-
- The Editor:
Dr. Paul Finger - The Editor:
| Patient Information: Retinal
Diseases Contacts: |
|
Usher's Syndrome When retinitis pigmentosa (RP) is associated with hearing loss, it is called Usher's Syndrome. There are two types of Usher's Syndrome. Usher Type 1: Early damage to the cochlea of the ear results in deafness which starts from birth. The retinitis also starts early with a progression from night blindness to tunnel vision within the first 10 years of life. Usher Type 2: These patients are more fortunate. The loss of hearing is less severe and the retinitis pigmentosa becomes more evident during the child's late teens and twenties. Unlike most patients with Ushers Type 1, Type 2 patients are more likely to benefit from hearing aids and lip reading.
Diagnosis: Hearing loss usually comes before night blindness and tunnel vision of RP. If a child is slow to start speaking, it may also have trouble hearing. All children with hearing problems should have eye and ear examinations. Treatment: Though there is no current treatment to stop the progression of Usher's Syndrome, it is essential to recognize affected patients as early as possible. Early intervention can help patients with hearing aids, sign language and lip reading training. In general, more can be done to help the patients when Usher's Syndrome is diagnosed before hearing and sight are lost. Genetic testing and counseling can let patients and their parents understand the probability that they will pass along this genetic predisposition to their children. Please
report any problems to: While this WWW site is intended to provide general information as a public service, the content is not intended to represent official policies and practices of the institution or to substitute for health care provided by a clinician. This
Web site and bulletin board has been provided by as a public service.
If you feel that you have benefited from this Web site, Content
and design copyright © Paul
T. Finger, MD 1999-2003 pfinger@macular-degeneration.org ALL RIGHTS RESERVED
|