In more than two-thirds of cases, severe or profound deafness is genetic in origin. It was in 1995 that the first gene responsible for deafness was isolated at the Pasteur Institute in Paris. Since then, more than a hundred genetic changes have been found to be associated with as many different forms of hearing loss. They affect the cochlea, and more specifically, the hair cells or auditory nerve.
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These discoveries revolutionized hearing research and paved the way for the development of therapies to restore the inner ear, most notably gene therapy, which consists of injecting a functional version of a missing or altered gene directly into cells.
“Among the pathways studied, the most advanced work concerns DFNB9 deafness, which is caused by the absence of a protein called otoferrin, which is important for sound transmission in the auditory nerve. This rare congenital deafness affects about 5% of deaf people.noted…
Source: Le Figaro
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