Elsevier

The Lancet

Volume 352, Issue 9145, 19–26 December 1998, Pages 1957-1964
The Lancet

Articles
Controlled trial of universal neonatal screening for early identification of permanent childhood hearing impairment

https://doi.org/10.1016/S0140-6736(98)06359-4Get rights and content

Summary

Background

Congenital permanent childhood hearing impairment (PCHI) impairs communication skills and, possibly, mental health and employment prospects. Management within 1 year of birth can alleviate most of its adverse effects. Neonatal screening for this disorder is feasible but its benefit for all babies is disputed. We investigated whether neonatal screening of all babies born in hospital, in addition to the standard health visitor distraction test, would increase the rates of early referral, confirmation, and management.

Methods

Between 1993 and 1996, two teams of four part-time testers and equipment moved between two pairs of hospitals to achieve four periods with neonatal screening and four without neonatal screening, each of 4–6 months' duration. Babies did or did not undergo neonatal screening dependent on during which periods they were born. We used a transient evoked otoacoustic emissions test and, in babies who failed this test, an automated auditory brainstem response test on the same day. We referred babies with positive results for audiological assessment.

Findings

53 781 babies were included in the trial, and 25 609 were born during periods with neonatal screening. Neonatal screening achieved 87% coverage of births, with a false-alarm rate of 1·5%, and an overall yield of 90 cases of bilateral PCHI of 40 dB or more relative to hearing threshold level per 100 000 target population, equivalent to 80% of the expected prevalence of the disorder in the population. 71 more babies with moderate or severe PCHI per 100 000 target population were referred before age 6 months during periods with neonatal screening than during periods without. Early confirmation and management of PCHI were significantly increased. The rate of false-negative results from neonatal screening was significantly lower than that for the distraction test (4 vs 27% p=0·041).

Interpretation

Neonatal screening is effective in identification of congenital PCHI early and may be particularly useful for babies with moderate and severe PCHI for whom early management may have the most benefit.

Introduction

Congenital bilateral permanent childhood hearing impairment (PCHI) of 40 dB or more relative to hearing threshold level can be expected to affect 112 babies per 100 000 births in Europe1 and the USA,2 equivalent in the UK to 840 children per year. Early management of hearing impairment may be of greatest benefit to these children, including improved language, communication, mental health, and employment prospects.1, 3

Screening for all infants for hearing impairment is not provided in most European countries or the USA but is undertaken in almost every district in the UK with the health visitor distraction test, at around age 7 months. One person, the distractor, attracts the infant's attention with a visual stimulus directly in front of the child and removes the visual stimulus from the child's view as the tester, out of view, tries to attract the child's attention with a soft auditory stimulus. Evidence of localisation by the child of a series of pre-specified auditory stimuli constitutes a pass. Many districts have reported low specifity, low sensitivity, and poor yield from this test.1 As a consequence, about half of children with PCHI in the UK are not identified by age 18 months, and about a quarter remain undiagnosed at age 42 months.1 The average age of identification of PCHI in the USA in 1993 was about 3 years.2

Neonatal screening for congenital PCHI could avoid delays in detection and intervention. Methods of neonatal screening are available2, 3, 4, 5, 6, 7 and technological advances have increased their effectiveness, especially for detection of transient evoked otoacoustic emissions and automated measurement of the auditory brainstem response.1, 8

Transient evoked otoacoustic emissions, also known as cochlear echoes, are low-intensity sounds originating from the outer hair cells in the cochlea and can be elicited in response to clicks presented to the ear via a lightweight probe. Their detection by a microphone within the same probe is a simple and rapid clinical test for the normal functioning of the middle and inner ear. The auditory brainstem response is an electrical response to auditory stimuli that is generally recorded with three electrodes on the scalp and is a well-established method for assessment of the functioning of the eighth cranial nerve and the auditory pathway in the brainstem

Quality standards and consensus statements for early identification of PCHI have been proposed in the USA,2, 9 the UK,10 and a European Consensus Statement on Neonatal Hearing Screening was made in May, 1998. Especially notable were the recommendations of the National Institutes of Health that all infants should be screened for congenital PCHI before age 3 months and that transient evoked otoacoustic emissions detection and auditory brainstem response measurement be used sequentially as a two-stage universal neonatal screen.2

Although just less than two-thirds of all districts in England and Wales now have some sort of neonatal hearing screening or assessment programmes, they are confined in almost all districts to the 10% of the infant population at increased risk of PCHI because of a history of admission to neonatal intensive-care units or special baby units or a family history of hearing impairment. Such targeted screening, even if completely effective in the high-risk group, would not detect 50% of PCHI that occurs in infants with no identified risk factor.1, 2, 8, 11, 12 In practice, only one in three of the high-risk subpopulation is screened in most programmes in the UK.1 Neonatal screening of all infants has been introduced in two districts in the UK,4, 13 and in some hospitals or regions in Denmark,14 Austria,15 Germany,16 and the USA,11, 17 but its benefit is disputed.18, 19, 20, 21 There have been no controlled trials on the effectiveness of hearing screening in childhood, especially none comparing rates of early diagnosis of congenital PCHI with and without a neonatal screen.

We have found previously that two-stage neonatal screening with transient evoked otoacoustic emissions detection followed by automated auditory brainstem response measurement is more than 99% specific7 and feasible as a universal neonatal screen.22 There are, however, no data on the incremental effect on the early diagnosis of PCHI of such screening. We did a controlled trial to investigate whether the addition of neonatal screening to the traditional health visitor distraction test compared with the distraction test alone at 8 months would increase detection and improve early management of babies with PCHI.

Section snippets

Study design

We included all infants born in the four participating hospitals from October, 1993, to October, 1996. We trained eight testers in September, 1993. All but two were qualified nursery nurses, but none had previous audiology experience. The testers formed two teams of four, and each team worked the equivalent hours each week of 2·5 full-time staff. The team aimed to cover 6 days per week (not Sundays), colleagues' leave, and national holidays except Christmas day.

We estimated, from a previous

Results

There were 53 367 live births in the four participating hospitals during 3 years of the trial. Of these, 25 609 were born during periods of neonatal screening. 21 279 (83·1%) babies underwent neonatal screening, including 82·3% of infants nursed in special-care baby units or neonatal intensive-care units. 1724 (8·1%) screened babies (5·1% on the postnatal wards, 3·0% in special units) had risk factors for PCHI, as defined in National Institutes of Health consensus statements24 (table 2). Birth

Discussion

We have previously reported that this type of screening would be feasible as a screen for all babies, including lowrisk babies,22 who account for about half of the confirmed cases of PCHI.2, 8, 11, 12 In this study, at several hospital sites, there were clear benefits with neonatal screening. The performance of this and other neonatal screens for PCHI have been reported but not been directly compared with detection of PCHI without such screening.7, 11, 13, 14, 25, 26, 27

Randomisation of

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