DULON Didier

Institut de l'Audition - 2019

Laboratory research grant - 2015

2019 Institut de l'Audition

Project status: active

Neurosensory hearing loss will soon benefit from genuine molecular and cellular therapies, and will also involve “smart” hearing aids integrating state-of-the-art signal processing methods. Two critical points for the validation of these new therapies will be addressed by the “Clinical and Translational Exploration of Auditory Synaptopathies” team led by Didier Dulon and Hung Thai Van:

  • The first is a precise diagnosis of the molecular mechanisms responsible for hearing loss.
  • The second point involves establishing the factors determining individual performance, based on a fine-grained phenotype of each subject.

2015 Research grant

Project status: closed

The role of otoferlin in the transmission of auditory information to the brain.

The ear is a complex and fragile organ that transforms sounds into information for the brain. Hearing is only possible thanks to the combined work of the ears and the brain. Without ears, sounds cannot be processed, and without a brain, they cannot be perceived. The transmission of information from the inner ear to the auditory nerve therefore plays a critical role in this process.

Dr. Dulon is studying the mechanisms of certain genetic forms of severe hearing loss, where the transmission of information to the brain by the inner ear is distorted. In particular, he is exploring the role of otoferlin, a protein involved in DFNB9 (a genetic form of severe hearing loss), situated at the interface between the inner ear and the auditory nerve. Otorferlin’s interaction with certain proteins involved in Usher syndrome, a rare disease that is the most common cause of combined vision and hearing loss, is also being explored in this research project.

This research will help understand the distorted mechanisms in the transmission of information to the brain in genetic forms of severe hearing loss. Ultimately, this will enable the development of gene therapies to correct the observed defects.

 

Docteur Didier Dulon
Investigator
“Neurophysiology of the auditory synapse,” Bordeaux University
Institut de l'Audition, Paris, France

Related scientific publication(s):