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