Objective: To assess the performance of the universal newborn hearing screen in England. Design: Retrospective analysis of population screening records. Study sample: A total of 4 645 823 children born 1 April 2004 to 31 March 2013. Results: 97.5% of the eligible population complete screening by 4/5 weeks of age and 98.9% complete screening by three months of age. The refer rate for the 12/13 birth cohort is 2.6%. The percentage of screen positive (i.e. referred) babies commencing follow up by four weeks of age and six months of age is 82.5% and 95.8% respectively. The yield of bilateral PCHL from the screen is around 1/1000. For bilateral PCHL in the 12/13 birth cohort the median age is nine days at screen completion, 30 days at entry into follow up, 49 days at confirmation, 50 days at referral to early intervention, and 82 days at hearing-aid fitting. Conclusion: The performance of the newborn hearing screening programme has improved continuously. The yield of bilateral PCHL from the screen is about 1/1000 as expected. The age of identification and management is well within the first six months of life, although there remains scope for further improvement with respect to timely entry into follow up.

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We have used a comprehensive register of hearing-impaired children born in the former Oxford Health Region to study risk factors for sensorineural hearing loss. The occurrence of a wide variety of risk factors was documented from the case notes of 145 children; these were all the cases known at the time of the study with all degrees of hearing loss born between 1984 and 1988. Comparison with the normal Regional population showed that maternal age over 35 years and Asian ethnic origin were significant risk factors for congenital (non-acquired) hearing loss (odds ratio 1.7 and 2.5 respectively). Black/Asian children were also significantly more likely to have acquired losses. Low birthweight (below 2500 g) also gave a significantly increased risk, with an odds ratio of 4.5, rising to 9.6 for birthweight less than 1500 g. We also found that significantly more hearing-impaired cases were in lower social classes compared with the general population. A high proportion of cases (24%) had cranio-facial abnormalities (CFA), including many non-aural abnormalities and dysmorphic features, which therefore should be counted as high risk. Hearing losses acquired due to perinatal causes were almost all mild or moderate. Four factors-admission to special care baby unit for more than 72 hours, CFA, family history, and meningitis-accounted for 69% of all cases in this study. Targeted neonatal screening based on the first three factors, plus obligatory testing following meningitis, therefore, should be highly efficient at detecting deafness early.

Otoacoustic emission (OAE) screening and oto-admittance testing (678 Hz probe tone) were performed on both ears of 84 special care neonates, as part of a larger study of middle-ear effusion in neonates and infants. OAE results, tympanometry, and acoustic reflex results are all strongly and significantly associated. No evidence was found of any maturational effects in the results. Based on the findings, a tentative classification scheme for neonatal tympanograms is suggested. We conclude that 678 Hz tympanometry is a useful indicator of middle-ear status in very young babies, and that middle-ear effusion does strongly affect OAEs in neonates. OAEs are also strongly affected by negative middle-ear pressure (MEP), and mean MEP in ears failing OAE screens was significantly more negative than in those passing. The prevalence of abnormal tympanometry, which may indicate middle-ear effusion or dysfunction, was 20% of ears (29% of babies) in this group. It appears that middle-ear effusion could account for about half of the ears failing an OAE screen on the special care baby unit. We also find that length of stay on the special care baby unit is an important risk factor for development of middle-ear effusion: those on the unit for over 30 days have about four times the risk of bilateral abnormal tympanometry.

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