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Usher Syndrome

Usher Syndrome: Introduction

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

Home Diagnostic Testing

Home medical testing related to Usher Syndrome:

Wrongly Diagnosed with Usher Syndrome?

Usher Syndrome: Related Patient Stories

Types of Usher Syndrome

Diagnostic Tests for Usher Syndrome

Test for Usher Syndrome in your own home

Click for Tests
  • Hearing tests
  • Electronystagmography (ENG) - for balance
  • Retinal examination
  • Electroretinography (ERG) - for retinitis pigmentosa
  • more tests...»

Usher Syndrome: Complications

Review possible medical complications related to Usher Syndrome:

Causes of Usher Syndrome

Read more about causes of Usher Syndrome.

Disease Topics Related To Usher Syndrome

Research the causes of these diseases that are similar to, or related to, Usher Syndrome:

Less Common Symptoms of Usher Syndrome

Usher Syndrome: Undiagnosed Conditions

Commonly undiagnosed diseases in related medical categories:

Misdiagnosis and Usher Syndrome

Vitamin B12 deficiency under-diagnosed: The condition of Vitamin B12 deficiency is a possible misdiagnosis of various conditions, such as multiple sclerosis (see ...read more »

Usher Syndrome: Research Doctors & Specialists

Research related physicians and medical specialists:

Other doctor, physician and specialist research services:

Usher Syndrome: Animations

Prognosis for Usher Syndrome

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

Research 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:

Statistics for Usher Syndrome

Usher Syndrome: Broader Related Topics

Usher Syndrome Message Boards

Related forums and medical stories:

User Interactive Forums

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

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:

A hereditary disorder characterized by deaf-mutism, retinitis pigmentosa, and occasional mental retardation. Early cases were reported mainly in Jews in Germany but later observations came from Finland, Norway, France, England, Israel, Louisiana. (the Acadian type affecting 4.4 per 100,000), and other parts. Several types are recognized: Type I. Synonyms: Usher syndrome type I (US1, USH1) Usher syndrome type IA (US1A, USH1A) Usher syndrome, French type Type IB Synonyms: Usher syndrome type IB (US1B, USH1B) Usher syndrome, non-Acadian variety Type IC Synonyms: Usher syndrome type IC (US1C, USH1C) Usher syndrome, Acadian variety Profound congenital deafness with onset of retinitis pigmentosa by the age of 10 years. Type II Synonyms: Usher syndrome type II (US2, USH2) Type IIB Synonyms: Usher syndrome IIB (US2B, USH2B) Type III Synonyms: Usher syndrome type III (US3, USH3) Retinitis pigmentosa first noted at puberty with progressive hearing loss. Schizophrenia reported in some cases. Type IV Synonyms: Usher syndrome type IV (US4, USH4) Retinitis pigmentosa and deafness possibly transmitted as an X-linked trait. - (Source - Diseases Database)

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

 

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