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Diseases » Usher Syndrome » Summary

What is Usher Syndrome?

What is Usher Syndrome?

  • Usher Syndrome: A rare inherited disorder characterized by sensorineural deafness and progressive vision loss.
  • 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

Usher Syndrome: Introduction

Types of Usher Syndrome:

Types of Usher Syndrome:

Broader types of Usher Syndrome:

How many people get Usher Syndrome?

Prevalance of Usher Syndrome: 16,000 Americans
Prevalance Rate of Usher Syndrome: approx 1 in 17,000 or 0.01% or 16,000 people in USA [about data]

How serious is Usher Syndrome?

Prognosis of Usher Syndrome: some suffers retain a portion of central vision
Complications of Usher Syndrome: see complications of Usher Syndrome

What causes Usher Syndrome?

Causes of Usher Syndrome: see causes of Usher Syndrome

What are the symptoms of Usher Syndrome?

Symptoms of Usher Syndrome: see symptoms of Usher Syndrome

Complications of Usher Syndrome: see complications of Usher Syndrome

Can anyone else get Usher Syndrome?

Inheritance: see inheritance of Usher Syndrome

Inheritance of genetic conditions: see details in inheritance of genetic diseases.

Usher Syndrome: Testing

Diagnostic testing: see tests for Usher Syndrome.

Misdiagnosis: see misdiagnosis and Usher Syndrome.

How is it treated?

Doctors and Medical Specialists for Usher Syndrome: Medical Geneticist, Otolaryngologist, Ophthalmologist ; see also doctors and medical specialists for Usher Syndrome.
Treatments for Usher Syndrome: see treatments for Usher Syndrome
Research for Usher Syndrome: see research for Usher Syndrome

Organs Affected by Usher Syndrome:

Organs and body systems related to Usher Syndrome include:

Name and Aliases of Usher Syndrome

Main name of condition: Usher Syndrome

Class of Condition for Usher Syndrome: genetic autosomal recessive

Other names or spellings for Usher Syndrome:

hereditary deafness [retinitis pigmentosa], retinitis pigmentosa and congenital deafness, Hallgren syndrome, Retinitis pigmentosa [deafness syndrome], Graefe-Usher syndrome, dystrophia retinae pigmentosa [dysostosis syndrome], deafness-retinitis pigmentosa syndrome, Retinitis pigmentosa-deafness

Usher Syndrome: Related Conditions

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


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