Usher syndrome type 1112/5/2024 A deficiency in clarin-1 protein is known to cause Usher syndrome type III The success of a cochlear implant is therefore largely dependent on the preservation of auditory neurons, as this ensures optimal transmission of the electrical impulses generated by the implant to the auditory cortex. These devices bypass damaged auditory sensory cells or hair cells and directly stimulate the auditory neurons, which transmit acoustic signals, converted into electrical signals, to the auditory cortex. To date, cochlear implants are the only available treatment for patients with hearing loss. Hearing impairment is more common in older people, affecting a third of the population over the age of 60. In France, about 6 million people have difficulties conversing because of a congenital or acquired hearing impairment. Hearing loss is the most common sensory deficit in humans. This discovery offers new prospects for the therapeutic treatment of individuals with hearing loss due to hair cell synaptopathies. The absence of clarin-1 leads to a progressive hearing impairment, which could be prevented by gene therapy that targets the sensory hair cells in the inner ear. ![]() Scientists from the Institut Pasteur, Inserm, Sorbonne University and the Collège de France, working in close collaboration with scientists from the University of Bordeaux, have recently elucidated the mechanisms of hearing loss associated with mutations in clarin-1, a molecule that plays an essential role in the auditory system.
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