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Hearing and Vision Loss Associated with Down Syndrome

by Kate Moss, Family Support Coordinator, TSBVI Deafblind Outreach

Each year approximately 3,000 - 5,000 children are born with Down syndrome. (Pueschel) Most of us are familiar with this syndrome, but may not be aware that a large number of these children have vision and/or hearing loss. I certainly wasn't until I began to notice the number of children reported on the deafblind census with an etiology of Down syndrome. It occurred to me that we should know more about the vision and hearing issues these children face and how these impairments impact their educational programming.

Physical characteristics associated with Down syndrome

General physical characteristics

Down syndrome is related to a specific type of a chromosome abnormality. There are three main types of these abnormalities in the syndrome: trisomy 21, about 95% of children with Down syndrome have an extra 21 chromosome; translocation, where the extra 21 chromosome is attached on to another chromosome; and mosiacism, where some cells have 47 chromosomes and others have 46 chromosomes. Many theories exist about the cause of the chromosome abnormality, but currently no one knows why it occurs.

Smaller stature along with slower development physically and mentally are typical of the child with Down syndrome. Although some children with Down syndrome are not mentally retarded, most of these children function in the mild to moderate range of mental retardation. Some children may be severely mentally retarded. It is important for parents to remember that whatever the child's cognitive ability he has the option of being educated in a variety of instructional settings.

Children with Down syndrome have distinct physical characteristics which are important primarily in helping physicians to make a clinical diagnosis. Not all children will exhibit all of the characteristics associated with the syndrome. It is important to be aware of specific medical issues that may exist for the child with Down syndrome so that appropriate health management can be planned. Forty to 45% of the children with Down syndrome have congenital heart disease. Intestinal abnormalities, thyroid dysfunctions and skeletal problems occur at a higher frequency in Down Syndrome. Many of the children fail to thrive in infancy, and on the other hand, obesity is often noted during adolescence and early adulthood. Other concerns for individuals with Down syndrome include immunologic concerns, leukemia, Alzheimer disease, seizure disorders, sleep apnea and skin disorders. (Pueschel) Even though a child has significant health issues, medical interventions available today mean these conditions can be very successfully treated.

Of most concern to us at Texas Deafblind Outreach is the high percentages of children who have vision and hearing impairments. Though these losses may appear mild they need to be considered when making programming decisions. When a child has both vision and hearing loss, modifications to programming becomes a critical issue if the child is going to be successful in the school setting.

Hearing loss

"Sixty to 80% of children with Down syndrome have hearing deficits." (Pueschel) Individuals with Down syndrome may have sensorineural loss, conductive loss related to otitis media, or both. Small ear canals are associated with this syndrome. (Cohen) This means that examinations are more difficult for these children and may have implications when fitting earmolds. It has been suggested by some audiologists that the child with Down syndrome produces an excessive amount of earwax. Sinusitis also is frequently a problem.

In his article "Health Care Guidelines for Individuals with Down Syndrome" Dr. William Cohen makes recommendations regarding medical management. Since loss of hearing, even minor loss, can greatly impact language development and learning, thorough and ongoing evaluation is critical.

The chart which follows shows his recommendations related to evaluating hearing function
with children diagnosed as having Down Syndrome.
Age Recommended hearing evaluations
birth to 2 months Refer for auditory brainstem response (ABR) test or other objective assessment of hearing to assess hearing by 6 months of age, if not performed at birth.
2 to 12 months Provide brainstem response test (ABR) or other objective assessment of hearing by 6 months of age if not performed previously or if previous results are suspicious. Evaluation by ENT specialist for recurrent otitis media.
1 to 12 years Yearly auditory testing for children 1-3 years old and every two years for children 3-13 years old.
12 to 18 years Hearing evaluations every other year.
over 18 years Continue auditory testing every two years.

Additionally, parents and school nurses will want to monitor for ongoing problems with fluid in the middle ear. Generally parents can tell if a child has fluid in his middle ear and if it is infected because the child will cry, act fussy, or tug at his ears. He may also have other symptoms such as fever, diarrhea, or a runny nose. If the fluid is not infected, it may be harder to tell. (Texas Department of Health) However, if your child seems to be ignoring you more than normal or saying "huh" a lot or giving any indication that he or she might not be hearing very well, get it checked out. Don't accept the excuse that he is just being stubborn and not responding. He may not be hearing you.

Children with Down syndrome may also have sensorineural hearing loss. They may have processing difficulties that affect their perception of words and their auditory short-term memory usually has limited capacity for storing and processing units of information they hear, for example, words and their meanings, instructions and numbers. (Buckley & Bird, 1994) Good assessment is very important in both of these areas as well, especially when planning instructional intervention.

Vision loss

Children with Down syndrome may also have vision impairments that may include such conditions as congenital cataracts, crossed-eyes or strabismus, nearsightedness, farsightedness, inflammation of the eyelids (blepharitis), and conjunctivitis. These conditions can be very successfully addressed both medically and from the standpoint of educational modifications and low vision adaptations.

Dr. Cohen recommendations for vision evaluations is shown on page 35. Once again the school nurse and the parents will want to keep an eye on conditions that might occur with the child such as conjunctivitis (pink eye) and see that the child receives appropriate treatment should that condition occur.

Dr. Cohen's Chart
Age Recommendations for vision evaluations
birth to 2 months Refer to vision/ophthalmological evaluation by six to twelve months of age for screening purposes.
2 to 12 months Pediatric ophthalmology evaluation by six to twelve months of age (earlier if nystagmus, strabismus or indications of poor vision are present).
1 to 12 years Continue regular eye exams every two years if normal, or more frequently as indicated.
12 to 18 years Vision evaluations every other year.
over 18 years Ophthalmologic evaluation every two years (looking especially for keratoconus and cataracts).

Addressing hearing and vision related issues in an educational setting

The vision and hearing loss which frequently occurs, especially when combined with other issues associated with this syndrome, can mean significant problems for these children in any educational setting. Buckley and Bird offer some specific suggestions for addressing both vision and hearing loss in educational settings in their book Meeting the Educational Needs of Children with Down Syndrome. Information from their book can be found on Downsnet at <www.downsnet.org>, and parents and professionals working with the child who has Down syndrome may want to review this literature.

Speaking and listening

"Children with Down syndrome have a range of specific learning difficulties that, combined together, make learning to speak in sentences very hard to achieve. Although their expressive language difficulties are greater than their comprehension difficulties, they do have delays in developing comprehension that may be made worse by hearing loss." (Buckley & Bird 1994) A child with a conductive loss brought about by otitis media may be able to hear the teacher some days and other days will not. It just depends on whether or not there is fluid in the middle ear. We have all experienced this phenomenon from time to time when we have had a bad head cold.

If the otitis media persists and the fluid thickens the child may have a constant mild hearing loss. This can effect the child's ability to perceive the differences between some consonant sounds. This means that if I said, for example, "bat the ball" the child might think I said "pat the doll". In other words, the child will have to figure out what I said based on the context of our conversation. He may also miss information that would help him to understand plural endings (plate/plates) or verb endings related to tense (stop/stopped). Single, short instructions may be easy for the child to figure out, but longer sentences may prove to be confusing. Language development can be impacted negatively.

Of course, the child may also have a sensorineural hearing loss alone or in combination with the conductive hearing loss. Evaluation to determine if there would be a benefit to using hearing aids is important, as well as the need for services of a speech/language therapist. Many children with Down syndrome benefit from using sign language (even if they are not hearing impaired). It is important to understand the impact of the child's hearing loss in accessing information in the classroom. Mild hearing losses, even a 15 decibel loss, have been shown to affect children's progress in school. (Buckley & Bird, 1994) Some strategies discussed by Buckley and Bird that should be employed in classrooms to help children with mild hearing loss include:

Children with Down syndrome may also have processing difficulties that affect their perception of words, even if they do not have a hearing loss. They typically have auditory short-term memory deficits that impact their ability to remember what they hear. This can effect all areas of instruction including language development, reading, math, etc. "Language supported by symbolic movements, such as sign, gesture or fingerspelling, and by visual methods, such as pictures, symbols, words, and sentences (multisensory methods) will help the children to remember information." (Buckley and Bird, 1994)

Of course, consideration should be given to any visual deficits the child might have; so it would be beneficial to discuss these multisensory methods with a teacher of the vision impaired. Information supplied by the functional vision evaluation and the learning media assessment can guide the selection of appropriate instructional materials and classroom adaptations.

Buckley and Bird recommend completing a digit span test to provide a rough guide for the teacher about the units of information the child can process. This will help her determine strategies for adapting her instruction. A speech/language pathologist should be consulted regarding this assessment.

Vision concerns

The types of vision issues for the child with Down syndrome may vary and must be addressed on an individual basis with support from a teacher of the visually impaired. The conditions that are associated with Down Syndrome include: nystagmus, strabismus, farsightedness, nearsightedness, keratoconus, congenital cataracts, and conditions like blepharitis and conjuctivitis. The information which follows is taken from Low Vision: a Resource Guide with Adaptations for Students with Visual Impairments written by Nancy Levack and published by Texas School for the Blind and Visually Impaired.

from Low Vision: a Resource Guide with Adaptations for Students with Visual Impairments by Nancy Levack
Eye Condition Adaptations Educational Considerations
Cataracts: Opacity or cloudiness of the lens which restricts the passage of light, usually bilateral. Immature or incipient cataracts are only slightly opaque; while mature cataracts are so opaque that the fundus cannot be seen and the pupil may be white. Magnification, enlargement or bringing materials closer to the eyes. Eccentric viewing may be helpful. Lighting should come from behind the student and glare should be avoided. If cataracts are centrally located, near vision will be affected and bright light may be a major problem. Low level of illumination may be preferred. If cataracts are in the peripheral area, bright light may be needed to close the pupil and allow the iris to cover most of the cataract area. Teacher should not stand in front of the window or the light source when teaching or speaking to the student. Lights with rheostats and adjustable arms are helpful for close work. If contact lenses or glasses are prescribed, they should be worn. Time may be needed for adjustment to different lighting situations. Rest periods may be needed when doing close work and variation of near and distant tasks can prevent tiring.
Conjuctivitis (membrane that lines the eyelid): An inflammation of the conjunctiva, most common eye disease of the Western Hemisphere, causing red, painful, irritated eyes, tearing and discharge. Lighting should come from behind the student and glare should be avoided. Rest periods may be needed when doing close work. Time may be needed to adjust to different lighting situations.
Farsightedness (Hyperopia: A refractive error in which the focal point for light rays is behind the retina; shortness of the eyeball. If not corrected, close work may cause nausea, headache, dizziness and eye rubbing. Corrective lenses, magnifiers. Students may tire easily when reading and doing close work. Variation in near and far tasks can pre-vent tiring. Students may prefer physical education activities and activities that require distance vision.
Keratoconus: The cornea becomes cone shaped. Can be found with retinitis pig-mentosa, Down's syndrome, Marfan's syndrome, and aniridia. Seems to be con-genital and bilateral. Usually has onset in young adulthood. Contact lenses are used to retard the bulging of the cornea in the early stages. Good contrast and lighting; avoid glare. Avoid activities that could cause corneal damage such as contact sports and swimming in heavily chlorinated water.
Nearsightedness (Myopia): A refractive error where the image of a distant object is formed in front of the retina and cannot be seen distinctly; elongation of the eyeball. Corrective lenses; high illumination with minimal glare, contact lenses. With degenerative myopia, students may need to move closer to see the blackboard and classroom demonstra-tions. Students who have progressive myopia should observe precautions for retinal detachment. Students may not be interested in activities that require distance vision especially physical education activities.
Nystagmus: Involuntary eye movements which can be horizontal, vertical, circular or mixed. Can be elicited when someone watches certain kind of moving objects. Shifting gaze or head tilting may help to find the "null point" which slows the nystagmus. Stress and spinning or other rhythmic movements in-crease nystagmus, and should be avoided when visual functioning needs to be maximized. Close work causes fatigue and visual tasks should be varied to provide rest for the eyes. Line markers, rulers, typo-scopes and other templates may be helpful to keep the place on the page. Good lighting and contrast are helpful.

Conclusion

Children with Down syndrome have some unique challenges in the educational environment, especially if they have vision and hearing loss. It is important that parents and educators work together to get accurate information about hearing and vision function. This information should be reassessed periodically and there should be regular monitoring of their health, vision, and hearing. When the child has both hearing and vision loss, even in mild forms, the teacher of the visually impaired and the teacher of the deaf and hearing impaired should lend their expertise to any considerations by the IEP team. Appropriate classroom modifications and adaptations to instruction should be made to meet the child's needs in being able to access information in his or her educational environment. A child with Down syndrome typically has great potential, but he must have the appropriate supports to be successful in a school placement.

Resources and references

Buckley, S. & Bird, G. 1994 (2nd ed.). Meeting the Educational Needs of Children with Down Syndrome. Available on-line only at <www.downsnet.org> from The Down Syndrome Educational Trust, England.

Cohen, William I. 1996. Health care guidelines for individuals with Down syndrome. Down Syndrome Quarterly, Volume 1, Number 2, June, 1996.

Levack, N. 1991. Low Vision: a Resource Guide with Adaptations for Students with Visual Impairments. Texas School for the Blind and Visually Impaired, Austin, TX.

Pueschel, Siegfried M., March 1992. I. Published on The Arc website <www.thearc.org>.

Texas Department of Health, March 1994. Ear infections: what you need to know to help your child. Texas Department of Health Publication Number 1-86, Austin, TX.

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