Vestibular Impairment and Postural Development in Children With Bilateral Profound Hearing Loss.

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.

Bone Conduction Cervical Vestibular Evoked Myogenic Potentials as an Alternative in Children with Middle Ear Effusion.

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.

Cervical vestibular evoked myogenic potentials in healthy children: Normative values for bone and air conduction.

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.

Audiovestibular Consequences of Congenital Cytomegalovirus Infection: Greater Vulnerability of the Vestibular Part of the Inner Ear.

OBJECTIVES: To investigate the impact of congenital cytomegalovirus infection on cochlear and vestibular function. DESIGN: This retrospective study conducted between March 2014 and March 2020 included children with confirmed congenital cytomegalovirus infection who underwent a complete audio-vestibular evaluation. It included a bithermal caloric test, a video head impulse test and a cervical vestibular evoked myogenic potential associated with a complete hearing assessment. RESULTS: The cohort of 130 children included in the study had a median age of 21 months (interquartile range: 12 to 37 months). Eighty-three children (64%) showed an inner ear impairment (both cochlear and vestibular). The vestibular part of the inner ear was significantly more frequently impaired than the cochlear part (ρ = 0.003). Sixty-two children (48%) showed confirmed hearing impairment. The severity of hearing loss was variable, with a high proportion of profound hearing loss (30/62, 48%), which was often bilateral (47/62, 76%). The vestibular assessment showed a canal function disorder in 67 children (88%) and an otolith function disorder in 63 children (83%; ρ = 0.36). The video head impulse test was significantly less altered (64%) compared with the bithermal caloric test (80%; ρ = 0.02) and the cervical vestibular evoked myogenic potential (83%; ρ = 0.009). Only seven out of 83 children (8%) showed hearing loss without vestibular dysfunction, of which only one had a normal hearing screening test at birth. For the children who passed the hearing screening test at birth and presented an inner ear impairment [n = 36, median age: 16 (11 to 34) months], vestibular disorders were later found in 35 children (97%) and 17 of them (47%) developed hearing loss secondarily. This underlines the importance of assessing both vestibular and auditory parts of the inner ear. When comparing the agreement of cochlear and vestibular impairment, the severity and the laterality of the impairment were low [Cohen's kappa 0.31 (0.22 to 0.40) and 0.43 (0.32 to 0.55), respectively]. CONCLUSION: In our study, we demonstrated that although both cochlear and vestibular parts of the inner ear can be impaired by congenital cytomegalovirus infection, the vestibular part seems more often impaired compared with the cochlear part. This underlines the importance of vestibular evaluation in the follow-up of cytomegalovirus-infected children associated with hearing assessment.

Predictors of cochleovestibular dysfunction in children with congenital cytomegalovirus infection.

The purpose of this study is to assess the predictive factors of both hearing and vestibular impairment in congenitally cytomegalovirus-infected children (cCMV) through a multivariate analysis of clinical and imaging characteristics collected during pregnancy and at birth. This retrospective study was conducted between March 2014 and March 2020, including confirmed congenitally CMV-infected children with a complete vestibular and hearing assessment. Data concerning pregnancy, date of infection, clinical characteristics, and symptomatology at birth were collected. In total, 130 children were included, with a median age of 21 months. Eighty-three children (64%) presented with an inner ear impairment (both cochlear and vestibular impairment). Sex, modality of maternal infection (seroconversion or reactivation), pregnancy term, weight and head circumference at birth, neonatal clinical signs of infection, and treatment were not significantly correlated with inner ear impairment. However, multivariate analysis confirmed that there are two independent predictive factors of inner ear impairment: antenatal imaging lesions (ORa = 8.02 [1.74; 60.27], p-value = 0.01) and infection during the first trimester (ORa = 4.47 [1.21; 19.22], p-value = 0.03). Conversely, infections occurring during the second trimester were rarely associated with inner ear impairment: 4/13 (31%) in our series, with vestibular impairment alone (4/4) and no hearing loss. None of the children infected during the third trimester developed inner ear dysfunction. CONCLUSION: Besides the symptomatic status of the CMV infection at birth, we found that antenatal imaging brain damage and early infection (mainly during the first trimester) constitute the two best independent predictive factors of inner ear involvement in congenitally CMV-infected children. WHAT IS KNOWN: • Congenital cytomegalovirus infection is the leading infectious cause of neurological disabilities and sensorineural hearing loss in children and responsible of vestibular disorders, which are probably underestimated. • No articles have yet defined the predictive factors of the entire inner ear impairment (vestibule and cochlea). WHAT IS NEW: • The timing of the infection during pregnancy (first and second trimester, ORa=4.47) and antenatal imaging lesions (ORa=8.02) are independently predictive (in a multivariate analysis) of inner ear involvement. • The symptomatic status at birth is a poor predictor of inner ear impairment.

Olfactory function in congenital cytomegalovirus infection: a prospective study.

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.

Vestibular Migraine of Childhood and Recurrent Vertigo of Childhood: Diagnostic criteria Consensus document of the Committee for the Classification of Vestibular Disorders of the Bárány Society and the International Headache Society.

This paper describes the diagnostic criteria for "Vestibular Migraine of Childhood", "probable Vestibular Migraine of Childhood" and "Recurrent Vertigo of Childhood" as put forth by the Committee for the Classification of Vestibular Disorders of the Bárány Society (ICVD) and the Migraine Classification subgroup of the International Headache Society. Migraine plays an important role in some subgroups of children with recurrent vertigo. In this classification paper a spectrum of three disorders is described in which the migraine component varies from definite to possibly absent. These three disorders are: Vestibular Migraine of Childhood, probable Vestibular Migraine of Childhood and Recurrent Vertigo of Childhood. The criteria for Vestibular Migraine of Childhood (VMC) include (A) at least five episodes with vestibular symptoms of moderate or severe intensity, lasting between five minutes and 72 hours, (B) a current or past history of migraine with or without aura, and (C) at least half of episodes are associated with at least one migraine feature. Probable Vestibular Migraine of Childhood (probable VMC) is considered when at least three episodes with vestibular symptoms of moderate or severe intensity, lasting between five minutes and 72 hours, are accompanied by at least criterion B or C from the VMC criteria. Recurrent Vertigo of Childhood (RVC) is diagnosed in case of at least three episodes with vestibular symptoms of moderate or severe intensity, lasting between 1 minute and 72 hours, and none of the criteria B and C for VMC are applicable. For all disorders, the age of the individual needs to be below 18 years old. It is recommended that future research should particularly focus on RVC, in order to investigate and identify possible subtypes and its links or its absence thereof with migraine.

Visual searching capabilities in Age-Related Macular Degeneration (AMD) subjects.

A decrease in visual perception with age was observed due to a decline in neurocognitive and visual functions. Previous studies showed that aging affects visual processes and contextual perceptual phenomena. The aim of our study was to explore the effect of age as well as the effect of Age-Related Macular Degeneration (AMD) on the visual searching task. Three groups of twenty-one subjects were recruited: AMD subjects (mean age 72.29 ± 4.83 years); healthy elderly (72.55 ± 4.95); and young healthy volunteers (28.38 ± 2.77). Visual perception functions were evaluated with a modified barrage test developed by Metrisquare. Our results showed that AMD patients and elderly do not differ in terms of time, omissions and errors. However both were slower to perform the visual perception tests when compared with young adults. Regarding the number of errors and omissions, we only found significant differences between the young and the AMD subjects, the later making more omissions and errors respect to young healthy volunteers. Despite AMD patients made more errors likely because to their poor fixation capability, we could conclude that AMD patients, as well as healthy elderly subjects, could compensate their altered visual strategies by taking more time to perform the visual searching tasks, probably due to cortical plasticity.HighlightsAMD patients and healthy elderly subjects do not differ in terms of time, omissions and errors.AMD patients, as well as healthy elderly subjects could compensate their visual difficulties by taking more time to perform the visual searching tasks when compared to young healthy volunteers. It is probably due to cortical plasticity.Note, however that AMD patients could make more errors when compared to young healthy volunteers, probably due to their poor fixation capability.The number of omissions and errors not different in healthy young and in elderly subjects.

Subjective visual vertical in patients with Usher syndrome.

BACKGROUND: Verticality, or more precisely the ability to perceive spatial orientation with regard to gravity, is based on the integration of visual, vestibular and somesthetic information. OBJECTIVE: The purpose of the present study was to compare the subjective visual vertical (SVV) in patients with Usher (type I and type II) with visual or vestibular impairment, and in healthy participants, in order to explore the importance of the visual and vestibular functions on the vertical's perception. METHODS: We evaluated the SVV using a wall housing which projects on the opposite wall a red-light line of about 2 meters, obtained by laser cannon. The evaluation was carried out under two tilt conditions: clockwise and counter-clockwise randomly performed five times in each direction. The response to the SVV task was quantified by the mean of the absolute values of the SVV. RESULTS: Responses to the SVV were significantly less accurate in patients with Usher with respect to healthy participants while it was similar for the two groups of patients with Usher. CONCLUSIONS: We hypothesize that visual inputs play a very important role in the perception of verticality and that the symmetrical bilateral vestibular deficit in Usher type I does not have a strong impact in perception of verticality.

cVEMPs and oVEMPs normative data in Malaysian preschool and primary school-aged children.

OBJECTIVE: Vestibular assessments in children are essential for the early identification of vestibular and balance dysfunctions. Vestibular evoked myogenic potentials, cervical (cVEMPs) and ocular (oVEMPs) have been reported to be feasible and effective when assessing otolith function in children. The main aim of the study was to obtain normative data for cVEMPs and oVEMPs from preschool and primary school-aged Malaysian children. METHODS: A group of 33 healthy children, aged from 5 years 9 months-12 years 4 months (mean ± SD = 8.83 ± 1.92 years), was recruited. Their otolith saccular function was assessed using 750 Hz tone burst for cVEMPs (with ER3A insert phone), while their utricular function was assessed using Brüel & Kjaer Mini-shaker Type 4810 (Naerum, Denmark) for oVEMPs. RESULTS: For cVEMPs, the mean value of P13 latency, N23 latency, P13-N23 interamplitude and asymmetry ratio were 12.62 ± 1.38 ms, 19.85 ± 1.95 ms, 92.47 ± 50.35 µV and 14.03 ± 9.75%, respectively. For oVEMPs, the mean value of N10 latency, P15 latency, N10-P15 interamplitude and asymmetry ratio were 9.23 ± 1.07 ms, 14.41 ± 1.04 ms, 10.32 ± 5.65 µV and 15.84 ± 11.49%, respectively. Two-way ANOVA analysis found that ear laterality and gender had no significant effect on all cVEMPs and oVEMPs parameters. No significant correlation was found between age and all VEMPs parameters. CONCLUSIONS: The normative data for cVEMPs and oVEMPs obtained in this study can be used as a guide by health professionals to assess saccular and utricular functions among children age from 5 to 12 years of age.

Postural Instability in Subjects With Usher Syndrome.

This study investigated postural performances and vestibular impairment in Usher patients. The three groups studied were: 11 patients with Usher type I (with visual and vestibular impairment), 14 patients with Usher type II (with only visual impairment), and 14 healthy control subjects. Postural stability was measured with a Framiral Multitest Equilibre platform with three visual conditions: eyes open (EO), eyes closed (EC), and vision disturbed by optokinetic stimulation (OPT), and two different postural conditions: stable or unstable platform. The surface and mean velocity of the center of pressure displacement (CoP) were measured and a postural instability index (PII) was calculated. Usher type I and II patients were more unstable than control subjects, but only for the unstable platform. Patients with Usher type I (with severe vestibular impairment) were also significantly more unstable than patients with Usher type II (with normal vestibular function) on the unstable platform. The severity of the vestibular impairment was correlated with the surface of the CoP displacement. We suggest that poor postural control of Usher patients is due to the abnormalities in their visual and, when defective, vestibular inputs. Measurements of postural stability on an unstable platform can distinguish type I from type II Usher patients. We emphasize the importance of multisensory evaluation in these patients to guide development of personalized visuo-vestibular rehabilitation techniques to improve their postural stability and improve their quality of life.

Dizziness and Convergence Insufficiency in Children: Screening and Management.

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 <15 min, associated with or alternating with headache (50%). Their exposure to small video screens and TV was intensive (∼3.6 h per day). At M0, all orthoptic and oculomotor parameters were statistically different in patients relative to controls (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.

Changes of spatial and temporal characteristics of dynamic postural control in children with typical neurodevelopment with age: Results of a multicenter pediatric study.

BACKGROUND: The aim of this multicenter study is to investigate the effect of chronological age and gender in postural control. METHODS: To approach an ecological model, we used a multicenter posturography assessment. We analyzed postural control with surface, mean velocity of center of pressure [CoP] and temporal analysis, with Postural Instability Index [PII] being a more sensitive parameter in postural evaluation. A large sample of 156 age- and gender-matched healthy children recruited in several pediatrics hospitals, participated. RESULTS: Our current results showed a significant decrease of all postural parameters (surface, mean velocity of CoP and PII) with age, and only on stable support condition. Our study additionally described a gender effect in conditions where all sensory inputs are most challenged with a mean velocity of CoP being significantly smaller in girls with respect to boys. CONCLUSION: We concluded that postural control improves with age linked with maturation process. Moreover, this maturation process seems not yet achieved at 16.08 years and still ongoing beyond. Interestingly, our result reported specificities linked with gender effect. Indeed, girls and boys do not proceed in the same way to maintain their postural control. We could make hypothesis that more children maintain their postural control efficiently; with a low energy cost, the more they could allocate attention to learning during childhood.

Reduced Balance Restoration Capacities Following Unilateral Vestibular Insult in Elderly Mice.

Acute vestibular syndrome (AVS) is characterized by severe posturo-locomotor and vestibulo-oculomotor impairment and accompanies several types of peripheral vestibulopathies (PVP). We know very little about its etiology, how its various symptoms are expressed and how it evolves with age. Robust repair capabilities of primary vestibular synapses have recently been shown to restore behavioral functionality. In this study, we used a mouse model of an excitotoxically induced unilateral vestibular lesion to compare the ability to restore balance and posture between old and young adult mice. We compared the temporal evolution of the evoked vestibular syndrome using a battery of behavioral tests to follow the evolution of postural-locomotor alterations and equilibrium. For the first time, we show that young adult (3 months) and elderly (22 months) mice are together able to restore normal postural-locomotor function following transient unilateral excitotoxic vestibular insult, though with different time courses. This animal study paves way for future, more detailed studies of how the early postural and locomotor disturbances following a unilateral insult are compensated for by various plasticity mechanisms, and in particular how age influences these mechanisms.

Epidemiology of Vestibular Impairments in a Pediatric Population.

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.

Video Head Impulse Tests with a Remote Camera System: Normative Values of Semicircular Canal Vestibulo-Ocular Reflex Gain in Infants and Children.

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. Vestibulo-ocular reflex (VOR) gain was measured for all canals of 303 healthy subjects, including 274 children (aged 2.6 months-15 years) and 26 adults (aged 16-67). We used the Synapsys® (Marseilles, France) VHIT Ulmer system whose remote camera measures head and eye movements. HITs were performed at high velocities. Testing typically lasts 5-10 min. 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 are more variable among very young children and for the vertical canals, but showed no difference for right versus left head rotations. 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.

Developmental study identifies the ages at which the processes involved in the perception of verticality and in postural stability occur.

AIM: The aim of this study was to understand the role played by visual information on the development of verticality and postural stability in healthy children. METHODS: The study comprised 66 healthy children from 4.0 to 15.7 years of age. Postural performances were recorded with a TechnoConcept platform. At the same time, the children's perception of subjective visual vertical (SVV) was recorded while they adjusted a vertical fluorescent line, either in the dark or in the presence of perturbing visual stimuli. Two testing control conditions without an SVV task were also performed by all of the children: static posturographic recording with open eyes and closed eyes. RESULTS: Postural measurements provided evidence of a correlation between the children's age and the tasks performed. Postural stability improved with age until eight to nine years, and SVV performance improved after 10-11 years. After these ages, postural and SVV capabilities did not change until at least 15 years of age. CONCLUSION: Our findings suggest that the maturation of cortical and central processes involved in both the perception of verticality and in postural stability took place during childhood. However, maturation occurred later for vertical perception, which could imply delayed maturation of sensory integration processes.

Up/down anisotropies of vertical saccades in healthy children depending on the mode and the depth of execution.

While the overall development of saccades in children has recently gained increasing interest, the precise characteristics of vertical saccades remain understudied. The few adult studies existing showed up/down anisotropies on various parameters. This study focuses on the development of vertical saccades and their interaction with vergence movements, according to the mode of initiation of the saccades (automatic and controlled). Eighty six children (9.57±3.15years) performed vertical saccades with an eccentricity of 7.5°, at 40cm and at 150cm viewing distance, in a gap paradigm - automatic saccades - and in an overlap paradigm - more voluntary or controlled saccades. Task and direction effects: latency was overall longer in the overlap than in the gap task, duration was longer for upward than downward saccades, conjugate vertical drift was larger after upward than downward saccades, horizontal vergence was higher during and after downward than upward saccades. Age effects: For upward saccades, amplitude, mean and peak velocity of upward saccades increased with age, while the vertical conjugate drift after downward saccades at far distance decreased with age; for downward saccades in the overlap task, the horizontal convergence increased with age concomitantly with an increase of the duration. The results are discussed in the context of hypothetical differential circuits of automatic and controlled saccades maturing progressively in children and interacting with direction (up/down). We suggest that the up/down asymmetries, more pronounced in the overlap task, are built progressively in mutual interaction with a perceptive peripheral bias, up being perceived as far and down as near.

A case of mild CHARGE syndrome associated with a splice site mutation in CHD7.

CHARGE syndrome (MIM#214800) (Coloboma, Heart defect, Atresia of choanae, Retarded growth and development, Genital hypoplasia, Ear abnormalities/deafness) is caused by heterozygous mutation of CHD7 transmitted in an autosomal dominant manner. In this report, we describe a patient with bilateral hearing impairment, unusually-shaped ears, no intellectual disability and a patent ductus arteriosus. Further investigation showed abnormal semicircular canals and the presence of olfactory bulbs. He does not fulfill the Blake or the Verloes criteria for CHARGE. A de novo mutation at the donor splice site of intron 33 was identified (c.7164 + 1G > A). It is of importance to diagnose mildly affected patients for appropriate genetic counselling and to better understand the mild end of the phenotypic spectrum of CHARGE syndrome.

Vestibular Disorders in Children With Congenital Cytomegalovirus Infection.

BACKGROUND: Congenital cytomegalovirus (CMV) infection is the leading infectious cause of neurologic disabilities and sensorineural hearing loss in children. Sensorineural hearing loss prevalence in CMV suggests a viral tropism for the inner ear. Vestibular disorders induced by CMV infection are underestimated. This is the largest and most thorough study to assess the incidence of vestibular disorders and their correlation with hearing thresholds in children with CMV. METHODS: This retrospective study assessed a cohort of 52 children with congenital CMV infection and sensorineural impairment who received a complete hearing and vestibular assessment. Vestibular evaluation included clinical examination, caloric bithermal test, earth vertical axis rotation, off-vertical axis rotation, and vestibular evoked myogenic potential. The prevalence, progression, and clinical impact of vestibular disorders were studied and correlated with hearing thresholds and the severity of congenital CMV infection. RESULTS: Forty-eight children (92.3%) had hearing loss and vestibular disorders. Of those, vestibular disorders were complete and bilateral in 33.3%, partial and bilateral in 43.7%, and partial and unilateral in 22.9%. Serial testing in 14 children showed stable vestibular function in 50% and deterioration in 50%. Congenital CMV infection has a negative impact on postural development that is correlated with neurologic and vestibular impairment. Vestibular disorders were significantly associated with hearing disorders, but their respective severities showed no concordance. CONCLUSIONS: Vestibular disorders are frequent and severe in CMV-infected children. Routine screening and appropriate management of vestibular lesions is essential to initiate adapted care.