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

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

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USHER SYNDROME

Causes, types and implications


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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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Types of Usher Syndrome


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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.


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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.


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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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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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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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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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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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Needs contrast:

Has difficulty reading light copies Often spills when pouring liquids Unable to see stars at night


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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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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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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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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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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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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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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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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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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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But remember to emphasise…………………...

Source: Sense

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