What Causes Hereditary Hearing Loss
There are more than 11,000 hair cells in each of your ears, deep within the inner ear, or spiral-shaped cochlea. They are the sound receptors of your ear, and each one plays an important part in transmitting sounds from the outside world to your brain. In acquired hearing loss, these hair cells get damaged with age or exposure to loud noises, and they are not able to function properly.
In many cases of hereditary hearing loss these hair cells have not developed properly due to a gene mutation present before birth. Without functioning hair cells, the ear cant work properly. Even though there is nothing wrong with the outer ear or with the neural pathway, sounds are never transmitted to the brain. The mutation is of a gene called Gap Junction Beta 2, and its the reason behind hereditary deafness and hearing loss in 1 in every 1,000 children.
Stem Cell Research For Hereditary Hearing Loss
A leader in stem cell research is professor Kazusaku Kamiya, a specialist in ear diseases who runs a lab at Juntendo University in Tokyo, Japan. The team at Juntendo University are growing stem cells to replace the mutated hair cells that are responsible for hereditary hearing loss. We hope this work will lead to a cure for a form of hereditary deafness says Kamiya, who reports that their lab has already found a way to make cochlear stem cells. This is a far better solution for hereditary hearing loss than a cochlear implant since stem cells will restore the natural function of the hair cells in the inner ear.
Snhl Definition And Type Of Studies
In the current study, SNHL is a type of hearing loss in which the root cause lies in the vestibulocochlear nerve or the inner ear. SNHL is characterized by a loss of receptor hair cells and/or spiral ganglion neurons, which carry afferent signals from the cochlea . There are a variety of causes of SNHL, most commonly genetic dysfunction, ototoxic drugs like aminoglycosides and loop diuretics, noise exposure, and aging . In preclinical studies, SNHL is induced via pharmacological agent, immunological conditioning, hereditary dysfunction, and noise induction .
The inclusion criteria are as follows: full-text original papers, controlled animal intervention studies that evaluate the therapeutic efficacy of mesenchymal stem cells for sensorineural hearing loss in animals, regardless of methodological quality.
The exclusion criteria are as follows: review and editorial articles, non-intervention studies, no control group, human studies, exclusive in vitro work, or co-intervention studies.
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Stem Cells And Hearing Loss
Stem cells have an extraordinary power: the ability to divide for indefinite periods in culture and to give rise to specialized cells. Countless research studies have focused on stem cells and the many conditions that could potentially be treated using them, which has led audiologists to wonder about the relationship between stem cells and hearing loss. Do stem cells hold the key to a cure for hearing loss or tinnitus?
Possible Hereditary Deafness Cure Using Stem Cells
Researchers continue to amaze us with new discoveries and treatments that can change lives. The latest buzz is from the field of stem cell research, and this is exciting news for those whove been deaf since birth, or have young children suffering from hereditary hearing loss. Scientists think they may be close to finding a cure for hearing loss!
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New Research On Hearing Loss
by Envoy Medical Staff Member, on February 27, 2021
Having lived with hearing loss for decades, I have always longed for progress towards a cure. Yet it seems for decades I have been told the same thing – “scientists are working on a cure”.
To be fair, I have said the same thing when I’ve been asked about research. It is hard to tell someone there is not a cure for their condition and kind of helps soften the blow.
However, to truly empower ourselves and make important decisions around our hearing health we need to take into account how research has evolved and incorporate it into our own unique journey. It can be hard to weed through all of the propaganda one encounters as a consumer and when we have hearing loss, we are more a consumer than a medical patient so the responsibility to educate ourselves falls on us.
While there is not a cure for sensorineural hearing loss today, there are lots of different treatment options including hearing aids, amplifiers, middle ear implants, cochlear implants, and other types of assisted listening devices. Lack of insurance coverage has created a confusing situation in which an individual is medically diagnosed with a condition by an ENT Specialist yet treatment for the diagnosis ,whether surgical or non-surgical is rarely covered. This has caused people to learn only about a narrow range of options and everything tends to fall under the antiquated hearing aid umbrella.
Harvard Stem Cell Institute
Pharmaceuticals and Gene Therapy
Other Hearing Loss Treatments
Until a cure for hearing loss is found, it is best to be mindful of maintaining your hearing health and prevent further irreparable hearing loss by seeking help from a hearing care professional. In a hearing consultation, the hearing care professional will evaluate your hearing health and recommend suitable treatment, and they might recommend a hearing aid.
Learn more about different hearing aid types by reading about the Top 6 Most Common Hearing Aid Styles.
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What Are Stem Cells
Stem cells are special cells that serve as the building blocks of our bodies. Present in everything from our vital organs to our hair, stem cells are unique because they can transform into many different types of cells. This gives them amazing regenerative effects and the ability to heal areas damaged due to disease or injury. By harnessing the power of stem cells, researchers have developed therapies that may be used to treat a wide array of ailments, from Parkinsons disease to hearing loss.
There are many different kinds of stem cells found throughout the body that are collected and used in several ways. At Broward ENT & Aesthetics, we use peripheral and adipose-derived stem cells harvested from a patients own blood or bone marrow and fat tissues. These stem cells are abundant, therapeutically active and able to differentiate in many different types of cells. When applied in stem cell treatments, peripheral and adipose-derived stem cells can exert many beneficial effects and help regenerate damaged tissues in treated areas.
The Role Of The Gfi1 Protein In The Development Of New Hearing Hair Cells
Researchers from the University of Maryland School of Medicine identified the role of a protein in the development of new hearing hair cells. The result shows that in the absence of this protein, called GFI1, embryonic hair cells cannot develop into fully functional adult cells. The genes expressed by these cells are likely to develop into neuron-like cells. They believe that once the complex pathway to normal hearing is found, there is an opportunity to reverse hearing loss.
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Stem Cell Program Grows
The California Institute of Regenerative Medicine has awarded $25 million to the School of Medicine to advance its research in human embryonic stem cells. The state agency handed out its first rounds of research awards this winter, with the medical school receiving 19 awards worth $25 million — more than any other single institution.
Among other things, the funding will launch an effort to create new stem cell lines using a process known as somatic cell nuclear transfer. These new cell lines are expected to provide insight into a variety of diseases — from diabetes to Parkinson’s disease — and open the way to developing new therapies.
In other funded projects, researchers will work to isolate heart and blood stem cells from embryonic stem cells, and to generate inner ear cells, nerve cells or cells for tissue grafts in the heart. The research effort is led by Irving Weissman, MD, director of the Stanford Institute for Stem Cell Biology and Regenerative Medicine.
James Battey, MD, director of the National Institute on Deafness and Other Communication Disorders, lauded Heller as “one of the leading auditory neuroscientists” and points to his stem cell regeneration research as a high priority for the institute.
Since coming to Stanford from Harvard two years ago, Heller has been focused on two paths: drug therapy — which could be as simple as an application of ear drops — and stem cell transplantation into the inner ear to remedy hearing loss.
Contact Broward Ent & Aesthetics
Making the decision to enroll in stem cell therapy is an exciting and promising opportunity with the potential to help patients with hearing loss and tinnitus regain their auditory senses. Specializing in ear, nose and throat treatments, board-certified otolaryngologist Dr. Wilson DuMornay and his dedicated team are excited to help you learn more about stem cell therapy and what it can do for you. Contact or call Broward ENT & Aesthetics at to get started today.
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Can Stem Cell Therapy Cure Tinnitus
Some patients testify to improvements due to tinnitus treatment with MSCs. Stem cell therapy tinnitus treatments generally improve the quality of life of these patients.
MSCs are capable of repairing damaged hair cellular entities due to producing a vast array of bioactive factors such as chemokines, cytokines, and growth factors. These factors are essential in the critical biologic processes like wound healing, tissue regeneration, biologic processes, angiogenesis, and others.
The overall health-promoting characteristics of stem cell-based therapy contribute to helping patients in diverse ways. Ways it positively influences patients are through:
- Stress relief
- Overall improvement of the health condition
Hearing Loss Study Results
The scientists turned the Lgr5+ cells found in mice into new hair cells by responding to a series of signals known as the Wnt pathway. They extracted the Lgr5+ cells from the inner ears of mice, placed them in a dish and used a combination of drugs and growth factors to multiply them. The new Lgr5+ cells were treated to a second drug cocktail that regenerated them into large populations of hair cells.
These results have helped the scientists identify a useful combination of drugs for first expanding Lgr5+ cells in the inner ear and converting them into hair cells. This provides a source of hope for the development of better therapies in the future to restore hearing to those with acquired forms of deafness.
Acquired Sensorineural Hearing Loss And Stem Cells
Unfortunately there is no treatment currently available which can repair or reverse acquired hearing loss. However, recent trials using human umbilical cord blood in animal models have shown regrowth of hair cells and some recovery of the auditory brainstem response. These trials with animal models have now developed into a human trial. The trial aims to determine safety, efficacy, and if an infusion of the childs own cord blood stem cells can improve their hearing, inner ear function, and language development.
There are currently 11 clinical trials investigating the application of stem cells in hearing loss
When Will Stem Cell Therapy Be Available
The next stage of the research is to find a way to safely implant these stem cells into the ear. Through surgery, they hope to plant stem cells into the cochlea where they will fuse with the cells of the inner ear and develop as normal hair cells, restoring natural hearing.
If there was a cure for your childs hearing loss, would you jump at the chance? Were all eagerly waiting for this revolutionary stem cell research to develop into a viable treatment option. Researchers say it may be as little as 5 to 10 years before stem cell treatment is available for hereditary deafness, or even as a treatment for sensorineural hearing loss later in life. Correcting gene mutations with stem cells might be the key to restoring hearing, and could change the lives of children born deaf.
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What Is Stem Cell Treatment
Stem cell treatment is essential in the therapy of some diseases and several medical conditions. It primarily targets the repair of damaged cellular entities in certain parts of the body. This treatment offers an alternative form of cure for tinnitus. Please note that tinnitus is caused by damage to the auditory hair cells or the auditory nerve.
During the treatment, there are several ways of implantation. The technique depends on the patientâs disease or physical condition. Typically, patients get between three to six procedures with cell-based products during the treatment.
Induced Pluripotent Stem Cells
Scientists have learned how to create a new type of stem cell that is proving to be very useful in research. They’ve discovered how to reprogram human skin cells so that they become pluripotent stem cells that resemble those from an embryo. The altered cells have the ability to produce any type of cell if they are stimulated correctly, including hair cells. They are known as induced pluripotent stem cells or iPS cells.
They are two advantages to working with iPS cells instead of embryonic ones. The skin cells that are reprogrammed are obtained without destroying embryos. In addition, the cells can be obtained from the eventual recipient of the reprogrammed iPS cells or of the cells that they produce. This reduces the chance that they will be rejected by the recipient’s body after a transplant. The body generally rejects cells that don’t belong to it.
Birds and some other vertebrates regenerate inner ear hair cells that are destroyed. Mammals don’t. Researchers are exploring the reasons for this difference.
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Can Hearing Loss Be Cured
Have you noticed any recent changes to your hearing abilities? You may be struggling to follow conversations in places with a lot of background noise or find yourself turning up the volume on the TV for the third time this week. Maybe you feel like the people around you have started mumbling, or that you cant hear the kids playing in the backyard. As you grapple with hearing loss, you may wonder if hearing loss can be cured.
Types of Hearing Loss
There are two main types of hearing loss, and the hearing loss you have will determine your treatment options. Conductive hearing loss is due to a blockage in the ear canal or the middle ear that stops sounds from reaching the inner ear. Its often caused by a buildup of earwax in the ear canal, or fluid in the ear canal or middle ear from an ear infection or other illness. This hearing loss can sometimes be reversed by treating the cause of the hearing loss, such as removing impacted earwax, or treating an infection to drain the ear and restore hearing.
Sensorineural hearing loss is the most common kind of hearing loss, and its caused by damage to the cells of the cochlea, or inner ear. This type of hearing loss is the result of exposure to loud noise, or develops as part of the aging process. Sensorineural hearing loss cant be cured.
Hearing Loss is Permanent
Researching Cures for Hearing Loss
Treating Hearing Loss
Is There A Cure For Hearing Loss
After realising they suffer from hearing loss, some patients seek medical help in hopes of fully recovering from hearing-impairment. However, hearing loss is complex because numerous potential causes require different treatment methods. Unfortunately, there is often no known surgery or drug that could cure hearing loss.
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Types Of Preclinical Animal Models
We will include preclinical in vivo models of SNHL that represent the pathophysiological features of human SNHL. Eligible animal models include healthy mammals of all genders and ages. A variety of methods to induce SNHL exist, which include surgical injection of neomycin and oubain mixture into the cochlea, animals with hereditary SNHL, and -tubulin immunization . These models may require surgery or extensive manipulation that is subject to technical variability. However, these models provide a predictable disease phenotype and share a common feature to human SNHL: histological evidence of cochlear cell loss or elevated auditory brainstem response thresholds.
Using Light To Improve Cochlear Implants
- Bionics Institute, Melbourne, Australia
If you are profoundly deaf, a cochlear implant can restore a sensation of hearing by working in place of the hair cells that have been lost. It generates electrical signals that activate the auditory nerve cells which carry signals to the brain, allowing you to hear. But it can still be difficult to hear in some situations, especially when there is a lot of background noise.
Professor Richardson is working to improve cochlear implants by using light, instead of electrical signals, to activate the hearing nerve cells. This could help people with implants hear with a much better and more natural quality of sound.
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The Role Of Neural Stem Cells In Hearing Regeneration
In recent years, many scientists around the world have explored the application of neural stem cell therapy in the inner ear and have achieved many inspiring results. The main direction is to induce the regeneration of auditory hair cells and spiral ganglion cells to replace damaged cells and attempt to treat SNHL . Neural stem cells in the inner ear can differentiate into auditory neurons, hair cells, and supporting cells. Therefore, after the inner ear is damaged by noise, neural stem cells can make up for the damaged cells, meanwhile reduce the apoptosis of spiral ganglion cells . Iguchi et al. found that the effectiveness of cochlear implantation relies on residual spiral ganglion cells, and neural stem cells can differentiate into glial cells and neuronal cells after CI. GDNF and BDNF can nourish spiral ganglion cells to enhance hearing improvement after CI . The application of stem cell therapy in the inner ear mainly includes two aspects: stimulating the proliferation and differentiation of endogenous stem cells in the inner ear and implanting exogenous stem cells .
Figure 1. Mechanism of neural stem cell transplantation for the treatment of hearing loss.