1. The Usher Syndrome Page An in depth article by Mary Guest, Head of Usher Services at Sense, about usher syndrome. Includes detailed description of what it is, the symptoms, genetics, transmission, the problems associated with it and what can be done. http://www.deafblind.com/usher.html

Go Back To info Page. Usher Syndrome A Condition which affects Hearing and Sight. by Mary Guest, Head of Usher Services at Sense. What is it ? Usher syndrome is an inherited condition which results in hearing loss and a progressive loss of vision from Retinitis pigmentosa (RP). The hearing loss is thought to be congenital, and ranges from moderate to profound. RP can occur without hearing loss. The condition is named after a British ophthalmologist, C.H. Usher, who in a paper in 1914 described several cases in which the link between congenital deafness and RP was stressed. However, as far back as 1860 workers such as von Graef and Liebreich in Berlin were aware of the link between congenital deafness and RP, especially in consanguinous marriages. Retinitis Pigmentosa, This term describes not one disease but a group of hereditary diseases of the retina. The retina is the light sensitive tissue inside the eye in which the first stages of 'seeing' take place. With RP the retina slowly degenerates and loses its ability to transmit pictures to the brain. In advanced stages characteristic clumps of pigment appear on the retina and can be seen through an ophthalmoscope. Symptoms

2. TARP Usher Syndrome What is usher syndrome There may even be a rare usher syndrome TypeIV, not yet well characterized. What Causes these Disorders? http://www.geocities.com/HotSprings/7815/tarp6.htm

Main Founder Ushers Syndrome Getting Support ... Contact Us This page last updated 12/11/97 7:49:00 PM Created by John Wenberg http://www.jwen.com What is Usher Syndrome The Usher syndromes (USH) are a large group of inherited disorders which combine simultaneously both the hearing impairment and progressive loss of vision (similar to "Retinitis Pigmentosa" [RP]). Type 1 (USH1) is characterized by a congenital (present at birth), severe to profound and preverbal deafness, absent vestibular deterioration (balance) function and early onset of an RP-like retinal deterioration (typically by age 5 or 6 years and almost always before age 10 years.) Type II (USH2) has a milder (post-verbal) hearing loss, apparently present from birth, and a later onset (or detection) of an RP-like retinal degeneration (typically between ages 10-20 years). Balance functions are normal and stable, but the hearing impairment deteriorates very slowly, by some estimates an average of about 1 decibel (Db) per decade. Although still controversial, the existence of at least a third type (USH3), distinguished from USH2 by the rapid and progressive nature of its hearing loss, has been suggested. USH3 seems to account for about 40% of Usher syndrome patients from eastern Finland; there is good genetics evidence that the gene for USH3 is located on a different chromosome than the locations of USH1 and USH2.

3. Retinitis Pigmentosa - Questions A description of this disease from Retinal Preservation Foundation of South Africa. http://www.rpsa.org.za/a6.htm

What is Usher Syndrome? Usher Syndrome is Retinitis Pigmentosa accompanied by hearing loss. The hearing loss is congenital, stable and usually quite severe, although severity can vary in different individuals. Hearing can usually be assisted by modern electronic aids, hearing-assisted telephones, TV aids, etc. Usher Syndrome is a recessively inherited condition. One of the greatest difficulties experienced by sufferers is increased isolation as the restrictions on both spoken and visual communication increases. Contact the Retinal Preservation Foundation for further information. Created and maintained by Intekom works Intekom

4. USHER SYNDROME LINKS usher syndrome LINKS. usher syndrome Page from SENSE TARP Ushers SyndromePage. usher syndrome - from The Family Village. Usher http://www.helenkeller.org/national/usher.htm

USHER SYNDROME LINKS Usher Syndrome Page -from SENSE TARP Ushers Syndrome Page Usher Syndrome from The Family Village Usher Syndrome - from Research Registry for Hereditary Hearing Loss What is Usher Syndrome ? - from Boystown Genetics Dept. Hearing Loss and Balance Problems in Usher Syndrome - from Hereditary Hearing Impairment Resource Registry Usher Bibliography - from DB-LINK

5. Usher Syndrome usher syndrome. Who to Contact. Where to Go to Chat with Others http://www.familyvillage.wisc.edu/lib_ushe.htm

Usher Syndrome Who to Contact Where to Go to Chat with Others Personal Accounts Learn More About It ... Web Sites Who to Contact Usher Family Support 4918 42nd Avenue, S Minneapolis, MN 55417 kadbmn@aol.com Where to Go to Chat with Others The Usher Syndrome Mailing List The Deaf-Blind List Personal Accounts My Life with Usher Syndrome - By Lawrence W. Lee Learn More About It Usher syndrome: A Condition which affects Hearing and Sight Services for Children With Deaf-Blindness Usher Syndrome Usher Bibliography - General Information ... Fine Mapping of the Usher Syndrome Web Sites The Deafblindness Web Resource Usher's Syndrome Page DB-LINK - The National Information Clearinghouse on Children Who Are Deaf-Blind The Deaf-Blind Association - Australia Usher Forum Page - Russia Back to [ U - V Family Village Home Library Coffee Shop ... Information Last Updated April 6, 1998 by familyvillage@waisman.wisc.edu Document Source: http://www.familyvillage.wisc.edu/lib_ushe.htm

6. Usher Syndrome - Parents - Boys Town National Research Hospital perhaps an equal number of hardof-hearing children have usher syndrome, which itself is more than one genetic condition. http://www.boystownhospital.org/parents/info/genetics/usher.asp

Genetics and Deafness - Usher Syndrome Table 1: Types of Usher Syndrome Symptom Type I Type II Type III Hearing Loss Born deaf with profound hearing loss Born hard of hearing with a sloping sensorineural loss from mild loss in low frequencies to severe-profound loss in high frequencies. Born with good hearing or mild hearing loss which gets worse over a decade or more. Looks like Type II in teenagers and young adults; looks like Type I in older people. Balance Absent inner ear balance Normal inner ear balance Progressive balance disturbance Vision Loss from RP Night blindness in infancy or early childhood. Tunnel vision by age 16, usually

7. Usher Syndrome Resource Guide and their families in searching for general information about usher syndrome. An eye care professional who has examined http://www.nei.nih.gov/health/ushers

Health Funding News Laboratories ... Health Information Usher Syndrome On this page: What Is Usher Syndrome? How is Usher syndrome inherited? How is Usher syndrome diagnosed? Is genetic testing for Usher syndrome available? ... Medical Literature Related Topics: Diagram of the Eye Vision-Related TermsSelected Online Sources Finding an Eye Care Professional Talking to Your Doctor ... U.S. Government Sources List The information provided in this Resource Guide was developed by the National Eye Institute (NEI) to help patients and their families in searching for general information about Usher syndrome. An eye care professional who has examined the patient's eyes and is familiar with his or her medical history is the best person to answer specific questions. What Is 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. Researchers have described three types of Usher syndrome-type I, type II and type III.

8. NIDCD Health Information: Usher Syndrome usher syndrome. What is usher syndrome? usher syndrome (US) is the theirchildren. Top. Who is affected by usher syndrome? More than half http://www.nidcd.nih.gov/health/hearing/usher.asp

Home Health Information Hearing, Ear Infections, and Deafness Usher Syndrome On this page: What is Usher syndrome? Who is affected by Usher syndrome? What causes Usher syndrome? What are the types of Usher syndrome? ... Where can I get additional information? What is Usher syndrome? Usher syndrome (US) is the most common condition that involves both hearing and vision problems. A syndrome is a disease or disorder that has more than one feature or symptom. The major symptoms of US are hearing impairment and an eye disorder called retinitis pigmentosa, in which vision worsens over time. Some people with US also have balance problems. There are three different types of US. Although it was first described by Albrecht Von Graefe in 1858, US was named for Charles Usher, a British eye doctor, who believed that this condition was inherited or passed from parents to their children. Top Who is affected by Usher syndrome? More than half of the estimated 16,000 deaf-blind people in the United States are believed to have US. Top What causes Usher syndrome?

9. RetNet - Retinal Information Network Provides tables of genes causing inherited retinal diseases, such as retinitis pigmentosa, macular degeneration and usher syndrome, and related information. http://www.sph.uth.tmc.edu/Retnet/

Table of Contents: Diseases: Cloned and/or Mapped Genes Causing Retinal Diseases Summaries: Summary Tables ( Genes Diseases , or Graph Symbols: List of Disease Symbols References: References for Disease Tables Links: Resources for Research on Retinal Degeneration What's New: New and Updated Disease Genes RetNet provides tables of genes causing inherited retinal diseases, such as retinitis pigmentosa, macular degeneration and Usher syndrome, and related information. This information is provided to the research community and other interested individuals for research purposes only. The information should not be used for medical or commercial purposes. Although we strive for accuracy and completeness, we cannot guarantee that all information is correct and complete. We welcome comments and suggestions RetNet , the Retinal Information Network, is a service of the Laboratory for the Molecular Diagnosis of Inherited Eye Diseases, a joint program of ... The Hermann Eye Center, Dept. of Ophthalmology and Visual Science , and The Human Genetics Center School of Public Health , at The University of Texas-Houston Health Science Center The RetNet web site was developed by: Stephen P. Daiger, PhD

10. Did You Know??? - Usher Syndrome Did You Know ? usher syndrome. More than half of the estimated cases of deafblindpeople in the United States are believed to have usher syndrome (US). http://www.helenkeller.org/national/upd-didyouknow.htm

Did You Know ? Usher Syndrome A leading cause of deaf-blindness More than half of the estimated cases of deaf-blind people in the United States are believed to have Usher syndrome (US). Usher Syndrome is the most common condition that involves both hearing and vision problems. The main symptoms of US are hearing impairment and an eye disorder known as Retinitis Pigmentosa, in which vision gradually diminishes over time. The loss of hearing and vision can vary from mild to total loss of each. It is often described by those who are deaf-blind as ‘a slow and emotionally painful loss of communication and interaction with the hearing and sighted world around them.’ Usher Syndrome is inherited or passed from parents to their children in genes. Some genes specify traits such as hair color, development of body parts, such as the ear or how parts of the body work. Each person inherits two copies of each gene; one gene comes from each parent. Sometimes genes are altered or mutated and this can cause cells to act differently than expected. Each parent of a child with US usually has one standard and one mutated US gene. A child with US receives two mutated genes, one from each parent. Usually parents are unaware that they have or carry a US gene. There are three types of Usher Syndrome, USI, USII, and USIII, each having their own characteristics. People with USI are born profoundly deaf and have severe balance problems. For most, sign language becomes their primary form of communication. Because of the balance problems, children with USI are slow to sit without support and rarely learn to walk before they are 18 months old. These children usually begin to develop vision problems by the time they are ten and tend to progress rapidly until the individual is completely blind.

11. Sense - UK Deafblind Charity - Information And Support UK deaf and blind charity provides information and advice about usher syndrome, rubella in pregnancy and CHARGE support. Make donations online to Sense http://www.sense.org.uk

Enter Viewing Options Sense is the UK's leading organisation for people who are deafblind or have associated disabilities. Deafblindness - a combination of sight and hearing loss - is one of the most challenging disabilities someone can face. Yet given the right services and support, deafblind people can develop their talents and choose how they wish to live their lives.

12. Usher Syndrome The Adviser Network The Sympathetic Hearing Scheme Contact Hearing Concern What is usher syndrome? usher syndrome is an hereditary condition which affects both hearing and sight. http://web.ukonline.co.uk/hearing.concern/fush.htm

13. Dobrodosli / Welcome @ Dodir's Homepage About Croatian deafblind people, news, information about deafblindness and usher syndrome, photo album. Croatian, English http://www.dodir.hr

Posjetitelj / Visitor No:

14. Usher Materials - Medical - 27 Records to information and resources for usher syndrome. NORD usher syndrome - Includes the synonyms, a general discussion http://www.tr.wou.edu/dblink/ushmed.htm

DB-LINK Home To print this document, you may want to switch to the text only version. DB-LINK The National Information Clearinghouse On Children Who Are Deaf-Blind About DB-LINK DB-LINK Publications DB-LINK Databases Selected Topics ... Search the DB-LINK Website Updated 02/2003 Usher Syndrome - Medical This is only a partial listing from the DB-LINK database. If you are unable to locate the information you need, please contact DB-LINK, (800) 438-9376 or (800) 854-7013 (TTY). Study Shows Vitamin A Slows Blindness: RP Foundation Launches Worldwide Education Campaign / RP Foundation Fighting Blindness. RP Foundation Fighting Blindness News, June 14, 1993, 1993. This announcement advocates taking 15,000 IU of vitamin A (and avoiding high doses of vitamin E) as the first useful treatment that may help people with RP to prolong their vision. Patients and health care professionals are advised to contact the RP Foundation for complete information at 1-(800) 683-5555. Defective Myosin VIIA Gene Responsible for Usher Syndrome Type 1B: Weil, Dominique;Blanchard, Stephane;Kaplan, Josseline;Guilford, Parry;Gibson, Fernando;Walsh, James;Mburu, Philomena;Varela, Anabel;Levilliers, Jacqueline;Weston, Michael D.;Kelley, Phillip M.;Kimberling, William J.;Wagenaar, Mariette;Levi-Acobas, Fabienne;Larget-Piet, Dominique;Munnich, Arnold;Steel, Karen P.;Brown, Steve D. M.;Petit, Christine. NATURE, vol. 374, March 2, 1995, pp. 60-61., 1995.

15. What Is Usher Syndrome? Whatis usher syndrome? usher syndrome is a genetic disorder with hearing lossand retinitispigmentosa which causes a progressive loss of vision. http://www.deafblind.com/whatushe.html

Go Back toThe Archives Page. Whatis Usher Syndrome? Usher Syndrome is a genetic disorder with hearing loss and retinitispigmentosa which causes a progressive loss of vision. Some individualsalso have balance problems. There are three types of Usher Syndrome. Type I: born with a profound hearing loss, retinitis pigmentosa, andbalance problems. Type II: born with a moderate to severe hearing loss, retinitis pigmentosa,and no balance problems. Type III: hearing loss that gets worse over time, retinitis pigmentosa,and may have balance problems. Hearing loss Individuals with Type I Usher Syndrome have a profound hearing lossin all frequencies and are considered to be deaf from birth. Individuals with Type II Usher Syndrome have a moderate hearing lossin the lower frequencies. In the higher frequencies it is severe or profound.The loss does not get worse as the person ages. These persons are consideredto be hard-of-hearing and usually find hearing aids to be useful. Individuals with Type III Usher Syndrome have a progressive hearingloss which gets significantly worse as the person ages. Most of the documentedType III cases are in Finland. Retinitis Pigmentosa (RP) RP is an eye disease that causes gradual loss of vision. The eye becomesless able to adjust to low light, resulting in night blindness. As RP progresses,the field of vision narrows until only central vision remains. This iscalled "tunnel vision". Many persons with Usher Syndrome will retain atleast some central vision for a long time.

16. Macular Degeneration, Retinitis Pigmentosa, Stargardt, Usher Syndrome : Usher Sy usher syndrome. The Foundation Fighting Blindness is a publiclysupported FFBHome usher syndrome Click here to go to FFB Research Two http://www.blindness.org/disease/default.asp?type=4

17. Macular Degeneration, Retinitis Pigmentosa, Stargardt, Usher Syndrome : Usher Sy Blindness is a publiclysupported charity raising money to fund research for maculardegeneration, retinitis pigmentosa (RP), usher syndrome, Stagardt disease http://www.blindness.org/visiondisorders/causes.asp?type=4

18. Sensory Impairment Information - UK Deafblind Charity - Sense UK deaf and blind charity provides information and advice about sensory impairment,usher syndrome, rubella in pregnancy and CHARGE support. usher syndrome. http://www.sense.org.uk/sensory_impairment/usher.html

Usher syndrome Usher syndrome is a major cause of deafblindness in adults which affects 3-6% of the people in the UK who were born deaf or partially hearing. People with Usher have a genetic condition which means they are born deaf or hard of hearing, then gradually start to lose their sight. The sight loss often begins in late childhood and is caused by an eye condition known as Retinitis Pigmentosa (RP) . RP is a disease of the retina. The retina slowly degenerates and loses its ability to transmit pictures to the brain. The early symptoms include difficulty seeing in the dark and in different lighting conditions. Over time vision gradually deteriorates until tunnel vision develops. Many people do not realise they have Usher until they begin to have problems with their sight. They will have grown up as deaf or partially hearing, and coming to terms with losing their sight as well can be very hard. But despite the enormous challenges this brings, people with Usher can achieve remarkable things. With the right support many people can go to college, get jobs, find a partner and enjoy their leisure. Read Factsheet 2: About Usher Syndrome For more information Get Adobe Acrobat here.

19. Sense - Sensory Impairment usher syndrome. What is usher syndrome? usher syndrome is one of themost challenging disabilities that anyone can face. People with http://www.sense.org.uk/large/sensory_impairment/usher.html

Text only Home About Usher People with Usher ... Contact Usher syndrome What is Usher Syndrome? Usher syndr ome is one of the most challenging disabilities that anyone can face. People with Usher have a genetic condition which means they are born deaf or hard of hearing, then gradually start to lose their sight. But despite the enormous challenges this brings, people with Usher can achieve remarkable things. With the right support many people can go to college, get jobs, find a partner and enjoy their leisure. The sight loss often begins in late childhood and is caused by an eye condition known as Retinitis Pigmentosa (RP) . This is a major cause of sight loss in people under 40. The early symptoms include difficulty seeing in the dark and in different lighting conditions. Over time vision gradually deteriorates until tunnel vision develops. When someone finds out that they have Usher syndrome the news can be devastating. Many people do not realise they have Usher until they are teenagers and begin to have problems with their sight. They will have grown up as deaf or partially hearing, and coming to terms with losing their sight as well can be very hard. Sense offers free information support and advice to people with Usher, their families and professionals working in the field.

20. Untitled Similar pages www.nih.gov/nidcd/usher.htm Similar pages More results from www.nih.gov usher syndrome Concern. usher syndrome, What is usher syndrome? usher syndrome isan hereditary condition which affects both hearing and sight. The http://www.nih.gov/nidcd/health/pubs_hb/usher.htm

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