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What is Usher's Syndrome?

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  • Written By: Mary McMahon
  • Edited By: Bronwyn Harris
  • Last Modified Date: 08 September 2018
  • Copyright Protected:
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    Conjecture Corporation
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Usher's Syndrome, or Usher Syndrome, is an inherited disability which causes both deafness and blindness. Deaf-blindness can be a very severe disability, as the tools used to help many blind people cannot be used by the deaf, and things designed to assist deaf people are often not accessible to the blind. As a result, someone with Usher's Syndrome can become extremely frustrated and isolated as he or she struggles with the condition, which currently has no cure.

There are three types of Usher's Syndrome. The first Type, I, is the most severe. People with Type I Usher's Syndrome are typically born deaf, and they often have severe problems balancing and walking. By the time they are 10, their vision deteriorates to the point of blindness. Type II patients are born with hearing impairments but not deafness, and dark spots start to appear in their vision in their teens, ultimately resulting in blindness.

In Type III Usher's Syndrome, someone may appear unimpaired at birth, but develop hearing and vision problems later in life. Complete deafness and blindness may not set in until the patient is middle aged, and the first sign of Usher's Syndrome is often night blindness. The mild symptoms at onset can make Usher's Syndrome difficult to diagnose in Type III patients.

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This extremely rare syndrome is named for Dr. Charles Usher, a British physician who first published studies on the condition in the early 20th century. Usher suggested that the disease might be congenital in nature, laying the groundwork for further study. Geneticists have determined that Usher's Syndrome is a recessive genetic condition, meaning that impaired genes must be passed down from both parents for symptoms to manifest. The severity of the condition is determined by which genes are affected; around 20 appear to be vulnerable to mutations which cause Usher's Syndrome.

This condition cannot be treated, but it can be managed. Since deafness and blindness rarely appear together, most doctors assume that patients who present with both symptoms have Usher's Syndrome. If the condition is caught early, before the patient starts to lose visual acuity, he or she can learn various tips and techniques which will be helpful when total blindness sets in. Some very prominent activists in both the deaf and blind community have Usher's Syndrome, demonstrating that people can live fulfilling, active, and happy lives with this condition, even though it can be very challenging.

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