Prevalence of permanent childhood hearing loss detected at the universal newborn hearing screen: Systematic review and meta-analysis

Abstract

Context Permanent childhood hearing loss (PCHL) can affect speech, language, and wider outcomes. Adverse effects are mitigated through universal newborn hearing screening (UNHS) and early intervention. Objective We 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 sources Multiple electronic databases were interrogated in January 2017, with further reports identified from article citations and unpublished literature (November 2017). Study selection UNHS reports from very highly-developed (VHD) countries with relevant prevalence and performance data; no language or date restrictions. Data extraction Three reviewers independently extracted data and assessed quality. Results We 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; I 2 = 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%. Limitations Results are generalisable to VHD countries only. Estimates and inferences were limited by available data. Conclusions In 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.

Results of a Government-supported Newborn Hearing Screening Pilot Project in the 17 Cities and Provinces from 2014 to 2018 in Korea

“…Subsequently, the overall hearing loss adjusted prevalence with ABR rate was 0.64% (0.35% on one side, 0.29% on both sides). The prevalence of UNHS-detected hearing loss in highly developed countries is 0.11% 20 ; however, it is difficult to simply compare the prevalence of hearing loss because the definition of hearing loss and the diagnostic ABR rate varies by countries and papers. The government needs to make efforts to increase the ABR performing rate after an NHS test, not to fade the meaning of EHDI to help children with hearing loss.…”

Arterial spin labeling brain MRI study to evaluate the impact of deafness on cerebral perfusion in 79 children before cochlear implantation

“…One in 1000 newborns are identified with severe to profound hearing loss at birth ( Butcher et al, 2019 ). Approximately 80% of prelingual deafness is due to genetic factors ( Marlin et al, 2005 ) with about a hundred identified gene abnormalities ( Ideura et al, 2019 , Bai et al, 2019 ).…”

A new approach to estimating the prevalence of hereditary hearing loss: An analysis of the distribution of sign language users based on census data in Russia

“…Universal newborn hearing screening (UNHS) programs are potentially the best source of data on the prevalence of congenital deafness [4][5][6]. The rate of congenitally deaf infants is generally considered to vary from 1 to 3 per 1,000 newborns in developed countries and up to 24 per 1,000 newborns in developing countries due to the higher presence of risk factors [4][5][6][7][8][9]. Despite all benefits of the UNHS data, a very limited number of countries (mostly highly developed countries) have massively adopted such programs, and the reported rates of congenital deafness are not applicable for other parts of the world [4,7,9].…”

“…The rate of congenitally deaf infants is generally considered to vary from 1 to 3 per 1,000 newborns in developed countries and up to 24 per 1,000 newborns in developing countries due to the higher presence of risk factors [4][5][6][7][8][9]. Despite all benefits of the UNHS data, a very limited number of countries (mostly highly developed countries) have massively adopted such programs, and the reported rates of congenital deafness are not applicable for other parts of the world [4,7,9]. The lack of systematic reporting and analysis of data also greatly affects the correct evaluation of congenital HL prevalence by the UNHS [4,7,9].…”

“…Despite all benefits of the UNHS data, a very limited number of countries (mostly highly developed countries) have massively adopted such programs, and the reported rates of congenital deafness are not applicable for other parts of the world [ 4 , 7 , 9 ]. The lack of systematic reporting and analysis of data also greatly affects the correct evaluation of congenital HL prevalence by the UNHS [ 4 , 7 , 9 ].…”