Usher Syndrome

Anatomy of an Ear Infection Slideshow

• What is Usher syndrome?
• Who is affected by Usher syndrome?
• What causes Usher syndrome?
• What are the characteristics of the three types of Usher syndrome?
• How is Usher syndrome diagnosed?
• Is genetic testing for Usher syndrome available?
• How is Usher syndrome treated?
• What research is being conducted on Usher syndrome?
• What are some of the latest research findings?

What is Usher syndrome?

Usher syndrome is the most common condition that affects both hearing and vision. A syndrome is a disease or disorder that has more than one feature or symptom. The major symptoms of Usher syndrome are hearing loss and an eye disorder called retinitis pigmentosa, or RP. RP causes night-blindness and a loss of peripheral vision (side vision) through the progressive degeneration of the retina. The retina is a light-sensitive tissue at the back of the eye and is crucial for vision. As RP progresses, the field of vision narrows - a condition known as "tunnel vision" - until only central vision (the ability to see straight ahead) remains. Many people with Usher syndrome also have severe balance problems.

There are three clinical types of Usher syndrome:

• type 1,
  
  
• type 2, and
  
  
• type 3.

In the United States, types 1 and 2 are the most common types. Together, they account for approximately 90 to 95 percent of all cases of children who have Usher syndrome.

Photograph of the retina of a patient with Usher syndrome (left) compared to a normal retina (right). The optic nerve (arrow) looks very pale, the vessels (stars) are very thin and there is characteristic pigment, called bone spicules (double arrows).

Suggested Reading by Our Doctors

• Visual Field Test - Read about visual field testing and vision tests (Amsler grid, Humphrey-Zeiss and Goldman perimetry tests) for glaucoma and macular degeneration detection.
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• Electroretinography - Read about electroretinography (ERG), an eye test where electrodes placed on the cornea measure electrical responses to light to try to detect abnormal retinal function.

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