USHER SYNDROME

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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