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Topic: Usher syndrome


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  Usher Syndrome
Usher syndrome is the most common condition that involves both hearing and vision problems.
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.
Usher syndrome is inherited or passed from parents to their children through genes.
www.nidcd.nih.gov /health/hearing/usher.asp   (1286 words)

  
  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 to the brain (sensorineual hearing loss) accompanied by retinitis pigmentosa, a disorder that causes progressive loss of vision.
Usher syndrome is inherited as an autosomal recessive genetic trait.
Usher syndrome is characterized by nerve deafness due to an impaired ability of the auditory nerves to transmit sensory input to the brain (sensorineural hearing loss) as well as abnormal accumulation of colored (pigmented) material on the nerve-rich membrane lining the eyes (retinitis pigmentosa).
hw.healthdialog.com /kbase/nord/nord529.htm   (2610 words)

  
 The Foundation Fighting Blindess - Usher Syndrome
Usher syndrome is the name given to a group of inherited disorders that cause hearing and vision loss.
Usher syndrome accounts for approximately three percent of the congenitally deaf (deaf at birth) and over 50 percent of the deaf-blind community.
Usher syndrome is caused by defects (mutations) in certain genes that are necessary for normal vision and hearing.
www.ffb.ca /disease_usher.php?hc=0   (789 words)

  
 Usher syndrome - Genetics Home Reference
The signs and symptoms of Usher syndrome vary in their severity and age of onset; most are present from birth or shortly thereafter, while a few begin later in childhood or adolescence.
Usher syndrome is thought to be responsible for 3 percent to 6 percent of all childhood deafness and about 50 percent of deaf-blindness in adults.
Mutations in the CDH23, GPR98, MYO7A, PCDH15, USH1C, USH1G, USH2A, and USH3A genes cause Usher syndrome.
ghr.nlm.nih.gov /condition=ushersyndrome   (836 words)

  
 Information About Usher Syndrome - The National Center for the Study & Treatment of Usher Syndrome - Boys Town ...
The RP may look the same even to an experienced eye doctor except that the symptoms seem to begin earlier in Type I. Usher syndrome is one of several conditions in which both hearing loss and retinitis pigmentosa (RP) are present.
Usher Type III is particularly common in Finland but is also seen in the rest of Europe and the United States in about 5 - 10 percent of all Usher cases.
Other syndromes have eye findings or hearing loss patterns which are different from those in Usher syndrome as well as abnormalities of other body parts.
www.boystownhospital.org /Usher/information.asp   (2217 words)

  
 Usher Syndrome Type I
Each subsequent pregnancy of a couple who has had a child with Usher syndrome type I has a 25% chance of resulting in an affected child, a 50% chance of resulting in an unaffected child who is a carrier, and a 25% chance of resulting in an unaffected child who is not a carrier.
Usher syndrome type II is characterized by (1) congenital, bilateral sensorineural hearing loss predominantly in the higher frequencies that ranges from mild to severe; (2) normal vestibular function; and (3) adolescent-to-adult onset of retinitis pigmentosa.
The parents of an individual with Usher syndrome type I are obligate heterozygotes and therefore carry a single copy of a disease-causing mutation in an Usher syndrome type I gene.
www.geneclinics.org /profiles/usher1/details.html   (4315 words)

  
 Retinal degenerative diseases - Usher syndrome
Usher syndrome is an inherited condition which results in hearing loss and a progressive loss of vision from Retinitis pigmentosa (RP).
In the Usher syndrome form of the disease the retinal condition tends to be diagnosed later rather than earlier in the life of the deaf person and affected individuals may not know they have RP until they have reached young adulthood.
Usher is transmitted genetically by an autosomal recessive gene.
www.retina-international.org /usher.htm   (2693 words)

  
 Usher's Syndrome and Retinitis Pigmentosa: Hear-it
Usher's syndrome is a disease that affects both hearing and sight.
Usher´s syndrome may also be referred to as Retinitis Pigmentosa.
In another form of Usher´s syndrome, the person will be suffering from partial hearing loss, and Retinitis Pigmentosa will be present before the patient reaches 20 years of age.
www.hear-it.org /page.dsp?page=330   (269 words)

  
 MN DeafBlind Technical Assistance Project - Usher Syndrome
Usher Syndrome means a child is born with deafness or hearing loss and then looses vision due to RP (Retinitis Pigmentosa).  Both conditions must be present and together are caused by a single gene.
Usher Syndrome only affects the eyes and ears!  If something else is present, like mental retardation, obesity, extra fingers, etc., another syndrome may be the cause.  A number of conditions may be confused with Usher Syndrome.
Sometimes the diagnosis of Usher Syndrome is given by an eye doctor or ear doctor who may not be aware of these other syndromes or is not aware that the patient they are examining has other problems.
www.dbproject.mn.org /ushersyndrome.html   (2185 words)

  
 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.
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.
www.theitinerantconnection.com /usher_syndrome.htm   (918 words)

  
 Usher Syndrome in the School Setting
Some students, especially those with Usher syndrome, Type 1, learn quite young that the members of the Deaf community are not always comfortable and sensitive to a student with a vision loss.
It is important for the counselor to be cognizant of the interaction between deafness and blindness and to be aware of the psychosocial aspects of Usher syndrome.
At the same time students with Usher syndrome are worrying about their decreasing visual fields, they are still students who share the concerns of all young people of their age: popularity and attraction to others, sports, ambitions for the future.
www.dblink.org /lib/topics/usher-IV.htm   (1592 words)

  
 Usher syndrome type II - Genetics Home Reference
Usher syndrome type II is characterized by hearing loss from birth and progressive vision loss that begins in adolescence or adulthood.
Usher syndrome type II is a subtype of Usher syndrome.
Usher syndrome type II appears to be the most common type of Usher syndrome.
ghr.nlm.nih.gov /condition=ushersyndrometypeii   (868 words)

  
 Usher Syndrome - Causes, Symptoms & Treatment
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).
Individuals with Usher syndrome type I are nearly or completely deaf and experience problems with balance from a young age.
Individuals with Usher syndrome type III are born with normal hearing but develop RP and then progressive hearing loss.
www.healthnewsflash.com /conditions/usher_syndrome.htm   (882 words)

  
 Usher Syndrome in the School Setting
Usher syndrome if they are to feel good about themselves and if they are to have any sense of their own selves in the future.
Usher syndrome is the name given to a group of genetic conditions involving both hearing loss and retinitis pigmentosa, a progressive, degenerative eye
Students with Usher syndrome usually experience a slow and progressive loss of peripheral vision, and this may be followed by a loss of central vision.
www.msab.state.mn.us /DBC/usher_syndrome_in_the_school_set.htm   (10433 words)

  
 Tipsheet: Counseling Students Who Have Usher's Syndrome
Usher Syndrome is an autosomal recessive genetic disorder characterized by congenital hearing loss and gradually developing retinitis pigmentosa leading to the loss of vision.
Type I Usher Syndrome is characterized by profound congenital deafness, vision problems starting in early childhood, and severe balance problems.
Historically, most students with Usher Syndrome Type I attended residential schools and colleges for the deaf, while individuals with Type II attended regular public schools and universities.
www.netac.rit.edu /publication/tipsheet/ushers1.html   (2040 words)

  
 Usher Syndrome in the School Setting
People with Usher syndrome and other deaf-blind conditions are able to go to college and vocational programs and to work at all kinds of jobs.
A diagnosis of Usher syndrome should not be the only factor when determining a future course of study.
People with Usher syndrome may be able to work for many years at their first choice of occupation.
www.dblink.org /lib/topics/usher-VI.htm   (1079 words)

  
 ushernet.org - What is Usher-Syndrome   (Site not responding. Last check: 2007-10-29)
Usher-Syndrome is named after a British doctor, Charles Usher, who first described the symptoms at the beginning of this century.
Usher Type I: The individual is born with a very profound hearing deficiency or deafness, Retinitis Pigmentosa and balance problems.
Usher-Syndrome is passed to succeeding family generations through the autosomal recessive inheritance pattern, which means that two defective Usher genes, one from each parent, is required for a person to suffer from it.
www.ushernet.org /en/ushersyndrome   (333 words)

  
 Information About Usher Syndrome
Usher Syndrome is a genetic disorder involving the loss of both sight and hearing.
Usher Syndrome is an autosomal recessive gene, and both parents must pass the gene to their child in order for the child to have the condition.
Usher Syndrome accounts for over 50% of all cases of deaf-blindness, with approximately 10,000 known cases in the United States.
www.tsbvi.edu /Outreach/deafblind/usher.htm   (1852 words)

  
 BBC - Health - Conditions - Usher Syndrome
It's rare for a child to have problems with both their hearing and their vision but when this happens the most common cause is Usher syndrome.
Usher syndrome is a genetic condition, which is inherited in an autosomal recessive fashion.
In most cases each parent only carries one Usher syndrome gene, so they don't have the condition themselves and are often unaware that they're a carrier.
www.bbc.co.uk /health/conditions/ushers1.shtml   (673 words)

  
 Usher Syndrome Resource Guide [NEI Health Information]
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).
Individuals with Usher syndrome type III are born with normal hearing but develop RP and then progressive hearing loss.
A person with Usher syndrome must pass on one disease gene to each of his or her children.
www.nei.nih.gov /health/ushers/index.asp   (1248 words)

  
 Your Health: Trover Foundation
Usher syndrome is an inherited disorder that involves loss of both hearing and vision.
Usher syndrome is inherited as an autosomal recessive trait.
Usher Types I and II are the most common, accounting for 90-95 percent of all cases, according to NIDCD.
www.stayinginshape.com /4troverfoundation/libv/k40.shtml   (882 words)

  
 Usher Syndrome : Risk Factors
However, the effect of pregnancy on Usher syndrome has not been clinically studied.
It is caused by mutations in genes that are active in the retina and in the ear.
Usher syndrome is not caused by injury, infection or exposure to any toxic substance.
www.blindness.org /disease/riskfactors.asp?type=4   (197 words)

  
 What is Usher Syndrome?
Usher Syndrome is a genetic disorder with hearing loss and retinitispigmentosa which causes a progressive loss of vision.
Individuals with Type I Usher Syndrome have a profound hearing lossin all frequencies and are considered to be deaf from birth.
Usher Syndrome is called a recessive condition because a person musthave a pair of altered genes before they have the syndrome.
www.deafblind.com /whatushe.html   (1030 words)

  
 HKNC Usher Syndrome
A common cause of deaf-blindness is a genetic condition called Usher syndrome.
People with Usher syndrome are born with hearing loss and retinitis pigmentosa.
Usher List is an Internet based listserv for people with Usher syndrome, their parents, friends, spouses and service providers.
www.hknc.org /WhoWeServeUsher.htm   (165 words)

  
 About Usher syndrome
People with Usher have a genetic condition which means they are born deaf or hard of hearing, then gradually start to lose their sight.
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.
Sense provides advice and support to people with Usher and their families - particularly when the condition is first diagnosed.
www.sense.org.uk /deafblindness/usher   (258 words)

  
 Information about usher syndrome
Usher syndrome is an inherited condition that causes a serious hearing loss that is usually present at birth or shortly thereafter and progressive vision loss caused by retinitis pigmentosa (RP).
Retinitis pigmentosa is a group of inherited diseases that cause night-blindness and 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.
Since individuals with Usher syndrome have both hearing and visual symptoms, tests are aimed at the hearing and visual systems.
www.mamashealth.com /syndrome/usher.asp   (552 words)

  
 Usher Syndrome information from Wikipedia - WrongDiagnosis.com
The Usher syndrome is defined as innate reciprocal deafness and progressing loss of vision due to Retinopathia pigmentosa (RP, formerly known as “Retinitis pigmentosa”).
For the patient the RP is noticeable in the beginning with night blindness, proceeding into a slow, progressive restriction of the visual field („tunnel vision“), which usually leads in a later stage to complete blindness.
C. Usher: On the inheritance of Retinitis pigmentosa with notes of cases.
www.wrongdiagnosis.com /u/usher_syndrome/wiki.htm   (1272 words)

  
 Patient Profiles - The National Center for the Study & Treatment of Usher Syndrome - Boys Town National Research ...
David has Usher syndrome, a devastating genetic disorder that robs its victims of their hearing and then slowly takes away their vision as they approach adulthood.
"The type of Usher's I have causes deafness from birth and causes me to be legally blind," say David.
Although they knew her hearing and vision would deteriorate in the years ahead, they delayed sharing the diagnosis with her until she turned 16 and could understand what was happening to her.
www.boystownhospital.org /Usher/patientprofiles.asp   (878 words)

  
 Usher Syndrome
Usher Syndrome Symposium Slated for October in Omaha
Leading experts in Usher syndrome will convene October 4-6, in Omaha, Nebraska, for the First International Symposium on Usher Syndrome and Related Disorders.
- Usher syndrome is an inherited disorder that is characterized by moderate to profound hearing impairment, and progressive vision loss due to retinitis pigmentosa.
www.blindness.org /Usher   (177 words)

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