Dataset Information

Etiology of Prelingual Hearing Loss in the Universal Newborn Hearing Screening Era: A Scoping Review.

ABSTRACT:

Objective

To conduct a scoping review on etiologic investigation of prelingual hearing loss among children <2 years of age in the era of universal newborn hearing screening (UNHS).

Data sources

PubMed, Embase, PsycInfo, CINAHL, and Cochrane Library databases.

Review methods

We searched for articles published from January 1, 1998, to February 19, 2020. We reviewed studies that (1) included children identified with either congenital or delayed-onset hearing loss before 2 years of age among cohorts who had undergone UNHS and (2) investigated ≥1 etiologies of hearing loss. We defined hearing loss as congenital when confirmed after UNHS failure and as delayed onset when diagnosed after ≥1 assessments with normal hearing.

Results

Among 2069 unique citations, 115 studies met criteria for full-text assessment, and 20 met our inclusion criteria. Six studies tested children diagnosed with hearing loss for genetic etiology, 9 for congenital cytomegalovirus (CMV) infection, and 5 for both. Among 1787 children with congenital hearing loss and etiologic investigation, 933 (52.2%) were tested for genetic mutations and 1021 (57.1%) for congenital CMV infection. The proportion of congenital hearing loss cases attributable to genetic etiology ranged between 7.7% and 83.3% and to congenital CMV infection between 0.0% and 32.0%.

Conclusion

Data are lacking on the identification and etiology of delayed-onset hearing loss in children <2 years of age in the UNHS era. The proportion of congenital hearing loss cases attributable to genetic etiologies and congenital CMV infection appears to vary widely.

SUBMITTER: Satterfield-Nash A

PROVIDER: S-EPMC7541667 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:BackgroundPermanent bilateral hearing loss (PBHL) is a serious condition in newborns, 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).MethodsWe registered our study protocol 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 the certainty of evidence using standard methods.ResultsThe search retrieved 2834 records. We identified five studies reporting on the effects of UNHS vs no UNHS in 1 023 610 newborns. The relative risk of 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, 5.85, one 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, -0.01, two 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, 1.14, three studies, 1 023 497 newborns, low certainty evidence). At the 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, 1.13, one study, 101 children, low certainty evidence) and the mean difference in developmental quotients was 7.72 (95% CI = -0.03, 15.47, three studies, 334 children, very low certainty evidence). The SMD in expressive language development was 0.39 z scores (95% CI = -0.20, 0.97, one study, 87 children, low certainty evidence) and the mean difference in developmental quotients was 10.10 scores (95% CI = 1.47, 18.73, 3 studies, 334 children, very low certainty evidence).ConclusionsUNHS programs result in earlier identification of PBHL and may improve neurodevelopment. UNHS should be implemented across high-, middle-, and low-income countries.RegistrationPROSPERO (CRD42020175451).

Project description:Background An effective newborn hearing screening programme has low referral rate and low loss to follow-up (LTFU) rate after referral from initial screening. This systematic review identified studies evaluating the effect of protocol and programme factors on these two outcomes, including the screening method used and the infant group. Methods Five databases were searched (latest: April 2021). Included studies reported original data from newborn hearing screening and described the target outcomes against a protocol or programme level factor. Studies were excluded if results were only available for one risk condition, for each ear, or for < 100 infants, or if methodological bias was observed. Included studies were evaluated for quality across three domains: sample, screening and outcome, using modified criteria from the Ottawa-Newcastle and QUADAS-2 scales. Findings from the included studies were synthesised in tables, figures and text. Results Fifty-eight studies reported on referral rate, 8 on LTFU rate, and 35 on both. Only 15 studies defined LTFU. Substantial diversity in referral and LTFU rate was observed across studies. Twelve of fourteen studies that evaluated screening method showed lower referral rates with aABR compared to TEOAE for well babies (WB). Rescreening before hospital discharge and screening after 3 days of age reduced referral rates. Studies investigating LTFU reported lower rates for programmes that had audiologist involvement, did not require fees for step 2, were embedded in a larger regional or national programme, and scheduled follow-up in a location accessible to the families. In programmes with low overall LTFU, higher LTFU was observed for infants from the NICU compared to WB. Conclusion Although poor reporting and exclusion of non-English articles may limit the generalisability from this review, key influential factors for referral and LTFU rates were identified. Including aABR in WB screening can effectively reduce referral rates, but it is not the only solution. The reported referral and LTFU rates vary largely across studies, implying the contribution of several parameters identified in this review and the context in which the programme is performed. Extra attention should be paid to infants with higher risk for hearing impairment to ensure their return to follow-up. Supplementary Information The online version contains supplementary material available at 10.1186/s12887-022-03218-0.