Usher Syndrome - General Information

This is a partial list of materials on this topic available from DB-LINK. In most instances, DB-LINK is able to provide a copy of the complete article. For this and other questions or information that you may need, please contact DB-LINK, (800) 438-9376 or (800) 854-7013 (TTY) or email: info@nationaldb.org

Updated 9/2009

2008-0226
Emma and Drew --Moller, Bettina U. NEWS - The Information Center for Acquired Deafblindness, NO. 1, Spring 2007, pp. 19-20. (2007) This article describes Emma, an information officer in Usher Services at Sense and her guide and hearing dog, Drew. This means that Drew is not only trained to guide Emma, who was born with Usher 1, around obstacles she cannot see, but also help her manage things in her environment which she cannot hear.

2004-0156
An Evaluation of the Sense Peer Mentoring Project --Sherlock, Christine. Sense. (2002) This booklet describes the background, history, and development of the peer mentoring project for people with Usher Syndrome. The project aims to train a core group of deafblind people to use their unique life experience to benefit others with acquired deafblindness. Describes how the mentors were recruited, trained, and provided follow-up support. Describes advertising for the project, and the two evaluations that were used to determine if the project goals were being met. Describes results of the project including problems encountered. Appendices include a sample agenda of the mentor training, and the questionnaires that were administered as evaluative tools before and after the project. Includes a peer mentoring flyer, beginnings checklist, and follow-up training document.

2009-0034
Experiences from People With Deafblindness : Nordic Project: Theory and Methods --Olesen, Birgitte Ravn; Jansbol, Kirsten. Information Center for Acquired Deafblindness. (2005) This booklet describes a five year Nordic project focusing on the personal experiences of persons with acquired deafblindness. The project objectives are explained and the theoretical and methodological reflections carried out both before and during the execution of the project are included. This is a collection of the most important points from the five booklets that cover the main issues in a deafblind person’s life. The aim was to let a group of deafblind persons talk about the consequences the progressive hearing and sight loss has on living the life that they wish to live. The five year project was designed to gather insight on the possibilities of creating and maintaining realtionships and of participating in society and to build up knowledge that can guide professionals. Available on the web: http://www.dbcent.dk/cgi-bin/vcfdbb/uploads/media/pdfs/Bog_1_GB.pdf

2008-0379
Independent and Social Living with Usher Syndrome - I Do It --Cloutier, Danielle; Lachance, Jean-Paul. 14th DbI World Conference on Deafblindness Conference Proceedings, September 25-30, 2007, Perth, Australia. (2007) This is text of a workshop presentation given at the 14th DbI World Conference on Deaf-Blindness. This presentation is a case study of a 58 year old woman with Usher syndrome.

2008-0287
Interactive Self-Study Material of the Psycho-Social Consequences for Adults with Usher Syndrome for Service Providers --Moller, Kerstin; Hammarstedt, Lena. 14th DbI World Conference on Deafblindness Conference Proceedings, September 25-30, 2007, Perth, Australia. (2007) This is the text of a workshop presentation given at the 14th DbI World Conference on Deaf-Blindness. The presentation describes the production of an interactive information CD to show the problem people with Usher syndrome face and their need of service and support in order to be as independent as possible and participate fully in life.

2006-0210
Maintaining and Expanding an Usher Network Across Europe and Beyond --Kilsby, Marylin. Brantford, Ontario: Canadian Deafblind and Rubella Association. 13th DbI World Conference on Deafblindness Conference Proceedings, August 5-10, 2003, Mississauga, Ontario, Canada. (2003) This is the text of a workshop presentation given at the 13th DbI World Conference on Deaf-Blindness. The paper describes the European Usher Syndrome Network.

2008-0301
Making Connections - Usher Contact, Support and Social Groups --Kilsby, Marylin. 14th DbI World Conference on Deafblindness Conference Proceedings, September 25-30, 2007, Perth, Australia. (2007) This is text of a workshop presentation given at the 14th DbI World Conference on Deaf-Blindness. This presentation describes how people with Usher syndrome have worked to overcome isolation through two different forms of social contact, face to face social groups and Usher e-groups.

2007-0281
Making Progress with Usher Syndrome --Scarola, Dawn. Volta Voices, March/April, 2007, 34-36. (2007) This article offers a brief overview of Usher Syndrome with a focus on information for the general public. It discusses developments in early identification and promising treatments.

2002-0261
Nebraska Usher Syndrome Screening Project --Coonts, Teresa; Giittinger, Sally; Kimberling, Bill; Srb, Steve. Omaha, Nebraska: Nebraska Project for Children and Youth with Deaf-Blindness. (2002) This manual was designed to help school personnel conduct Usher Syndrome screenings. It provides information about Usher Syndrome, screening instructions, screening forms (available in Spanish), and state and national resources. It includes descriptions of Usher Types I, II, and III, causes of Usher syndrome, roles of the screener and interpreter, and information about how to interpret screening results. For copies, contact Teresa Coonts, Project Coordinator, at 402-595-1810 or tcoonts@esu3.org.

2007-0168
Nordic Project on the Experiences of People With Deafblindness --Olesen, Brigitte Ravn; Jansbol, Kirsten. Information Center for Acquired Deafblindness. (2005) The Nordic Project on the Experiences of Deafblind People is a joint cooperation between the Nordic countries. Twenty deafblind persons in Denmark, Sweden, Norway and Iceland have been interviewed once a year for a five year period. During the five years the participants have shared their experiences as people who are deafblind and who have a progressive visual and hearing impairment. The structured interviews have focused on the person in different life situations, in the family, at work, in their free time etc. and have resulted in six booklets on different themes. Booklet 1: Theory and Method; Booklet 2: Receiving a Diagnosis; Booklet 3: Getting Support; Booklet 4: Being Active; Booklet 5: Getting an Education and Work; Booklet 6: Narratives of Everyday Life.
Available on the web: http://www.dbcent.dk/vcfdbb/subpage105.aspx

2008-0364
Occupational Chances of People with Usher Syndrome Type I in Germany --Scheele, Andrea. 14th DbI World Conference on Deafblindness Conference Proceedings, September 25-30, 2007, Perth, Australia. (2007) This is text of a presentation given at the 14th DbI World Conference on Deaf-Blindness. This presentation describes a questionnaire sent out to people with Usher syndrome type I in Germany about their careers and a model developed for improvement and future prospects outlined.

2008-0285
Out of Being Caged to Being Independent and Leading an Active Social Life by Training Dogs to Aid the Deaf/Deafblind --Meirovich, Thamara; van Eek, Tracy. 14th DbI World Conference on Deafblindness Conference Proceedings, September 25-30, 2007, Perth, Australia. (2007) This is the text of a workshop presentation given at the 14th DbI World Conference on Deaf-Blindness. The presentation describes a person with Usher syndrome gaining independence and enjoying a social life using a dog for assistance in Israel.

2005-0295
Psychological and Social Issues of Children with Type I Usher Syndrome --Metcalf, Chad. DEAF-BLIND AMERICAN, vol. 44, #1, Jan-Mar 2005, pp. 38-44. (2005) By exploring the physical and psychological dimensions of Usher Syndrome, this article seeks to help the reader identify better with children who have Usher Syndrome. It describes briefly the different types of Usher and symptoms associated with the syndrome. It also discusses informing the child about their diagnosis and the impact that could have including describing the five stages of grief and how that applies to a child with Usher Syndrome. Social issues such as friendship, independence and social activities are discussed.

2006-0114
Quality of life and Cochlear Implantation in Usher Syndrome Type I --Damen, Godelieve W. J. A.; Pennings, Ronald J. E.; Snik, Ad F. M.; Mylanus, Emmanuel A. M. LARYNGOSCOPE, vol. 116, May 2006, pp. 723-728. (2006) This study from the Netherlands evaluated quality of life (QoL), hearing, and vision in patients with Usher syndrome type 1, with and without cochlear implants. QoL of 14 patients (7 adults, 7 children) who had cochlear implants was compared with QoL of 14 patients (12 adults, 2 children) who did not have cochlear implants using three questionnaires and the Usher Lifestyle Survey. A significant benefit of CI was seen in the hearing-specific questionnaire. The Usher Lifestyle Survey indicated that patients with the CI tended to be able to live an independent life more easily than the profoundly deaf unimplanted patients. Overall, the authors concluded that QoL can be enhanced by CI in patients with Usher 1, although effects are mostly seen in hearing-related QoL items.

2007-0416
Rapper sings in sign --Bishara, Mike. Auckland: Sunday Star Times. Stuff.co.nz, August 9, 2007, 1-2. (2007) Story of Terry Richards who translated rap lyrics into sign language and performs as a signing rap artist. Richards who has Usher Syndrome, also has keratoconus which limits his central vision. Available on the web: http://www.stuff.co.nz/northland/4158527a23016.html Publisher's web site: http://www.stuff.co.nz

2004-0230
Siblings Are Doing It for Themselves --Kersten, Sabine. TALKING SENSE, Summer 2003, pp. 28-30. (2003) This article describes the experiences of a woman who is a sibling of a man with Usher Type I. She talks about the impact on her own childhood, her relationship with her brother, and their future. She also discusses a new sibling network that she is starting.

2004-0487
A Sign to Remember --Podmore, Ron. Tacoma, WA: Puget Sound Publications. (2002) Fictional story of a teenage boy as he adjusts to changes related to having Usher Syndrome

2007-0345
Silence with a Touch : Living with Usher Syndrome Rochester, NY: National Technical Institute for the Deaf. (2006) This DVD introduces several individuals, from early adolescence to adulthood, who are living with Usher Syndrome, a genetic condition that causes both hearing loss and progressive vision loss. The individuals profiled are shown in a variety of settings including at school, at home, and in places of employment. All speak about how Usher Sydnrome has changed their lives and about the adjustments and challenges they face. It was produced by the National Technical Institute for the Deaf in cooperation with the New York State Technical Assistance Project Serving Children and Youth Who Are Deafblind. To order, send a check or money order payable to Teachers College for $20.00 (U.S.) or $25.00 (International). The cost includes postage and handling fees. Mail to New York State Technical Assistance Project, Teachers College Columbia University, 525 West 120th Street, Box 223, New York, NY 10027. For further information, call 212-678-8188, e-mail nystap@tc.edu, or go to http://www.tc.columbia.edu/nystap/detail.asp?id=dvd. Publisher's web site: www.tc.columbia.edu

2008-0520
Spotlight on the Deaf-Blind Community : Hands, Heart and Soul --Spiers, Elizabeth. AADB TODAY, December 22nd Edition [online] (2008) Tells the personal story of William Suggs, from Baltimore. Mr. Suggs, who has Usher Syndrome Type 1, is one of the major participants in the choir of the Baltimore-based Christ United Methodist Church of the Deaf. This article describes his history, his current work with the choir, his involvement with the American Association for the Deaf-Blind, and his struggle to find employment. Available on the web: http://aadb.convio.net/site/MessageViewer?em_id=1721.0&dlv_id=6361

2005-0413
Success Story : Debra Whittier, San Diego --Mansfield, Jennifer. AT Network and California Assistive Technology Systems. AT JOURNAL-THE LATEST NEWS & RESOURCES IN
ASSISTIVE TECHNOLOGY, vol. 87, December 15, 2003. (2003) This story is about Debra Whittier of San Diego and her life after diagnosis of Usher syndrome. Whittier found a role model and mentor in Assistive Technology Advocate Mark Carlson of the Access Center at Deaf Community Services in San Diego. Available on the web: http://www.atnet.org/news/2003/dec03/121502.htm Publisher's web site: http://www.atnet.org/

2005-0414
Success Story Updates : George Sharp, Susan Harrison and Debra Whittier --Mansfield, Jennifer. AT Network and California Assistive Technology Systems. AT JOURNAL-THE LATEST NEWS & RESOURCES IN
ASSISTIVE TECHNOLOGY, vol. 106, January, 2005. (2003) The AT Journal has published many educational and entertaining success stories since the first journal in November 2002 and the consumers highlighted have no doubt done wonderful things in their lives, due in part to assistive technology devices obtained. This article recaps three past success stories and updates on where they are today. Debra Whittier has Usher syndrome. Available on the web: http://www.atnet.org/journal/010103.htm Publisher's web site: http://www.atnet.org/

2007-0246
Successful Transition of Students with Usher Syndrome from School to Work or Continuing Education --Miles, Constance; Davidson, Roseanna. --ICEVI. (2002) This is a paper presented at the International Council for Education of People with Visual Impairment (ICEVI) world conference in 2002. With ongoing opportunities for independence, self-confidence, and communication the student with Usher Syndrome is able to realize her strengths. Successful job placement occurs when these strengths are combined with assistive devices, education and rehabilitation professionals, and opportunities that serve as a bridge from the school to work environment. Once the bridge is crossed, the student has an opportunity to use the skills that she acquired during her school years. Available on the web: http://www.icevi.org/publications/ICEVI-WC2002/papers/02-topic/02-miles.htm

2006-0173
They Should Know They Have Usher Syndrome Around Here [Dissertation] : College Students Who Are Deafblind --Arndt, Katrina. Ann Arbor, MI: UMI Dissertation Services. (2005) This study examined how post-secondary education students who have Usher Syndrome negotiate identity, social relationships and services in higher education settings.

2004-0061
Touching Lives : Portraits of Deaf-Blind People --Gordon, Myles; Hajjar, Susan. Navada Productions. (2002) This video is a documentary by a deaf-blind interpreter. Susan Hajjar, grew up with three siblings who are deaf-blind and tells the story of how their influence affected her life. The video features Jamie Lard, a deaf-blind woman who advocates on behalf of deafblind people. Jamie describes her upbringing including her time as a student at Perkins School for the Blind, and now as an adult living independently. It also features, Harry Anderson, president of the American Association of Deafblind (AADB), and Ona Stewart, a deaf-blind woman with Usher syndrome who lives and works independently in a large city. Barbara Stein, who is orally trained and uses fingerspelling, is a software engineer who is unemployed at the time of the taping. She describes the difficulties she faces in gaining employment especially in a declining labor market of information technology. Describes how many people with deafblindness face isolation and loneliness. Other profiles include Chuck Ferraro, and the Tracy family. A transcript of the video is also available. Download the order form for this video at http://dblink.org/lib/docs/touchingLivesInvoice05.doc

2004-0167
Touching Lives Full Script --Gordon, Myles; Hajjar, Susan. (2002) This is the full text script of the video documentary "Touching Lives: Portraits of Deaf-Blind People" that describes the experiences of Susan Hajjar, a deaf-blind interpreter who grew up with three siblings who are deaf-blind. It also tells the stories of a number of deaf-blind individuals including Jamie Lard, a deaf-blind woman who advocates on behalf of deafblind people; Harry Anderson, president of the American Association of the Deaf-Blind; Ona Stewart, a woman with Usher syndrome who lives and works independently in a large city; and Barbara Stein, a software engineer who was unemployed at the time of the taping. Describes how many people with deafblindness face isolation and loneliness.

2006-0126
Trust me, I'm a doctor! --Leicester, Howard. Talking Sense, Summer 2004, pp. 16-17. (2004) Completing a PhD is notoriously tough - and if you have Usher syndrome then the odds are definitely stacked against you. Dr. Howard Leicester describes how he finally achieved his goal.

2009-0221
Update on Usher Syndrome --Saihan, Zubin; Webster, Andrew R.; Luxon, Linda; Bitner-Glindzicz, Maria. CURRENT OPINION IN NEUROLOGY, vol. 22, pp. 19-27. (2009) This technical article reviews recent developments in the understanding of Usher syndrome. It includes a description of the various types of Usher syndrome and its characteristics (hearing loss, retinitis pigmentosa, vestibular dysfunction) as well as a detailed discussion of its mechanisms and genetics. It describes recent molecular findings that have provided more information regarding the pathogenesis of Usher Syndrome and the wide phenotypic variation in both the audiovestibular and/or visual systems. Evidence has begun to emerge supporting a theory of a protein interactome involving the Usher proteins in both the inner ear and the retina.

2005-0231
Usher 2 - How is it for you? --Butler, SJ. Sense. TALKING SENSE, Autumn/Winter, 2004, p. 22-30. (2004) Profiles a number of adults with Usher 2. Many of those diagnosed had never heard of Usher 2 until they were diagnosed much later in their lives. Describes services available through Sense.

2008-0227
Usher Children Project --Moller, Bettina U. NEWS - The Information Center for Acquired Deafblindness, NO. 1, Spring 2007, p. 21. (2007) This article describes a project, set up in 2005 with the help of the Information Center (Denmark), which worked on finding new models for optimizing the professional guidance and advising of parents of children born with Usher syndrome. The hope was that there could be a movement from linear consulting with many separate stages to a more circular form, which included both the past and the future.

2007-0143
Usher Lifestyle Survey: Maintaining Independence: A Multi-Centre Study --Damen, Godelieve, W.J.A.; Krabbe, Paul F.M.; Kilsby, M.; Mylanus, Emmanuel A.M. INTERNATIONAL JOURNAL OF REHABILITATION RESEARCH, vol. 28, #4, 2005, pp. 309-320. (2005) Patients with Usher syndrome face a special set of challenges in order to maintain their independence when their sight and hearing worsen. Three different types of Usher (I, II and III) are distinguished by differences in onset, progression and severity of hearing loss, and by the presence or absence of balance problems. In this study 93 Usher patients from seven European countries filled out a questionnaire on maintaining independence (60 patients type I, 25 patients type II, four patients type III and four patients type unknown). Results of Usher type I and II patients are presented. Following the Nordic definition of maintaining independence in deaf-blindness, three domains are investigated: access to information, communication and mobility. Research variables in this study are: age and type of Usher, considered hearing loss- and the number of retinitis pigmentosa-related sight problems. Usher type I patients tend to need more help than Usher type II patients and the amount of help that they need grows when patients get older or when considered hearing loss worsens. No patterns in results were seen for the number of retinitis pigmentosa related sight problems.

2006-0272
Usher Study Group --Guest, Mary; Miles, Connie. Brantford, Ontario: Canadian Deafblind and Rubella Association. 13th DbI World Conference on Deafblindness Conference Proceedings, August 5-10, 2003, Mississauga, Ontario, Canada. (2003) This is the text of a workshop presentation given at the 13th DbI World Conference on Deaf-Blindness. The paper describes a meeting held at the conference of the European Usher Study Group focusing at the Canadian conference on issues of transition as well as medical science.

2008-0213
Usher Symposium Speaker Talks --Boys Town National Research Hospital. Speeches from the First International Symposium on Usher Syndrome and Related Disorders, Omaha, NE, October 2006. (2006) This DVD contains speeches given by leading experts at the First International Symposium on Usher Syndrome and Related Disorders on October 4-6, 2006 in Omaha, NE. The Symposium took place at Boys Town National Research Hospital. The event was primarily an exchange of information between scientists and clinicians, though people affected by Usher syndrome and their families were welcome to attend. Presentation and discussion topics included: treatment and rehabilitation, ophthalmology and audiology, balance and vestibular issues, psychosocial aspects, and molecular genetics.

2005-0108
Usher Syndrome --Davenport, Sandra L.H. Austin, TX: Texas Deafblind Project. 2005 Texas Symposium on Deafblindness. (2005) Discusses Usher Syndrome, its symptoms, different types of the syndrome, gene studies and resources for Usher Syndrome.

2007-0035
Vision Care for All --Guest, Mary. NB MAGAZINE, Issue 1, January 2006, pp.31-35. (2006) This article gives the background to new guidelines which highlight the need for lifelong vigilance in checking the vision of deaf people. In April 2004, Vision care for all deaf children and young people was launched at the British Library in London by the National Deaf Children's Society and Sense, the National Deafblind and Rubella Association. This publication was the result of five years of collaboration between NDCS and Sense, and with a working party drawn from a wide cross section of people involved with the care of deaf children.

2007-0094
What You Should Know About US! (Understanding Usher Syndrome) --Miller, Peggy; Weiser, Amanda. Austin, TX: Texas Deafblind Project. 2007 Texas Symposium on Deafblindness. (2007) This presentation includes a brief summary about Usher syndrome; a chart listing types of Usher syndrome; a vision screening tool; suggestions for Home/Dorm for Students with Usher syndrome; and Do's and Don'ts for Working With Deaf People Who Have Retinitis Pigmentosa. There is also a section on links to resources, books, materials and research articles.

2005-0028
Words in My Hands : A Teacher, a Deaf-Blind Man, an Unforgettable Journey --Chambers, Diane. Conifer, CO: Ellexa Press LLC. (2004) After his wife died when he was 86, Bert Reidel, a man with Usher Syndrome, moved to Colorado to live with his son and daughter-in-law. Although Bert was an expert Braille reader, he had never learned sign language and his wife had been his “eyes and ears” to the world. This book tells the story of Bert’s life and how he learned sign language beginning at age 86. It illustrates that it is never too late to learn as it describes how sign language transformed not only Bert’s life, but the lives of his family, friends, and the interpreter who was his sign language teacher. Cost $15.95. Available from Ellexa Press LLC, 32262 Steven Way, Conifer, CO 80433. Fax: 303- 838-7010. E-mail: dlc@ellexapress.CityMax.com. Available on the web: http://www.ellexapress.citymax.com