Docstoc

USHER SYNDROME

Document Sample
USHER SYNDROME Powered By Docstoc
					USHER
SYNDROME
Causes, types and implications




1         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

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




3    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.


4              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.


5             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.

6            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



7                    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.)


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


9          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


10              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




11          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


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



13         St. Paul's Way School Deaf Support Base
                            C.Dew
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.
14         St. Paul's Way School Deaf Support Base
                            C.Dew
Problems with visual acuity:


 Holds book close to eyes, or bends to
  read
 Places face close to desk while writing

 Sits near whiteboard




15         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




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

17         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

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


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

20             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

21             St. Paul's Way School Deaf Support Base
                                C.Dew
But remember to
emphasise…………………...




                                               Source: Sense

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

				
DOCUMENT INFO