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Beethoven, Ratatouille, and a Krisper – Preventing Deafness

This is some really cool and promising scientific news regarding hearing loss that came out on the 20th of last month. In the science journal Nature, scientists at Broad Institute, MIT, and Harvard published a study showing promising gene-editing techniques for preventing hearing loss in laboratory animals. While the research is still underway, the scientists say this could go a long way someday towards preventing deafness in children born to families with histories of genetic hearing loss.

The study was done by performing experiments on a type of mouse called the Beethoven Mouse, named for the famously deaf composer Ludwig von Beethoven. The mice carry a defect that causes them to lose their hearing. The gene defect that the mice carry is the same defect that can cause children to be profoundly deaf by late childhood if a bad copy of it is found in the body.

To run the experiment the scientists used a gene-editing technique called CRISPR Cas-9 to effectively block out the defective gene. This gene when unblocked will seek to destroy the tiny hairs in the inner ear which are needed for hearing. The gene-editor that was created using the CRISPR was then injected into one of the ears of Beethoven Mice shortly after they were born. Once in the ear the gene editor can seek out the defective genes and cut their DNA, effectively neutralizing them.

The experiment showed that while the untreated ears on the mice had the normal expected hearing loss, the ears injected with the gene-editor were remarkably better. The scientists involved in this study are continuing their research and hope that similar approaches can be created to prevent babies being born deaf due to inherited genetic mutations.


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