Hearing Instability in Children with Congenital Cytomegalovirus: Evidence and Neural Consequences.

OBJECTIVE/HYPOTHESIS: Sensorineural hearing loss (SNHL) is a common sequela of congenital cytomegalovirus (cCMV), potentially exacerbating neurocognitive delay. The objectives of this study were to assess: (1) age at which SNHL in children with cCMV; (2) stimulability of the auditory system in children with cCMV following cochlear implantation (CI); and (3) whether features of magnetic resonance imaging (MRI) potentially are predictive of hearing outcomes. METHODS: In this retrospective study of a prospectively acquired cohort, 123 children with cCMV who were referred for hearing loss at a single tertiary referral hospital over 20 years were compared with an unmatched comparative group of 90 children with GJB2-related deafness. Outcome measures were results of newborn hearing screening (NHS), behavioral audiograms, and, in a subgroup of cochlear implant (CI) users, responses from the auditory nerve and brainstem evoked by CI at initial activation, as well as lesional volume of FLAIR-hyperintense signal alterations on MRI. RESULTS: All but 3 of 123 children with cCMV had confirmed and persistent SNHL. At birth, 113 children with cCMV underwent NHS, 31 (27%) passed in both ears and 23 (20%) passed in one ear (no NHS data in 10 children). At the first audiologic assessment, 32 of 123 (26%) had normal hearing bilaterally; 35 of 123 (28%) had unilateral SNHL; and 57 of 123 (46%) had bilateral SNHL. More than half (67 of 123, 54%) experienced hearing deterioration in at least one ear. Survival analyses suggested that 60% of children who developed SNHL did so by 2.5 years and 80% by 5 years. In the children who passed NHS in one or both ears, 50% developed hearing loss by 3.5 years in the ear, which passed unilaterally (n = 23 ears), and 50% by 5 years in bilateral passes (n = 62 ears). Hearing loss was significant enough in all but one child with isolated high-frequency loss for rehabilitation to be indicated. Hearing thresholds in individual ears were in the CI range in 83% (102 of 123), although duration of deafness was sufficient to preclude implantation at our center in 13 children with unilateral SNHL. Hearing aids were indicated in 16% (20 of 123). Responses from the auditory nerve and brainstem to initial CI stimulation were similar in children with cCMV-related SNHL compared with GJB2-related SNHL. Characteristic white matter changes on MRI were seen in all children with cCMV-related SNHL (n = 91), but the lesion volume in each cortical hemisphere did not predict degree of SNHL. CONCLUSIONS: cCMV-related SNHL is often not detected by NHS but occurs with high prevalence in early childhood. Electrophysiological measures suggest equivalent stimulability of the auditory nerve and brainstem with CI in children with cCMV and GJB2-related SNHL. Hyperintense white matter lesions on FLAIR MRI are consistently present in children with cCMV-related SNHL but cannot be used to predict its time course or degree. Combined, the data show early and rapid deterioration of hearing in children with cCMV-related SNHL with potential for good CI outcomes if SNHL is identified and managed without delay. Findings support universal newborn screening for cCMV followed by careful audiological monitoring. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:S1-S24, 2022.

Exposure to Spoken Communication in Children With Cochlear Implants During the COVID-19 Lockdown.

Importance: The coronavirus disease 2019 (COVID-19) lockdowns in Ontario, Canada in the spring of 2020 created unprecedented changes in the lives of all children, including children with hearing loss. Objective: To quantify how these lockdowns changed the spoken communication environments of children with cochlear implants by comparing the sounds they were exposed to before the Ontario provincial state of emergency in March 2020 and during the resulting closures of schools and nonessential businesses. Design, Setting, and Participants: This experimental cohort study comprised children with hearing loss who used cochlear implants to hear. These children were chosen because (1) their devices monitor and catalog levels and types of sounds during hourly use per day (datalogs), and (2) this group is particularly vulnerable to reduced sound exposure. Children were recruited from the Cochlear Implant Program at a tertiary pediatric hospital in Ontario, Canada. Children whose cochlear implant datalogs were captured between February 1 and March 16, 2020, shortly before lockdown (pre-COVID-19), were identified. Repeated measures were collected in 45 children during initial easing of lockdown restrictions (stages 1-2 of the provincial recovery plan); resulting datalogs encompassed the lockdown period (peri-COVID-19). Main Outcomes and Measures: Hours of sound captured by the Cochlear Nucleus datalogging system (Cochlear Corporation) in 6 categories of input levels (<40, 40-49, 50-59, 60-69, 70-79, ≥80 A-weighted dB sound pressure levels [dBA]) and 6 auditory scene categories (quiet, speech, speech-in-noise, music, noise, and other). Mixed-model regression analyses revealed main effects with post hoc adjustment of confidence intervals using the Satterthwaite method. Results: A total of 45 children (mean [SD] age, 7.7 [5.0] years; 23 girls [51.1%]) participated in this cohort study. Results showed similar daily use of cochlear implants during the pre- and peri-COVID-19 periods (9.80 mean hours pre-COVID-19 and 9.34 mean hours peri-COVID-19). Despite consistent device use, these children experienced significant quieting of input sound levels peri-COVID-19 by 0.49 hour (95% CI, 0.21-0.80 hour) at 60 to 69 dBA and 1.70 hours (95% CI, 1.42-1.99 hours) at 70 to 79 dBA with clear reductions in speech exposure by 0.98 hour (95% CI, 0.49-1.47 hours). This outcome translated into a reduction of speech:quiet from 1.6:1.0 pre-COVID-19 to 0.9:1.0 during lockdowns. The greatest reductions in percentage of daily speech occurred in school-aged children (elementary, 12.32% [95% CI, 7.15%-17.49%]; middle school, 11.76% [95% CI, 5.00%-18.52%]; and high school, 9.60% [95% CI, 3.27%-15.93%]). Increased daily percentage of quiet (7.00% [95% CI, 4.27%-9.74%]) was most prevalent for children who had fewer numbers of people in their household (estimate [SE] = -1.12% [0.50%] per person; Cohen f = 0.31). Conclusions and Relevance: The findings of this cohort study indicate a clear association of COVID-19 lockdowns with a reduction in children's access to spoken communication.