Usher Syndrome

Introduction: Usher Syndrome

Usher Syndrome: Usher syndrome is an inherited condition that causes 1) a serious hearing loss that is usually present at birth or shortly thereafter ... more about Usher Syndrome.

Usher Syndrome: A rare inherited disorder characterized by sensorineural deafness and progressive vision loss. More detailed information about the symptoms, causes, and treatments of Usher Syndrome is available below.

Symptoms of Usher Syndrome

• Hearing impairment
• Retinitis pigmentosa - this leads to symptoms such as:
  • Night-blindness
  • Peripheral vision loss
  • Progressive retinal degeneration

See full list of 17 symptoms of Usher Syndrome

Medical Textbooks Online about Usher Syndrome

Book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

Less Common Symptoms of Usher Syndrome

• Mental retardation
• Cataracts

Read more about symptoms of Usher Syndrome

Diagnostic Tests for Usher Syndrome

• Hearing tests
• Electronystagmography (ENG) - for balance
• Retinal examination
• Electroretinography (ERG) - for retinitis pigmentosa

Read more about tests for Usher Syndrome

Causes of Usher Syndrome

Read more about causes of Usher Syndrome.

Videos for Usher Syndrome

Patient Surveys for Usher Syndrome

• Patient Profile Survey
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• Survey about the symptoms of your Usher Syndrome
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Prognosis for Usher Syndrome

Prognosis for Usher Syndrome: some suffers retain a portion of central vision

More about prognosis of Usher Syndrome

Reseach about Usher Syndrome

Visit our research pages for current research about Usher Syndrome treatments.

Clinical Trials for Usher Syndrome

The US based website ClinicalTrials.gov lists information on both federally and privately supported clinical trials using human volunteers.

Some of the clinical trials listed on ClinicalTrials.gov for Usher Syndrome include:

• Natural History and Genetic Studies of Usher Syndrome - This study is currently recruiting patients (Current: 23 Nov 2006)
• Study of Dietary N-3 Fatty Acids in Patients With Retinitis Pigmentosa and Usher Syndrome - This study has been terminated (Current: 23 Nov 2006)
• A Genetic Analysis of Usher Syndrome in Ashkenazi Jews - This study has been completed (Current: 23 Nov 2006)
• S Tudy of Usher Syndromes, Type 1 and Type 2 - This study has been completed (Current: 23 Nov 2006)
• Molecular Genetics of Retinal Degenerations - This study is currently recruiting patients (Current: 23 Nov 2006)

Read more about Clinical Trials for Usher Syndrome

Statistics for Usher Syndrome

• Medical statistics for Usher Syndrome
• Prevalence and incidence statistics for Usher Syndrome
• Death and Mortality statistics for Usher Syndrome
• Society statistics for Usher Syndrome

Types of Usher Syndrome

• Usher Syndrome Type 1 - deafness, balance problems, RP in early adolescence
• Usher Syndrome Type 2 - moderate hearing loss, no balance problems, RP later in adolescence
• Usher Syndrome Type 3 - normal hearing at birth, progressive hearing loss and RP

Read more about Types of Usher Syndrome

Stories from Users Related to Usher Syndrome

• Retinitis Pigmentosa and genetics
• Possible Usher Syndrome - Loss of peripheal vision
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User Interactive Forums

Read about other experiences, ask a question about Usher Syndrome, or answer someone else's question, on our message boards:

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Article Excerpts about Usher Syndrome

Usher syndrome is an inherited condition that causes 1) a serious hearing loss that is usually present at birth or shortly thereafter and 2) progressive vision loss caused by retinitis pigmentosa (RP). RP is a group of inherited diseases that cause night-blindness and peripheral (side) vision loss through the progressive degeneration of the retina, the light-sensitive tissue at the back of the eye that is crucial for vision. (Source: excerpt from Usher Syndrome Resource Guide: NEI)

Definitions of Usher Syndrome:

Usher Syndrome is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Usher Syndrome, or a subtype of Usher Syndrome, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Ophanet, a consortium of European partners, currently defines a condition rare when it affects 1 person per 2,000. They list Usher Syndrome as a "rare disease".
Source - Orphanet