Gene Mutation Linked to Deafness

Recently scientists at The Scripps Research Institute (TSRI) have discovered how there is one gene  in particular essential to hearing. By studying this gene they believe they have uncovered a new cause of deafness. Tmie is the name of the gene believed to be held responsible. Mutations in this gene can cause deafness from birth. After reintroducing the gene to mice and seeing promising results towards restoring hearing,  Professor Ulrich Müller says "This raises hopes that we could, in principle, use gene-therapy approaches to restore function in hair cells and thus develop new treatment options for hearing loss". Mechanotransduction is the process that allows us to hear. Mechanical sounds waves are converted into electrical signals for the brain. The signals are passed from fine hairs within the eye along ion channels to the brain. After 6 months of studying genes, the team eventually found the protein TMIE which interacted with the top link protein and acts as the connections the links to the ion channel. This study will hopefully open the door towards new genetic gene replacement therapy and the curing of deafness in certain individuals.

http://www.medicalnewstoday.com/releases/285938.php

Gene Therapy Holds Promise for Reversing Congenital Hearing Loss

In recent news from Science daily gene therapy has come out with a new type of discovery in mice. A new gene has been found which could reverse hearing loss. Hearing loss is one of the most common human sensory deficits. This mainly results from damage from the hair cells in the inner part of the ear. About half have been recognized as genetic defects. But with the help of a gene coding protein called VGLUT3, (vesicular glutamate transporter-3), there has been enabling of hearing.

This was tested in the mouse model and the cause by the genetic defect in the mouse model of congenital deafness. The different treatments put out for patients with hearing loss can be resulted into simply enhancing sounds but not fully restore the normal hearing levels. Studies have been done using mice with the deafness in the protein VGLUT3. This protein is important in order to send signals to ensure hearing is enabled. This protein was injected and all the mice had hearing enable after two weeks.

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This therapy was ensured without any side effects, and proved to be successful. The therapy also corrected any other damages in the mice’s ears. This has been performed on mice and will not eventually be introduced into humans. With all the best research and constant experimenting there can be finally a way to inject this protein into humans who suffer from the deafness.

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