How to Identify Usher Syndrome in a Young Child

Also known as Retinitis pigmentosa, Usher syndrome results in a reduction of vision in an already-deaf child. Although many children with hearing loss will never experience the loss of their vision, they are in a high-risk group, in need of ongoing monitoring.

Difficulty: Challenging

Instructions

1. Notice if your child’s vision worsens in the dark. Night blindness is one of the first symptoms of Usher syndrome to appear. The retina begins to deteriorate slowly and transmission of light to the brain reduces.

2. Test your child for the loss of his peripheral vision. Although he may be able to see in front of him quite clearly, his field of vision begins to narrow as the condition progresses.

3. Examine a child who has a sibling with Retinitis pigmentosa or Usher syndrome. Parents of an affected child will both carry the recessive gene. If only one parent carries it, the child will probably have no symptoms.

4. Check your child’s vision for loss of central acuity. Often seen in the elderly, the condition, known as macula degeneration, is diagnosed by a loss of the central portion of vision. The child will see the surrounding area but not the object in the middle.

5. Get genetic counseling for a child who has Usher syndrome. If he conceives a child later in life with a person who is a carrier, all the children are likely to have the condition. Alternately, if he marries someone who is not a carrier, none of the children will have Usher, but they will be carriers.

6. Monitor all deaf children for vision impairment on an ongoing basis. Although there is no treatment for Usher syndrome, children should begin training as young as possible to deal with the condition and be encouraged to lead full and happy lives.

Tips & Warnings

• Join an Usher mailing list for ongoing research information. (See Resources)

[HowardBThiname]

on 8/8/2009 Thank you, this is good information.