CONGENITAL RUBELLA DEAFNESS: A PREVENTABLE DISEASE

258

illustrative of

a new force likely to have a profound influence on psychiatry in the U.S.A. Not only has President Carter’s Commission on Mental Health61 highlighted many of the deficiencies in current practice and outlined amore scientific and socially responsible approach to psychiatry, but the administration has brought in a new breed of academic psychiatrists to high-level posts in the powerful federal agencies. It is to cooperation between such agencies and academic departments that American psychiatry must look for further research advances and for the integration of such advances into broad-based training programmes and eventually into everyday practice. Meanwhile, one can confidently state that the dark clouds which enveloped American psychiatry in the post-war period have now

largely dispersed. 1. Webster, T. G. in Working Papers of the Conference on Psychiatry and Medical Education. American Psychiatric Association, Washington, 1969. 2. Brown, B. Am. J. Psychiat. 1977, 134, March suppl. p. 1. 3. Arthur, R. J. ibid. July suppl. p. 2. 4. Shepherd, M. ibid. 1957, 114, 417. 5. Shepherd, M. Compreh. Psychiat. 1971, 12, 302. 6. Pulver, S. E. Am. J. Psychiat. 1974, 131, 1400. 7. Blackwell, B., Allen, A., Wales, E., Paper read at the Maurice Levine Society, Cincinnati, September, 1977. 8. Armor, D. J., Klerman, G. L. J. Hlth soc. Behav. 1968, 9, 243. 9. Stone, W., Stein, L. S., Green, B. Archs gen. Psychiat. 1971, 24, 468. 10. Casariego, J. I., Greden, J. F. Compreh. Psychiat. 1978, 19, 241. 11. Kernberg, O. F., Burstein, E. D., Coyne, L., Appelbaum, A., Horwitz, L.

Voth, H. Bull. Menninger Clin. 1972, 36, 1. R. B., Staples, F. R., Cristol, A. H., Yorkston, N. J., Whipple, K. Psychotherapy Versus Behavioural Therapy. Cambridge, Mass., 1975. 13. Bergin, A. E., Strupp, H. H. Changing Frontiers in the Science of Psychotherapy. Chicago, 1972. 14. West, N. D., Walsh, M. A. Am. J. Psychiat. 1975, 132, 1318. 15. Kline, W. Paper read at the Royal College of Psychiatrists, London, July, Sloane,

1975. 16. Redlich, F., Kellert, S. R. Am. J. Psychiat. 1978, 135, 22. 17. Coryell, W., Wetzel, R. D. ibid. p. 732. 18. Clare, A. W. Listener. Nov. 9, 1978, p. 608. 19. Maher, J. quoted by Clare.18 20. Vaillant, G. Archs gen. Psychiat. 1976, 33, 535. 21. Menninger, K. The Vital Balance: The Life Process in Mental Health and Illness. New York, 1963. 22. Kuriansky, J. B., Gurland, B. J., Spitzer, R. L., Endicott, J. Am. J. Psychiat.

1977, 134, 631. 23. World Health Organisation. International Pilot Study on Schizophrenia, vol. I. Geneva, 1973. 24. Baldessarini, R. J. Am. J. Psychiat. 1970, 127, 759. 25. Dunne, D. L., Fieve, R. R. in Psychiatric Diagnosis: Exploration of Biological Predictors (edited by H. S. Akiskal and W. L. Webb). New York, 1977. 26. Rosenhan, D. Science, 1973, 179, 250. 27. Clare, A. W. Psychiatry in Dissent. London, 1976. 28. Feighner, J. P., Robins, E., Guze, S. B., Woodruff, R. A., Winokur, G.,

Munoz, R. Archs gen. Psychiat. 1972, 26, 57. R. L., Endicott, J., Robins, E. Research Diagnostic Criteria. New York State Psychiatric Institute, Biometrics Research, New York, 1975. 30. Rakoff, V. M., Stancer, H., Kedward, H. B. Psychiatric Diagnosis. New York, 1977. 31. Akiskal, H. S., Webb, W. L. Psychiatric Diagnosis: Exploration of Biological Predictors. New York, 1977. 32. Spitzer, R. L., Klein, D. Critical Issues in Psychiatric Diagnosis. New York, 29.

CONGENITAL RUBELLA DEAFNESS: A PREVENTABLE DISEASE CATHERINE S. PECKHAM

Department of Medicine, Charing Cross Hospital Medical School, Fulham Palace Road, London W6 8RF

J.

A. M. MARTIN

Nuffield Hearing and Speech Centre, Royal National Throat, Nose and Ear Hospital, Gray’s Inn Road, London WC1 W. C. MARSHALL

REFERENCES

12.

Preventive Medicine

Department of

J. A. DUDGEON

Microbiology, Institute London WC1

of Child Health,

Over a 4-year period (1972-75) an unselected sample of 568 children aged under 4 years attending the Nuffield Hearing and Speech Centre were tested for rubella antibody. Sensorineural deafness was subsequently diagnosed in 349 of these children, and 83 (24%) of this group had rubella antibody. In contrast, only 19 (9%) of the remaining 219 children in whom sensorineural deafness was excluded had rubella antibody. The seropositive and seronegative children with sensorineural deafness showed striking differences in family history, history of maternal rubella, adverse perinatal events, and presence of other defects. Congenital rubella is an important cause of deafness, and the rubella vaccination programme must be pursued more vigorously if this serious defect is to be prevented in the future.

Summary

INTRODUCTION

SENSORINEURAL deafness is the

most common abnorassociated with mality congenital rubella, and it is the defect caused this fetal infection which occurs only by alone with any consistency. 1-4 It has been suggested that the number of children with hearing impairment due to intrauterine rubella has been seriously underestimated,5 probably because of the high incidence of subclinical

Spitzer,

1978.

33. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders. New York, 1977. 34. Mednick, S., Schulsinger, F., Higgins, J., Bell, B. (editors). Genetics, Environment and Psychopathology. Amsterdam, 1974. 35. Gottesman, I. I., Shields, J. Schizophrenia and Genetics. New York, 1972. 36. Goodwin, D. W., Schulsinger, F., Moller, N. Hermansen, L., Winokur, G., Guze, S. Archs gen. Psychiat. 1974, 31, 164. 37. Levine, J. in Psychopharmacology: A Generation of Progress (edited by M. A. Lipton, A. DiMascio, and K. F. Killam). New York, 1978. 38. Usdin, E., Hamburg, D. A., Barchas, T. Neuroregulators and Psychiatric Disorders. New York, 1977. 39. Hanin, I., Usdin, E. Animal Models in Psychiatry and Neurology. Oxford, 1977. 40. Lipton, M. A., DiMascio, A., Killam, K. F. (editors). Psychopharmacology: A Generation of Progress. New York, 1978. 41. Kety. S. ibid. 42. Bunney, W. E Am. J. Psychiat. 1978, 135, July suppl., p. 8.

43. Koran, L. M. ibid. 1976, 133, 1052. 44. National Institute of Mental Health. Staffing Patterns in Mental Health Facilities. N.I.M.H., Series B., Washington, 1972. 45. Ilfeld, F. W. Archs gen. Psychiat. 1978, 35, 716. 46. Kramer, M. Paper given at the Institute of Psychiatry, London, April, 1976. 47. Torrey, E. F. Washington Post, Sept. 4, 1977. 48. Hustead, E. C., Sharfstein, S. S. Am. J. Psychiat. 1978, 135, 315. 49. Arthur, R. J. ibid. 1973, 130, 841. 50. Zussman, J., Lamb, H. R. ibid. 1978, 134, 887. 51. Weissman, M. M., Klerman, G. L. Archs gen. Psychiat. 1978, 35, 705. 52. Robins, L. ibid. p. 697. 53. Gregg, A. Am. J. Psychiat. 1944, 101, 285. 54. Kubie, L. S. Archs Neurol. Psychiat. 1957, 78, 283. 55. Wedge, B. Science, 1967, 157, 281. 56. Freedman, A. M., Kaplan, H. I., Sadeck, B. J. Comprehensive Textbook of

Psychiatry. Baltimore, 1975. 57. Miller, H. G. World Med. 1969, 5, 44. 58. Cerrolaza, M. Compreh. Psychiat. 1973, 14, 299. 59. Kety, S. Am. J. Psychiat. 1974, 131, 957. 60. Klerman, G. L., quoted by Lebensohn, Z. M. Psychiat. Ann. 1975, 5, 376. 61. President’s Commission on Mental Health. Report for the President. Wash-

ington, 1978.

259

during pregnancy. Rubella deafness is theoretically preventable by active immunisation, and if rubella vaccines are effective and if they are taken up by the none-immune female population the number rubella infection

now

of rubella-deaf children should decline over the next decade. This paper reports the findings of a clinical and laboratory study of children attending a large hospital department concerned with the diagnosis and management of early-onset hearing loss. METHOD

Over the 4-year period, 1972-75, an unselected sample of 568 children aged 6 months to 4 years who were referred for assessment to the Nuffield Hearing and Speech Centre were tested for rubella antibody. Since rubella antibody is seldom acquired from postnatal infection before the age of 4 years, the detection of antibody in young children was used to make a retrospective diagnosis of congenital rubella infection.6 Capillary blood was taken from both mother and child onto absorbent paper and tested for rubella haemagglutination-inhibition (H.!.) antibodies as previously described.6 Children with rubellaH.I. antibody titres of 1:16 or greater were regarded as seropositive and those with titres of <1:16 as seronegative. Details of the type of deafness, the maternal and family history, birth-weight, the condition of the child at birth, and the presence of any other defects were obtained from clinical records of all patients.

findings in the 83 rubella-seropositive children are compared with those in the 266 children who were seronegative. Between 19% and 28% of deaf children born each year were seropositive. When the months of birth of the seropositive and seronegative groups were compared the majority (57%) of the seropositive children were born during the winter months (October to February) but no such seasonal pattern was present in the seronegative group (35% were born between October and February). This finding reflects the seasonal incidence of rubella: the majority of children with congenital rubella are born in the winter months to women who have acquired the infection in late spring or early 8 summer during the first trimester of pregnancy. 7 The mothers of only 40% of the children who were seropositive gave a history of rubella illness with rash in pregnancy, but a positive history was also obtained from the mothers of 10 (4%) of the seronegative children. The and the

of deafness in these 10 children was not established, but other possible aetiological factors were present in 6 of them: 2 had a family history of deafness, 2

cause

TABLE I--COMPARISON OF BIRTH-WEIGHTS OF CHILDREN WITH SENSORINEURAL DEAFNESS AND CHILDREN IN THE NATIONAL

PERINATAL

STUDY 10

RESULTS

Of the 568 children tested for rubella antibody, 102 (18%) were found to be seropositive and 466 (82%)

seronegative. Scrutiny of individual records without prior knowledge of rubella-antibody status showed that 349 (61%) of the 568 children had sensorineural deafness. 83 (24%) of the 349 children with sensorineural deafness were seropositive, whereas only 19 (9%) of 219 children without sensorineural deafness were seropositive (P<0’001) figure). Only those children with sensorineural deafness are included in the subsequent analysis.

(see

Children with Sensorineural Deafness There

were

349 children with sensorineural

deafness,

*Singletons and survivors. had had neonatal convulsions, 1 had a birth-weight of less than 2000 g, and 1 had been nursed in an incubator for several days after birth. These associations are possible causes of deafness; on the other hand, these children could represent the very small group with conhad genital rubella infection in whom rubella antibody declined to undetectable levels by the age of testing.99

Birth-weight shows the birth-weight distribution of the seropositive and seronegative deaf children and for comparison the children in the National Perinatal Study who were born in one week in March, 1970. The mean birth-weight of the seropositive group was significantly lower, at the 5% level, than that of the seronegative group. When comparisons were made between the mean birth-weight of the seronegative group and that of the infants in the National Perinatal Study, the seronegative group had a significantly lower mean birth-weight, at the 1% level.

Table

Rubella-antibody status of children aged 6 m0--4 yr. ’Conductive deafness (6), congenital heart defect and developmental delay (1), family history of deafness (1), maternal history of rubella and/or contact (1), referred for speech delay and/or suspected hearing loss but hearing normal (10). fConductive deafness (47), developmental delay (22), cleft palate ),, meningitis (4), family history of deafness (28), maternal history of rubella contact and/or rash (25), referred for speech delay or suspected heanng loss but hearing normal (69).

i

260 Associated Defects anda bnormalities Information on a large number of factors was available after detailed scrutiny of the patients’ medical records. A standard questionnaire completed for all children included information about perinatal problems (t.’&bgr;., jaundice, condition at birth, type of delivery), a medical and developmental history, and a family history. The presence of a wide variety of other defects was recorded at the time of the medical examination. The comparisons between the two groups were revealing, but clearly caution is required in interpreting results because the information is retrospective. 69 (83%) of the 83 seropositive deaf children had no relevant medical or family history and no additional defect. In contrast, only 82 (31%) of the 266 seronegative deaf children had no relevant history and no additional defects (table n). 11 (13%) of the seropositive children had other defects, and in all but 1 case, a child with Down syndrome, these were compatible with congenital rubella infection. The defects included congenital heart defects, cataracts, microphthalmos, and mental retardation. In contrast 5(20%) of the 266 seronegative children had additional defects, and these were of quite a different nature from those among the seropositive group (table ill). In only 2 of the seropositive group of 83 children was there a history of adverse perinatal events, and 1 child had a family history of deafness. In the group of seronegative deaf children a much more complex picture emerged in relation to other defects, medical history, and perinatal events (table n). 184 children (69%) had at least one additional defect, relevant medical or family history, or a perinatal problem. ,In 115 this occurred as a single additional association TABLE II-ASSOCIATED

DEFECT(S) AND PERINATAL HISTORY IN

CHILDREN WITH SENSORINEURAL DEAFNESS

TABLE III-ADDITIONAL DEFECTS AMONG

266

SERONEGATIVE

CHILDREN WITH SENSORINEURAL DEAFNESS

*Cytomegalovirus isolated from urine of 1 child. in the remaining 51 there were two or more additional associations, which illustrates the extreme complexity of the situation. The role of these adverse factors in the aetiology of deafness, particularly where there was more than one factor, is impossible to determine in this type of study. Nevertheless, as a group the seronegative children with sensorineural deafness were strikingly different from those who were seropositive, although it is not possible to establish whether any of the features we elicited were the cause of deafness. Although a history of infection, trauma, or possible harmful perinatal events should not be given too much significance, since the information was gathered retrospectively, the data are helpful in suggesting possible aetiological relations between early events and sensorineural deafness. Any bias would be reflected equally in both seropositive and seronegative groups, since parents of deaf children are more likely to remember positive events which may explain their child’s deafness.

and

DISCUSSION

study confirms previous observations that conrubella is an important cause of congenital sensorineural deafness35 and that rubella deafness is often the sole manifestation of congenital infection.48 Many of the children in this study who had congenital rubella deafness would never have been identified as such if reliance had been placed solely on a history of maternal rubella infection. Similarly, a diagnosis based on the presence of other rubella-type defects such as cataracts or congenital heart-disease would have identified only 10 (2-9%) children from the 349 with sensorineural deafThis

genital

ness.

*Nos. in parenthesis indicate single association.

Our findings highlight the importance of screening deaf children for rubella antibodies at an early age, to

261

identify those with rubella deafness. Since acquired postnatal infection is uncommon in children aged under 4,6 the presence of rubella antibody in a deaf child of this age suggests a diagnosis of congenital rubella. It is never possible to be certain about the diagnosis in an individual child, particularly when there are no additional rubella-type defects and a negative maternal history, but the earlier the antibody test is done after the age of 6 months, when antibody from the mother will no longer be detected, the more indicative a positive result is of congenital infection. Rubella serology is useful in epidemiological studies to examine the role of rubella as a of sensorineural deafness, because the number of acquired infections can be estimated from data gathered on controls. The significance this has for the confirmation of a diagnosis of rubella embryopathy in the individual child depends, however, on a number of factors. If a young child and the mother are seropositive it is advisable to examine the fundi for retinopathy, since cause

this is one of the most consistent features associated with rubella embryopathy.7 However, this is not a routine part of the examination of a deaf child and requires considerable skill and experience. The seropositive deaf children differed from the seronegative group in that far fewer of them had associated defects, a family history of deafness, or adverse perinatal events. In the seronegative group there was a higher proportion of very light infants, which probably reflects prematurity, often associated with jaundice and adverse perinatal events, as a cause of deafness in some of the seronegative children. However, it was not the purpose of this investigation to determine the causes of sensorineural deafness, and this information has been presented only to show the striking difference between the seropositive and seronegative deaf children. The findings that the seropositive children were more likely to be born in the winter months and to have no maternal history of rubella or rubella contact during pregnancy and that deafness was often the sole manifestation of rubella embryopathy is consistent with the observations on children with congenital rubella notified to the National Congenital Rubella Surveillance Programme.8 Rubella embryopathy is numerically by far the most important known prenatally acquired cause of profound childhood deafness, and the highlighting of such an "avoidable" cause of deafness shows clearly the urgent need for an effective immunisation policy. The prevalence of significant hearing loss in childhood is difficult to determine and varies according to the criteria used to identify such cases. Estimates based on children receiving special education for the deaf or partially hearing result in a prevalence of 1 per 1000 children," whereas rates based on studies on all schoolchildren include less severe forms of deafness and result in a much higher prevalence. In a national follow-up of all children in England, Scotland, and Wales who were born in one week in March, 1958,12 2.5 per 1000 children at age 7 were judged from audiometric screening and clinical assessment to require special education for the partially hearing or a school for the deaf. Half these children had already been placed in such establishments." Thus in a population with 650 000 live births, between 650 and 1625 children will have a significant bilateral hearing loss. The total number of cases of congential rubella deafness will be substantial and in epidemic years could rise to catastrophic figures. 14

and an effort must be made to ensure that the present rubella vaccination programme in the United Kingdom is pursued more actively. Since rubella is numerically such an important cause of deafness, if should be possible to assess the efficacy of rubella vaccine and the selective immunisation policy that has been adopted in this country by monitoring the annual incidence of congenital rubella deafness. If the vaccines used are effective there should be a gradual decline, allowing for fluctuation of rubella in the community. This would not only prevent unnecessary suffering for individual children and their families; it should also reduce the number of children requiring special education for the deaf or partially hearing.

Congenital

rubella is

now

preventable,

This work was carried out when C. S. P. was in receipt of a grant from the Medical Research Council while working at the Institute of Child Health. W. C. M. was supported by a research grant from the Wellcome Trust. We thank the staff of the Nuffield Hearing and Speech Centre for participating in the study, Miss M. Whelan for her help in the analyses, and Miss P. Parekh for preparing the manuscript.

’Requests for reprints should be addressed to C. S. P. REFERENCES 1. 2.

3. 4. 5.

6. 7. 8. 9. 10.

W. P. D., Loy, R. M. Rep. publ. Hlth med. Subj., Lond. no.101. H.M. Stationery Office, 1960. Lundström, R. Acta pœdiat. scand. 1962, 51, suppl. 113, 1. Karmody, C. S. New Engl. J. Med. 1968, 278, 809 Peckham, C. S. Archs Dis. Childh. 1972, 47, 571. Gumpel, S. M., Hayes, K., Dudgeon, J. A. Br. med. J. 1971, ii, 300. Dudgeon, J. A., Peckham, C. S., Marshall, W. C., Smithells, R. W., Sheppard, S. Hlth Trends, 1975,5,75. Frazer, G. R. The Causes of Profound Deafness in Childhood. London, 1976. Sheppard, S., Smithells, R. W., Peckham, C. S., Dudgeon, J. A., Marshall, W. C. Hlth Trends, 1977,9, 38. Dudgeon, J. A., Marshall, W. C., Peckham, C. S. Lancet, 1972, ii, 480. Chamberlain, R. (editor) British Births 1970, vol. 1, The First Week of Life.

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London,1975. 11. Commission of the European Community, Childhood Deafness in the European Community (in the press). 12. Davie, R., Butler, N., Goldstein, H. From Birth to Seven. London, 1972. 13. Sheridan, M. D. Devl. Med. Child Neurol. 1972, 14, 296. 14. Baldursson, G., Bjarnason, O., Halldorsson, S., Júliusdóttir, E., Kjeld, M. Scand. Audiol. 1972, 1, 3.

Diagnosis of Death The Conference of Medical Royal Colleges and their Faculties in the U.K. has prepared the following memorandum. It supplements the report’ on the diagnosis of brain death which appeared in 1976 and which made no

reference to organ transplantation2. 1. In October, 1976, the Conference of Royal Colleges and their Faculties (U.K.) published a report’ unanimously expressing the opinion that "brain death", when it had occurred, could be diagnosed with certainty. The report has been widely accepted. The Conference was not at that time asked whether or not it believed that death itself should be presumed to occur when brain death takes place or whether it would come to some other conclusion. The present report examines this point and should be considered as an addendum to the original report. 2.

Exceptionally,

as a

instantaneously

or

result of massive trauma, death

near-instantaneously.

1. Br. med. J. 1976, ii, 1187; Lancet, 2. Lancet, 1976, ii, 1064.

1976, ii, 1069.

Far

occurs

more

com-