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What is Usher-Syndrome

Usher-Syndrome is named after a British doctor, Charles Usher, who first described the symptoms at the beginning of this century.

It is the combination of two illnesses: deafness or hearing deficiency and the progressive degeneration of the retina (so called Retinitis Pigmentosa ("RP")).

RP is a hereditary disorder whose common feature is a gradual deterioration of the light sensitive cells of the retina (the "rods and cones").

The first effects of the deterioration of the rods will usually be noticed between the ages of 10 and 15 when vision in darkness is gradually lost, eventually leading to night blindness. Between the ages of 15 and 20, the deterioration of the cones usually commences affecting the ability to see colours and outlines. Some individuals will also have balance problems.

Usher-Syndrome Types

There are three types of Usher-Syndrome:

Usher Type I:

The individual is born with a very profound hearing deficiency or deafness , Retinitis Pigmentosa and balance problems.

Usher Type II:

The individual is born with a moderate to severe hearing loss, Retinitis Pigmentosa but no balance problems.

Usher Type III:

The individual suffers from hearing deficiency which becomes more severe over time. He or she will also have Retinitis Pigmentosa and may in addition have balance problems.

Usher-Syndrome is passed to succeeding family generations through the autosomal recessive inheritance pattern, which means that two defective Usher genes, one from each parent, is required for a person to suffer from it. Where only one copy of the gene is carried within an individual the disorder will not surface.

It is estimated that approximately one to three million people worldwide suffer from RP and that roughly 10% of them have Usher-Syndrome.

So far, there has been little research on Usher-Syndrome itself and unfortunately there is currently no prevention or treatment for this illness available. The hopes of those suffering from this illness therefore depend on the research which we aim to support.

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