose of the therapy corrects deafness for at least six months out of the life of the animal which is about two years of age," said Dr. Jennifer J. Lentz, the lead author on the paper and an assistant professor of otolaryngology and biocommunications at LSU Health Sciences Center.
Dr. Lentz worked for years to create the mouse model with the human Usher's gene. Now she and her team at the LSU Neuroscience Center have discovered an injection that tells the defective gene in Usher syndrome to express, or to do what it is supposed to do if it were not defective. This finding is just released in one of the most prestigious publications, Nature Medicine.
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Local medical research team makes breakthrough in curing deafness