DEFECTS OF VISION AND HEARING BERNARD W. BARRON, M.D.
One true test of our competence as physicians for the whole child is demonstrated by our ability to recognize early, evaluate properly, and start the multidisciplined wheels of help rolling for our patients with either visual or hearing problems. We also have responsibilities to the parents of such a child. After recognizing and evaluating the difficulty we must be sufficiently informed to explain the problem in terms that parents can follow-its nature and how it developed, if possible, and, most important, how the child can be helped. Parents of any handicapped child often experience feelings of guilt or shame. Attitudes of rejection or overprotection may follow these reactions. Parental cooperation will often depend upon how quickly we anticipate and efficiently handle these attitudes. To the best of our knowledge we must quickly and honestly attempt to predict the course we expect the deficiency to take. We should attempt to estimate how much it will cost to help the child and know what community resources are available for this help. The purpose of this paper is to review the clinical aspects of visual and hearing problems in children and to relate how they are handled in a typical urban pediatric practice in a fairly typical city. EYE DISABILITIES
We use our eyes not only to see, to form visual impressions, but also to express feelings and emotions. Hence any abnormality either in visual function of our eyes or in their appearance may constitute a handicap. Eye disabilities may be classified as follows: SIGHTED CHILDREN WITH EYE PROBLEMS. This group consists of children with vision 20170 or better in either eye after correction. It also includes those with eye muscle imbalance problems (strabismus or heterophoria) as well as those with color vision defects, cataracts, cosmetic defects or ptosis. PARTIALLY SEEING CHILDREN. These are children who see 20170 or less after correction, but have enough vision to use sight for learning. 18 5
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BLIND CHILDREN. These have 20/200 vision or less. The magnitude of this problem is difficult to appraise. The best estimates are as follows: One in 4 United States school children has an eye problem necessitating care (estimated 8.5 million children). One in 500 preschool and school children is classified as partially seeing (estimated 68,000 children). One in 4000 preschool children is blind (5800 children). One in 5000 school children (5600 children) is estimated to be blind. Causes of Eye Defects
It is often difficult to understand the underlying causes of eye defects. Such factors as heredity, infection and trauma may be responsible. In 1951-2 The National Society for Prevention of Blindness reported 7000 partially seeing children. Forty-nine per cent had difficulties due to refractive errors. Twenty-two per cent had difficulties due to developmental anomalies of structure. Eighteen per cent had defects of muscle function, and 11 per cent had other diseases or defects such as infection, trauma, tumor, or eye problems associated with generalized disease. Of 7000 children who were blind (3000 in the preschool group), 38 per cent had blindness due to prenatal causes, 47 per cent were blind because of retrolental fibroplasia, 4 per cent from tumors, and 5 per cent unspecified. Of the remaining 4000 (school children), 60 per cent had blindness due to prenatal causes, 9 per cent due to retrolental fibroplasia, 11 per cent to infection, 6 per cent to injuries, 5 per cent to tumors, and 9 per cent unspecified. Retrolental fibroplasia has been a main cause of blindness in the past 10 years. This should be reduced in the very near future with better knowledge of the problem and the judicious use of oxygen in premature infants. Loss of sight from infectious diseases is a decreasing problem in this country. Blindness from injury is also on the decline, owing to public education and safety legislation. Recognition of Eye Defects
Complete ophthalmologic examinations should be done on all prematures whose birth weight was less than 3Y2 pounds. The same is true of infants born to mothers who had rubella in the first trimester of pregnancy or of infants of parents with a history of familial eye disease. One should routinely attempt to evaluate the eyes of newborns, looking specifically for several things. The general facial appearance of the infant should be noted. Look especially for symmetry of the orbits and abnormalities of the lids. The lids should be separated and the conjunctiva inspected, the globe itself visualized. The sclerae are observed for hemorrhages, icterus or blue-white color. Check the reaction of the infant to the flashing of a bright light. The normal response of blinking, frowning or other withdrawal movements indicates that the infant has some vision in that eye. The red reflex should be demonstrated with the ophthalmoscope set at "0" diopter and held approximately 10 inches from the infant. The presence
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of this reflex indicates that the lens is clear. Subsequent well-baby visits offer an opportunity to follow the development of the patient's vision. Gesell notes that monocular fixation starts in a few weeks and continues to about 16 weeks. After the fifth to sixth week the infant begins to have some binocular fixation. Fusion is weak for the next three or four months, and deviations are frequent. By six months of age a moderate degree of binocular fixation has developed. Convergence and visual following movements are well developed by the third and fourth months. By the fifth to sixth month hand and eye coordination begins to appear. Keeney states that visual acuity at 4 months is 61100 to 6170, at 9 months 6170, at one year 6/60, at 2 years 6112, and at 3 years 6/9. Macular development is complete at six to seven years. Vision of 20/30 to 20/40 is probably normal for the 4- to 5-year-old group; 20/20 vision is probably not attained until 6 to 7 years. Children do not usually complain of ocular difficulties, so that one must rely upon observation and history taken from the parents. The presence of red eyes, swollen lids, crossed eyes or nystagmus indicates some difficulty. Look for aimless wanderings of the eyes in infants, unusual head positions, changes while inspecting objects, squinting at distant objects, or unusually close holding of near objects. Visual defects can delay a child's developmental activities and should be considered a possible cause of retarded motor development. In an older school-age child parents and teacher observations on position of reading matter, ability to see work on the blackboard, difficulties with reading or arithmetic, poor alignment of written work, unusual use of color in art classes, and recurring headaches at the end of the school day may all be helpful in detecting visual difficulties. Visual Screening
It is a policy in our practice to do a visual screening test on all preschool children as part of their school entrance physical examination. We use the Snellen illiterate E chart incorporated with a properly illuminated cabinet at a test distance of 20 feet (Fig. 16) (this is
Fig. 16. Snellen chart in illuminated cabinet. (Courtesy of Green Test Cabinet Co., St. Louis, Missouri.)
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the Green Test Cabinet Company model, No. 103). Children who see 20/40 or worse before entry into the first grade should be referred to an ophthalmologist. It is our policy to refer strabismus patients for evaluation as early as possible. Subsequent follow-up is done jointly with the ophthalmologist. The actual management of the ophthalmologic problem is the province of the eye specialist. Nevertheless better parent and patient acceptance of the treatment is attained if the referring physician maintains an active interest. HEARING DISABILITIES
"My child doesn't answer when I call him from another room." "Why does my baby only smile when she sees me smile first?" "My daughter sits so close to the television and turns the volume so loud. Is that just a phase she is going through?" "My seven-year-old's teacher says he just sits in class and pays no attention to her." "Johnny is four years old, and I can't understand a word he says. Is there anything wrong about that?" "Doctor, since Dorothy had measles, she can't seem to hear too well." "Peter says he can hear the teacher all right, but he can't hear the other children; they recite too quietly." These are typical complaints which are heard by pediatricians. Some hearing impairment could be behind each of them. Listening and talking are so much a part of life and learning that we take them for granted. Few of us realize how much a child who is deaf can miss. Types of Hearing Impairment
CONDUCTIVE HEARING DEFECTS. These are due to impairment in conduction of sound to the inner ear. Hereditary. Example, otosclerosis-fixation of the ossicular chainnot commonly seen in children. Congenital. Malformations that may run the range from minor defects of the outer ear to complete absence of the otic mechanism. Acquired. (a) Upper respiratory tract infections leading to otitis media with injury to the otic mechanism; (b) otitis externa with injury to the otic mechanism; (c) allergies; (d) mechanical obstruction of the ear canal, due to cerumen or foreign body; (e) trauma; (f) aerotitis with resultant pressure changes which result in conduction difficulties. PERCEPTrvE HEARING DEFECTS. These are due to disturbances in the inner ear, the auditory nerve, or hearing centers in the brain. Hereditary. The absence of brain centers, auditory nerve, or the organ of Corti. Congenital. Infections in utero during the first trimester. An example is rubella or possibly other virus infections at that time. It is possible that high temperatures in the mother have an adverse effect upon the fetal cochlear nerves or auditory cortex.
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Acquired. (a) Brain damage from kernicterus; (b) birth trauma and neonatal anoxia; (C) childhood infections affecting the auditory nerve-meningitis, measles, mumps, chickenpox, pertussis, scarlet fever and pneumonia have all been implicated; (d) toxic effects of drugs: e.g. quinine, dihydrostreptomycin, neomycin, streptomycin, kanamycin; (e) trauma; (f) neoplasm; (g) cerebrovascular accidents; (h) degenerative central nervous system diseases.
Extent of Hearing Defects
The extent of the hearing problem is difficult to determine. Five per cent of school children who received screening tests have enough loss to warrant special study. Between 1.5 and 3 per cent have enough loss to warrant special care and educational help. One tenth of 1 per cent of school children are totally deaf. Degrees of hearing loss are classified as follows: MILD TO MARGINAL. These children have a loss of 20 to 40 decibels in the better ear. They can hear and learn speech by ear in a good conversational situation, but have trouble at a distance or in a group. They benefit from preferential seating in regular school. Those children who have over 35 per cent loss benefit from amplification by hearingaid and speech reading training. MODERATE Loss. A loss of 40 to 60 decibels in the better ear. These children can learn speech by ear with amplification and visual aids. They have a limited vocabulary and frequent errors in speech. They may attend regular school with a hearing-aid, but require intensive speech training. SEVERE Loss. A loss of 60 to 75 decibels in the better ear. These children have trainable, residual hearing, but require special training to learn to communicate. In each case one must decide whether these children need special schooling or whether they may attend regular school with special help for language and communication. PROFOUND Loss. A loss greater than 75 decibels in both ears. These children have voices of poor quality and cannot learn language and speech without help. They require early special schooling. Early detection of hearing loss, especially in the moderate or severe category, is vital in order to obtain maximum benefit from training and prevent the development of serious psychological problems. Again, the pediatrician, having the opportunity to see his patients throughout infancy and childhood, becomes the logical one to discover these handicapped children as he encounters them in his practice. By taking careful histories from our new patients and following up our old ones, we can be alert to those children who already have hearing problems or may be expected to develop them. Family history of deafness, complicated pregnancy, labor, delivery or neonatal period-all should make us alert to possible hearing problems in such children. The history of repeated upper respiratory tract infections with otitis media should make us watchful for hearing loss.
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Hearing Testing
One can routinely test hearing during the newborn period with a fair degree of accuracy by eliciting the Moro reflex, first by jarring the infant, and then by a sudden loud noise. We can compare the response to both types of stimulation. If the response to sudden loud noise is absent while the jarring Moro reflex is active, we may assume that the child was unable to hear. The oral-palpebral reflex has been utilized. The infant responds to noise by blinking of the eyes. This test is not generally considered reliable, however. At four to six months the child responds to familiar known voices by turning to look. At 6 to 12 months the child with normal hearing recognizes meaningful sounds. Children from one to two years of age who can hear begin to understand words and have some vocabulary of their own. Older children should be able to follow commands given by someone who is out of their line of sight. Children of the preschool group, age four to six years, who have a mild or moderate hearing loss seem to be more attentive to movement than sound. They seem to watch lips, may show signs of strain at listening, or they may seem inattentive. Their speech may have been slow in developing. They often confuse words with similar sounds. In our office we use a crude method of testing these children. The child is told to stand facing the wall and put a finger in one ear. Simple words are spoken by the examiner at different degrees of loudness, and he is to repeat each word he hears. Simple twosyllable words such as "baseball," "hotdog," "cowboy," "football," "pancake," are used. The examiner's voice is modulated from a whisper to whatever intensity is required to make him hear. This provides fairly good testing for sounds in the spoken range. Higher frequencies are tested by having the child listen to the ticking of a watch or clicking of coins at various distances from the ear. School children are best tested by audiometric methods utilizing either speech audiometers or, preferably, pure tone audiometers. During 1955 to 1956 in our community in Louisville approximately 40,000 school children were individually tested by the Sweep Check Test. These tests were conducted by the parents, who were trained in the use of the audiometer by the school authorities. Approximately 4 per cent of the children were found to have some degree of hearing loss and were referred to their physicians. In our practice conductive hearing loss, secondary to acute otitis media, is evaluated grossly at the time of diagnosis. Appropriate therapy for the infection is instituted, and we follow them up closely with repeated evaluation of hearing in the infected ear. If hearing fails to improve with resolution of the infection or if there is a history of recurring episodes of otitis media, we refer the patient to an otolaryngologist. In cases in which hearing loss is found without significant ear, nose or throat disease, some element of perceptive or nerve damage may be present. These patients are generally referred to an audiology clinic for complete testing, including psychological tests and recommendations. Upon completion of these tests an educational program is planned for the patient. Our com-
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munity is fortunate in having a well staffed audiology clinic, a deaforal preschool, as well as special classes for the hard of hearing and deaf in our public schools. SUMMARY
One of the duties of the child's physician is to recognize, evaluate and arrange for the management of the patient with visual problems or hearing difficulties. So far as the general physician is concerned, these problems and difficulties center around recognition, counseling and referral. These should be sought routinely well before school age. The school child with an undetected visual or hearing problem is at an enormous disadvantage. REFERENCES
1. American Public Health Association Committee on Child Health: Services for Children with Hearing Impairment. New York, American Public Health Association, Inc., 1956. 2. American Public Health Association Committee on Child Health, and the National Society for the Prevention of Blindness: Services for Children with Vision and Eye Problems. New York, American Public Health Association, Inc., 1956. 3. Gesell, A. L.: The Developmental Aspect of Child Vision. J. Pediat., 35:310, 1949. 4. Green, M., and Richmond, J.: Pediatric Diagnosis. Philadelphia, W. B. Saunders Company, 1954, p. 28.
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