I’ve been super busy for the last few months and unfortunately had to ignore updating this blog. I was busy finishing my new book called “WHAT and HOW Does This Child See?” – a book I co-wrote with Namita Jacob. I’m excited about this book as it features a collection of my lectures over the last 35 years regarding the assessment of Visual Functioning for Development and Learning. You can check out the link on my homepage where you will be able to see the contents of the book. If you would like to order a copy, the book is being sold by Good-Lite.The book also comes with a special disc that includes the full text of the book, the examination videos mentioned in the book as well as PowerPoint lectures covering each of the chapters.
If you would like to learn more about this puzzle, please check out the link below (it takes you to 7 videos and an extensive write up.
Dual sensory impairment as a peripheral impairment
Dual sensory impairment caused by disorders in the peripheral parts of both systems, eyes and ears, is uncommon in children. The changes in the peripheral parts of the visual system are structural changes in the eyes and optic nerves and in the auditory system structural changes in the outer, middle and/or inner ear. There are two different groups among these children based on their communication and educational needs:
1) Children with congenital infections (rubella, toxoplasmosis, syphilis, CMV, herpes meningitis, encephalitis), rare syndromes or an unknown cause that has affected development of both eyes and ears, is one group. This group of children has a great variation in their communication techniques. Many depend on tactile sign language, many have limited language skills but there are also children with normal spoken language and good, although stressful communication using hearing aids and trying to decipher visual communication. Severity of brain damage varies and is difficult to assess during the first and second year because we cannot be sure about the degree and type of deprivation.
2) Children who have hearing impairment from early on and develop visual symptoms later. Usher syndrome is the most common cause of this type of combined loss of vision and hearing. In communication these children use hearing aids, speech, visual sign language, speech reading and body language. Vision loss is gradual and visual communication usually remains fairly good through school age. Children who had hearing in preschool age and learned to speak can now get a cochlear implant in many countries when their progressing hearing loss is no longer helped with hearing aids.
Deaf infants are receiving cochlear implants very early.
If both vision and hearing are impaired from birth, the child and his parents need intensive training in interaction, communication and exploration. The communication techniques of this group of children vary so much that the early
intervention teams and teachers need to have good information from the medical experts, psychologists and optometrist who in turn need to know this special population.
with coloboma and hearing impairment
If a child with hearing impairment has large colobomas in both eyes like the infant in the video below, it is often difficult for them to get adequate visual information from the lips of people who are speaking. The central retina is often pulled upward from its normal location. Therefore, when the infant is looking at the adult person’s face, he seems to be looking at or above the hairline. This is naturally disconcerting to those interacting with the child, as they may feel that he is not looking at them and this can pose a further challenge to the development of communication unless it is explained clearly.
The next photo shows how the therapist gently guides the infant to study his legs and feet with his hands. Note that the infant also looks in the direction of his feet when touching them. At a moment that the therapist felt good for testing, a high contrast toy (seen in the next photo) was slowly moved in front of the infant who followed it with combined head and eye movements 180 degrees.
The responses had three observers: the therapist, her consulting experienced therapist (the NDT therapist behind the camera) and the ophthalmologist (giving the test object, a high contrast ring).
Video recording of test situations like this gives useful material for follow-up of the development. This infant with large colobomas had been assessed as totally blind the day before. The parents of infants with syndromes that cause loss of vision and large anatomic changes in the eyes, nearly always hear “this infant is not going to see.”
Toddlers and children with moderate to severe impairment of vision and poor hearing because of rare syndromes may have intellectual disabilities but may as well have normal intelligence and may develop compensatory functions so that they often seem to see better than they really do. They use cues that they pick in the environment to remember their routes, keep their devices and toys well arranged to find them with ease. Therefore they may be required to function at a much higher visual level than where they actually are.
In school, apart from the challenges of keeping up in the classroom, it is important to recognize that the break time can be very stressful for children with vision and hearing impairment. The noise levels increase and the movements of children are greater, faster and more unpredictable than at any other time of the day. Provisions should be made so that these children have the opportunity to have access to a quieter space for at least some parts of the day to enable them to recharge their energies.
Toddlers and young children, who do not start to talk, are taught using various communication strategies. Sign language is used in many countries to support delayed development of speech, or as an alternative to speech, but is not accepted in all countries or some communities of a country as a technique of expressive language. In these places, hearing-impaired children, for example, are asked to learn speech reading although it has not been examined, whether the children can perceive lip movements. This should be tested in all children before starting to train speech reading. Picture communication may also be started before perception and recognition of pictures has been assessed. Thorough assessment of visual functions should, actually MUST be a prerequisite for choosing communication techniques.
Children with Usher’s Syndrome are a special group among the deaf-blind children.
Most of them are born with a severe hearing defect but some have useful hearing until school age (Usher syndrome type III) and then develop progressive sensory-neural hearing defects. Visual impairment is caused by congenital retinal degeneration that starts to cause symptoms before or in school age. The first symptom is often “night blindness” (actually not blindness but slow adaptation to low luminance levels), which can be diagnosed during the first year by observing the infant in daylight and twilight.
Changes in the visual field start as small islands of decreased sensitivity, slowly become confluent and form a ring scotoma, a ring-shaped poorly functioning area around the central visual field. Peripheral vision disappears very slowly and a limited central field of vision usually remains until adulthood or even past 60 years. Photophobia and continuous flickering in the visual image bother some of these children. A young child seldom notices his visual problems before they are explained to him because he does not know how other people see. Vision development in all deaf and hard of hearing children should be followed for possible changes as a part of school health.
The confirmation of the diagnosis of Usher syndrome can be made in eye hospitals with electroretinography by measuring the responses of the retina to different stimuli.
Functional diagnosis of a delay in the adaptation time of the cone cells is possible early with the CONE Adaptation test (Good-Lite) that measures the time needed to start seeing in twilight. The measurement takes less than twenty seconds, and the instructions to the child, just a few minutes. It is important to be aware of the delay in cone adaptation so that the child gets a good torch/flashlight when sent to get something from a dark place and extra lamps in the closets. Extra lamps outside the house and strategies for moving in twilight using a torch, later with a cane, help these children in developing a full participation and independent moving.
The assessment of progressive visual defects requires ability to communicate with deaf children. To improve the assessment situations, all deaf children should learn the basic vocabulary related to vision and vision examination but also the testers should learn at least rudimentary communication using signs during assessments. Deaf children with normal vision also need this information to understand their deaf-blind peers.
Central auditory and visual processing disorders
A combined processing disorder is rare but should be remembered when assessing children with brain damage. A brain damage can affect processing of both visual and auditory information. Functions of eyes and ears and the pathways may be normal but the processing of information has defects. Dual central processing disorders are usually a part of an extensive brain damage but can also occur in otherwise normally developing children with mild or no motor problems.
If an infant has learned to move at the appropriate age, finds his way in the house and on the yard and seems to hear even the weakest sounds, most people do not notice that the child does not have any interest in toys, except knocking them, does not recognize family members or facial expressions, has never copied gestures and does not speak a word. If the parents express worries to doctors, the diagnosis of “autism” is regularly given because it is so much more common than dual loss of processing functions. There are few places were correct diagnoses can be given during the first or second year, and thus, sensory deprivation and lack of communication may make the child frustrated and angry.
The event is free but you can RSVP online or by calling (316) 440-1514.
Reservations are requested by March 4, 2011.
When: Thursday, March 10, 2011 6:30 PM – 8:00 PM
Where: Envision, INTRUST Bank Community Services Hall, 610 N. Main, Wichita, KS 67203
To find the online RSVP link, click here.
From Dr. Lea’s daugher Liisa:
You can now find Dr. Lea Hyvarinen and Lea-Test also on Facebook. Just follow this link. Hope you will click on “like” once you get to that page and tell others interested in children’s vision development about this additional way to connect with Dr. Lea.
Multiple problems are more common in children with congenital visual impairment than in children who become visually impaired later in life. In different parts of the world 60–80% of vision impaired children have at least one other impairment or chronic illness. Some have more than four impairments, each causing disability. In most countries, visual impairment of multi-disabled children is not registered because only the “primary” impairment can be registered and that is usually intellectual, motor or auditory disability because they are diagnosed early. It is important to be aware of the variations of visual development in the groups of children with several impairments so that visual disability is not interpreted as a behavioral problem or an intellectual disability.
Better health care across the world has ensured greater survival rates for the premature and very low birth weight infants, children with syndromes, and those who have severe damage in accidents. As a result we see an increase in the number of children with visual impairment and additional disabilities, but neither medical nor rehabilitation services have received enough resources for intervention to enable better quality of life for all visually impaired children.
Children with multiple disabilities often have poor assessment and intervention because many practitioners find it hard to imagine the potential for development when they see the combination of several impairments. However, often infants, who did not seem to have almost any potential for development during the first few months, surprise specialists with their skills in many areas.
When assessing a child for the first time it is wise to ask the child’s therapist to start the testing so that the child gets an opportunity to hear the new voice without anything painful happening (as it was usual at the hospital). Infant’s own therapist can perform the first test situations, where she brings an illuminated ball (thin plastic ball on a penlight) from the side toward the mid-line to test the size of the visual or attention field. This infant responded only in the mid-line.
Here is the video from the testing situation:
Note in the video how the therapist is asked to bring the illuminated ball below the hands of the infant, first the better functioning hand then the hemiplegic hand, so that fingers on the ball create a grating pattern, an effective
visual stimulus. When the infant is getting accustomed to the new voice the doctor can go on with the assessment.
Since no accommodation could be measured, near correction was tried and the girl had a brief eye contact with the tester.
We learned more about the girl’s vision and capacity when her twin brother was examined. The girl showed very clearly that she was accustomed to be the center of attention and she did not like a situation, where the new person was playing with her brother. It was obvious that she had to be able to see and hear more than what the tests had shown; she had opinions and she could express her opinions. What else should a 5-month old infant be able to communicate?
The early activation and continuous support of visual development is especially important in all young infants with hypotonia or hemiplegic conditions. Infants with motor problems can develop their ocular motor functions better if they are included in physiotherapy.
Here is the video showing the child’s progress:
Ten weeks after the physiotherapy was started, this infant had learned to use her hemiplegic side quite well, played with both hands in the midline and had good visual communication with her therapist. Note that during this therapy session she turned from her back to her stomach for the first time when trying to get her favorite toy. Visual information was used to entice the infant to try a new motor function.
When her right eye started to become a lazy eye training was included in her physiotherapy. During training of the less functioning right arm, the baby held a toy and moved it in front of her right eye. The left eye was patched.
During therapies and the visits of an early intervention teacher/worker the use of tactile and kinaesthetic information should be observed to enrich information during interaction. The little hands should be given opportunities to explore faces. Father’s face is exciting to explore: in the morning before the father has shaved, his face can be studied in detail. Then when he has shaved one half of the face, the difference between the two halves of the face is investigated; the comparison is repeated when the other half of the face is shaved. This gives the infant much to ponder during the day until the father comes home, again a bit different. Mothers’ face never changes.
Many tests cannot be used exactly as designed. Some children cannot speak and will point with their eyes, head, hand or foot. Some children will need support for their bodies or head, help in bringing their hands forward or keeping their bodies relaxed in order to use their visual system. Other children will be able to give visual responses only when they are given adequate time and no disturbance of the other senses – no noise or touch. These needs vary from child to child and are learned through observation and conversations with those who work closely with the child. Changes in medication often affect children’s wakefulness and therefore parents should inform orthoptists and ophthalmologists about changes in child’s functioning.
Use of eye glasses
When near correction is found helpful, the use of eye glasses is started in communication situations and during therapy. If the side parts of the glasses are thin and don’t bother the infant during head movements, glasses can be used during the whole day. Since strabismus often develops in infants with brain damage, therapists can keep an eye on the infant and inform the ophthalmologist at the first signs of squinting (strabismus). Training of the less used eye can be included in the infant’s therapy.
When planning spectacle correction, it should enable the use of the best visual image at a distance relevant to the child. Spectacles known as reading correction for a very close distance is useful for tiny infants; an unusually long distance from the eye to the foot is necessary later for students who use their feet to type. We should consider also the typical position of the child’s head and use supports to maintain these positions when selecting spectacle frames. In many medical services children with intellectual disabilities do not get spectacles. Spectacle corrections are especially important for these children who have so few options for compensating the lack of spectacles. Finding the optimal spectacle correction is often challenging and requires thinking out of the box.
Spectacle corrections, when given, may not be accepted. There are several reasons. If a high minus correction is given as full correction, the dramatic change in the size of objects and distances may be too much to be tolerated. Hyperopic correction (= plus glasses) may be 1-2 dioptres over-corrected, sometimes more if the child’s visual sphere (cognitive sphere), within which the child can use vision is limited. It is then important to notice that when the child starts to pull the plus glasses away this can be due to an increase in the cognitive space and thus bifocal or monovision glasses may be needed (monovision = one lens is fitted for close distances and the other for distances up to a meter). The introduction of a spectacle correction may be easier if the infant has used empty spectacle frames first. Problems with glasses are individual; some children accept full correction of astigmatic error and spherical correction, some children do not.
Some infants with intellectual disability and myopia learn to use the “pin hole” effect to see clearly. They turn their head and look through the small hole created by the edge of the pupil and the corner of the eyelid. A small hole (pinhole) effectively compensates for refractive errors and gives a clear enough image for orientation. The child sees where to go and once close enough to the object can see it quite well because of myopia, nearsightedness. In cases like this, glasses may be acceptable only outside. This phenomenon is quite common in children with Cornelia de Lange Syndrome. If you have difficulty in believing this paragraph, make a small hole in dark coloured paper, take your glasses off if you need them to see at close distances or take “wrong” eye glasses that blur your vision and look through them and the small hole, keeping it close to your leading eye. You are likely to see enough to read, not as well as with your spectacles, but well enough to see many important details.
In the correction of refractive errors the functional visual sphere should be kept in mind and the spectacle correction fitted to function within that sphere. Distance glasses become useful only when the cognitive sphere of the child increases to include objects at greater distances.
If a child does not accept spectacles, all demanding tasks must be kept at the distance where the child sees best, and within the dimension of the uncorrected visual acuity for that distance. Families and caretakers should get information so that they are aware of this distance. In developing countries there are too many infants and young children with severe physical disabilities fitted with big and thick spectacle lenses that then press against the child’s cornea as these children spend most of the day lying on their side or back.
Visual stimulation as passive stimulation is appropriate when an infant/child is barely aware of vision. Once the response is obtained, the goal of intervention is to develop the child’s interest in actively using vision and a confidence in the vision as a source of information about the world. This is not learned through watching blinking lights in a dark room. It is learned by enabling the use of vision through the day in a variety of situations and for a variety of reasons – to better understand communication, to learn about toys, to understand information from other senses, to explore spaces and observe the effect of her actions on the world.