Hereditary hearing loss: the ear and auditory brain are both affected

Scientists from the Institut Pasteur, Inserm, the Collège de France and Pierre & Marie Curie University have recently demonstrated that mutations in three genes responsible for Usher Syndrome – a hereditary condition that affects both hearing and sight – influence not only the workings of the ear, specifically the function of sensory cells in the cochlea, but also the development of the auditory cortex. Their discovery could explain why some patients, even after being fitted with a cochlear implant (an electro-acoustic device that bypasses the defective cochlea), still have difficulties understanding speech. The findings are reported in a paper in the Proceedings of the National Academy of Sciences of the USA.

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