Génétique et Physiologie de l’audition

The TherapUsher project funded by Retina-France is aimed at creating appropriate animal models for the USH1 retinal dystrophy. The Usher syndrome (USH) is the most prevalent cause of inherited deaf-blindness in humans. Three clinical […]

The enlightenment of the deafness: an holistic approach of Usher syndrome Recherche Hospitalo-Universitaire en Santé Professor Christine Petit (Institut Pasteur, Collège de France, Inserm, UPMC) and Dr. Aziz El-Amraoui (Institut Pasteur, Inserm, UPMC) coordinate the […]

Pr Christine PETIT was the coordinator of this 36 month project which included Pr E. Sylvester Vizi and Dr Tibor Zelles (partners from Institute of experimental medicine, Hungarian Academy of Science, Budapest, Hungary) as […]

Mutations in the gene encoding Vlgr1 (Very large G-coupled Receptor protein 1) areresponsible for Usher Syndrome type 2C (USH2C). This autosomal recessive syndrome is characterized by congenital, moderate to severe hearing impairment and a […]

Programme Grands investissements d’avenir Sensory disorders significantly impact cognition, education, employment, autonomy and are associated with a heavy socio-economic burden (e.g. the combined cost of visual and auditory impairment is estimated at 600 billion […]

Assembling the puzzle of the operating auditory hairbundle The hair bundles of the sensory cells play a central role in the processing of sounds by the cochlea. Various performances of auditory perception depend upon […]

Human and mouse hereditary deafness Severe to profound deafness affects one in 700 newborns. This disability also affects an additional one child in 1000 before adulthood, and 2.3% of the general population between 60 […]

All genes that we have identified as associated with deafness, except for the gene encoding pejvakin which is responsible for DFNB59, lead to a primary deficit in the peripheral auditory sensory system. An in-depth […]

Severe to profound deafness affects one in 700 newborns. This disability also affects an additional one child in 1000 before adulthood, and 2.3% of the general population between 60 and 70. Moreover, 40% of […]