Latest News
26.06.2006 Hereditary deafness - Gene discovered
Hereditary deafness – Gene discovered, which disturbs the communication between the ear and the brain.
The team of Professor Christine Petit, from the Genetics of the Sensory Deficits Unit of the Pasteur Institute associated with Inserm and the College de France, has identified a gene which transformation, oppositely to all the other genes of deafness identified up to now, do not involve a dysfunction of the auditive sensor, the cochlea. Thanks to the development of an animal model, the researchers of Pasteur Institute showed that, in this form of deafness, the sensory cells of the cochlea function correctly on the contrary of the neurons of the auditive ways. In these latter, the propagation of the nerve impulse is delayed and desynchronized. The nervous message, translation of the sound scene, which arrives at the brain, is deeply modified. The discovered gene is the first human gene implied in a hereditary deafness due to an attack of the neurons of the auditive ways. This work, realized in collaboration with Paul Avan of the laboratory of sensory Biophysics of the Medicine Faculty of Clermont-Ferrand and 3 other laboratories of the European consortium EuroHear has been published in the magazine "Nature Genetics".
Thanks to the team work of Professor Christine Petit at the Pasteur Institute, it is well known that the majority of deafness of children in developed countries is hereditary. Children who have a major deafness due to the hurt of the cochlea can benefit from the cochlear implants.Those cochlear implants replace the weak sensor organ by converting the sound message into electric signals which they deliver directly to the auditive neurons. These neurons transmit them, via the central auditive ways, to the brain. There are however certain failures of the cochlear implant which could source the form of deafness whose gene has just been discovered.
Thanks to the molecular diagnosis developed by the researchers, the clinicians will have a tool which should enable them to evaluate the benefit of the cochlear implant for this form of deafness. Pioneer in the study of hereditary deafness, the team of Professor Christine Petit identified genes responsible for a group of twenty forms of deafness. These discoveries clarify also the defective mechanisms in these various forms, and opened the way to the knowledge of the molecular bases of hearing.
Press Contact
Institut Pasteur: Nadine Peyrolo, Tel. +33 (0)1 45 68 81 47
