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09.05.2014
Washington, October 26 : US researchers have identified a genetic biomarker for age-related hearing loss, a major breakthrough in understanding and preventing a condition of aging that millions of people and greatly diminishes their quality of life.
In a nine-year study that was a collaboration between University of South Florida's Global Center for Hearing and Speech Research and the National Technical Institute for the Deaf at the Rochester Institute of Technology, researchers were able to identify the first genetic biomarker for presbycusis.
The genetic mutation carried by those who ultimately suffer from age-related hearing loss is linked to speech processing abilities in older people. In collaboration with the House Ear Institute in Los Angeles, the researchers discovered a gene that produces a key protein in the inner ear - the cochlea - called glutamate receptor metabotropic 7 (GRM7).
Now having identified the gene, the researchers said people can be tested and takes steps earlier in life - such as avoiding loud noises, wearing ear protection and avoiding certain medicines known to damage hearing - to protect their hearing.
Our sense of hearing relies upon many factors within the ear, but none so much as the cochlea hair cells. With the development of gene therapy, I believe many individuals who lose hearing due to the damage or loss of hair cells might regain some hair cell functioning and reduce their hearing loss. Oxford University defines gene therapy as “the transplantation of normal genes into cells in place of missing or defective ones in order to correct genetic disorders.” Yet, this term encompasses a large scope. Other studies have shown that aiming at the supporting cells around the hair cells will also result in the restoration and help to prevent hearing loss. If your student has trouble making inferences, knowing why the student struggles is the first step in deciding on a treatment strategy.
Musicians stand to benefit from audiologists’ support to preserve hearing and to bolster performance. Amid renewed debate about teaching signed versus spoken language to children who are deaf, a cadre of professionals calls for emphasizing all forms of early language access. The ASHA Leader Blog is the official blog of the American Speech-Language-Hearing Association. If you'd like to advertise on The ASHA Leader Blog, please contact Pam Leppin or visit ASHA's marketing site.
Hearing loss in later life and early deafness are caused by mutation in hearing-related gene. The TSRI study, published in the journal Neuron, reveals how mutations in a gene called Tmie can cause deafness right from birth or hearing decline in later years. Researchers were able to examine the hair cells of so-called knock-out mice, using electrophysiological techniques over six months. Of course, scientists have long known that mutations in the Tmie gene could cause deafness, yet they’ve never been sure how that could be. When a sound wave enters our ear, the uneven ends – stereocilia – of the inner ear’s hair cells are pushed back. Nevertheless, this process of converting mechanical force into electrical activity – what’s called mechanotransduction – is a mystery to researchers. Did you know that one of the most common birth defects is hearing loss or congenital deafness?
In the past 10 years, advances in molecular biology and genetics have helped us to better understand the development, function, and pathology of the inner ear.


As a result, screening tests for the GJB2 gene are available to people so that they can determine their risk of having a child with hearing problems.
Now that researchers have learned how mutations in the gene Tmie can cause hearing difficulties, the challenge will be to find out how the many components of the mechanotransduction system take shape in the inner ear’s hair cells. We have all accepted that as we age, things simply don’t work the same way they used to – including hearing. On any matter relating to your health or well-being, please check with an appropriate health professional. The GRM7 protein is intimately involved in converting sound into the code of the nervous system, in the cochlea, which is then sent to the parts of the brain used for hearing and speech processing. Ears need inner and outer hair cells to create the electromotile responses needed to collect information from incoming sound to enable us to hear. A study by the National Institute on Deafness and Other Communication Disorders says nearly 26 million Americans suffer from hearing loss attributed to high levels of noise exposure.
Stanford School of Medicine gives a bit more clarity to gene therapy as it relates to hearing loss: a focus on re-establishment of the three genes that cause hearing loss. Columbia University Medical Center conducted a study using mice with genetic mutations to their hearing. Treated by Hinrich Staecker, Robb Gerk underwent gene therapy for a hearing loss condition caused by spinal meningitis. Definitive results aren’t available yet, but preliminary findings lean to the positive as Gerk reports better balance—a function fundamentally controlled by the hearing nerve—along with other subtle improvements.
It is intended to inspire discussion about issues related to the fields of audiology and speech-language pathology, and features posts from a variety of authors, including communication sciences and disorders (CSD) professionals and ASHA staff. The research in recent years have shown progress in stem cell therapies that allow damaged cells to regenerate. Scientists from the University of California, San Francisco used mice with congenital deafness caused by a mutation in the genetic code to deal with the challenge of treatment.
While yet the effect is temporary, the results give significant promise for future success in humans. Scientists at the Scripps Research Institute (TSRI) recently determined that a single gene is responsible for our hearing.
They then reintroduced the Tmie gene in the population before bringing back the very process that reinforces hearing. It basically changes mechanical sound waves into electric signals in order for our brains to process the message. Genetic defects play a key role in congenital hearing loss, and this contributes to roughly 60 percent of deafness that happens in babies. Well, essentially, they’re a road map for the synthesis of proteins, which are the building blocks for everything in our bodies, like hair, eyes, heart, lungs – and ears. If some of these inherited genes are defective, a health disorder like hearing loss or deafness can develop early or later in life, according to the American Academy of Otolaryngology.
Researchers have pinpointed various genes that are behind hereditary deafness and hearing loss – most notably, the GJB2 gene mutation.


In the future, we’ll also need to understand the biophysical principles through which these same proteins turn mechanical signals into electrical ones in the first place.
Many of us just expect hearing to fade and simply believe there is nothing we can do about it. Family history, head trauma, an illness, and even simply aging can all contribute to hearing loss. No statement herein is to be construed as a diagnosis, treatment, preventative, or cure for any disease, disorder or abnormal physical state. Stem cell gene therapy treatments, specifically through the forced development of ATOH1, might supplement these three genes, however.
Cunningham says that supporting cells “must survive in order to transduce sound.” In her study of heat shock proteins (HSPs), she found that HSP70 resisted hearing loss and hair cell loss.
Development of cells and implementation of genetically developed cells in the mice resulted in the regeneration of a significant amount of hair cells. Posts written by ASHA members reflect their own thoughts and opinions, and publication does not imply ASHA’s endorsement of the products or services they may write about. These are hair cells, whose work is to transform sound vibrations in the fluid of the cochlea into electrical signals which are then transmitted on the aural nerve to the brain.
They’ve also discovered the exact cause of deafness while suggesting new avenues for hearing therapies. This kind of movement creates tension in the strings of proteins that connect the stereocilia. But there is a surprising connection between a common illness and hearing loss that you may not have been familiar with.
The statements herein have not been evaluated by the Foods and Drugs Administration or Health Canada. Specifically, the number of healthy outer hair cells that push against the tectoral membrane becomes smaller and smaller. It becomes more difficult to hear certain sounds or in certain situations. Where age doesn’t play a factor, noise-induced hearing loss makes up a significant portion of the rest of the 10 million people. By regenerating the HSP70 through gene therapy, hair cells could have a slower rate of deterioration resulting in reduced hearing loss.
Mice share a similar genetic makeup with humans, so many audiologists and scientists predict that this might be the breakthrough genetic science needs to boost technology for preventing hearing loss.
In fact, there are necessary steps that you can take in order to protect your precious hearing.



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