Effective strategies for home and school for the student with usher syndrome
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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. Usher Syndrome Overview.

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Effective strategies for home and school for the student with usher syndrome

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

Usher syndrome overview

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)

Retinitis pigmentosa rp

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

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)

Rp continued1

RP- continued

  • 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.


  • 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

Usher syndrome type l

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

Usher syndrome type ll

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

Usher syndrome type lll

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

Behavioral symptoms for parents and teachers us l

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

Behavioral picture continued

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

Behavioral picture continued1

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

Behavioral picture continued2

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.

School behaviors and considerations

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.

School behaviors and considerations continued

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

Educational needs

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

Educational needs continued

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

Classroom modifications

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.

Classroom modifications continued

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.

Classroom modifications continued1

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.

Home modifications

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

Home modifications continued

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

Home modifications continued1

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

Emotional considerations for individual and family

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

Emotional considerations for individual and family1

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 16-22 year olds.

  • Individual will need to learn self-advocacy skills



  • 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

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