Transcript USHER SYNDROME - St Paul's Way Trust School

USHER
SYNDROME
Causes, types and implications
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What is Usher Syndrome?
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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:
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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:
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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 nonverbal 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 behaviourappears 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:
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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:
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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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