Sylvette R. Wiener-Vacher

Importance: Children with profound hearing loss (HL) and vestibular impairment have worse cochlear implant outcomes compared with those without vestibular impairment. However, the decision for cochlear implantation is rarely based on vestibular function assessment as a complement to audiologic testing.Objectives: To identify the prevalence of vestibular impairment according to HL origin and to assess the association between vestibular impairment and delayed posturomotor development in children with profound HL.Design, Setting, and Participants: This cohort study was conducted in a pediatric referral center for cochlear implantation in Paris, France, using medical records data on HL origin, vestibular assessment, and ages of developmental milestone achievement. The cohort included children with profound HL (loss >90 dB HL) who completed vestibular assessment prior to cochlear implantation between January 1, 2009, and December 31, 2019. Data analyses were conducted between January and June 2023.Main Outcomes and Measures: The primary outcome was prevalence of vestibular impairment according to HL origin. Children were classified into 3 groups according to their responses to vestibular testing: normal vestibular function (NVF), partially impaired vestibular function (PVF), and complete bilateral vestibular loss (CBVL). Generalized logit models were performed to evaluate the association between vestibular impairment and causes of HL as well as posturomotor development delay.Results: A total of 592 children were included (308 males [52.0%]; mean [SD] age, 38 [34] months). In children with documented HL origin (n = 266), 45.1% (120) had HL with genetic origin, 50.0% of which were syndromic (mainly Usher and Waardenburg syndromes) and 50.0% were nonsyndromic (mainly associated with connexin 26). Among patients with infectious HL origin (n = 74), 70.3% (52) had cytomegalovirus (CMV) infection. Vestibular impairment was found in 44.4% (263 of 592) of the children; it was mostly symmetrical in 88.9% (526) and was CBVL in 5.7% (34) of the cases. Vestibular impairment was present in 78.3% (47) of children with genetic syndromic HL (56.7% [34] with PVF; 21.7% [13] with CBVL) and in 69.2% (36) of children with CMV infection (57.7% [30] with PVF; 11.5% [6] with CBVL). Genetic syndromic HL origin was found to be more often associated with both PVF and CBVL than other HL causes. The odds of having delays in 4 developmental milestones (head holding, sitting, standing with support, and independent walking) were higher in both PVF and CBVL (eg, head-holding odds ratios: 2.55 and 4.79) compared with NVF, and the age of achieving these milestones was higher in CBVL than PVF (eg, head holding: 7.33 vs 4.03 years; P < .001). All 4 developmental milestones were associated with the degree of vestibular impairment.Conclusions and Relevance: This cohort study found that among children with profound HL, vestibular impairment was prevalent, varied according to HL origin, and associated with posturomotor development; while all developmental milestones were associated with vestibular impairment severity, not all HL causes were associated with vestibular impairment severity. Children with profound HL may benefit from complete vestibular assessment before cochlear implantation, which would support early and adapted management, such as physical therapy for CBVL and cochlear implantation strategy.

Objective: To compare the amplitude ratio and P-wave latency of cervical vestibular evoked myogenic potentials (c-VEMPs) for bone conduction (BC) and air conduction (AC) stimulation in children with otitis media with effusion (OME). Material and methods: This is an observational study of a cohort of 27 children and 46 ears with OME. The c-VEMP amplitude ratio and P-wave latency were compared between BC and AC in children with OME and healthy age-matched children. Results: The c-VEMP response rate in children with OME was 100% when using BC stimulation and 11% when using AC stimulation. The amplitude ratio for BC was significantly higher in the OME group than the age-matched healthy control group (p = 0.004). When focusing on ears with an AC c-VEMP response (n = 5), there was a significant difference in the amplitude ratio between the AC and BC stimulation modes, but there was no significant difference in the AC results between the OME group and the age-matched control group. Conclusions: BC stimulation allows for reliable vestibular otolith testing in children with middle ear effusion. Given the high prevalence of OME in children, clinicians should be aware that recording c-VEMPs with AC stimulation may lead to misinterpretation of otolith dysfunction in pediatric settings.

Objectives: To characterize cervical vestibular evoked myogenic potentials (c-VEMPs) in bone conduction (BC) and air conduction (AC) in healthy children, to compare the responses to adults and to provide normative values according to age and sex.Design: Observational study in a large cohort of healthy children (n = 118) and adults (n = 41). The c-VEMPs were normalized with the individual EMG traces, the amplitude ratios were modeled with the Royston-Wright method.Results: In children, the amplitude ratios of AC and BC c-VEMP were correlated (r = 0.6, p < 0.001) and their medians were not significantly different (p = 0.05). The amplitude ratio was higher in men than in women for AC (p = 0.04) and BC (p = 0.03). Children had significantly higher amplitude ratios than adults for AC (p = 0.01) and BC (p < 0.001). Normative values for children are shown. Amplitude ratio is age-dependent for AC more than for BC. Confidence limits of interaural amplitude ratio asymmetries were less than 32%. Thresholds were not different between AC and BC (88 ± 5 and 86 ± 6 dB nHL, p = 0.99). Mean latencies for AC and BC were for P-wave 13.0 and 13.2 msec and for N-wave 19.3 and 19.4 msec.Conclusion: The present study provides age- and sex-specific normative data for c-VEMP for children (6 months to 15 years of age) for AC and BC stimulation. Up to the age of 15 years, c-VEMP responses can be obtained equally well with both stimulation modes. Thus, BC represents a valid alternative for vestibular otolith testing, especially in case of air conduction disorders.

Congenital cytomegalovirus (CMV) infection leads to olfactory bulb lesions in the fetus, yet little is known about its impact on olfaction after birth. Here, we have assessed in a prospective study conducted on children in two French hospitals from 2016 to 2019, infection severity and olfactory performance after congenital CMV infection. Children with congenital CMV infection aged 3 to 10 years and healthy controls (CTL) matched for age and sex to CMV children symptomatic at birth (sCMV) were enrolled. Olfactory discrimination was assessed using mono-odorants and binary mixtures. Data were analyzed for 54 children with PCR-confirmed congenital CMV infection, including 34 sCMV (median [IQR] age, 6 [5–8] years; 19 [55.9%] male), and 20 CMV asymptomatic at birth (aCMV, median [IQR] age, 4 [3–6] years; 12 [60.0%] male). sCMV were compared to 34 CTL children. Olfactory scores in CMV-infected children were independent from vestibular deficit and hearing loss. The olfactory score was efficient to discriminate between CTL and sCMV for children > 6 years (area under the receiver-operating characteristic curve (AUC, 0.85; P = 0.0006), but not for children < 7 years. For children > 6 years, the proportion of children with total olfactory score < 4 differed between sCMV and CTL groups (91.2% and 18.7%, P < 0.001), but not between aCMV and age-matched healthy control groups.   Conclusion: Congenital CMV infection is associated with reduced olfactory performance in children with infection symptoms at birth.   Clinical trial registration: NCT02782988 (registration date: May 26, 2016). What is Known: •Congenital cytomegalovirus infection leads to olfactory bulb lesions in the fetus, yet little is known about its impact on olfaction after birth. •Depending on neonatal clinical presentation, children are either categorized as having a symptomatic or asymptomatic infection at birth. What is New: •Congenital cytomegalovirus infection is associated with reduced olfactory performance in children with infection symptoms at birth.

Objective: In children screened for dizziness with vergence disorders, we tested short and long term efficacy of orthoptic vergence training (OVT) and instructions to reduce screen usage. Methods: Prospective study: Of the 179 children referred for vertigo or dizziness (over 3 years) with ophthalmological disorder as the only problem after complete oto-neuro-vestibular testing, 69 presented vergence insufficiency, and 49 accepted to participate in this study. 109 healthy children served as controls. All subjects had classic orthoptic evaluation and video binocular movement recordings during various oculomotor tasks. Patients were evaluated before OVT (M0), 3 months after the end of OVT (M3) and 9 months after the end of OVT (M9). Statistics compared orthoptic and oculomotor parameters between patients and controls over time with one-way ANCOVA, and mixed models, controlling for age and gender. Results: Patients reported vertigo that was usually rotatory, lasting p < 0.0001) except for divergence. At M3, vertigo symptoms had disappeared in all of the patients, and all eye movement parameters improved significantly (p < 0.0001). At M9, this improvement remained stable or continued. Conclusion: Vergence disorders (assessed by abnormal orthoptic and oculomotor parameters) can generate symptoms of dizziness in children. Orthoptic treatment and instruction to reduce screen usage has a significant and long term effect on vertigo symptoms as well as oculomotor performances. Dizzy children should be screened for vergence disorders. WHAT THIS STUDY ADDS Dizziness in children can be associated exclusively with insufficient convergence. Orthoptic training and instructions to reduce screen exposure made dizziness symptoms disappear and improved all eye movement parameters for 6 months. Vergence disorders should be screened for in dizzy children.

The purpose of this study was to report the prevalence of vestibular impairment (VI) in children (n = 2,528) referred for complete vestibular testing because of balance disorders (BD) or hearing loss (H). A VI was shown in 51.5% of the children tested (1,304/2,528). For BD (e.g., vertigo, dizziness, instability, delay in posturomotor development), VI was found in 36.5% (n = 379/1,037). The most frequent causes of BD with VI included inner ear malformation (13.5%), delay in posturomotor development (13.4%), hearing loss revealed with vertigo (3.9%), trauma (3.9%), vestibular neuritis (3.3%), meningitis (2.5%), Meniere-like syndrome (1.1%), BPPV posttrauma (1%), labyrinthitis (0.4%), and unknown etiology (19.6%). Normal responses to the complete battery of tests (n = 658, 63.5%) excluded a vestibular origin to BD, leading to other diagnoses: principally migraine (15.6%), ophthalmological disorders (15.1%), neurological disorders (including delay in posturomotor development; 14.4%), orthostatic hypotension, or somatoform dizziness (<1%). Of the children referred for hearing loss (n = 1,491), 68.5% were tested without cochlear implantation (CI; n = 1,022). In this group, 54.5% presented with VI (n = 557). This was mostly found in cytomegalovirus infection, inner ear malformation, and genetic syndromes. Profound hearing loss candidates for cochlear implants had complete bilateral vestibular loss in 20% and delay in posturomotor development, and 80% had partial or normal vestibular function and normal posturomotor development. VI was found after CI in 50% on the side of the implant (partial in 41% and complete in 9%). VI is present in 36.5% of children referred to our center for BDs and 54.5% for hearing loss. Vestibular testing permits ruling out peripheral VI and hence seeking other causes for BDs such as migraine and ophthalmological disorders and also helps lower the risk of inducing bilateral complete vestibular loss in CI protocols.

The Video Head Impulse Test (VHIT) is widely used to identify semicircular canal function impairments in adults. But classical VHIT testing systems attach goggles tightly to the head, which is not tolerated by infants. Remote video detection of head and eye movements resolves this issue and here we report VHIT protocols and normative values for children. VOR gain was measured for all canals of 303 healthy subjects including 274 children (aged 2.6 months to 15 years) and 26 adults (aged 16 to 67). We used the Synapsys® (Marseille, France) VHIT Ulmer system whose remote camera measures head and eye movements. HITs were performed at high velocities. Testing typically lasts 5-10 minutes. In infants as young as 3 months old VHIT yielded good inter-measure replicability. VOR gain increases rapidly until about the age of 6 years (with variation among canals) then progresses more slowly to reach adult values by the age of 16. Values obtained are more variable among very young children and for the vertical canals, but showed no difference for right vs. left head rotations. VHIT reliably measures VOR in very young children. Normative values of VOR gain are presented to help detect vestibular impairment in patients. VHIT testing prior to cochlear implants could help prevent total vestibular loss and the resulting grave impairments of motor and cognitive development in patients with residual unilateral vestibular function.

Verticality is essential in our life, especially for postural stability. Subjective vertical as well as postural stability depends on different sensorial information: visual, vestibular and somesthesic. They help to build the spatial referentials and create a central representation of verticality. Children are more visuo-dependant than adults; however, we did not find any study focusing on how children develop their sense of verticality. We studied two groups of subjects: 10 children (from 6 to 8 years) and 12 young adults. We recorded postural stability with a Techno Concept plateform and perception of subjective visual vertical in the following conditions: while adjusting the vertical in the dark or with visual perturbation, while fixating the vertical bar, and with eyes closed. Children are more instable than adults in terms of postural parameters, and also while performing a double task, especially when no visual references are present. They also present a higher variability and lower accuracy than adults in reporting their perception of true vertical reference. Children might have limited attentional resources, and focus their attention on the more demanding task, corresponding to the U-shaped non-linear model.

Vestibular signals play an essential role in oculomotor and static and dynamic posturomotor functions. Increasing attention is now focusing on their impact on spatial and non-spatial cognitive functions. Movements of the head in space evoke vestibular signals that make important contributions during the development of brain representations of body parts relative to one another as well as representations of body orientation and position within the environment. A central nervous system pathway relays signals from the vestibular nuclei to the hippocampus where this input is indispensable for neuronal responses selective for the position and orientation of the head in space. One aspect of hippocampal systems' processing to create episodic and contextual memories is its role in spatial orientation and navigation behaviors that require processing of relations between background cues. These are also impaired in adult patients with vestibular deficits, however little is known about the impact of vestibular loss on cognitive development in children. This is investigated here with a particular emphasis upon the hypothetical mechanisms and potential impact of vestibular loss at critical ages on the development of respective spatial and non-spatial cognitive processes and their brain substrates.