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Purpose: Showing the interest of vestibular evoked myogenic potentials in paediatric neurological vestibulospinal pathology detection and followup. Materials and methods: The vestibular evoked myogenic potentials testing apparatus presented is now commonly used in ENT clinics for patients from 1 month of age. Our system and protocol permits control to evoke and select the best EMG level and makes possible a comparison of data from different sides or level of stimulation or different sessions. Normal vestibular evoked myogenic potentials latencies obtained with tone bursts were remarkably stable (P: 13 +/- 0.8 ms, N: 19.6 +/- 1.6 ms). The reported case illustrates abnormal vestibular evoked myogenic potentials latencies in neuropathy. Results: A 6 y.o. child with progressive imbalance was referred to the ENT department for vestibular functional evaluation. Abnormally long latencies in the vestibular evoked myogenic potentials and neurological examination oriented the diagnosis towards Guillain-Barre syndrome and immediate referral to a neurology department. Vestibular evoked myogenic potentials also helped to monitor the neurological recovery. Conclusion: The present case shows the potential value of vestibular evoked myogenic potentials in diagnosis and evaluation of descending brainstem pathways in neuropathies like Guillain-Barre syndrome in complement to neurological evaluation. [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]

Introduction Chez l’enfant dyslexique (Kapoula et Bucci 2007), ainsi que chez l’enfant strabique (Gaertner et al., 2013), l’exécution de mouvements actifs de vergence entre une cible proche et une lointaine améliore la stabilité posturale. Cette étude développementale explore l’effet de la vergence sur la stabilité posturale chez des enfants de 6 à 17 ans présentant un trouble de vergence. Matériel et méthode Trente-sept enfants (11 ± 3 ans) se plaignant de vertiges mais sans troubles vestibulaire ont réalisé trois conditions expérimentales : fixation proche à 40 cm (angle de vergence 9°) fixation lointaine à 150 cm (angle de vergence 2°), et mouvements actifs de vergence entre deux diodes à 20 cm (18° de convergence) et 70 cm (5°). La posturographie en position orthostatique a été enregistrée avec la plate-forme Techno Concept (25,6 s). Les tests ont été réalisés avant et après rééducation orthoptique de la vergence (12 séances). Résultats La surface est significativement plus faible en condition vergence active comparé à la fixation lointaine après rééducation seulement ( p < 0,009). La surface d’oscillation est significativement plus faible dans la condition de fixation proche (179 ± 173 mm 2 ) après la rééducation orthoptique qu’avant (211 ± 122 mm 2 ). De plus, la variance de la vitesse est plus faible après la rééducation qu’avant en fixation lointaine. Discussion – conclusion Ces résultats confirment que la vergence, même dysfonctionnant, joue un rôle important dans la posture. La rééducation a un effet bénéfique sur la stabilité posturale, surtout en condition de fixation proche ou l’angle de vergence requis est fortement convergent. [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.

Saccades and vergence were studied in adults with vertigo but without abnormal vestibular function; vertigo was associated with headaches and occurred mostly after prolonged computer work. Horizontal eye movements from both eyes were recorded simultaneously with the IRIS SKALAR device. Several of the specific characteristics of eye movements described previously in controls were not present in vertigo adults: latency of convergence was not longer than that of divergence; components of combined saccade-vergence movements did not have longer latency than pure saccades or vergence; vergence velocity was not accelerated by the saccade during combined movements. Relative to control adults, vertigo subjects showed longer latencies of saccades to distant targets, divergence and convergence (tendency), slower speed for convergence, divergence and vergence components of combined movements. Eye movement abnormalities could be useful for the differential diagnosis of pseudo-vestibular syndrome (without vestibular dysfunction). Oculomotor training is suggested for such subjects with vertigo to improve their abnormal eye movements and reduce symptoms. [ABSTRACT FROM AUTHOR]

CHARGE syndrome (OMIM #214800) is a multiple malformation syndrome with distinctive diagnostic criteria, usually because of CHD7 (chromodomain helicase DNA binding 7) haploinsufficiency. Familial occurrence of CHARGE syndrome is rare. We report six patients from two Caucasian families (both with one parent and two children) affected by mild to severe CHARGE syndrome. Direct sequencing of the CHD7 gene was performed in these two unrelated families. A mutation in exon 8 (c.2501C>T – p.S834F) in first chromodomain was found in family A and a nonsense mutation in exon 2 (c.469C>T – p.R157X) in family B. Both mutations are de novo in the parents. In family A, the elder son had bilateral cleft lip and palate, esophageal atresia with fistula, complex heart defect and vertebral abnormalities, while the younger had a posterior coloboma. Their mother had asymptomatic vestibular dysfunction and retinal coloboma, identified after the molecular diagnosis of her children. In family B, both affected children had severe expression of CHARGE syndrome. The father carrying the mutation only had asymmetric anomaly of the pinnae. These familial reports describe the intrafamilial variability of CHARGE syndrome, and underline the presence of CHD7 mutations in patients who do not fit the ‘classical clinical criteria’ for CHARGE syndrome. [ABSTRACT FROM AUTHOR]

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]

Summary: Vestibular syndrome is not frequently described in patients with sickle cell disease. We report the case of a teenager with sickle cell disease who had a vestibular syndrome with vertigo that successfully responded to exchange transfusion. We discuss guidelines and review the literature in view of this case report. Sensorineural disorders should be considered as stroke syndromes. They require urgent treatment consisting of exchange transfusion or maintaining optimal hydration associated with blood withdrawal. Treatment of vestibular syndrome in sickle cell disease is urgent. [Copyright &y& Elsevier]