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.

Outcomes and Management of Infants Who Refer Newborn Hearing Screening

“…A rate of 11.8% (2/17) for SNHL due to cCMV is comparable with the 5% reported by Stehel et al 3 The incidence of cCMV as a cause of congenital SNHL varies in the literature but it has been well documented that targeted screening of patients who fail UNHS contributes to earlier diagnosis and potentially beneficial treatments. 1,14,15 This study also supports the previously reported finding that OME and cerumen/vernix are likely underestimated causes of failed screening. 16 In this review, 35 patients (7.8%) had OME as a diagnosis, sometimes in combination with cerumen or SNHL.…”

“…A rate of 11.8% (2/17) for SNHL due to cCMV is comparable with the 5% reported by Stehel et al 3 The incidence of cCMV as a cause of congenital SNHL varies in the literature but it has been well documented that targeted screening of patients who fail UNHS contributes to earlier diagnosis and potentially beneficial treatments. 1,14,15…”

Assessment of Autism Spectrum Disorder in Deaf Adults with Intellectual Disability: Feasibility and Psychometric Properties of an Adapted Version of the Autism Diagnostic Observation Schedule (ADOS-2)

“…Moreover, it is the level of cognitive impairment and not the degree of hearing loss that is associated with ASD severity (Jure et al, 1991 ). For non-syndromic hearing loss (about 60% of congenital hearing loss) the genetic basis is identified for up to 70% of the cases (Satterfield-Nash et al, 2020 ) and does not show any overlap with the far more complex polygenetic architecture of ASD, making it difficult to suggest that deafness increases the risk for autism in general (Szymanski et al, 2012 ). Sensory deprivation per se as causal for ASD is considered refuted (Zafeiriou et al, 2007 ).…”

Newborn Hearing Screening Results of Infants Born To Mothers Who Had COVID-19 Disease During Pregnancy: A Retrospective Cohort Study

“…It is known that some viral infections during pregnancy can affect the developing fetus and cause anomalies. Sensorineural defects are one of the more serious complications of intrauterine exposure to certain viruses, such as the cytomegalovirus (CMV), rubella virus, and varicella-zoster virus, and to certain parasites, such as the toxoplasma gondii (Beswick et al 2012;Gurlek & Colak 2019;Satterfield-Nash et al 2020;Buca et al 2021). The timing of the viral infection is also a significant factor as the placental transmission of some infections is more likely in particular trimesters, and the trimester when exposure occurs will alter the risk for developing an anomaly.…”