1. Effective Strategies for Home and School for the Student with Usher Syndrome
Molly McLaughlin OTR/L, M.A.
Project for NM Children and Youth who are Deaf-blind
UNM Center for Development and Disability

2. Usher Syndrome Overview
Usher syndrome- defined as hearing loss and an eye disorder called retinitis pigmentosa, or RP.
Inherited recessive genetic disorder- must get the gene from both parents. 1-4 chance of having a child with US if both parents carry the gene
About 25,000 people in the U.S. are affected by (US)
Estimated that 3-6% of the deaf/hh population have US
Usually leads to legal, if not complete blindness from RP
At least three types exist (Type l, ll, and lll)

3. Retinitis Pigmentosa “RP”
RP causes night-blindness and a loss of peripheral vision (side vision) through the progressive degeneration of the retina.
RP may be diagnosed at any age and can lead to complete blindness
RP affects the photoreceptor cells (rods and cones) in the retina. These cells gradually deteriorate and die.
Rods help us see in dim light and at night
Cones help us with our day vision, seeing fine details and color

4. RP-continued
As RP progresses, the field of vision narrows—a condition known as “tunnel vision” -until only central vision (the ability to see straight ahead) remains.
With RP the rods deteriorate first- first symptom is difficulty seeing in dim light or at night
Later as cones start to die, will get blind spots in the peripheral vision
With “tunnel vision” good vision may remain in the center- (central vision)

5. RP- continued Testing:
Person is considered “legally blind” when only 20 degrees of central vision remain.
Some people may retain 5-10 degrees of good vision into old age.
Testing:
USM Chip-microchip that can test a saliva sample (65-75 % accurate)
ERG-Electroretinography – measurement of nerve impulses in the retina (95% accurate)
Visual Field Test- side/peripheral vision

6. Usher Syndrome- Type l Most common-estimated at 90%
Born with severe to profound hearing loss in both ears
Balance problems- absent vestibular function
Night blindness in infancy or early childhood
Usually ASL signers
Delayed developmental milestones of lifting head, crawling, walking
Tunnel vision usually by age 16

7. Usher Syndrome- Type ll
Born with moderate to severe hearing loss-both ears show similar loss
Benefit from hearing aids
RP- Night blindness begins in teenage years- with tunnel vision present by late teens to early 20’s.
No balance issues
Normal motor milestones
Usually oral communicators- rely on lip-reading

8. Usher Syndrome- Type lll
Born with good hearing or mild hearing loss
Hearing loss progresses over time- hearing aids may need to be changed.
Begin as oral communicators- as hearing loss progresses- ASL communicators.
RP- Night blindness in childhood or teens- tunnel vision in the 20’s- 30’s
Can have a progressive balance disturbance

9. Behavioral Symptoms for Parents and Teachers- (US-l)
Young children - harder time learning how to sit without support, crawl and walk. May prefer rolling to being on all fours. May have 5-point crawl-head down
Walking usually delayed (18 months or later)
Seen as “clumsy” children
May love twirling, spinning- don’t get dizzy

10. Behavioral Picture - continued
As RP progresses:
May naturally or automatically turn their head to scan visual field- not fully recognizing the extent of vision loss.
Bumps into things in front of their feet.
Tripping over curbs, stairs, people
May reach for something in front of them that they see clearly and knock something else over.
Spills when pouring liquids

11. Behavioral Picture- continued
Bump into open doors- not see the edge of the door, or hits head on kitchen cabinet
Reluctance to play in low light or outdoors at twilight/dark
May request that lights be left on at night, in hallways etc.
Difficulty adjusting to changes in light- going from low light to sunny outdoors- vice versa.
Avoids outdoor sports when sun is bright

12. Behavioral Picture- continued
Wears sunglasses- even inside
Likes to enter a room that is dark early- movie theater.
Avoids conversations in a darkened area.
Difficulty with riding a bicycle
When walking along a road at night, may stagger or lose balance after an oncoming car has passed.

13. School Behaviors and Considerations
Needs good contrast- has difficulty reading light copies
Turns head while reading
Uses fingers to mark place
Holds book close to the eyes or bends to read
Places face close to desk while writing
Sits near blackboard
Fails to understand or miss group instruction- may position self to one side of the group. Often last at completing group activities.

14. School Behaviors and Considerations-continued
May have repetitive behavior- likes to do things the same way. Routines are comforting, predictable and increase success!
Appears to ignore others standing to the side
Prefers conversation at 4-6 feet
Becomes anxious in unfamiliar areas or with new tasks.
Episodes of anger, frustration, emotional outbursts-normal grieving process. Can/will reoccur as vision decreases over time

15. Educational Needs Schools need to provide:
- academics- with modifications/adaptations
- orientation and mobility instruction
- athletics/sports- good for student as long as it
doesn’t compromise safety.
- vision teacher/consultant- help with modifications

16. Educational Needs-continued
- communication- ASL, Braille, tactile
signing, captioning-need black box with
text for good contrast
- vocational exploration and training- skills
for future
- support/counseling- reduce isolation,
educate peers

17. Classroom Modifications
Teachers need to consider future dual-sensory impairment when identifying skills to teach- not just current status.
Full spectrum lighting if possible- helps us see fine details more easily, color matching, reduces glare, Helps lesson eye fatigue and strain when performing visually demanding tasks.
Seat student where they are comfortable- (front-side) so they can see chalkboard, teacher and other students in class.

18. Classroom Modifications- continued
Teacher/assistant needs to direct attention of the student to other students in class that are asking or answering questions. Can only see 1 person at a time.
Windows should be behind the student. Teacher should avoid standing in front of window while communicating
Teacher should provide instruction using a non-cluttered background area-with good contrast
Furniture arrangement- Keep room the same if possible. Keep doors and drawers closed. Let the student know in advance of any changes.

19. Classroom Modifications-continued
Print materials should be maximum contrast. Use non-glare paper. Yellow transparency overlay is helpful to reduce glare and eye fatigue
Whiteboard- need dark markers- black, blue or purple best. Yellow difficult to see.
Students may need individual copies of graphs, charts, assignments to examine close up.
Students need additional time to complete tasks- “time and a half” minimum. May consider reducing the # of questions or problems to equalize the time spent on task.

20. Home Modifications
Lighting- have child/youth experiment with different lighting options- full spectrum, goose-neck lamps, natural light, etc. to figure out what is best, where, and at what time of day. Use curtains/ shades to decrease glare
Good contrast helpful- tables, contact paper, place mats. Black and white usually best
Avoid visual clutter- on tables, bed
Use contrasting colors to differentiate between walls and floors- contrast molding can be helpful. Flat paint provides less glare

21. Home Modifications- continued
Outline doorways with contrasting border of color- tape, paint
Use paint or tape of contrasting color on edges of steps. Top and bottom step can be marked with contrasting stripe. Use of handrail on stairs
Provide a consistent and organized environment. Keep furniture and objects in consistent places. Keep walking areas free of low objects to run into or trip over.
Keep doors and cabinets fully open or closed

22. Home Modifications- continued
Use of flashlight in dimly lit areas
Plan for and allow more time for eyes to adjust to changes in light
Go early to events
Teach child/youth to advocate for themselves and what they need to function best
Identify strengths and interests

23. Emotional Considerations for Individual and Family
Can be very difficult to cope with initial diagnosis of US- debate about when to tell a child. Child may ask questions and want to know.
Questions should be answered honestly but constructively. Emphasize strengths.
Most professionals believe that students should know future implications by high school so that student can make educational or vocational choices that will compensate for eventual dual-sensory loss
Watch for increased isolation, talking or thinking of suicide
May not be safe to drive- HUGE issue for teenagers and adults

24. Emotional Considerations for Individual and Family
Anger, depression and denial- cycle between, before acceptance. Recycle as vision or hearing loss changes
When grieving, academic focus may decrease
Peers need education- deaf friends may think that the student with US is deliberately ignoring them or acting rude and start to pull away
Can be helpful to find a older mentor with US to help support child/youth, provide positive role model
Take advantage of resources- state and national-HKNC- 2 summer programs for year olds.
Individual will need to learn self-advocacy skills

25. Conclusion
Early identification- offers more opportunity for learning adaptations/modifications
Helps with realistic vocational goals and learning independent life skills
Genetic counseling- may want-especially when considering children
Start treatment to slow down or arrest the progression of US