Usher Syndrome
What is Usher Syndrome?
Usher Syndrome is the most common genetic cause of combined deafness and blindness. In this case, your hearing and your vision can be affected as well as depending on the type of Usher Syndrome you have, your balance may also be affected. It also changes over time.
The main symptoms of Usher syndrome are hearing loss and an eye disorder called retinitis pigmentosa (RP).
RP causes night blindness and a loss of peripheral vision through the progressive degeneration of the retina. As RP progresses the field of vision narrows – a condition sometimes known as ‘tunnel vision’ – until only the ability to see straight ahead remains.
More than 400,000 people are affected by this condition worldwide. Currently there is no cure for Usher Syndrome.
Usher Syndrome impacts three major senses in the body
Vision
Vision loss in Usher syndrome is caused by a progressive vision disorder known as Retinitis Pigmentosa (RP). RP causes the light-sensing cells in the retina to gradually deteriorate, initially resulting in night blindness, followed by a narrowing of the visual field, commonly known as tunnel vision.
Hearing
Children with Usher syndrome are born with or develop hearing loss. It is estimated that upward of 10 percent of individuals with congenital bilateral, sensorineural hearing loss have Usher syndrome.
Balance
Balance is achieved and maintained through input from your eyes, the vestibular organs in the inner ear and the sensory systems of the body, such as the skin, muscles and joints. People with Usher syndrome suffer from severe balance issues due to vestibular dysfunction.
There are three different types of Usher Syndrome
Type 1
Are profoundly deaf from birth.
Have severe balance problems.
Develop vision problems before age 10, usually in early childhood.
Begins with night vision problems.
Type 2
Have moderate to severe hearing loss at birth.
Have normal balance.
Can benefit from hearing aids.
Develop night vision problems as teenagers.
Progresses more slowly.
Type 3
Have almost normal balance.
Sometimes have balance problems later on.
Have gradual hearing and vision loss.
Hearing loss is apparent by teens.
Can benefit from hearing aids after hearing loss begins.
Night blindness starts in puberty.
Blind spots occur by late teens/young adulthood.
Is there a cure?
At the moment, no. The researchers are working at the moment.
How can I help?
You are helping. Understanding Usher Syndrome is a great step forward.
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