Background: Permanent bilateral hearing loss (PBHL)  is a serious newborn condition with a prevalence of at least one per 1000 live births.  However, there has been no recent systematic review and meta-analysis of the effectiveness of universal newborn hearing screening programs (UNHS).

Methods: Our protocol was registered on PROSPERO   CRD42020175451. Primary outcomes were any identification of PBHL (ie PBHL diagnosed at any time), age of identification of PBHL and neurodevelopment. Two reviewers searched standard databases to March 2022 and extracted data.  We used fixed and random effects meta-analysis to pool data and graded certainty of evidence using standard methods.

Results : The search resulted in 2,834 records. We identified five studies of 1,023,610 newborns that reported on the effects of UNHS versus no UNHS. The relative risk of infants being identified with PBHL before nine months in infants with UNHS compared to infants without UNHS was 3.28 (95% confidence interval [95% CI] 1.84 to 5.85, 1 study, 1,023,497 newborns, low certainty evidence). The mean difference in the age of identification of PBHL in infants with UNHS compared to infants without UNHS was 13.2 months earlier  (95% CI -26.3 to -0.01,  2 studies, 197 newborns, very low certainty evidence). The relative risk of infants eventually being identified with PBHL in infants with UNHS compared to infants without UNHS was 1.01 (95% CI 0.89 to 1.14, 3 studies, 1,023,497 newborns, low certainty evidence). At latest follow up at 3-8 years the standardised mean difference (SMD) in receptive language development between infants with UNHS compared to infants without UNHS was 0.60 z scores  (95%CI 0.07 to 1.13, 1 study, 101 children, low certainty evidence) and the mean difference in developmental quotients was 7.72 (95%CI -0.03 to 15.47, 3 studies, 334 children, very low certainty evidence). The SMD in expressive language development was 0.39 z scores  (95%CI -0.20 to 0.97, 1 study, 87 children, low certainty evidence) and the mean difference in developmental quotients was 10.10 scores (95%CI 1.47 to 18.73, 3 studies, 334 children, very low certainty evidence).

Conclusions: UNHS programs result in earlier identification of PBHL and may improve neurodevelopment.  UNHS should be implemented across high, middle and low income countries.