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Texas School for the Blind & Visually Impaired Outreach Department. Presents TETN # 30,238 Usher Syndrome: An Overview. Events in February. February 1-2 nd - Mentor Training at TSBVI February 13 th - TETN O&M for Babies Who Are Non-Mobile

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Texas school for the blind visually impaired outreach department
Texas School for the Blind & Visually Impaired Outreach Department

Presents TETN # 30,238

Usher Syndrome: An Overview

Events in february
Events in February . . . Department

  • February 1-2nd - Mentor Training at TSBVI

  • February 13th - TETN O&M for Babies Who Are Non-Mobile

  • February 15-16th – VI Touch Workshop with Barbara Miles

  • February 18-19th – Intervener Statewide Workshop in Austin

  • February 24-26th – Mentor Center at TSBVI

Don t forget to fax to 512 206 9320
Don’t Forget to Fax to Department512-206-9320

  • Sign-in Sheets

  • Include evaluations

  • Print e-mail addresses clearly if you want to receive your SBEC certificate

Acvrep credit
ACVREP Credit Department

  • ACVREP approval pending. Certificates will be sent once approval has been received.

  • ACVREP CEU Certificate Request Form (original broadcast date only) located on TSBVI website at www.tsbvi.edu/Outreach/ACVREPcert_request.htm

Usher syndrome an overview

Usher Syndrome: An Overview Department

Presented by

Kate Moss, Statewide Staff Development Coordinator

Edgenie Bellah, Family Specialist

TSBVI Outreach

With Special Guest, Linda Carter

What is usher syndrome
What is Usher Syndrome? Department

  • Hereditary Syndrome

  • Hearing loss

  • Progressive vision loss as a result of Retinitis Pigmentosa (RP)

  • Combined vision and hearing loss (deafblindness)

What is retinitis pigmentosa
What is Retinitis Pigmentosa? Department

  • Progressive vision loss

  • Rods of the retina (responsible for night vision) impacted first

  • In some cases early cone degeneration in macula leads to central loss

  • In most cases loss in the peripheral fields making donut shape and progresses to tunnel vision measured in degrees (10 degree fields)

  • Usually results in total vision loss

Statistics on occurrence

3-6% congenitally deaf or hard of hearing Department

50% of all cases of deafblindness; leading cause of combined vision & hearing loss in USA

Type 1 - 90% of all Usher (most common)

Type 2 - 10% of all Usher

Type 3 – seems to account for about 40% of Usher in eastern Finland;

Statistics on occurrence

How is it diagnosed

Ophthalmological exam that includes field testing as well as acuity testing

Boys Town Research Hospital

Dr. William Kimberling, Center for the Study & Treatment of Usher SyndromeBoys Town National Research Hospital – Omaha

Phone – 402.498.6713email - [email protected]

How is it diagnosed?

Hereditary pattern
Hereditary Pattern acuity testing

  • Autosomal recessive gene: both parents must pass gene for condition to occur

  • Each pregnancy 1 in 4 chance of US and 2 in 4 chance unaffected carrier

What treatment is available
What treatment is available? acuity testing

  • Gene therapy – preclinical settings

  • Nutritional therapy - vitamin A palmitate in some RP and US 2.  Docosahexaenoic acid (DHA)— can enhance effect of vitamin A. www.blindness.org

  • A Phase II/lll human clinical trial underway to test encapsulated cell technology (ECT) for delivery of a vision-preserving, therapeutic agent CNTF to retina.

  • Artificial retinal implants and transplants

Educational issues
Educational Issues acuity testing

  • Nightblindness (dark to light & light to dark transitions inside and outside)

  • Restricted fields (loss of peripheral information for communication, travel & social interactions)

  • Possible acuity problems (need for glasses, LP, issues with seating)

  • Glare sensitivity (need for sunglasses, hats, problems with overheads)

  • Need for high contrast (travel and print)

Educational issues1
Educational Issues acuity testing

  • Functional Vision Assessment or Evaluation (FVE)

  • Learning Media Assessment (LMA

  • Communication – Part B

  • Orientation & Mobility Assessment

Tool for assessment
Tool for assessment acuity testing

  • Use for completing the FVE, LMA and Communication Part-B assessments

  • http://www.dblink.org/pdf/adamls.pdf

Educational issues2
Educational Issues acuity testing

  • Travel cane

  • Driver’s training & driver’s license

  • One-on-one interpreter

  • Support Service Provider (SSP)

Deaf blind perspectives www tr wou edu tr dbp
Deaf-Blind Perspectives acuity testingwww.tr.wou.edu/tr/dbp

Db perspectives vol 9 issue 1
DB-Perspectives,Vol.9, Issue 1 acuity testing

“What’s My Role?” A Comparison of the Responsibilities of Interpreters, Interveners, and Support Service Providers

by Susannah Morgan

Educational issues3

Balance problems for Type 1 and 3 may contribute to overall clumsiness

May produce a wide-based gait although vision loss contributes

Certain sports may be difficult for the students

Infants and toddlers may be delayed in acquiring certain motor skills and may crawl with a “5 point stance”.

Educational Issues

Emotional support issues
Emotional Support Issues clumsiness

  • Being different

  • Being uninformed

  • Being left out of games / activities

  • Fearing the future

  • Feeling insecure

Don ts for usher syndrome

S clumsinesstand too close when fingerspelling, signing, speaking

Wave at the person from the sides to get attention

Point at another person who may want the attention

Grab the person’s arm to guide in the dark

Conversed with light coming directly behind you

Use large, wide-movements while using sign language

Attempt to carry on conversation in poor or dim light

Point vaguely in general direction of what you talk about.

Assume that person sees low obstacles.

Be afraid to ask if help is needed.

DON’Ts for Usher Syndrome

Dos for usher syndrome
DOs for Usher Syndrome clumsiness

  • Stand at reasonable distance (4-5 ft.) when fingerspelling, signing and speaking.

  • Walk up to or ask person nearest him to call his attention.

  • Say name of person wanting attention/where person is.

  • Offer your arm for guidance in the dark.

  • Keep direction of the light at the side or behind

  • Confine fingerspelling and signs, preferably to chest level.

  • Converse in a well-lighted area, if possible.

  • Point out/specify where/to what you are referring.

  • Be ready to warn about low obstacles/unexpected steps.

  • Feel free to ask if he or she needs help.

For parents
For Parents clumsiness

  • Read as much as you can about Usher Syndrome, but especially learn what the adults with Usher have to share – go to http://www.tr.wou.edu/dblink/lib/topics/topics.cfm

  • Go to training events with other parents http://www.tsbvi.edu/Outreach/maillist.htm

  • Help your child meet others with Usher

  • Prepare yourself and your child

  • Never loose HOPE

Some important resources
Some important resources clumsiness

  • Boys Town National Research Hospital: National Center for the Study and Treatment of Usher Syndrome www.boystownhospital.org

  • Foundation Fighting Blindness www.blindness.org

  • DB-Link: National Consortium on Deaf-Blindness www.tr.wou.edu/dblink/

  • Texas Deafblind Outreach www.tsbvi.edu

  • Texas School for the Deaf www.tsd.state.tx.us

Don t forget to fax to 512 206 93201
Don’t Forget to Fax to clumsiness512-206-9320

  • Sign-in Sheets

  • Include evaluations

  • Print e-mail addresses clearly if you want to receive your SBEC certificate

Thanks for clumsiness

joining the

Usher Syndrome