EuroHear (contract LSHG-CT-2004-512063) is supported by funding under the Sixth Research Framework Programme of the European Commission.

CONTEXT

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.
DOWNLOADING INFORMATION
Understanding the Genetics of Deafness : A Guide for Patients and Families (311 ko - pdf)

(by courtesy of Harvard Medical School Center for Hereditary Deafness)

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
 

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.