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U 844

U 1051

Team 1
Genetics and therapy of retinal blindness and optic nerve

Team 2
Pathophysiology and therapy of the inner ear

Team 3
Neural differentiation and connectivity in the somatosensory system

Team 4
Plasticity, stem cells and glial tumors

Team 5 (in process)

Avenir Team 1
Somato-sensory specification

Avenir Team 2
Molecular mechanisms of myelination/demyelination and gene therapy approaches in peripheral nerves

Avenir Team 3
The Ubiquitin Proteasome System in neurodegeneration and cytoskeleton architecture


 

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Clinical Transfert

 

 

Clinical study of tinnitus

 
A specialist tinnitus clinic has been created by Dr Cécile Nicolas-Puel within the ENT department directed by Pr. Alain Uziel. Ethiological and semiological analysis have been already performed by these clinicians on 900 patients suffering of tinnitus. Besides a better understanding of the mechanisms of tinnitus, these clinical studies will allow us to determine which patients present enough similarities with our animal models to integrate future clinical assay based on local therapy.
Van Gogh painting known as "Self portrait with the bandaged ear" (1889). In 1889, the painter severed his right ear in an attempt to stop tinnitus (Bridgeman Art Library, London).
 
In addition to clinical studies, we are developing an objective method to detect tinnitus in humans by recording the spontaneous noise of the auditory nerve via an electrode placed on the promontory through the tympanic membrane.

Epidemiology and genetic of the presbycusis

Presbycusis is a progressive hearing loss which appears during aging. This phenomenon is probably due to a genetic susceptibility but also to environmental factors such as noise or ototoxic drugs (cisplatin, quinine and some antibiotics).


Presbycusis also results from other disease like heart insufficiency, high blood pressure, vascular problems or diabetes. Consequently, Dr Catherine Blanchet and Pr. Michel Mondain are studying the audiogram and the environmental risk factors for hearing (noise and ototoxic drugs exposure) in a 3-cities cohort (Montpellier, Bordeaux, and Dijon). Persons composing this cohort are older than 70 and have been phenotyped (blood pressure, cholesterol…) and tested for Alzheimer disease (cognitive tests…) by Dr Claudine Berr (Inserm U888). Until now, data collection has been performed on more than 800 subjects (clinical records, audiograms, and DNA collection). This genetic study is directed by Dominique Hillaire.

Cochlear implants

 
Cochlear implants are used to restore hearing in profoundly deaf patients by the electrical stimulation of the auditory nerve fibres. When residual hearing can be preserved, the implant performance largely surpasses those of a conventional hearing aid. To date, the use of "soft surgery" techniques and adapted devices such as soft tip electrodes allows the preservation of a certain residual hearing in a large number of patients, thus increasing hearing performance and the patient quality of life.

During the last decade, we closely worked with pharmaceutical companies in order to test neuroprotective molecules. To date, the better understanding of the molecular mechanisms involved in cochlear neurons and hair cell degeneration, and the innovative therapeutic strategies to protect them, attract implant manufacturers who want to minimize the traumatism of the electrode insertion and preserve residual hearing after cochlear implantation.


Selected recent references:


Venail F, Wang J, Ruel J, Ballana E, Rebillard G, Eybalin M, Arbones M, Bosch A, Puel JL. Coxsackie adenovirus receptor and alpha nu beta3/alpha nu beta5 integrins in adenovirus gene transfer of rat cochlea. Gene Ther. 2007, 14: 30-37.


Nicolas-Puel C, Akbaraly T, Lloyd R, Berr C, Uziel A, Rebillard G, Puel JL.
Characteristics of tinnitus in a population of 555 patients: specificities of tinnitus induced by noise trauma. Int. Tinnitus J. 2006, 12: 64-70.


Uziel A, Mondain M, Hagen P, Dejean F, Doucet G.
Rehabilitation for high-frequency sensorineural hearing impairment in adults with the symphonix vibrant soundbridge: a comparative study. Otol. Neurotol. 2003, 24: 775-783.


Nicolas-Puel C, Faulconbridge RL, Guitton M, Puel JL, Mondain M, Uziel A. Characteristics of tinnitus and etiology of associated hearing loss: a study of 123 patients. Int. Tinnitus J. 2002, 8: 37-44.

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