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. 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

3. Usher Syndrome / Family Village usher syndrome. Who to Contact. Where to Go to Chat with Others Learn More About It. usher syndrome A Condition which affects Hearing and Sight http://www.familyvillage.wisc.edu/lib_ushe.htm

Usher Syndrome Who to Contact Where to Go to Chat with Others 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 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 10/31/2003 by familyvillage@waisman.wisc.edu Document Source: http://www.familyvillage.wisc.edu/lib_ushe.htm

4. Usher Syndrome - Parents - Boys Town National Research Hospital Genetics and Deafness usher syndrome. About 3-6 percent of all deaf children and perhaps an equal number of hard-of-hearing children have usher syndrome, which itself is more than one genetic condition. earlier in Type I. usher syndrome is one of several conditions in 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

5. Usher Syndrome usher syndrome. The Foundation Fighting Blindness is a nonprofit national organization that funds research for macular degeneration, retinitis pigmentosa (RP), usher syndrome, Stargardt disease, http://www.blindness.org/usher-syndrome.asp

Text Only Macular Degeneration Retinitis Pigmentosa Usher ... Future Microsoft WebSpeak has been installed to read content on this site aloud. When you see this speaker icon, use your mouse to click on it and the wizard will be activated. Email Newsletter Sign up here for the latest research and science news, and FDA-approved clinical trials related to retinal degenerative diseases. Login Register Benefit Usher Syndrome Usher syndrome is an inherited disorder that is characterized by moderate to profound hearing impairment, which is present at birth or shortly thereafter, and progressive vision loss due to retinitis pigmentosa (RP), a degeneration of the retina. It is the major cause of deaf blindness. About 10-15,000 people in the United States have Usher syndrome Clinical Description There are three known forms of Usher syndrome . Individuals with Usher syndrome type I are born profoundly (completely) deaf and experience problems with balance. In adolescence, they usually begin to exhibit the first signs of RP: night blindness and loss of peripheral vision. Individuals with Usher syndrome type II experience moderate to severe hearing impairment at birth, but they do not have balance problems. Symptoms of RP develop later in adolescence. With

6. Usher Syndrome Resource Guide [NEI] Publication about usher syndrome, an inherited condition that causes a serious hearing loss and progressive vision loss. families in searching for general information about usher syndrome. An eye 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 ... Eye Health Organizations Database New! 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.

7. Usher Materials - Medical - 27 Records Defective Myosin VIIA Gene Responsible for usher syndrome Type 1B Weil, Dominique;Blanchard, Stephane;Kaplan Cochlear Implants in Young Children with Usher's Syndrome Young, N 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.

8. Usher Syndrome [NIDCD Health Information] This fact sheet provides a brief, easyto-understand reference for the public on the communication disorder known as usher syndrome. usher syndrome. http://www.nidcd.nih.gov/health/hearing/usher.asp

Change text size: S M L Home ... 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 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 Usher syndrome are hearing impairment and retinitis pigmentosa, an eye disorder that causes a person's vision to worsen over time. Some people with Usher syndrome also have balance problems. There are three general types of Usher syndrome. Although the syndrome was first described by Albrecht Von Graefe in 1858, it was named for Charles Usher, a British eye doctor, who believed that the condition was inherited or passed from parents to their children. Top Who is affected by Usher syndrome? Approximately 3-6 percent of all deaf children and perhaps another 3-6 percent of hard-of-hearing children have Usher syndrome. In developed countries such as the United States, about 4 babies in every 100,000 births have Usher syndrome. Top What causes Usher syndrome?

9. Usher Syndrome: New Insights Lead To Earlier Treatment [NIDCD Health Information Children with usher syndrome can now benefit from better treatment options , thanks to recent discovery of a method to identify this disorder at birth. http://www.nidcd.nih.gov/health/hearing/usher_new.asp

Change text size: S M L Home ... Hearing, Ear Infections, and Deafness Usher Syndrome: New Insights Lead to Earlier Treatment On this page: Early detection leads to earlier treatment A Puzzling Disorder, Passed from Parents to Children Zooming in on the Genetic Component Earlier Screening Is Best Medicine ... Where can I get additional information? Early detection leads to earlier treatment Children with an inherited hearing and vision disorder known as Usher syndrome can now benefit from better treatment options, thanks to a recent scientific discovery of a method to identify this disorder at birth. Those with Usher syndrome type 1 are usually born deaf or hearing-impaired in both ears. Vision problems often don't appear, however, until a child is olderaround age 10 for those with the most severe form of Usher syndrome and around age 20 for those with a less severe form. The disorder takes many by surprise. The parents of deaf children often do not know that they both carry the genes that cause Usher syndrome and its inevitable blindness in their children who inherit the Usher mutation from both parents. Approximately 5 percent of children born deaf or hearing-impaired have Usher syndrome, or about one in 25,000 of all people in the United States. An international team of researchers, zeroing in on one of the several genes thought to be responsible for Usher syndrome, found a telltale mutationa part of the gene not carrying out its biological orders properly. This mutation (called R245X) appears to account for a large proportion of the most severe form of Usher syndrome in the Ashkenazi Jewish population, a Jewish group descended from eastern European ancestors.

10. TARP Usher Syndrome What is usher syndrome. There may even be a rare usher syndrome Type IV, 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.

11. 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:

12. 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 RetNet maintenance and design: Stephen P. Daiger, PhD

13. What Is Usher Syndrome? Whatis usher syndrome? usher syndrome is a genetic disorder with hearing loss and 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.

14. Usheruk.org.uk Voluntary organisation run mainly by people with usher syndrome, promoting the interests and wellbeing of all people who have Usher and their families. Information on activities and events. http://www.usheruk.org.uk/

The website for usheruk.org.uk can be found by clicking here . usheruk.org.uk is registered through Easily.co.uk - get web site hosting or domain name registration here

15. Usher Syndrome usher syndrome. Retinitis pigmentosa is an eye disease that occurs rather often in the deaf and same inherited disorder that is called usher syndrome. Deafness or partial hearing http://med-aapos.bu.edu/leaweb/EYES&V2/usher.html

16. Usher Syndrome - Treatments usher syndrome TreatmentsWhile researchers are gaining new understandings about the precise genetic. causes of usher syndrome and the actual mechanisms of the disease, research http://www.blindness.org/pdfs/Usher_Syndrome_Treatments.pdf

17. National Center For The Study And Treatment Of Usher Syndrome usher syndrome is a devastating genetic disorder that first robs its victims of their hearing, and then slowly takes away their vision as they approach http://www.boystownhospital.org/UsherSyndrome/index.asp

View David's Story dial-up broadband Read David's Story Receive David's Story Usher syndrome is a devastating genetic disorder that first robs its victims of their hearing, and then slowly takes away their vision as they approach adulthood. The leading cause of combined deafness and blindness in the United States, Usher syndrome affects an estimated 30,000 to 40,000 Americans with as many as 30 million people who may be carriers of Usher genes. Insights from our Research Leaders Patient Profiles Make a Donation Basic Usher Syndrome Information Opening the World to Children With Usher Syndrome Children with Usher syndrome are born with varying degrees of hearing loss. Vision loss due to retinitis pigmentosa (RP), an eye disorder that results in gradual loss of vision, commonly is not identified until a child reaches adolescence. Loss of these two vital senses shuts these individuals out of the world around them and prevents them from experiencing many of life's pleasures and opportunities.

18. Health And Medical Information: Diseases And Conditions, Medical Dictionary, Pro usher syndrome A genetic disorder characterized by hearing impairment and an eye disorder called retinitis pigmentosa Some people with usher syndrome also have balance problems http://www.medterms.com/Script/Main/Art.asp?li=MT&ArticleKey=16123

19. 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 Registered Charity No: 289868 Registered Company No: 1825301

20. Publications, Advice And Services - UK Deafblind Charity - Sense UK deaf and blind charity provides information, advice and publications about usher syndrome, rubella in pregnancy and CHARGE support. ABOUT usher syndrome. http://www.sense.org.uk/word_documents/fact2.cfm

Back to previous page Publications Sense homepage Factsheet two ABOUT USHER SYNDROME Usher syndrome is one of the most challenging disabilities that anyone can face. People with Usher are born deaf or hard of hearing, then gradually start to lose their sight in late childhood. But despite the enormous challenges this brings, people with Usher can achieve a great deal. With the right support, many people can go to college, get jobs, find a partner, and enjoy their leisure, just like anyone else. What is Usher syndrome? Usher syndrome is a genetic condition which causes profound deafness or partial hearing from birth, and sight loss over a number of years. This sight loss often begins in late childhood and is caused by an eye condition known as Retinitis Pigmentosa (sometimes called RP). Usher is also a significant cause of deafblindness in adults. Deafness People who have Usher syndrome experience moderate to profound deafness. Children born profoundly deaf have average losses of 95 db and over, which means that they cannot hear continuous speech sounds, even with a powerful hearing aid. Profoundly deaf children tend to prefer sign language as their natural language, although some do develop speech. Many sign language users feel very much part of the deaf community and culture.

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