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Unlabelled: Recently, vestibular anomalies have been described as a frequent feature in children with coloboma-heart-atresia-retarded-genital-ear (CHARGE) syndrome. They are likely to play an important role in the psychomotor retardation affecting these children. In order to test this hypothesis, we prospectively performed complete vestibular investigations in a series of 17 CHARGE syndrome patients including inner ear CT scan and functional vestibular evaluation of both canal and otolith functions. These results were correlated with the postural anomalies observed during the children's development and showed that vestibular dysfunction is a constant feature in CHARGE syndrome and has very good sensitivity for confirming the diagnosis. Anomalies of semicircular canals were frequently found (94%), easily detectable on CT scan and associated with no response on canal function evaluation. They were considered as partly responsible for the retardation of postural stages. Vestibular functional tests were consistently abnormal but allowed detection of residual otolith function in most patients (94%). All children of this series had an atypical pattern of postural behaviour that we consider to be related to their vestibular anomalies. Residual otolith function seems to have a positive influence for postural development.Conclusion: Vestibular investigations are valuable for diagnosis, developmental assessment, and adaptation of specific rehabilitation programmes in CHARGE syndrome patients. [ABSTRACT FROM AUTHOR]

Objectives: Cervical vestibular evoked myogenic potentials (c- VEMPs) are feasible and reliable electrophysiological measures that are widely used in adults for evaluating the otolith vestibulospinal function. In children, normative values are still lacking. This study aimed to fully characterize c-VEMPs for bone conduction (BC) and air conduction (AC) c-VEMPs in healthy children. Material and methods: We used a recording protocol and analysis adapted to children as young as 2 months of age. Otolith receptors were stimulated with tone burst (750 Hz, 6.6 ms duration, 4.1 pps) delivered either by BC (B71vibrator on the mastoid) or AC (headphones). The c-VEMPs were normalized using EMG quantification from individual-trace EMGs. Measurements included N-P wave amplitude ratio, threshold, as well as P- and N-wave latency. 118 healthy (=236 ears) children with bilateral type A tympanogram (aged 2 months to 15 years; mean ±SD: 8.7±4.5 years; 60 boys) were included. Results: AC and BC c-VEMP amplitude ratios were significantly correlated (r=0.6, p<0.001) with no significant difference in the amplitude ratio (p>0.05) between BC and AC. There was no significant difference between BC and AC c-VEMP. Finally, there was no relevant difference (p=0.69) for P- and N-wave latency between BC and AC. Conclusions: Results indicate that BC and AC stimulation can be used indifferently for c-VEMP recordings up to 15 years of age but not in adults. Therefore, BC c-VEMP test should be part of the vestibular evaluation test battery in children with balance disorders. [ABSTRACT FROM AUTHOR]

The article evaluates clinical application, in terms of both fitting and everyday use, of the Auria Behind The Ear (BTE) sound processor in children. Particular attention was given to psychophysical program levels, speech perception abilities, comfort, ease of handling, confirmation of system function, battery life and ease of fitting.

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., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: In the context of the SARS-CoV-2 pandemic, patients may have been dissuaded from seeking consultation, thus exposing themselves to a risk of loss of chance. This guide aims to define how teleconsultation can assist in assessing vertiginous adults or children, and to gather the information needed to provide quick medical care., Methods: These recommendations rely on the authors' experience as well as on literature. A survey on otoneurologic approach via telemedicine has been conducted based on a literature search until March 2020., Results: The first clinical assessment of the vertiginous patient via teleconsultation can only be successful if the following conditions are met: initial contact to verify the feasibility of the assessment at a distance, the presence of a caregiver in order to assist the patient, the possibility of making video recordings. Medical history via telemedicine, as in a face-to-face assessment, allows to assess the characteristics, duration, frequency, and potential triggering factors of the vertigo, in both children and adults. During teleconsultation, the following tests can be carried out: oculomotricity evaluation, assessment of balance, simple neurological tests, checking for positional vertigo/nystagmus and, eventually to perform canalith-repositioning procedures. In children, the following should be searched for: history of hearing or visual impairment, a context of fever or trauma, otorrhea, signs of meningeal irritation., Conclusion: The neurotologic telemedicine relies on the accuracy of the clinical assessment, which is based on history taking and a few simple tests, encouraging the development of a decision-making algorithm adapted for teleconsultation. However, the latter has its limitations during an emergency examination of a new patient presenting vertigo, and, at least in some cases, cannot replace a face-to-face consultation. Teleconsultation is often adapted for follow-up consultations of previously selected vertiginous patients during face-to-face assessment., (Copyright © 2020. Published by Elsevier Masson SAS.)

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.

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.

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.

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., (Copyright © 2018. Published by Elsevier B.V.)

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.

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., (©2016 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

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., (Copyright © 2016. Published by Elsevier Ltd.)

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., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Eye movements affect postural stability in children. The present study focuses on the effect of different types of eye movements on postural stability in healthy children. Both eye movements and postural stability have been recorded in 51 healthy children from 6.3 to 15.5 years old. Eye movements were recorded binocularly with a video oculography (MobilEBT(®)), and postural stability was measured while child was standing on a force platform (TechnoConcept(®)). Children performed three oculomotor tasks: saccades, pursuits and reading a text silently. We measured the number of saccades made in the three oculomotor tasks, the number of words read, and the surface area, the length and mean velocity of the center of pressure (CoP). According to previous studies, postural control improves with age until 10-12 years. Saccades toward a target as well as during a reading task reduce significantly the CoP displacement and its velocity, while during pursuit eye movements all children increase postural parameters (i.e., the surface area, the length and mean velocity of the CoP). These results suggest the presence of an interaction between the oculomotor control and the postural system. Visual attention to perform saccades (to stationary targets or to words) influences postural stability more than the frequency of saccade triggering does., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

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., (Copyright © 2015 by the American Academy of Pediatrics.)

Purpose: We compared postural stability and subjective visual vertical performance in a group of very preterm-born children aged 3-4 years and in a group of age-matched full-term children., Materials and Methods: A platform (from TechnoConcept) was used to measure postural control in children. Perception of subjective visual vertical was also recorded with posture while the child had to adjust the vertical in the dark or with visual perturbation. Two other conditions (control conditions) were also recorded while the child was on the platform: for a fixation of the vertical bar, and in eyes closed condition., Results: Postural performance was poor in preterm-born children compared to that of age-matched full-term children: the surface area, the length in medio-lateral direction and the mean speed of the center of pressure (CoP) were significantly larger in the preterm-born children group (p < 0.04, p < 0.01, and p < 0.04, respectively). Dual task in both groups of children significantly affected postural control. The subjective visual vertical (SVV) values were more variable and less precise in preterm-born children., Discussion-Conclusions: We suggest that poor postural control as well as perception of verticality observed in preterm-born children could be due to immaturity of the cortical processes involved in the motor control and in the treatment of perception and orientation of verticality.

The aim of this study was to explore further the development of postural control in healthy children. The novelty of this study was to resort to both spatial and temporal analysis of the center of pressure (CoP). Forty-six healthy children from 4 to 16 years old (mean age: 9.1±3 years) and a group of 13 healthy adults (mean age: 25±3 years) participated to this study. Postural control was tested on both a stable and an unstable platform in three different visual conditions: eyes open fixating a target, under optocinetic stimulation, and eyes closed. Resul*ts showed a significant decrease of both surface area as well as mean velocity of the center of pressure (CoP) during childhood. With the children's increasing age, the spectral power indices decreased significantly and the canceling time increased significantly. Such improvement in postural control could be due to a better use of sensorial inputs and cerebellar integration during development, allowing subjects to achieve more efficient postural control., (Published by Elsevier Ltd.)

Objective: To examine the effect of proprioceptive information during postural control in strabismic children., Methods: Postural stability was recorded with a platform (Techno Concept®) in 12 strabismic children aged from 4.9 to 10 years and data were compared to that of 12 control age-matched children. Two postural positions were performed: Romberg and Tandem. Two postural conditions: without and with foam pad. We analyzed the surface area, the length, the mean speed of the center of pressure (CoP) and the effect of proprioceptive information., Results: Strabismic children are more instable than control age-matched children. The surface, the length and the mean speed of CoP are significantly higher in strabismic children than in control age-matched children. Both groups are more instable in Tandem position than in Romberg position. Finally, strabismic children use more proprioceptive information than control age-matched children., Conclusion: For both Romberg and Tandem position, strabismic children are more instable than control age-matched children. Strabismic children use proprioceptive information more than control age-matched children to control their posture., Significance: Proprioceptive inputs are important for control posture particularly for strabismic population.

Purpose: The objective of our study was to examine horizontal smooth pursuit performance in strabismic children and in children with vergence deficits, and to compare these data with those recorded in a group of control age-matched children., Methods: Binocular eye movements were recorded by video-oculography in ten strabismic children (mean age: 9.8±0.8) and seven children with vergence deficits (mean age: 10.8±0.6). Data were compared to that of age-matched control children (mean age: 9.8±0.8 years)., Results: Catch-up saccades amplitude in strabismic children and in children with vergence deficits were significantly higher than in control age-matched children. Moreover, in strabismic children the amplitude of catch-up saccades was significantly higher in rightward than in leftward direction. The number of catch-up saccades was also significantly higher in rightward than in leftward direction. The gain value of pursuits in rightward direction was significantly higher in the right eye than in the left one; for the right eye, the gain value was significantly higher in rightward than in leftward direction. Binocular coordination of pursuit was better in control age-matched children than in children with vergence deficits and than in strabismic children., Conclusions: Binocular coordination of pursuit is abnormal in children with vergence deficits and worse in strabismic children. Binocular vision plays an important role in improving binocular coordination of pursuit.

Introduction: Dual-task performance is known to affect postural stability in children. This study focused on the effect of oculomotor tasks like saccadic eye movements on postural stability, studied in a large population of children by recording simultaneously their eye movements and posture., Materials and Methods: Ninety-five healthy children from 5.8 to 17.6 years old were examined. All children were free of any vestibular, neurological, ophtalmologic and orthoptic abnormalities. Postural control was measured with a force platform TechnoConcept®, and eye movements with video oculography (MobilEBT®). Children performed two oculomotor tasks: fixation of a stable central target and horizontal saccades. We measured the saccade latency and the number of saccades during fixation as well as the surface, length and mean velocity of the center of pressure., Results: During postural measurement, we observed a correlation between the age on the one hand and a decrease in saccade latency as well as an improvement in the quality of fixation on the other. Postural sway decreases with age and is reduced in the dual task (saccades) in comparison with a simple task of fixation., Discussion - Conclusion: These results suggest a maturation of neural circuits controlling posture and eye movements during childhood. This study also shows the presence of an interaction between the oculomotor system and the postural system. Engaging in oculomotor tasks results in a reduction of postural sway.

Introduction: 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., Methods: 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., Results: 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., Discussion: Children might have limited attentional resources, and focus their attention on the more demanding task, corresponding to the U-shaped non-linear model.