Hearing Triggered by Molecular Gating ‘Spring’
Researchers have discovered the long-sought gating spring responsible for opening ion channels in sensory hair cells, essential for hearing.
Researchers have discovered the long-sought gating spring responsible for opening ion channels in sensory hair cells, essential for hearing.
Hearing loss due to aging, noise, and certain cancer therapy drugs and antibiotics has been irreversible because scientists have not been able to reprogram existing cells to develop into the outer and inner ear sensory cells — essential for hearing — once they die. But Northwestern Medicine scientists have discovered a single master gene that programs ear hair cells into either outer or inner ones, overcoming a major hurdle that had prevented the development of these cells to restore hearing, according to new research published in “Nature.”
Researchers at Uppsala University have created the “first 3D map of the hearing nerve showing where the various sound frequencies are captured,” according to an article on the university's website. Using what is known as synchrotron X-ray imaging, they were able to trace the fine nerve threads and the vibrating auditory organ, the cochlea, and find out exactly how the frequencies of incoming sound are distributed.
To achieve reprogramming, the scientists exposed fibroblasts and supporting cells to a cocktail of four transcription factors, which are molecules that help convey the instructions encoded in DNA. The scientists identified this cocktail by testing various combinations of 16 transcription factors that were highly active in the hair cells of newborn mice.
Read MoreTo hear, animals rely on hair cells in the inner ear, which bend under the pressure of sound waves and send electrical impulses to the brain. The recessive mutation to TMC1 that Liu and Yeh hoped to correct caused rapid deterioration of those hair cells, leading to profound deafness at just 4 weeks of age.
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Read MoreUsing zebrafish as a proxy, scientists have shed light on how changes to specific genes alter the coordinated direction that these cells are laid out.
Read MoreIn a Phase 1/2 study, FX-322 demonstrated a statistically significant and clinically meaningful improvement in key measures of hearing loss, including clarity of sound and word recognition, with no serious adverse events observed.
Read MoreThe researchers found that noise trauma causes substantially greater changes in neural processing of complex sounds compared with age-related metabolic loss, potentially explaining large differences in speech perception commonly seen between people with the same clinically defined degree of hearing loss based on an audiogram.
Read MoreUsing a zebrafish model, researchers at Case Western Reserve University School of Medicine have found that artemisinin, an anti-malarial drug, may help treat forms of hereditary hearing and vision loss associated with clarin1 mutations like Usher syndrome.
Read MoreUsing a zebrafish model, researchers at Case Western Reserve University School of Medicine have found that artemisinin, an anti-malarial drug, may help treat forms of hereditary hearing and vision loss associated with clarin1 mutations like Usher syndrome.
Read MoreThe book describes major advances in our understanding of the pathogenic processes underlying various forms of hearing loss and the emergence of treatments for deafness.
Read MoreFettiplace, a professor of neuroscience at the University of Wisconsin School of Medicine and Public Health (UW SMPH), won the award for showing how cochlear hair cells sense the tiny mechanical vibrations that sound produces in the inner ear.
Read MoreAfter accounting for the risk factors of age at diagnosis and treatment intensity, the analysis suggested that survivors with severe hearing loss struggled the most with slowed processing speed and phonological skills.
Read MoreTo identify new molecules involved in hearing loss, the researchers took a genetic approach and created 1,211 new mouse mutants. They screened each of these mice using a sensitive electrophysiological test, the auditory brainstem response, to find out how good their hearing was.
Read MoreThe auditory receptors of the inner ear, called hair cells, pick up sounds using a vibration-sensing antenna called the hair bundle. While much research into hearing loss has focused on the hair bundle, UVA’s discovery spotlights the foundations those antennas stand on.
Read MoreThe researcher’s new work reveals the identity and mechanism of a finely-tuned spring they believe is responsible for converting the deflection of hair cells into a force capable of opening ion channels.
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