Our team, composed of a mixture of clinicians and researchers, works on the neuropathies and synaptopathies of the inner ear. We try to answer fundamental questions essential for diagnosis and for the clinical rehabilitation of these diseases. How do the synaptic nerve contacts of the hair cells of the inner ear and their projections onto the central nervous system change with aging, acoustic trauma and certain genetic mutations? We characterize the cellular and molecular changes occurring in these diseases, with a view to
defining strategies for their treatment or the prevention of these neurological diseases of the inner ear.
Establishing large cohorts of patients for clinical trials
The audiological, electrophysiological and clinical data will be grouped together in a connected database available at the clinical sites of Necker Hospital (pediatric CeRCA), Pitié-Salpétrière Hospital, and the university hospitals of Lyons and Montpellier (in the framework of an ongoing collaboration). The data will be shared and connected, to include molecular data, with the BAMARA genetic database. We are already following large cohorts of hearing-impaired patients that have been well characterized genetically (e.g. 200 patients presenting congenitally enlarged vestibular aqueducts (EVAs)).
Validating new techniques for the objective exploration of hearing and the capacity to encode the sounds of speech
The short- and medium-term projects of the team are:
- Studying the plasticity of the afferent auditory pathway in patients with cochlear implants and/or hearing aids.
- Comparing the maturation of efferent auditory pathways in children with and without learning difficulties.
Objective functional and genetic exploration of patients with hearing aids/implants presenting a poor hearing performance
The objective of this team is to develop innovative diagnostic tests for adult patients suffering from presbycusis, in close partnership with other teams from the Hearing Institute. Given the continual increase in the number of patients suffering from hearing problems and the number of patients equipped with conventional hearing aids or implants, audiologists are having to deal with a growing percentage of treatment failures.
The researchers will address the question of neuroplasticity in hearing-aid users, by applying a direct stimulation method via the hearing aid rather than free-field stimulation, thereby creating a binaural and ecological stimulation based on speech sounds while preserving the temporal characteristics of the stimulatory signal.
Deciphering the molecular mechanisms of cochlear synaptopathies
The objective here is to resolve key fundamental questions relating to the diagnosis and clinical rehabilitation of synaptic disorders of hair cells. How do the synaptic nerve contacts of the hair cells of the inner ear and their projections onto the central nervous system change with aging, acoustic traumatism and certain genetic mutations? The molecular changes occurring in these disorders will be studied in mouse models, to define strategies for correcting or preventing these neurological conditions of the inner ear.