Long-term outcome of vestibular function and hearing in children with congenital cytomegalovirus infection: a prospective cohort study

Abstract

Purpose Congenital cytomegalovirus infection (cCMV) is the most frequent nonhereditary cause for sensorineural hearing loss (SNHL) in children. Data on vestibular function in children with cCMV are, however, scarce, although some evidence for cCMV-associated vestibular dysfunction exists. In this prospective cohort study, we evaluated long-term vestibular function and hearing outcomes in a cohort of children with cCMV. Methods Participants were 6–7-year-old children with cCMV from a large population-based screening study. Controls were age and gender matched healthy children, who were CMV-negative at birth. Hearing was examined with pure tone audiometry. Definition of hearing loss was pure-tone average > 20 dB. Vestibular function was assessed using the video head impulse test that provides a measure of semicircular canal function. Definition of vestibular dysfunction was lateral semicircular canal gain < 0.75. Results Vestibular dysfunction occurred in 7/36 (19.4%) of children with cCMV and in 1/31 (3.2%) of controls (p = 0.060). SNHL was recorded in 4/38 (10.5%) of children with cCMV and in 0/33 of controls (p = 0.118). Hearing loss was unilateral in all cases. In cCMV group, the two children with bilateral vestibular dysfunction also had SNHL, whereas those with unilateral vestibular dysfunction (n = 5) had normal hearing. Conclusions In this cohort of children with cCMV identified using newborn screening, vestibular dysfunction was more common than SNHL at 6 years of age. Vestibular dysfunction occurred both in children with and without SNHL. Based on these data, inclusion of vestibular tests in follow-up protocol of cCMV should be considered.

Vestibular Follow-up Program for Congenital Cytomegalovirus Based on 6 Years of Longitudinal Data Collection

“…This might be largely due to the fact that our proportion of symptomatic patients (45%) was considerably smaller compared with most previous studies in which the majority of patients were symptomatic (Pappas 1983;Karltorp et al 2014;Bernard et al 2015;Chebib et al 2022a). Indeed, a vestibular dysfunction rate (19%) similar to our rate was reported in a recent study of Kokkola et al (2023) in a group of 36 cCMV-patients identified through population-based cCMV screening.…”

“…In fact, as vestibular loss occurred in only 5% of second trimester seroconversions and none of the third trimester seroconversions, these patients and their parents can be fairly reassured. Apart from these 2 predictors that are, to a greater or lesser extent, already described in literature (Zagólski 2008; Karltorp et al 2014; Bernard et al 2015; Chatzakis et al 2020; Dhondt et al 2020; Chebib et al 2022a, 2022b; Kokkola et al 2023), the predictive value of periventricular cysts on MRI also looks promising. When available, these data could add to the accuracy to predict vestibular outcome.…”

“…Incidence rates for vestibular dysfunction in cCMV reported in literature cover a terribly wide range, from 14 to even 92% (Pappas 1983; Karltorp et al 2014; Bernard et al 2015; Dhondt et al 2020; Pinninti et al 2021; Shears et al 2022; Kokkola et al 2023), indicating the need for additional research in large, representative study samples (Shears et al 2022). The unpredictable nature of cCMV also holds true for vestibular outcome, as previous research has provided evidence for a wide range of vestibular dysfunctions, in terms of severity (uni- or bilateral, isolated parts of the vestibular organ or complete dysfunction of all five parts, mild function losses or complete areflexia), onset (present from the first examination onwards or delayed onset), and evolution (stable or progressive) (Pappas 1983; Huygen & Admiraal 1996; Zagólski 2008; Karltorp et al 2014; Bernard et al 2015; Maes et al 2017; Dhondt et al 2019b, 2020; Lazar et al 2021; Pinninti et al 2021; Chebib et al 2022b; Kokkola et al 2023). Although patients with SNHL seem to be most at risk, vestibular losses are also reported in asymptomatic and normal-hearing patients (Dhondt et al 2020; Pinninti et al 2021; Chebib et al 2022b; Kokkola et al 2023).…”

“…The unpredictable nature of cCMV also holds true for vestibular outcome, as previous research has provided evidence for a wide range of vestibular dysfunctions, in terms of severity (uni- or bilateral, isolated parts of the vestibular organ or complete dysfunction of all five parts, mild function losses or complete areflexia), onset (present from the first examination onwards or delayed onset), and evolution (stable or progressive) (Pappas 1983; Huygen & Admiraal 1996; Zagólski 2008; Karltorp et al 2014; Bernard et al 2015; Maes et al 2017; Dhondt et al 2019b, 2020; Lazar et al 2021; Pinninti et al 2021; Chebib et al 2022b; Kokkola et al 2023). Although patients with SNHL seem to be most at risk, vestibular losses are also reported in asymptomatic and normal-hearing patients (Dhondt et al 2020; Pinninti et al 2021; Chebib et al 2022b; Kokkola et al 2023). Taken together, these findings endorse the need for longitudinal follow-up of the vestibular function in all cCMV-patients, as is already well-established for the auditory function (Goderis et al 2014; Fletcher et al 2018; Vos et al 2021).…”

The Audiological Follow-Up of Children with Symptomatic Congenital Cytomegalovirus Infection: An Experience in Two Italian Centers

“…Long-term follow-up of children with cCMV is essential to identify those with late-onset hearing loss [44]. Moreover, as a recent study reported that vestibular dysfunction was more common than SNHL at 6 years of age, the inclusion of vestibular tests in the follow-up protocol for cCMV could also be considered in children with normal hearing [45].…”