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

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Presentation on theme: "St. Paul's Way School Deaf Support Base C.Dew 1 USHER SYNDROME Causes, types and implications."— Presentation transcript:

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

2 St. Paul's Way School Deaf Support Base C.Dew 2 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

3 St. Paul's Way School Deaf Support Base C.Dew 3 Types of Usher Syndrome

4 St. Paul's Way School Deaf Support Base C.Dew 4 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.

5 St. Paul's Way School Deaf Support Base C.Dew 5 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.

6 St. Paul's Way School Deaf Support Base C.Dew 6 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.

7 St. Paul's Way School Deaf Support Base C.Dew 7 Physical Signs (there is no way to predict timing or progression of the vision loss) Source: Centre on Deafness, Illinois & Deaf-blind Services, Illinois

8 St. Paul's Way School Deaf Support Base C.Dew 8 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.)

9 St. Paul's Way School Deaf Support Base C.Dew 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

10 St. Paul's Way School Deaf Support Base C.Dew 10 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

11 St. Paul's Way School Deaf Support Base C.Dew 11 Needs contrast: Has difficulty reading light copies Often spills when pouring liquids Unable to see stars at night

12 St. Paul's Way School Deaf Support Base C.Dew 12 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

13 St. Paul's Way School Deaf Support Base C.Dew 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

14 St. Paul's Way School Deaf Support Base C.Dew 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.

15 St. Paul's Way School Deaf Support Base C.Dew 15 Problems with visual acuity: Holds book close to eyes, or bends to read Places face close to desk while writing Sits near whiteboard

16 St. Paul's Way School Deaf Support Base C.Dew 16 Balance problems: Cannot ride a bicycle Is considered clumsy Loses balance easily in the dark

17 St. Paul's Way School Deaf Support Base C.Dew 17 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

18 St. Paul's Way School Deaf Support Base C.Dew 18 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

19 St. Paul's Way School Deaf Support Base C.Dew 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

20 St. Paul's Way School Deaf Support Base C.Dew 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

21 St. Paul's Way School Deaf Support Base C.Dew 21 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

22 St. Paul's Way School Deaf Support Base C.Dew 22 But remember to emphasise…………………... Source: Sense


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