61. About Usher usher syndrome is a major cause of deafblindness in adults which affects 36% usher syndrome is a genetic condition passed on by Recessive Inheritance. http://www.sense.org.uk/deafblindness/usher/about.htm

Skip navigation Viewing Options Sign In Search this site Home About deafblindness Causes of deafblindness CHARGE ... Usher syndrome About Usher About Usher 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.

62. Deafblindness - Usher Syndrome Deafblind alphabet. Did you know ? Deafblind Scotland arranges an usher syndrome support group meeting twice a year. http://www.deafblindscotland.org.uk/db/indexus.htm

Deafblindness Home About us What's new Support Us ... Deafblindness Usher Syndrome What is Usher Syndrome? Genetics in Usher Syndrome Interpreting for people with Usher Syndrome How to make best use of residual sight and hearing ... Guidelines for Designing Clear Text Did you know ...? Deafblind Scotland arranges an Usher Syndrome support group meeting twice a year. The association of deafblind and dual sensory impaired people

63. Usher Syndrome usher syndrome. What is usher syndrome? Genetics in usher syndrome Interpreting for people with usher syndrome How to make best use of residual sight http://www.deafblindscotland.org.uk/text/db/tindexus.htm

with graphics Home Deafblindness Usher Syndrome What is Usher Syndrome? Genetics in Usher Syndrome Interpreting for people with Usher Syndrome How to make best use of residual sight and hearing ... Communication Section of website Home About us What's new Support Us ... Deafblind alphabet Deafblind Scotland, 21 Alexandra Ave, Lenzie, Glasgow, G66 5BG, Scotland Tel/Text: 0141 777 6111 Fax: 0141 775 3311 Email: info@deafblindscotland.org.uk Website: http://www.deafblindscotland.org.uk

64. Usher Syndrome Definition - Medical Dictionary Definitions Of Popular Medical Te Online Medical Dictionary and glossary with medical definitions. http://www.medterms.com/script/main/art.asp?articlekey=16123

65. Deafblind UK - Help & Advice: Links To External Usher Syndrome Websites [text On Deafblind UK, the association of deafblind and dual sensory impaired people in the UK. http://www.deafblind.org.uk/text/advice/links/usherwebsites.html

with graphics Deafblind UK: In touch with deafblind people You are at: home Links to external usher syndrome websites Usher UK Usher UK is a voluntary organisation run mainly by Usher people. It promotes the interests, needs, welfare and well-being of all people who have Usher, their partners, families and friends. European Usher Website provide information on medical, research and rehabilitation aspects of the Usher Syndrome to Usher patients and their relatives as well as interested professionals. External links menu deafblind websites deaf websites blind websites usher syndrome websites ... disability websites Please note that deafblind UK cannot be held responsible for the content of any of these external websites Main sections of website Home page About us Deafblindness Services ... Donate Now Registered Charity No. 802976 Company Reg. No. 2426281 Site maintained by leisure sports international

66. Deafblind UK - Help & Advice: Usher Websites usher syndrome websites. Usher UK. Usher UK is a voluntary organisation run mainly by Usher people. It promotes the interests, needs, welfare and wellbeing http://www.deafblind.org.uk/advice/links/usherwebsites.html

[text only] about us deafblindness services ... disability websites Usher Syndrome websites Usher UK Usher UK is a voluntary organisation run mainly by Usher people. It promotes the interests, needs, welfare and well-being of all people who have Usher, their partners, families and friends. European Usher Website provide information on medical, research and rehabilitation aspects of the Usher Syndrome to Usher patients and their relatives as well as interested professionals. Please note that Deafblind UK cannot be held responsible for the content of any of these websites contact us Registered Charity No. 802976 Company Reg. No. 2426281 Site maintained by leisure sports international

67. Genes And You - Genetic Disorders - Usher Syndrome< usher syndrome is a genetic condition which causes a person to be born deaf and to The pattern of inheritance for usher syndrome is autosomal recessive. http://www.gig.org.uk/genesandyou_ushersyndrome.htm

Genetic Interest Group - Working to benefit all people affected by genetic disorders Home Latest News Publications Press ... Site Map How helpful is our site? Comment: Your email: Add to mailing list: Who are you? Choose.. Student Teacher Medical Scientist/Research Member of GIG Patient Family Member Carer Press Other You are here: Education Genes and You Contents Usher Syndrome Usher Syndrome WHAT IS USHER SYNDROME? Usher syndrome is a genetic condition which causes a person to be born deaf and to lose their sight over a number of years. Loss of vision often begins in late childhood and is caused by an eye condition known as Retinitis Pigmentosa (pronounced rett-in-eye-tis pig-men-toe-ser) or RP. HOW IS USHER SYNDROME PASSED ON? The pattern of inheritance for Usher syndrome is autosomal recessive. A person who inherits one faulty gene for Usher syndrome will be a carrier. Carriers are usually unaffected but can pass the faulty gene onto any children they may have. If one or both parents is a carrier, there is a 50% (1 in 2) chance that each child of theirs will also be a carrier. A child who inherits two copies of the faulty gene (one from each parent) will have Usher syndrome. If both parents are carriers, there is a 1 in 4 chance with each conception that a child will be born with Usher syndrome. Three types of Usher have been described: Type 1 is characterised by severe to profound deafness from birth, poor balance that can cause late walking and symptoms of RP before the age of 10.

68. Otolaryngology - Usher Syndrome usher syndrome is an inherited disorder that involves both a hearing impairment usher syndrome is passed from parents to their child(ren) genetically. http://uuhsc.utah.edu/healthinfo/adult/ent/usher.htm

Usher Syndrome What is Usher syndrome? Usher syndrome is an inherited disorder that involves both a hearing impairment and a vision impairment called retinitis pigmentosa. Some people also have varying problems with balance. Usher syndrome is passed from parents to their child(ren) genetically. What are the different types of Usher syndrome? There are three types of Usher syndrome: US type 1 (US1) - characteristics include: profoundly deaf from birth do not usually benefit from hearing aids severe balance problems vision problems begin by age 10 blindness eventually occurs US type 2 (US2) - characteristics include: moderate to severe hearing problems usually benefit from hearing aids use speech to communicate normal balance retinitis pigmentosa begins in teenage years US type 3 (US3) - characteristics include: born with normal hearing hearing problems develop in teenage years near normal balance deafness by late adulthood retinitis pigmentosa begins around puberty blindness by mid-adulthood How is Usher syndrome diagnosed? Special tests assist in the diagnosis of Usher syndrome, including:

69. Usher Syndrome@Everything2.com the vision loss associated with usher syndrome does not usually manifest than half of the estimated 16000 deafblind Americans have usher syndrome. http://www.everything2.com/index.pl?node_id=1165681

70. Retina Australia In usher syndrome Type 1, there is severe damage to the cochlea from a very early age and In usher syndrome Type 2, the hearing loss is more variable, http://www.retinaaustralia.com.au/AssocConditions.htm

USHER SYNDROME Symptoms and Cause This genetic condition causes hearing loss from birth and progressive loss of sight due to retinitis pigmentosa (RP), which causes degeneration of the retina. Often the first symptom of RP is night blindness, followed by narrowing side vision leading to what is called "tunnel vision". Two types of Usher syndrome In Usher Syndrome Type 1, there is severe damage to the cochlea from a very early age and therefore the child has severe deafness from birth. The child usually develops problems with night vision and tunnel vision due to RP in the first 10 years of life. People with this condition usually communicate by signing. This clearly can become more difficult as vision deteriorates. In Usher Syndrome Type 2, the hearing loss is more variable, ranging from mild to severe. Symptoms of RP usually develop from late adolescence to late twenties. Communication is enhanced in these people with hearing aids and lip reading. LARA'S STORY Lara has been diagnosed with Usher Syndrome Type 2. She is currently Co-ordinator of the Youth Group for Retina Australia Queensland Lara is currently studying meetings and events management, and is planning a career in media, focusing on motivational speaking.

71. Usher Syndrome Detailed information on usher syndrome, including types, diagnosis, and treatment at St. John’s Mercy Medical Center, St. Louis Missouri. http://www.stjohnsmercy.org/healthinfo/adult/ent/usher.asp

Home Contact Us Site Map Search for: WebNursery Health Info Find a Job Find a Physician ... Otolaryngology Printable Version Usher Syndrome What is Usher syndrome? Usher syndrome is an inherited disorder that involves both a hearing impairment and a vision impairment called retinitis pigmentosa. Some people also have varying problems with balance. Usher syndrome is passed from parents to their children genetically. What are the different types of Usher syndrome? There are three types of Usher syndrome: US type 1 (US1) - characteristics include: profoundly deaf from birth do not usually benefit from hearing aids severe balance problems vision problems begin by age 10 blindness eventually occurs US type 2 (US2) - characteristics include: moderate to severe hearing problems usually benefit from hearing aids use speech to communicate normal balance retinitis pigmentosa begins in teenage years US type 3 (US3) - characteristics include: born with normal hearing hearing problems develop in teenage years near normal balance deafness by late adulthood retinitis pigmentosa begins around puberty blindness by mid-adulthood How is Usher syndrome diagnosed?

72. Usher Syndrome usher syndrome is a rare inherited disorder primarily characterized by deafness due to an impaired ability of the auditory nerves to transmit sensory input http://www.bchealthguide.org/kbase/nord/nord529.htm

var hwPrint=1;var hwDocHWID="nord529";var hwDocTitle="Usher Syndrome";var hwRank="1";var hwSectionHWID="nord529-Header";var hwSource="en-caQ2_05";var hwDocType="Nord"; National Organization for Rare Disorders, Inc. Usher Syndrome Important It is possible that the main title of the report Usher Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms Hereditary Deafness-Retinitis Pigmentosa Retinitis Pigmentosa and Congenital Deafness Disorder Subdivisions Usher Type I Usher Type II Usher Type III Usher Type IV General Discussion Usher syndrome is a rare inherited disorder primarily characterized by deafness due to an impaired ability of the auditory nerves to transmit sensory input to the brain (sensorineual hearing loss) accompanied by retinitis pigmentosa, a disorder that causes progressive loss of vision. Researchers have identified three types of Usher syndrome and debated the existence of a fourth type. The age at which the disorder appears along with the severity of symptoms distinguishes the different types of Usher syndrome. Usher syndrome is inherited as an autosomal recessive genetic trait. The possible fourth type of Usher syndrome may be inherited as an X-linked genetic trait. Resources National Association for Visually Handicapped 22 West 21st Street New York, NY 10010

73. Usher Syndrome usher syndrome is a rare inherited disorder that involves loss of both hearing Three types of usher syndrome have been identified types I, II, and III. http://healthgate.partners.org/browsing/browseContent.asp?fileName=22833.xml&tit

74. Usher DEFINITION usher syndrome means a child is born with deafness or It has been estimated that about 1 in 10000 adults have usher syndrome (all types). http://www.dbproject.mn.org/syndromes/usher.htm

Home DB Overview Who We Are What We Do Causes (Etiologies) Education Families Interveners ... Contact Us includes map Minnesota DeafBlind Technical Assistance Project USHER Syndrome INFORMATION FOUND ON THIS WEB PAGE: Definition of Usher Syndrome Frequency in the population Types of Usher Syndrome RP ( Retinitis Pigmentosa Hearing Loss Balance Gene Studies ... Resources RELATED INFORMATION FOUND ON LINKED WEB PAGES: More information about RP ( Retinitis Pigmentosa More information about inner ear balance ( Vestibular Loss Educational implications of Usher Syndrome More information about genes in Usher Syndrome DEFINITION: Usher Syndrome means a child is born with deafness or hearing loss and then loses vision due to RP (Retinitis Pigmentosa). Both conditions must be present and together are caused by a single gene. FREQUENCY: About 3-6% 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. It has been estimated that about 1 in 10,000 adults have Usher Syndrome (all types). TYPES: On the basis of clinical findings at least three types exist ( Table 1 ) while gene localization studies show that one clinical type may be due to several different genes located on different chromosomes. The most important clinical distinctions are based on the differences in hearing and balance while the RP may look the same even to an experienced eye doctor except that the symptoms can begin earlier in Type I. Usher Syndrome is one of several conditions in which both hearing loss and retinitis pigmentosa (RP) are present. In this article the symptoms or RP, the various forms of Usher Syndrome and some of the features of other syndromes will be discussed. Suggestions will be given on where to go for further diagnosis and information.

75. Usher Syndrome usher syndrome is a rare inherited disorder primarily characterized by deafness due to an impaired ability http://my.webmd.com/hw/raising_a_family/nord529.asp

var guid_source = ""; var guid_source_id = ""; //unused var encodedurl = ""; WebMD Today Home WebMD News Center XML News via RSS Member Services WebMD University My WebMD Find a Physician Medical Info Check Symptoms Medical Library Health Tools Clinical Trials ... Women, Men, Lifestyle Who We Are About WebMD Site Map Health Topics Symptoms ... For a Complete Report Usher Syndrome Important It is possible that the main title of the report Usher Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms Hereditary Deafness-Retinitis Pigmentosa Retinitis Pigmentosa and Congenital Deafness Disorder Subdivisions Usher Type I Usher Type II Usher Type III Usher Type IV General Discussion Usher syndrome is a rare inherited disorder primarily characterized by deafness due to an impaired ability of the auditory nerves to transmit sensory input to the brain (sensorineual hearing loss) accompanied by retinitis pigmentosa, a disorder that causes progressive loss of vision. Researchers have identified three types of Usher syndrome and debated the existence of a fourth type. The age at which the disorder appears along with the severity of symptoms distinguishes the different types of Usher syndrome. Usher syndrome is inherited as an autosomal recessive genetic trait. The possible fourth type of Usher syndrome may be inherited as an X-linked genetic trait. Resources National Association for Visually Handicapped

76. Research Review 2003 - Making Sense Of Usher Syndrome Increasing knowledge of the severity of usher syndrome may help those suffering usher syndrome is a form of inherited deafness in which children also http://www.ich.ucl.ac.uk/publications/research_review03/30genes1.html

Publications Unit Research Reviews Annual Reports Corporate Plan Summary ... Roundabout Contact information Email: publications@gosh.nhs.uk Tel: 020 7829 7895 Genes, Development and Disease Dr Maria Bitner-Glindzicz I am a clinical and molecular geneticist with a research interest in deafness. I trained in adult and paediatric medicine, with a PhD in genetic deafness at the ICH where I am now Academic Head of the Clinical and Molecular Genetics Unit. I am also an Honorary Consultant in clinical genetics at GOSH. Making sense of Usher syndrome Increasing knowledge of the severity of Usher syndrome may help those suffering the later effects of this damaging disease. Usher syndrome is a form of inherited deafness in which children also progressively lose their sight. Restricted vision or night blindness may first become apparent when they are around seven or eight years old, and often progress to severe visual impairment during adolescence or young adulthood. Ongoing research has revealed that abnormalities in at least 13 genes can underlie Usher syndrome, and that in most cases the gene's normal role is in controlling the development of sensory hair cells of the inner ear. The role of these genes in the eye is less clear: they are not needed for very early development, as children with Usher syndrome are usually born without visual problems, but appear to be involved in maintaining photoreceptor cells needed for sight.

77. Usher Syndrome - Otolaryngology Health Guide Definition, types, diagnosing and treatment for usher syndrome, a common hearing disorder. http://www.umm.edu/ent/usher.htm

var MenuLinkedBy='AllWebMenus [2]', awmBN='530'; awmAltUrl=''; Otolaryngology Health Guide Ear, Nose, and Throat Disorders... Balance Disorders... Hearing Disorders... ... Index Related Resources Within UMM Otolaryngology Translate using Google to Spanish German French Italian Portuguese Otolaryngology Usher Syndrome What is Usher syndrome? Usher syndrome is an inherited disorder that involves both a hearing impairment and a vision impairment called retinitis pigmentosa. Some people also have varying problems with balance. Usher syndrome is passed from parents to their child(ren) genetically. What are the different types of Usher syndrome? There are three types of Usher syndrome: US type 1 (US1) - characteristics include: profoundly deaf from birth do not usually benefit from hearing aids severe balance problems vision problems begin by age 10 blindness eventually occurs US type 2 (US2) - characteristics include: moderate to severe hearing problems usually benefit from hearing aids use speech to communicate normal balance retinitis pigmentosa begins in teenage years US type 3 (US3) - characteristics include: born with normal hearing hearing problems develop in teenage years near normal balance deafness by late adulthood retinitis pigmentosa begins around puberty blindness by mid-adulthood How is Usher syndrome diagnosed?

78. Early Diagnosis Of Usher Syndrome Type 1 Made Possible By New Findings There are three clinical subtypes of usher syndrome, the most severe of which is Dr. BenYosef identified a mutation, R245X, of an usher syndrome type 1 http://www.pslgroup.com/dg/230ffa.htm

79. Support Group usher syndrome, Boystown; What is usher syndrome, Texas Association Retinitis Pigmentosa; usher syndrome Retinitis Pigmentosa Information Referrals, http://www.kumc.edu/gec/support/usher.html

Usher syndrome Usher Syndrome , Boystown What is Usher Syndrome , Texas Association Retinitis Pigmentosa , National Deafblind Organization Usher's Syndrome Page Also See: National organizations information on genetic conditions or birth defects Information for siblings and family members DB-LINK , National Information Clearinghouse on Children Who Are Deaf-Blind The Deafblindness Web Resource A-Z to Deafblindness (formerly Deafblind Link), United Kingdom Hard of Hearing / Deafness Resources , University of Kansas Medical Center To locate a genetic counselor or clinical geneticist in your area: Professional Genetics Societies Genetic clinics, centers, departments Genetic Societies Clinical Resources ... Search Genetics Education Center Debra Collins, M.S. CGC , Genetic Counselor, dcollins@kumc.edu This site subscribes to the principles of the HONcode (Health on the Net, Code of Conduct for Medical and Health Web Sites) of the Health On the Net Foundation

80. Health Information usher syndrome is an inherited disorder that involves both a hearing impairment and usher syndrome is passed from parents to their children genetically. http://www.baptisthealth.net/greystone/content.jsp?pageid=P00474

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