EuroHear (contract LSHG-CT-2004-512063) is supported by funding under the Sixth Research Framework Programme of the
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
Hearing loss not only affects young people: presbycusis is the most
deficit in the elderly. The probability of impairment increases with
% of the population of 40-50 years of age, compared to over 30% of the
above 70 years of age.
The handicap has a severe effect on the individual and those around him
as it becomes a major impediment to daily communication.
||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
of the molecular and cellular mechanisms underlying inner ear
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.
to address the above issues, the EuroHear project is organised into 2
- The identification of genes underlying sensorineural HI in humans and
- The analysis of the molecular and cellular mechanisms underlying the
and function of the major components i.e. the hair bundle, the ribbon
of the hair cell and outer hair cell electromotility, the ion channels,
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
the programme: providing experimental evidence that pharmacological
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
ear progenitor cells, suggest entirely new means for treating the inner
Within the next five years, EuroHear expects to prove this concept.