1. Causes, types and implications
USHER SYNDROME
Causes, types and implications
St. Paul's Way School Deaf Support Base C.Dew
Carolyn Dew

2. St. Paul's Way School Deaf Support Base C.Dew
What is Usher Syndrome?
A syndrome which affects both hearing and vision
It is an inherited condition; often parents are unaware that they both carry this faulty gene
It affects 3 – 6% of the deaf and partially hearing population in the UK
At present there is no treatment for this condition
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3. Types of Usher Syndrome
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4. St. Paul's Way School Deaf Support Base C.Dew
Type 1 (USH1)
Marked by profound deafness at birth and severe balance problems. Many will not benefit from hearing aids and so most will use sign language to communicate. These children begin to develop vision problems by the time they are 10 and usually start with difficulty seeing at night; this progresses rapidly until blindness eventually occurs.
St. Paul's Way School Deaf Support Base C.Dew

5. St. Paul's Way School Deaf Support Base C.Dew
Type 2 (USH2)
Marked by moderate to severe hearing loss at birth but no balance problems. Most of these children benefit from hearing aids and will use speech to communicate. The visual problems tend to progress more slowly than in USH1 and are characterised by blind spots that begin to appear during the teenage years. Gradually, the vision deteriorates to blindness.
St. Paul's Way School Deaf Support Base C.Dew

6. St. Paul's Way School Deaf Support Base C.Dew
Type 3 (USH3)
These children have normal hearing and near normal balance. They usually develop hearing problems during their teenage years and become deaf by mid to late adulthood. Night blindness usually starts during puberty, blind spots appear in early adulthood and the individual is usually blind by mid adulthood.
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7. St. Paul's Way School Deaf Support Base C.Dew
Physical Signs
(there is no way to predict timing or progression of the vision loss)
Source: Centre on Deafness, Illinois & Deaf-blind Services, Illinois
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8. St. Paul's Way School Deaf Support Base C.Dew
Night Blindness:
Difficulties seeing when coming in from bright sunlight
Trips over things when light changes or light is dim
Stays near a light in a dark room or at night
Moves a speaker so light falls on face
May want to enter a room before it is darkened (cinema etc.)
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9. Night Blindness (cont):
Avoids conversations in a darkened area
Whilst walking on the pavement, may appear to stagger or lose balance after an oncoming car has passed
Has problems reading under some lights or in dimly lit areas
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10. St. Paul's Way School Deaf Support Base C.Dew
Glare sensitive:
Squints and shades eyes in bright lights or fluorescent lighting - complains that the light hurts his/her eyes
Likes to wear sunglasses even in buildings, but especially in bright sunlight
Avoids participating in outdoor sports when the sun is very bright
May appear dazzled when leaving a building and going outside
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11. St. Paul's Way School Deaf Support Base C.Dew
Needs contrast:
Has difficulty reading light copies
Often spills when pouring liquids
Unable to see stars at night
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12. St. Paul's Way School Deaf Support Base C.Dew
Restricted field:
Startles easily
Seems to hold eyes in a different direction when looking at some things
Turns head while reading across a page
Uses finger to mark place while reading
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13. Restricted field (cont):
Unable to find small objects that have been dropped
Does not respond to verbal or non-verbal communication from the left or right
Bumps into people, tables, and chairs
Stumbles on stairs and curbs
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14. Restricted field (cont):
Is quiet in a large group or may edge to one side when placed in a group
Frequently fails to understand or misses group instruction. Often relies on friends for information
May appear to ignore others standing by his/her side
Prefers conversation at distances of 4 to 6 feet.
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15. Problems with visual acuity:
Holds book close to eyes, or bends to read
Places face close to desk while writing
Sits near whiteboard
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16. St. Paul's Way School Deaf Support Base C.Dew
Balance problems:
Cannot ride a bicycle
Is considered clumsy
Loses balance easily in the dark
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Other:
Frequently last in completing group activities
May appear anxious in new surroundings
Often last to enter the room
May have some repetitive behaviour- appears to do the same things in the same ways
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18. St. Paul's Way School Deaf Support Base C.Dew
Other (cont):
May not participate in group activities
Frequently hesitates at the top or bottom of stairs
Avoids walking or running in unfamiliar areas
Appears to be constantly visually scanning a group
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19. Classroom Adaptations 1:
Lighting should be adequate without glare
Close curtains to minimise glare
Windows should be behind students
The teacher should never be in front of windows
Avoid clutter on the walls and floor
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20. Classroom Adaptations 2:
Wall colour behind the teacher needs to be a solid colour
Floors and carpets should not be dark red or brown
Furniture should be arranged to provide easy movement in open space
Seat students where they are comfortable -possibly front side so they can see the whiteboard and other students in the class
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21. St. Paul's Way School Deaf Support Base C.Dew
Materials:
Print should be maximum contrast. Use 12 to 18 point type with non-glare (yellow) paper.
Students may need individual copies of graphs or charts, or they may need time to examine these charts close-up.
Tests may have to be adapted for individual use and extra time allowed
Keep the whiteboard clean and use high contrast colours
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22. But remember to emphasise…………………...
Source: Sense
St. Paul's Way School Deaf Support Base C.Dew