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Usher's Syndrome

What is Usher’s syndrome?
Usher’s syndrome affects both hearing and vision.  There are three types of Usher’s syndrome. 

People who have the first type are born with little or no hearing.  They have balance problems because their ears are not helping them with balance as they should.  People who have the first type of Usher’s syndrome begin to slowly lose some vision at around five or six years of age.  It is so slow that they have time to get used to it. 

People who have type two begin to lose their hearing when they are little children, after they are old enough to speak.  Balance is not a problem for them.  They can see when they are children.  Between the ages of ten and twenty they slowly lose some vision. 

People who have type three are also born with vision and hearing.  They lose their hearing more quickly when they are children.  They lose their vision more quickly when they are teenagers or young adults.

What causes Usher’s syndrome?
Usher’s syndrome is an inherited eye condition.  That means that a baby is born with it even if it does not show up at first.  When people have a baby, the baby’s body has many things that the parents’ bodies have.  For example, a mother may have curly hair, and her baby may have curly hair just like hers.  The father may have brown eyes, and his child will probably have brown eyes, too.  But it is possible that two parents who have curly hair could have a baby with straight hair.  Two parents who have brown eyes could have a baby who has blue eyes.  They have the gene that makes straight hair and blue eyes hidden in their bodies.

Parents who have children who have Usher’s syndrome may not have known that they had it in their bodies.  It was hidden.  They find out that Usher’s is in their body cells when they have a child who has it.  Or they may remember that someone else in the family had Usher’s.

What kind of vision do people have when they have Usher’s syndrome?
The vision part of Usher’s is called retinitis pigmentosa.  People who have retinitis pigmentosa see things from the center of their eyes.  They may see details in good light. They have trouble seeing anything in dim light.  They may not notice things moving to the side.  They may bump into things or trip on things.  They do not see things to the side or down below or up above.  Sometimes they feel like they are looking through a tube or a tunnel.  People call this “tunnel vision.”

People who have retinitis pigmentosa sometimes have myopia as well.  They can see things better when they are up close. 

It may take longer for people who have retinitis pigmentosa to see again when they go from bright light to dim light or from dim light to bright light.

Most people who have retinitis pigmentosa like to press their eyes.  If they press their eyes a lot, their eyes will look strange and may be injured.

What will help you if you have Usher’s syndrome?
1.  Glasses may be helpful.  Your doctor can tell you what glasses are right.  You can have them made just for you.
2.  Wear sunglasses and a hat in bright light.  It will be easier to go into dimmer light if you do.
3.  You may want to make things look bigger.  The easy way to make things look bigger is to move them close to you or move yourself close to them.  Use a bookstand to hold your book so that you can sit up to look at it up close.  Maybe a magnifier will help you see things close to you.  You can make print and pictures bigger on the computer, with a CCTV or with a copy machine. 
4.  Do not make things too big.  If they are too big, they will be outside the “tunnel” that you see in.  If you want to have a wider view of things, you can use a monocular telescope to do that. Hold the telescope backwards.  Things will look small, but more will fit into the view.  It is best to use a telescope that is made to make things look two to four times bigger for this.
5.  Learn to use a white cane so that you will not bump into things or trip.  At night or in a dark place it will help a lot to have a white cane.
6.  You may want to learn braille.  If you lose more vision, you will still be able to read.  You can read braille all of your life. 
7.  A monocular telescope may help you see things far away.  It may help you read signs outside and the board at school.
8.  If you like to press your eyes, but you do not want them to look strange, try this idea. Choose a special word, and only tell a few people that it means, “Stop pressing your eyes.”  Then you will have a secret code to keep your eyes looking good.
9.  If you lose more vision, you may feel sad about it.  Sometimes it helps to talk to someone about sad feelings.  Find someone you like to talk to about how it feels to lose vision.

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