ContextPermanent childhood hearing loss (PCHL) can affect speech, language, and wider outcomes. Adverse effects are mitigated through universal newborn hearing screening (UNHS) and early intervention.ObjectiveWe undertook a systematic review and meta-analysis to estimate prevalence of UNHS-detected PCHL (bilateral loss ≥26 dB HL) and its variation by admission to neonatal intensive care unit (NICU). A secondary objective was to report UNHS programme performance (PROSPERO: CRD42016051267).Data sourcesMultiple electronic databases were interrogated in January 2017, with further reports identified from article citations and unpublished literature (November 2017).Study selectionUNHS reports from very highly-developed (VHD) countries with relevant prevalence and performance data; no language or date restrictions.Data extractionThree reviewers independently extracted data and assessed quality.ResultsWe identified 41 eligible reports from 32 study populations (1799863 screened infants) in 6195 non-duplicate references. Pooled UNHS-detected PCHL prevalence was 1.1 per 1000 screened children (95% confidence interval [CI]: 0.9, 1.3; I2 = 89.2%). This was 6.9 times (95% CI: 3.8, 12.5) higher among those admitted to NICU. Smaller studies were significantly associated with higher prevalences (Egger’s test: p = 0.02). Sensitivity and specificity ranged from 89–100% and 92–100% respectively, positive predictive values from 2–84%, with all negative predictive values 100%.LimitationsResults are generalisable to VHD countries only. Estimates and inferences were limited by available data.ConclusionsIn VHD countries, 1 per 1000 screened newborns require referral to clinical services for PCHL. Prevalence is higher in those admitted to NICU. Improved reporting would support further examination of screen performance and child demographics.