DANIEL WONG, MD; CHANDRAKANT P. SHAH, MD, FRCP[C]

In the past, deafness was commonly referred to as deaf-mutism; for example, Aristotle and Hippocrates From the department of pediatrics, the Hospital for Sick Children, Toronto and the departments of pediatrics, preventive medicine and biostatistics, and health administration, University of Toronto Reprint requests to: Dr. C.P. Shah, Department of preventive medicine and biostatistics, Faculty of medicine, University of Toronto, 402 McMurrich Bldg., Toronto, Ont. M55 1A8

The cover photograph of 6-monthold Matthew with his mother was suppiled by Dr. C.P. Shah; the photography was by Mr. W. Bryson, department of visual education, the Hospital for Sick Children, Toronto.

foundly deaf infants are born each year in the United States.11 The incidence in Australia is 2.14 cases . of deafness per 1000 live which is close to the minimum world incidence (2 per 1000) suggested by the World Health Organization in 1966.14 In California the incidence of severe hearing loss is 2.5 per 1000 live births,13 and in Canada the incidence of ¶iny degree of congenital deafness is 1 in 1500.16

Etiology

The known causes of deafness in early childhood include genetic, prenatal and perinatal factors, infection, treatment with ototoxic drugs and trauma in early childhood. The proportion of cases due to unknown causes is smaller if relevant family, clinical and laboratory records are available. Budden and associates,1' who reviewed studies of the causes of deafness in childhood, found that the cause was known in 60% to 70% of these cases and in about 50% of their own cases in British Columbia in an 18-year period. Similarly, the cause has been identified in 55% of the cases in the hearing-assessment program at our hospital. When only one child in a family is congenitally deaf the commonest cause is recessive inheritance, either autosomal or X-linked. The next commonest cause appears to be dominant deafness due to a fresh mutation; all of these cases may represent true fresh mutations, however, and with reduced expressivity or incomplete penetrance of the dominant trait impaired hearing may not be evident in other carrier members of the family. An unknown but probably small proportion of children owe their deafness to congenital malformation of the middle or inner ear or both. Genetic factors

Congenital deafness in the absence of other prenatal or perinatal causes, with the exception of a family history of early severe deafness of unknown cause, accounts for one-fifth to one half of the cases of profound deafness in children. Heritability is said to be recessive in 75% to 88% of such cases;17'18

the ability to hear low-frequency tones is retained. Dominant inheritance, which accounts for about 12% to 25% of the cases of genetic deafness, is often complicated by decreased penetrance; for example, Waardenburg's syndrome, a dominant trait that accounts for 2.5% of childhood deafness,19 has a penetrance of only 20% *20 In this syndrome the audiogram and pathological findings are similar to those in recessively inherited deafness,18'21'22 but in cases of dominantly inherited deafness the audiogram is flat. X-linked inheritance is responsible for only about 1 % to 3% of the cases of genetically produced hearing impairment;17'23 some retention of hearing at all frequencies is common. Hereditary nerve deafness is usually bilateral. If unilateral, it is inherited as an autosomal dominant trait that is not always penetrantY427 There are at least 16 types of hereditary hearing loss in which nerve deafness is the sole abnormality, but more than a third of the cases of profound childhood deafness are associated with other anomalies..'28 Prenatal factors Viral infection: Rubella embryopathy is by far the commonest prenatal cause of profound childhood deafness.29'30 Congenital syphilis, a potential cause of deafness, is now less frequent, and maternal toxoplasmosis is a rare preceding event.31 Maternal influenza32 and cytomegalovirus disease3.1 have been reported to affect the ear adversely. Ototoxic drugs: Drugs ingested during pregnancy may damage the fetal ear. Streptomycin (especially its dihydro form),36 quinine37 and chloroquine phosphateM.3O cause deafness by destroying neural elements of the inner ear, whereas thalidomide can cause extensive damage to the auricle and the osseous structures of the middle and inner ear, and even total aplasia of the labyrinth.4.2 A remarkable case of drug-induced ototoxic effects in both mother and fetus has been reported.43 In the 28th week of pregnancy the mother received kanamycin, 1 g/d for 4½ days, for a Klebsiella infection and ethacrynic acid because of renal insufficiency; within 2 weeks she became

530 CMA JOURNAL/SEPTEMBER 8, 1979/VOL. 121

completely deaf. Her child appeared to be normal at birth but at 3 years of age had not started to talk and was found to be profoundly deaf. The combination of kanamycin and ethacrynic acid is more ototoxic than either drug alone in humans" and other mammals.42 Other maternal disorders: Rarely endocrine diseases in the mother, such as pseudohypoparathyroidism" and diabetes mellitus,47 may predispose her offspring to congenital deafness. The mechanism of this has not been established. Perinatal factors

Prematurity: Premature infants have an increased risk of deafness that has been attributed to intrapartum hemorrhage into the inner ear. As the survival rate of premature infants improves, their proportion in the population of deaf children is increasing; it has been reported to be 2% or more among the few survivors whose birth weights were less than 1400 g.48'11 Birth trauma: Hemorrhage into the inner ear as a result of intrapartum injury or stress may cause deafness. Damage to the organ of Corti is due to the toxic effects of extravasated blood and is irreversible.12 Traumatic obstetric procedures, such as forceps delivery and version followed by traction, account for the hemorrhage in many of these cases.13 Anoxia: Intrapartum asphyxia and anoxia may have a toxic effect on the cochlear nuclei and result in deafness.54 Such anoxic episodes may be manifested in the neonatal period as cyanotic spells, as the symptom complex known as cerebral irritation or as part of the respiratory distress syndrome. In many of these children deafness is accompanied by other evidence of neurologic damage, such as cerebral palsy. mental retardation, optic atrophy or epilepsy. Hyperbilirubinemia: Deafness due to kernicterus is the result of toxic damage to the cochlear nuclei or the central auditory pathways;11 athetoid neurologic sequelae are common. Since the advent of prophylaxis deafness due to hyperbilirubinemia has become rarer. Neonatal sepsis and meningitis: Deafness has been reported in an

infant in whom neonatal septicemia was treated with gentamicin.'6 Neonatal meningitis is commonly caused by gram-negative bacteria, particularly Escherichia ccli; other causal organisms are Pseudomonas, Kiebsiella and Proteus.'6 The prognosis is very poor, and 50% of survivors have severe neurologic . including hearing loss. These gramnegative organisms are sensitive to the newer aminoglycosides (e.g., gentamicin, tobramycin and amikacm), which are potentially ototoxic and must be administered with extreme caution, especially if renal function is impaired. infection in infancy or childhood Meningitis: The commonest cause of severe deafness acquired after the perinatal period is unquestionably meningitis, pyogenic or tuberculous, although the incidence of this infection as a cause of deafness is decreasing in affluent societies. Hearing loss has been reported in 5% to 35% of survivors, the higher figure may be closer to the actual incidence, as detection of the hearing loss, particularly if it is partial or subtle,61-63 may be very difficult, even with careful followup. In a retrospective review of 547 cases of meningitis treated at the Massachusetts General Hospital over a 14-year period59 it was found that of the 236 patients with bacterial meningitis 5% of the survivors under 2½ years of age and 21% of those aged 2½ years or more had a sensorineural hearing loss, and in these patients the organism was Neisseria meningitidis. Hearing was impaired in 3 of the 7 patients who had had fungal meningitis but was unimpaired in the survivors (303 in 304) of aseptic or viral meningitis. Sensorineural hearing loss after bacterial meningitis was bilateral in 77% of the patients and partial in approximately 70%; the cochlea was invariably the site of the lesion. Audiometry yielded uniformly high scores for speech discrimination and no evidence of tone decay in these patients, in contrast to those with defective sensorineural hearing after fungal meningitis. Viral infections: Measles and mumps may cause deafness in a small percentage of children who are not fully immunized. Measles

may cause viral labyrinthitis and resultant hearing loss by three mechanisms: the virus may enter the inner ear via the bloodstream or the central nervous system, or complicating purulent otitis media may spread to the inner ear, causing suppurative labyrinthitis and destruction of the inner ear. Mumps damages hearing in up to 5% of cases.84 Sudden hearing loss may precede the parotitis; invasion of the labyrinth is thought to occur during or shortly after the viremia. The deafness may be profound and permanent, but one review.' showed less severe loss and at least partial recovery in 50% to 90% of cases. Mumps has been considered the leading cause of unilateral sensorineural hearing loss in children. Chickenpox may be complicated by severe otitis media; the virus may then invade the labyrinth and cause hearing loss. Herpes zoster oticus in childhood is rare but may occur if the immune mechanisms are deficient. It is usually unilateral. The virus attacks the sensory roots of the eighth cranial nerve, and vesicles develop in the concha of the external ear. Deafness, facial paralysis and vestibular dysfunction occur separately or in combination. Other causal viral diseases include western equine encephalitis, which has a mortality of 5% to 15% and carries a high risk of neurologic sequelae and deafness in young children, rubella, poliomyelitis, influenza, infectious mononucleosis, viral hepatitis and adenovirus diseases. Otitis media: The incidence of acute otitis media is highest in the first 2 years of life and declines steeply with increasing age. Howie, Ploussard and Sloyer66 reported that the initial episode of otitis media was in the first year of life in 49% of infants and in the second year of life in only 12%. They also reported a 14% to 21% annual recurrence rate in children 2 to 7 years old, which is close to the 12% annual incidence reported by the Medical Research Council of Great Britain67 in children under 10 years of age. Recurring acute otitis media was found to increase the chance of permanent damage to the middle ear. Because of the availability of antibiotics, uncomplicated

acute otitis media should no longer result in hearing loss. However, chronic otitis media is very common in children that have a cleft palate and causes moderately severe hearing loss in many. Ototoxic drugs Deafness may develop up to 6 months after the ingestion of an ototoxic drug.68 It is more frequent with dihydrostreptomycin than streptomycin, and familial hypersensitivity or idiosyncrasy to streptomycin has been reported.69 Neomycin, which is also nephrotoxic,70'7' can cause profound deafness when given parenterally,7' intrapleurally, intraperitoneally, by aerosol73 or orally,71'74 and when used in solution to irrigate wounds.73'7' Bamford and Jones76 reported deafness in six infants aged 8 to 16 months after their full-thickness burns were sprayed with a combination of neomycin, bacitracin, polymyxin B and colistin. Lowry, May and Pastore77 described neomycin-induced destruction of both inner and outer hair cells of the organ of Corti. The hearing loss is usually progressive, with the high frequencies affected first, and ultimately perception of the entire frequency range is lost. Tinnitus may develop, but vertigo is rare (vestibular function is intact). Like kanamycin, neomycin penetrates the inner-ear fluids slowly and its elimination is difficult; therefore, cochlear damage is severe. The interval between the cessation of therapy and the onset of deafness may be considerable but is usually shorter than with kanamycin.76 Echeverria and colleagues79 reported that gentamicin-induced hearing loss occurred chiefly in patients whose serum concentration of the drug was greater than 12 ,.tg/ml. However, hearing loss also occurred in some children with serum concentrations previously considered safe, and the risk of this complication appeared to be greater in children who had renal failure or were taking diuretics concomitantly. Yow and associates80 were the first to report an ototoxic effect of kanamycin in children. This drug causes a hearing loss that is usually quite profound; it is mostly highfrequency sounds that can no longer

CMA JOURNAL/SEPTEMBER 8, 1979/VOL. 121

531

be detected. The deafness is of slow, insidious onset, and tinnitus is an early feature; prompt withdrawal of kanamycin may prevent further deterioration of hearing and permit the restoration of hearing to functional levels. Amikacin, an ami noglycoside, is both ototoxic and nephrotoxic; ototoxic effects have been reported to occur in 4.6% 91 to 7*4%82 of cases. Other drugs that may lead to reversible ototoxicity include salicylates83 and diuretics such as furosemide and ethacrynic acid.84 Deafness has also been reported after treatment with long-acting progesterone, possibly as a result of occlusion of the common cochlear artery.85 Trauma

Accidental trauma is a rare cause of deafness in infancy and childhood. Hearing does not improve when the fractures heal if they are transverse, but may become normal if the fractures are longitudinal.86 Problems of early identification There have been many reports of refusal by family physicians to consider seriously the parents' observations on their children's hearing impairment.34'8'9.87'88 All too often they reject the parents' observations in favour of their own findings on cursory examination of a child's ears.89 Some well meaning physicians reassure parents with inappropriate advice, believing that this is what the parents need; for example, "Don't worry yourself; he is just a bit slow."0 A survey in Australia showed that such advice significantly delayed diagnosis in one quarter of cases.3 It is time physicians realized that parents are the best "screening instruments" and listened to them; they should accept a provisional diagnosis of hearing loss until a qualified audiologist has given an opinion. A pilot study showed that only 28.3% of primary-care physicians questioned thought a diagnosis of deafness could be made by a child's first birthday, 40% thought a reliable diagnosis possible only after a child was 2 years old and 11.7% did not know or did not answer.6 With recent advances in screening

methods hearing loss can be identified in the neonatal period, and the degree of deafness can be established at 8 to 10 months. In many surveys the average age of children when their parents first suspected a hearing loss has been found to be 13 months. At this age, when most of these parents take the infant to a doctor, there is no possible explanation for further delay by the physician in identifying hearing loss. Misdiagnosis by family doctors accounted for delayed identification of deafness in up to 30% of cases in some reported series;9093 the interim diagnoses included mental retardation, aphasia, emotional disturbance, minimal brain damage and impaired learning ability. Some deaf children may be thought to be linguistically handicapped by virtue of their socioeconomic status or second-language problems.94'95 Parents, particularly mothers, are likely to detect hearing defects in their children; detection rates of 70% to 80% have been reported,6'96 and our observations are similar. A few parents persistently deny even obvious signs of hearing impairment in their children, and others are ambivalent about expressing such fears to their doctors.4'87 In these cases early identification of hearing impairment depends on the alertness of others. All parents of deaf children initially deny the deafness.97 A. family's detection of the condition is gradual and depends on the parents' personalities, their relations with their children, the state of their marriage, the order of the deaf child in the family by birth and the importance of verbal communication to the family and its culture.98 Communicative, authoritative parents who have a positive self-image are probably early detectors of deafness. The frequency of parental observations regarding hearing, talking and other behaviour is illustrated in Table I. Many infants with impaired hearing are quiet and sleep unusually well despite noise; they do not respond to rattles or peek-a-boo games, and either do not babble or decrease their early babbling. Suspicion should increase with certain idiosyncratic manifestations, such as those listed in Table I.

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A primary-care physician in office practice rarely sees a deaf child and, therefore, may not be alert to the possibility of hearing loss, but many physicians in Canada are aware of the inadequacy of their basic training in the diagnosis, treatment and prognosis of deafness in children. Although many medical educators recognize these deficits, it seems that only concentrated efforts by pressure groups (e.g., parents' associations) to present their case to these educators can bring about the necessary changes.2'99 Meanwhile, there should be wid& dissemination of information about deafness (preferably by family doctors) to alert the general public as well as health-care personnel to the magnitude of the problem of deafness in childhood. Family physicians should know

that comprehensive history-taking with attention to high-risk factors, coupled with simple screening of speech and hearing development, will identify a high proportion of cases. Even with the most sophisticated equipment in the best trained hands, diagnosis may be difficult, but referral for specialist advice in all cases can prevent many tragedies. In Canada early identification is less likely in rural than in urban populations. Partly because of limited access to medical diagnostic facilities, but also because of decreased awareness, parents in rural areas suspect hearing loss in their children and seek medical attention later than do urban parents."6'1. (Upfold,3 in Australia, however, found no significant difference between the two populations.) Mobile hearing clinics are one good solution; vans equipped for this purpose have brought to rural communities in Manitoba'01 and to isolated residents in northern Ontario100 the diagnostic and therapeutic facilities usually available only in large medical centres. Selective hearing loss presents a

serious challenge to early identification; this condition can cause confusion for parents, physicians, teachers and other health-care personnel. If the deafness is to sounds at the frequency of normal speech, failure to identify it can have tragic effects on speech and language acquisition. One child with this kind of deafness was variously considered to be mentally retarded, emotionally disturbed or brain damaged before the deafness was diagnosed at the age of 5½ years.90 There is no short-cut to the identification of selective hearing loss. Nonsensical, poorly enunciated speech and excessive gesturing are highly indicative of hearing impairment. In a child 2 years of age or older a delay in speech and language development is demonstrable by the Denver Developmental Screening Test, but partial hearing loss may be identifiable only by an audiologist. Mild hearing loss can be a child's largest problem during the early school years. It may be evidenced by inattentiveness, apparent laziness, day-dreaming or behaviour and learning problems. Its com-

monest cause is chronic serous otitis media, the resultant fluctuating hearing loss being the reason for the unusual behaviour and poor school performance. A substantial percentage of children thus affected do recover complete middle-ear function without medical or surgical intervention, but the others are left with middle-ear dysfunction and mild to moderate hearing loss. Program for prevention and early identification

The most important requisites of such a program are prenatal care, knowledge of genetic and perinatal factors, physical examination and audiologic screening at birth, follow-up assessment at 2 months of age and continuing pediatric care. Perinatal care Rubella immunization: In selected series,"'37 and in our experience, congenital deafness has been due to maternal rubella in about 15% of cases. Ideally all women of reproductive age should be protected against rubella; individuals not immunized during childhood

'.'.>K'..

The well-baby check-up is a chance to screen an infant at risk for a hearing deficit. CMA JOURNAL/SEPTEMBER 8, 1979/VOL. 121 535

should be vaccinated before they become pregnant - perhaps at a premarital physical examination. The financial cost of such a program would be only a minute fraction of the potential cost of dealing with a rubella epidemic and its consequences; the saving of human suffering is incalculable. For immunization to be successful we should use no vaccines after their expiry date and should vaccinate no child under 12 months of age and no woman who is or may be pregnant. Physicians and nurses must read the manufacturer's instructions. Only with such concern and awareness can we hope to reduce the appallingly high incidence of rubella-induced congenital deafness. Genetic counselling: Counselling as to the estimated risk of deafness is most important in families known to be at risk and in communities in which consanguineous marriages are common. Many parents of deaf children want to know the chance of deafness in subsequent children, and many deaf parents want to know the probability of transmitting the defect to their offspring. Such counselling requires the services of a geneticist, an audiologist, a psychologist and a social worker in addition to the family physician. Great care must be taken to guard the rights of each family member. Prenatal workshops: All pregnant women should have the benefit of regular prenatal examination. In addition, expectant parents should be encouraged to attend lectures or workshops on childbirth, child development and child care. Both parents should learn about hearing loss (conductive and sensorineural), its prevention and manifestations and its management, especially at home. They should know about ear infections - hearing impairment in 5-year-olds is due to chronic secretory otitis media in approximately 81% of cases.102 Parents must be encouraged to participate in health care, to detect any apparently abnormal physical events and behaviour, and to ask about possible consequences and early signs and symptoms of disease. A parent's awareness of the risk of deafness is a great aid to early identification; it can lead to earlier diagnosis, faster referral

by primary-care physicians and significantly less delay before definitive diagnosis and the institution of therapy.6 The Crib-O-Gram®:103 This is an automated method for screening in nurseries the hearing of infants at high risk. A motion-sensitive transducer placed beneath or in the infant's crib detects virtually every motion stronger than an eye-blink. A strip-chart records this motor activity before and after a test sound, shuts itself off, then repeats this process at set intervals 20 times in 24 hours. Follow-up of all babies in whom the test indicates impaired hearing begins soon after discharge from hospital. The pediatrician or family doctor is notified of the test result (and advised that false-positive results are common), and an appointment is made for rescreening at a lower sound intensity (20 dB) when the child is 6 months old. Infants who fail the second test are referred to an audiologist for evaluation; the names of those who pass are deleted from the highrisk register. Registration of neonates The Saskatoon Conference on Early Diagnosis of Hearing Lossl.k recommended in 1978 the registration of neonates as being at high risk for hearing impairment if any

of the following criteria applied: * A family history of onset of severe hearing loss in early childhood. * A significant illness during the mother's pregnancy (e.g., rubella or cytomegalovirus or herpesvirus infection). * A congenital anomaly of the skull, face, ear, nose or throat, including absent, malformed or lowset pinna, cleft lip, cleft palate and submucous cleft of the palate. * Prematurity, with a birth weight of less than 1500 g. * Neonatal icterus, with a serum bilirubin value of 20 mg/dl (342 ..tmol/l) or greater in a baby of normal weight or a lower value in a smaller baby. * Anoxia at birth, with persisting neurologic abnormalities (e.g., neonatal convulsions). The Saskatoon conference passed a resolution requesting provincial and local governments to make registration mandatory.1" A register would increase the probability of the early identification of hearingimpaired infants and would lead to supervision, including periodic screening, of hearing losses not apparent in the first months of a child's life. Registered infants should be referred for detailed audiologic evaluation before they are 2 months old; this would include an arousal test, Crib.O.Gram®

Before an infant is 7 months old the Crib-O-Gram® can he used to screen for and detect a hearing defect. (Courtesy of Telesensory Systems, Inc., Palo Alto, California.)

536 CMA JOURNAL/SEPTEMBER 8, 1979/VOL. 121

Table 11-Office testing of hearing in infants and young children Age Newborn 4 mo 6 mo 942 inn 13-30 me

2j-5 yr

Normal response Arousal from sleep (startle response) Eye-blInk or rudimentary head-turn Turns head directly toward side of sound Tries to locate sound-source beside orlielow Seeks to locate sound-source at side, below or above. Early in age range begins to respond to single spoken words; later shows understanding of simple commands Normal speech, using two4yllable Rasponds to simple commands and words normally in vocabulary of age qnestlons group, at a distance of 45 cm, with loudness decreased until tbe tpter can barely bear him- or herself

Testing material Loud cowbell or noIsemaker (70 48) Small bell or soft sqUeeze-toy (60 45) Small bell (60 dB) Small bell (0dB) er crinkling *f callophane wrapping (4l.dS) Small bell or normal speech

with deafness in children necessitate the cooperation of many healthcare professionals in identifying the condition and planning its management. At the Hospital for Sick Children assessment is done daily for 4 days. Children from out of town stay in a hostel beside the hospital. Members of the hearing-assessment program team interview the family, discuss their findings and recommend therapy. The primary team consists of an audiologist, an ophthalmologist, an otologist, a pediatrician, a psychologist, a public health nurse, a social worker, a teacher of the deaf and a representative from the local school authority. Consultants include a geneticist, a neurologist, a neuroradiologist, a psychiatrist and a speech therapist. Such an approach recognizes the special role of each profession and presumes that each member should contribute to the overall care of deaf children. Unfortunately the team approach is still relatively rare, which limits not only the provision of the best care but also research into deafness. Financial restraints and the shortage of certain specialists (particularly audiologists and speech pathologists) preclude team approaches in many areas, though most university health science centres in Canada have such a program. Hearing aids Hearing aids are used as soon as a tentative diagnosis of hearing loss is made, to capitalize on any residual hearing. Infants as young as 2 to 3 months have been fitted with hearing aids. Various electroacoustic criteria and clinical procedures are used to select the appropriate amplification. A child should be taught how to use a hearing aid properly; frequent instruction by the audiologist, parents and therapist is required. Hearing aids should be regularly refitted to accommodate the child's growth. The Canadian Hearing Society, at the recommendation of an otolaryngologist and an audiologist, dispenses hearing aids at manufacturers' prices. Many service clubs, churches and local philanthropic organizations give financial aid to parents that cannot afford hearing aids for their children.

Teaching communication

Each child should be given the opportunity to learn how to communicate by the most suitable method; rigid insistence on one method is not recommended. An aggressive home stimulation program, resulting in good communication between family members and child, is of particular importance during the child's formative years. Three primary methods are used for teaching a child to communicate. Auditory: The aim of this method is to deprive the partially deaf child of all channels of communication except hearing; this should force the child to use auditory instead of visual cues for communication. This method necessitates intensive training of the child by specialist teachers and a high degree of parental cooperation, but it tends to produce the best speech and language development. This approach

is being used in the educational therapy program (auditory training) at the Hospital for Sick Children. Oral: This method teaches a combination of lip-reading and auditory cues. It provides easier communication with the child but tends to result in poorer speech and language development. Total communication: In this method all available means of communication are used - residual hearing, lip-reading and manual speech. Although this method tends to yield the poorest speech and language development, it does allow the early and rapid development of a significant degree of communication. It is probably the most widely used method. Education Large cities across Canada operate day-school classes for deaf preschool-aged and school-aged children; in Ontario the oral method is generally used. Hearing-impaired

Early training of a hearing-impaired child increases the chance of a productive life. (Courtesy of the Canadian Hearing Society.)

540 CMA JOURNAL/SEPTEMBER 8, 1979/VOL. 121

children whose parents choose the thermore, few conditions are as oral method can be educated in amenable to correction or amelioraordinary schools; this has the ad- tion. Alberta has a comprehensive vantage of allowing the child a norpreschool surveillance program in mal life in his or her community. Most residential schools for the which a high-risk register is maindeaf practise total communication; tained and infants and children are in addition to boarding facilities, periodically screened for hearing these schools have travelling teach- and other developmental problems. ers that visit preschool children to In the United States, Massachusetts and New York State have passed give them instruction. legislation for setting up high-risk Help for the child's family registers. In California the legislaParents' groups are becoming a ture directed the state department dominant factor in therapy for deaf of health to study the cost of varichildren and in helping the families ous programs for testing hearing at deal with social and emotional birth and to outline ways of impleproblems. The members share ex- menting such a program. In Utah a periences and help those whose register is being used by an increaschildren are newly identified as ing number of hospitals, and its use deaf to cope with the problems that will become compulsory when all others have encountered. "Voice", hospitals have become involved. Six one such organization, in Ontario, other states - Kansas, Maryland, practises the auditory approach to Nevada, New Mexico, Nebraska education. and Wyoming - are planning to The two main Canadian volun- implement such a register."0 tary organizations working for the There is no single acceptable hearing-impaired are the Western method for routine screening of neoInstitute for the Deaf, which has at nates. Ideally a practical program least 20 branches in British Colum- of screening for hearing impairment bia, including several parents' and other disorders should be degroups that help with preschool veloped through interdisciplinary children, and the Canadian Hearing collaboration. Society, whose head office is in In addition to improving the Toronto and which provides serv- quality of their lives, the early idenices throughout Ontario. Other re- tification and treatment of deaf chilnowned institutions for parents of dren can increase their earning podeaf children are the John Tracey tential and save public money. Clinic and the Alexander Graham There is even a difference in earnBell Association for the Deaf, both ings between the congenitally deaf in the United States. A book many and those who become deaf at 3 parents and physicians may find years of age or later; the difference useful is "Learning to Listen"; it is represents the level of language obtainable from the Canadian skills a child can attain with as Hearing Society, 60 Bedford Rd., little as 3 years of normal hearing Toronto, Ont. M5R 2K2. and language development. There are approximately 1.3 million Existing screening and registration deaf persons in the United States; it is estimated that their identification programs at birth and subsequent language Where does screening for deaf- training would have resulted in an ness stand in comparison with that annual saving of at least US$360 for other diseases? All infants born million."' in Canada are screened for phenylketonuria, which has an incidence Conclusion of 1 in 15 000 live births. Quebec (for the past 4 years) and Ontario Many of the known causes of (since October 1978) screen all neo- childhood deafness, especially runates for hypothyroidism, which bella, mumps and ototoxic effects has an incidence of 1 in 5000. of drugs, are preventable. The probHowever, screening for deafness, lems of early identification will conwhich has a minimum worldwide tinue to exist but are not insurincidence of about 2 per 1000 live mountable. We believe that use of births, is relatively infrequent. Fur- a high-risk register would be the 542 CMA JOURNAL/SEPTEMBER 8, 1979/VOL. 121

greatest aid to early identification of deafness in preschool-aged children. It is high time delayed diagnosis of hearing impairment in early childhood became the exception rather than the rule. We thank Ms. Karen Freel of the Canadian Hearing Society for her helpful suggestions and the medical publications department at the Hospital for Sick Children for their help in the preparation of the manuscript. References 1. AMMAN JC: Surdus Loquens s. Methodus, qua qui Surdus Natus est, Loqui Discere Possit, Amsterdam, 1692; reprinted 1700 (The Talking Deaf Man, or a Method Proposed Whereby He Who is Born Deaf May Learn to Speak, FooT D (trans], Howkins, London, 1694) 2. SHAH CP, CHANDLER D, DALE R:

Delay in referral of children with impaired hearing. Volta Rev 80: 206, 1978 3. UPFOLD U: The early detection of

childhood deafness. Med J Aust 2: 507, 1978

4. LLJTERMAN DM, CHAsIN J: The pe-

diatrician and the parent of the deaf child. Pediatrics 45: 115, 1970 5. NORTHERN JL, DowNs MP (eds): Hearing in Children, Williams & Wilkins, Baltimore, Md, 1974 6. SHAH CP, DALE R, CHANDLER D:

The challenge of hearing impairments in children. Can Fain Physician 23: 175, 1977 7. WALLACE G (project director): Canadian Study of Hard of Hearing and Deaf, Canadian Rehabilitation

Council for the Disabled, Toronto, 1973 8. DE ScHwEINITz L, MILLER CA,

MILLER JB: Delays in the diagnosis of deafness among preschool children. Pediatrics 24: 462, 1959 9. BROWN MS: Approaches to hearing testing of children in the office. Comments on present status. Clin Pediatr (Phila) 14: 639, 1975 10. GERBER SE: Neonatal auditory testing: a review, in Proceeding of the Conference on Newborn Hearing Screening, San Francisco, Feb. 2325, Alexander Graham Bell Association for the Deaf, Washington,

DC, 1971, p 15 11. BUDDEN SS, RoBINsoN GC, MACLEAN CD, et al: Deafness in infants and preschool children: an analysis of etiology and associated handicaps.

Am Ann Deaf 119: 387, 1974 12. BERGSTROM

L,

HEMENWAY

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DowNs MP: A high risk registry to find congenital deafness. Otolaryngol Clin North Am 4: 369, 1971 13. UPFOLD U, FIFIELD DB, MURPHY

PA, et al: Permanent childhood deafness in Australia: incidence and trends, 1954-1971. Med I Aust 2: 57, 1973

14. World Health Organization: The Early Detection and Treatment of Handicapping Effects in Young Children. Report of a Working Group Convened by the Regional Office for Europe of the World Health Organization, WHO, Copenhagen, 1967 15. SIMMoNs FB: Automated screening test for newborns: The Crib-O-Gram, in Hearing Loss in Children. A Comprehensive Text, JAFFE BF (ed), Univ Park, Baltimore, Md, 1977, p 89 16. STEWART IF: Newborn infant hearing screening - a five year pilot project. I Otolaryngol 6: 477, 1977 17. CHUNG CS, RonIsoN OW, MORTON NE: A note on deaf mutism. Ann Hum Genet 23: 357, 1959 18. RosE SP: Genetic Studies of Profound Prelingual Deafness, thesis, Indiana University, Bloomington, 1975

19. FRASER GR: Profound childhood deafness. I Med Genet 1: 118, 1964 20. WAARDENBURG PJ: A new syndrome combining developmental anomalies of the eyelids, eyebrows and nose root with pigmentary defects of the iris and head hair and with congenital deafness. Am I Hum Genet 3: 195, 1951 21. ORMEROD FC: The pathology of congenital deafness. I Laryngol Otol 74: 919, 1960

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pneumonia of the mother with hem-

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INGS JO: Psychosocial problems ot deaf children and their families: a comparative study. Am Ann Deaf 120: 391, 1975 FREEMAN RD: Psychiatric aspects of sensory disorders and intervention, in Epidemiological Approaches in Child Psychiatry, GRAHAM P (ed), Acad Pr, New York, 1977, p 275 HOLM VA, THOMPSON G: Selective hearing loss: clues to early identification. Pediatrics 47: 447, 1971 HODGSON WR: Misdiagnosis of children with hearing loss. J Sch Health 39: 570, 1969 VERNON M: Psychological aspects of the diagnosis of deafness in a child. Ear Nose Throat J 52: 60, 1973 MEADOW KP: Parental response to the medical ambiguities of congenital deafness. J Health Soc Behav 9: 299, 1968 COLEMAN RO: Delayed identification of hearing loss in a six- and one-half year old child: a case study. J Commun Disord 5: 368, 1972

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rate in an urban speech and hearing clinic. ASHA 17: 455, 1975 counselling, 1961-1968. Volta Rei' 72: 51, 1970 97. BECKER 5: Initial concern and action in the detection and diagnosis of a hearing impairment in the child. Volta Rev 78: 105, 1976 98. MINDEL ED, VERNON M:

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cylate ototoxicity. A clinical and experimental study. Arch Otolaryngol 82: 483, 1965

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BOOKS I This list is an acknwledgement of books received It does not preclude review at a later date. ADJUVANT THERAPIES AND MARKERS OF POST-SURGICAL MINIMAL RESIDUAL DISEASE I. Markers and General Problems of Cancer Adjuvant Therapies. Edited by G. Bonadonna, G. Mathe and S.E. Salmon. 150 pp. lIlust. Springer-Verlag New York Inc., Secaucus, New Jersey, 1979. $27. ISBN 3-540-09291-9 ADVANCES IN CANCER CHEMOTHERAPY. Edited by Andre Rosowsky. 297 pp. Illust. Marcel Dekker, Inc., New York, 1979. $29.75. ISBN 0-8247-6806-X AnITUDE THERAPY FOR STRESS DISORDERS. Brian J. Gorman. 245 pp. Illust. Detselig Enterprises Ltd., Calgary, 1979. Price not stated. ISBN 0-920490-03-4 CANCER RESEARCH SINCE 1900. An Evaluation. Sigismund Peller. 394 pp. Philosophical Library Inc., New York, 1979. $15. ISBN 8022-2223-4 THE CHANGING YEARS. The Story of Toronto Hospital and the Fight against Tuberculosis. Godfrey L. Gale. 134 pp. Illust. West Park Hospital, Toronto, 1979. $10, hardbound; $5, paperbound: plus $1 to cover mailing charges. ISBN 0-9690035-0-1 COLOR ATLAS OF CANCER MORTALITY BY ADMINISTRATIVE AND OTHER CLASSIFIED DISTRICTS IN TAIWAN AREA: 1968-1976. Kung-Pei Chen, HsinVing Wu, Ching-Chuan Yeh and others. 109 pp. lIlust. NSC special publ. no. 2. National Science Council, Taipei, Taiwan, Republic of China, 1979. Price not stated