Objectives To assess the prevalence, characteristics, and risk of sensorineural hearing loss (SNHL) through 18 years of age in children with congenital CMV infection identified through hospital-based newborn screening who were asymptomatic at birth compared to uninfected children. Methods We included 92 case-patients and 51 controls assessed using auditory brainstem response and behavioral audiometry. We used Kaplan-Meier survival analysis to estimate prevalence of SNHL, defined as ≥25 dB hearing level (HL) at any frequency, and Cox proportional hazards regression analyses to compare SNHL risk between groups. Results At the end of follow-up, SNHL prevalence was 25% (95% CI: 17–36%) among case-patients and 8% (95% CI: 3–22%) in controls (hazard ratio (HR): 4.0; 95% CI: 1.2–14.5; p-value=0.02). Among children without SNHL by age 5 years, the risk of delayed-onset SNHL was not significantly greater for case-patients than for controls (HR: 1.6; 95% CI: 0.4–6.1; P=0.5). Among case-patients, the risk of delayed-onset SNHL was significantly greater among those with unilateral congenital/early-onset loss than those without (hazard ratio: 6.9; 95% CI: 2.5–19.1; P<0.01). At the end of follow-up, the prevalence of severe to profound bilateral SNHL among case-patients was 2% (95% CI: 1–9%). Conclusions Delayed-onset and progression of SNHL among children with asymptomatic congenital CMV infection continued to occur throughout adolescence. However, the risk of developing SNHL after age 5 years among case-patients was not different than in uninfected children. An estimated 2% of case-patients developed SNHL severe enough to be candidates for cochlear implantation.

ObjectiveTo assess long-term outcomes of children with symptomatic congenital cytomegalovirus (CMV) disease detected at birth.MethodsWe used Cox regression to assess risk factors for intellectual disability (intelligence quotient <70), sensorineural hearing loss (SNHL; hearing level ≥25 dB in any audiometric frequency), and vision impairment (best corrected visual acuity >20 or based on ophthalmologist report).ResultsAmong 76 case-patients followed through median age of 13 (range: 0–27) years, 56 (74%) had SNHL, 31 (43%, n=72) had intellectual disability, and 18 (27%, n=66) had vision impairment; 28 (43%, n=65) had intellectual disability and SNHL with/without vision impairment. Microcephaly was significantly associated with each of the three outcomes. Tissue destruction and dysplastic growth on head computed tomography scan at birth was significantly associated with intellectual disability and SNHL.ConclusionInfants with symptomatic congenital CMV disease may develop moderate to severe impairments, which were associated with presence of microcephaly and brain abnormalities.