Controlled trial of universal neonatal screening for early identification of permanent childhood hearing impairment: coverage, positive predictive value, effect on mothers and incremental yield

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Abstract
Object: Congenital bilateral permanent childhood hearing impairment (PCHI) impairs communication skills and, in some cases, mental health and employment prospects. Management of PCHI within the first year of life can alleviate most of its adverse effects. We investigated whether neonatal screening of all babies born in hospital, in addition to the standard Health Visitor Distraction Test (HVDT), would increase the rates of early diagnosis. 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 mo duration in each hospital. Babies did or did not undergo neonatal screening dependent on the periods during which they were born. We used a transient evoked oto-acoustic emissions (TEOAE) test followed, in infants who failed this test, by an automated auditory brainstem response (AABR) test on the same day. We referred babies with positive results for audiological assessment. Results: 53 781 infants were included in the trial, including 25609 born during periods of neonatal screening. The neonatal screen achieved 87% coverage of inborn births, with a false alarm rate of 1.5%, and an overall yield from the screen of 90 cases of bilateral PCHI ≥ 40dB HTL per 100000 target population, equivalent to 80% of the expected prevalence of the condition in the population. Seventy-one more babies with moderate or severe PCHI per 100000 target population were referred before age 6 mo during periods with neonatal screening than during periods without. Early confirmation and management of PCHI were significantly increased. The false-negative rate of neonatal screening was significantly lower than that of HVDT screening (4% vs 27%). Conclusions: Neonatal screening is effective in identification of congenital PCHI and may be particularly useful for babies with moderate and severe PCHI for whom early management may have the most benefit.

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