Visually Impaired Children with Multiple Disabilities, Part 2

Visual and intellectual disability

The severity of visual impairment varies in different types of intellectual disability.

Generally, the more severe the intellectual disability is the more severe is also visual impairment.

Differences in refractive error between children in a normal cohort (A) and in children with Down syndrome (B). Modified from a slide of JM Woodhouse.

The largest group of infants and children with a genetic cause of intellectual disability are children with Down syndrome. Visual problems of these children are well known: large refractive errors are more common than in typically developing children, accommodation is weak or lacking in hypotonic infants and visual acuity seldom develops to normal level. Since these deviations from the norm affect early development, they should be diagnosed during the first few weeks of life. Infants should get their eye glasses fitted for near vision because their visual sphere is limited and early learning occurs within their arms reach.

Insufficient accommodation is common in infants with delayed general development due to abnormal brain functions.

In most countries, there is no tradition of treating insufficient accommodation in infancy and early childhood, and treatment of strabismus is commonly postponed in children with developmental delay. This results in poor visual acuity at near in hyperopic children, and delays in communication and learning.

Here is a video that shows an infant react to being able to use her vision with proper eye glasses:

If children do not get their eye glasses early, they are accustomed to blurred images and may not accept their glasses when they start school. Compensation of poor accommodation with usual near correction (“reading lenses”) often momentarily changes infant’s behavior from sleepy and to well awake and active. Severely disabled infants may need several exposures to a sharp image on their retinas before they become aware of visual information.

A girl with Down syndrome needed to first feel the forms on the LEA puzzle. Then assisted, she moved her finger on the picture of the circle.

Children with intellectual impairment need to be trained in order to be able to participate in testing. Many children will learn to respond by pointing if trained early, consistently and initially using concrete representations of the optotypes (the LEA Puzzle by Good-Lite) and shaping the response until they are able to function with optotypes alone.

The measurement of the near

This training was necessary to help her understand that the puzzle could be used as an answering key. The more difficult forms “house” and “apple” were covered during this first vision screening situation at school.

vision acuities (single symbols and symbols in line) resulting in different values should alert the tester to test more carefully both ocular motor and processing functions. If the child cannot accommodate, visual acuity at near distance is worse than visual acuity at greater distances and needs to be improved with reading lenses. Few children with severe intellectual disability can perform in the near vision tests with tightly crowded optotypes.

Another child may perform well with a line test at near distance but cannot respond to it at the distance of three meters. Distance visual acuity at three meters needs then to be measured with single symbols instead. A response like this clearly demonstrates to the teacher that the child is unable to look at words, or other crowded information on the blackboard but may be able to perceive and recognize the same words at near.

While testing children with Down Syndrome you may see similar behavior as in this picture. The child had no difficulty in fixating a single optotype on a LEA Playing card and answered promptly.

Many syndromes cause changes in vision and a delay in general development. Changes may involve the eyes, visual pathways, cortical functions or all three. The next child, a boy, had a rare syndrome.  It often leads to death before eight months of age and therefore this boy was not referred for early intervention until 18 months, when it became obvious that he was going to survive.

He moved by rolling quite fast, noticed objects, including low contrast objects and grasped

When the same optotypes were shown on a line test at near the child hesitatingly points with his finger to the optotype he should read (= supports fixation) and then to the puzzle piece “ball”.

them. He explored the environment and reacted immediately when he heard his mother closing the zipper of her bag on the floor, rolled to the bag and opened the zipper. The mother explained that this was one of his favorite games at home.

It was obvious that he heard sounds but he did not response to words, not even his own name. His mother helped him to use the tactile sign “I want” and he seemed to grasp that it was a communicative activity combined with mother’s voice and expression. He had good visual communication with his mother and was eager to make himself

He fixated with his right myopic eye (–3.0) and had esotropia of the left eye. When looking at an adult’s face he seemed to look at the hairline.

understood. Support in the further development of his communication was arranged during an evaluation week at a rehabilitation center where also his orientation in space was observed.

He had deformed hands but grasped the gray LEA Rectangles on the gray surface, i.e. his contrast sensitivity was likely to be good.

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7 Comments

Filed under Early development of vision, Multiple disabilities, Video, Visually impaired children

7 responses to “Visually Impaired Children with Multiple Disabilities, Part 2

  1. Pingback: Tweets that mention Visually Impaired Children with Multiple Disabilities, Part 2 | Dr Lea & Children's Vision -- Topsy.com

  2. Jana

    Hi,
    We want to prepare multiple disabilities (severe and profound disabilities)children cognitive skills learning tools.
    Do you have any observations on color, size, shape and other characteristics of what we should consider?

  3. Angie Powers

    Hello Dr. Lea,

    I am so EXCITED to meet you next month when you come to Salina, KS. I’ve been reading all your stuff on the web and have learned a lot of course about CVI and all the things that go along with that condition.

    My daughter was born 3 month premature, took 8mins to intubate her, suffered from grade IV bilateral bleeds, has had 3 shunts in 3 months (the current one has been in place since Aug 2010), has cerebral palsy with motor delays and of course CVI. Dr. Linda Lawrence saw Jocelyn just recently and say that Jocelyn has sufficient accommodation and that glasses would not help her. Also, each of Jocelyn’s eyes turn inward independently at various times for short periods. Through months of work, we have achieved her being able to see some items up to 3-4ft away (items that are reflective…ie pom-poms) and other non-lighted or non-reflective several ft away. My daughter turns her head back-n-forth and up-n-down all the time…is this a common characteristic that you’ve found in CVI patients? I know you will get the chance to meet my daughter but Dr. Lawrence said that she had never seen that type of behavior and wanted to videotape her doing it. She felt as though she was trying so hard to see. From my understanding, I think she might be doing that to put her world “in motion” but I’m not sure. Anyways, just food for thought and thought I might seek other ways to help her. I so badly want to get her sitting up, crawling, etc and hoping that if I can help improve her vision, MAYBE that will increase her driving force???

    Again – so thrilled to meet you soon

    With extreme fondness,
    Angie Powers

  4. Pingback: Visually Impaired Children with Multiple Disabilities, Part 2 | Amanda's Blog

  5. Pingback: Visually Impaired Children with Multiple Disabilities part 1 2 and 3 | Amanda's Blog

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