There are 22.5 million hearing impaired people in Europe alone. Hearing impairment affects children before speech development (1 in 800-1000), and in their early childhood (1 in 400-500). This handicap can adversely affect a child’s educational performance and cognitive development.

Hearing loss not only affects young people: presbycusis is the most common sensory deficit in the elderly. The probability of impairment increases with age: 2.3 % of the population of 40-50 years of age, compared to over 30% of the population above 70 years of age.

The handicap has a severe effect on the individual and those around him or her, as it becomes a major impediment to daily communication.

 
European Research Project
EuroHear: A European research project on hereditary deafness, molecular, physiology and pathophysiology of the inner ear.


The 5 year project commenced on December 1st 2004 .

news_01.jpg EuroHear was officially launched in Paris on March 23, gathering more than 70 people in the Conference Centre of the Curie Institute.

This field of research has been supported by the European Commission for several years. Indeed this project, granted 12.5 M€ by the EC, follows programmes also led by Christine Petit which have brought to light the prominent role of heredity in isolated (non syndromic), congenital or early-onset childhood forms of deafness.

As a direct consequence of these investigations, molecular diagnosis for some forms of deafness is now implemented in developed countries, as well as in certain developing countries.

This aforesaid implementation results in greatly improved genetic counselling available to families. It allows them to be informed about the recurrence risks for future children, of the hearing impairment’s probable evolution, and of the possible relevance of cochlear implantation.

Twenty-two academic institutions and three small privately owned companies from ten different countries are working in partnership over the project, bringing together 250 scientists across the world - acknowledged experts including physicians, geneticists, molecular and cellular biologists, electrophysiologists, biophysicists and theoreticians.

EuroHear is expanding the field of investigation to include the elderly population: presbycusis, a sensorineural form of deafness, accounts for the majority of cases of sensorineural hearing loss occurring in people over the age of 40. The hypothesis that this age-related hearing loss has a genetic basis has been put forward for decades. Only recently has science proved that 50% of the loss can be attributed to genetic factors. Presbycusis is a complex trait influenced by the interplay between environmental and genetic risk factors.

New research strategies, utilising tools of various scientific disciplines, must be developed to unravel these complex mechanisms.

The understanding of the molecular mechanisms underlying the development and function of the inner ear will pave the way for research into therapeutic tools, a challenge also undertaken by EuroHear.
 
 
 
GENETIC APPROACH

The pioneering work of Christine Petit, which demonstrated evidence of genetic cause behind deafness, has opened new avenues of research on the genetic and molecular mechanisms underlying hearing impairment.

The aim of this project is to build on this knowledge, to further our understanding of the molecular and cellular mechanisms underlying inner ear functioning.


EUROHEAR PROJECT

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EuroHear has two closely inter-related objectives.
- to provide fundamental knowledge about the development and function of the inner ear, and
- to identify the molecular defects underlying hereditary hearing impairments (HI), including presbycusis, one of the most frequent forms of HI. Achieving these objectives will facilitate the development of therapies for alleviating HI.

In order to address the above issues, the EuroHear project is organised into 2 major axes:
- The identification of genes underlying sensorineural HI in humans and mice.
- The analysis of the molecular and cellular mechanisms underlying the development and function of the major components i.e. the hair bundle, the ribbon synapse of the hair cell and outer hair cell electromotility, the ion channels, ion transporters and gap junction channels that contribute to the potassium homeostasis.

The most recently developed tools of genomics and biotechnology will be implemented in the context of this research.

The outlook to new tools for preventing and curing HI is a significant part of the programme: providing experimental evidence that pharmacological compounds can significantly reduce the progression of hearing impairment will lead to clinical studies and more efficient treatment strategies.

Recent observations on cell and gene therapy, as well as the discovery of inner ear progenitor cells, suggest entirely new means for treating the inner ear. Within the next five years, EuroHear expects to prove this concept.

 
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