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.

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.

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.

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.

Vestibular impairment after bacterial meningitis delays infant posturomotor development.

OBJECTIVE: To examine the findings and impact of postmeningitis vestibular dysfunction on early posturomotor development. Meningitis in children is frequently associated with postural instability, which is often attributed to an undefined neurologic disorder but it could actually be due to vestibular impairment. STUDY DESIGN: In a retrospective cohort study, we compared groups with vestibular loss before versus after independent walking: 37 children (18 girls, 19 boys; median age: 2.3 years) hospitalized for bacterial meningitis and referred for postural instability. A complete vestibular evaluation included 3 tests for function of the 6 semicircular canals (caloric, earth vertical axis rotation, head impulse tests), 2 tests for otolith function assessment (vestibular evoked myogenic potentials, off vertical axis rotation), audiologic evaluation, neurologic examination, and brain and temporal bone imaging. RESULTS: Twenty-nine children (10.5% of the 276 children hospitalized with bacterial meningitis) had vestibular impairment. Vestibular loss was complete bilaterally in 16 of 37 children and partial in 13 of 37, and 8 cases had normal vestibular responses. Neurologically normal children who had meningitis before they walked independently and had complete bilateral vestibular loss walked significantly later and their postural instability lasted longer than children in the other groups. The degree of vestibular impairment correlated with the postural instability duration and with the degree of hearing loss. CONCLUSIONS: Bacterial meningitis in young children can impair vestibular function completely, leading to delayed posturomotor development if meningitis occurs before independent walking, even in absence of neurologic impairment. Vestibular evaluations are encouraged for postmeningitis evaluation, particularly in cases with postural instability exceeding 8 days, hearing impairment, and programmed cochlear implant.

Identification of CACNA1A large deletions in four patients with episodic ataxia.

Episodic ataxia is an autosomal dominant ion channel disorder characterized by paroxysmal attacks of incoordination. Episodic ataxia type 2 (EA2) is caused by mutations in CACNA1A. EA2 mutations are mostly nonsense and sometimes missense mutations. However, in some typical EA2 families, CACNA1A sequencing does not detect any point mutation. Herein, we have designed a quantitative multiplex polymerase chain reaction of short fluorescent fragment test to screen the 50 exons of CACNA1A and investigated 27 probands referred for molecular diagnosis of EA2 who did not show any point mutation in CACNA1A. We have identified four different exonic deletions in four patients with a typical EA2 phenotype. These results establish the need to complete sequencing analysis by a screening for deletions to ensure an accurate molecular diagnosis of EA2.

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.

Vestibular impairments pre- and post-cochlear implant in children.

OBJECTIVES: Determine prevalence and types of vestibular impairments in sensorineural hearing loss (SNHL) in a large population of pediatric candidates for cochlear implants. Evaluate impact of cochlear implants on vestibular function. STUDY DESIGN: Retrospective and prospective study. METHODS: Children with profound SNHL (n=224) underwent complete vestibular testing (clinical vestibular examination, bicaloric test, earth vertical axis rotation, off vertical axis rotation and vestibular evoked myogenic potentials) before cochlear implant. Changes in vestibular responses were measured after implants in 89 of these patients. RESULTS: In the SNHL population only 50% had normal bilateral vestibular function, while 20% had bilateral complete areflexia, 22.5% partial asymmetrical hypoexcitability and 7.5% partial symmetrical hypoexcitability. In the 71/89 follow-up patients showing vestibular responses prior to implant, 51 (71%) had changes in vestibular function including 7 (10%) who acquired ipsilateral areflexia. Others developed ipsilateral hypo- or hyperexcitability. Vestibular modifications occurred during the 3 months after surgery and were not clearly associated with clinical signs except for ipsilateral areflexia cases. In long-term follow-up, two of the 7 patients with ipsilateral areflexia partially recovered vestibular function. CONCLUSION: Since half of pediatric cochlear implant candidates have vestibular deficits and 51% of implants induce modifications of existing vestibular function, each implant should be preceded by canal and otolith functional tests to assure that the least functional vestibule is implanted. The tests provide baselines for follow-up monitoring of subsequent losses and recovery. This could be easily implemented with a clinical vestibular examination including the head thrust test associated with a bicaloric test and vestibular-evoked-myogenic-potentials.

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.

Vestibular disorders in children.

When vertigo and dizziness are diagnosed in a child, this is a matter of concern for the physician and the child's family. Prior to a comprehensive otological, neurological, and vestibular clinical examination, physicians often request CT or MRI scanning, although in most cases such expensive testing is unnecessary. The present work is based on the results of a 14-year study conducted with a sample of more than 2,000 children referred for vertigo and balance disorders to the functional vestibular evaluation unit of the ENT pediatric department at Robert Debre Hospital (Paris). The clinical signs of vestibular deficit and the most frequent aetiologies of vertigo and dizziness in children are detailed (migraine equivalent, ophthalmologic disorders, benign paroxysmal idiopathic pediatric vertigo, and temporal bone fracture), which has been helpful in determining the best diagnostic procedure and therapy for vertigo in children.

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.

Vertigo and balance problems in children--an epidemiologic study in Finland.

OBJECTIVES: There is only scant data about the frequency and characteristics of vertigo in children. The aim of the study was to determine the prevalence and characteristics of vertigo and balance problems in children aged between 1 and 15 years. METHOD: One thousand and fifty children aged from 1 to 15 years from one child welfare unit and three schools in Helsinki University Hospital District received a questionnaire acquiring about their dizzy symptoms. RESULTS: Of 1050 eligible children, 938 (89%) or a caregiver completed a simple screening questionnaire, 8% had experienced vertigo and 23% of these it was so severe vertigo that it prevented their present activity. Reason for vertigo was unknown in one third of the children and 69% could name a provocative factor for their vertigo. CONCLUSION: Balance problems are not rare in children and can limit their daily activities.

Cochlear implantation and vestibular function in children.

OBJECTIVES: To analyze vestibular function Q2 (VF) after cochlear implantation (CI) in children. METHODS: Retrospective cohort study at a tertiary pediatric referral center. 43 children that had vestibular testing (VT) after unilateral cochlear implantation, from 2001 to 2010, were included. CT scan of the temporal bone was done systematically before surgery. VT included three tests: head-impulse test, caloric tests, vestibular evoked myogenic potentials. VF was graded in: normal (type 1), partial dysfunction (type 2), severe dysfunction and areflexia (type 3). In 12 cases, VT was done before and after CI. Vestibular function was analyzed looking to side, etiology and preoperative status. RESULTS: Mean age at CI was 2.9 years. Bilateral inner ear malformation were retrieved in 16%. Before surgery, 50% of children had normal vestibular responses, 4/12 had bilateral type 2, two had asymmetrical VF. In this group, after surgery, 2 children had VF worsening, none on the CI side only. Considering all 43 patients, post operative VT showed normal response in 48.8% and type 2&3 in 16.2%. Children had asymmetrical poorer vestibular function on the side of CI in 19%. Among them, 75% had normal contralateral VF. Sensorineural hearing loss etiologies known to be associated with vestibulopathy (Usher/Meningitis/Inner Ear Malformations/CMV) were associated to abnormal vestibular function more frequently than in other causes (p=0.01). CONCLUSION: Half of the children had initial vestibular dysfunction. In our study, 20% of cochlear implantation could have worsened vestibular function. As vestibular function should be part of the choice in cochlear implantation, side of implantation and survey; and vestibular tests are uneasy to achieve in pediatric population, we propose a three-steps evaluation and gradation which allow easier comparison.

Vestibular activity and cognitive development in children: perspectives.

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 hippocampal system where this input is indispensable for neuronal responses selective for the position and orientation of the head in space. One aspect of the 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.

Visuo-oculomotor deficiency at early-stage idiopathic scoliosis in adolescent girls.

STUDY DESIGN: Cross-sectional study. OBJECTIVE: To determine whether adolescent idiopathic scoliosis (AIS) at onset is associated with oculomotor dysfunction and whether these oculomotor anomalies are correlated to the amplitude of the spine deformation. SUMMARY OF BACKGROUND DATA: AIS is related to abnormalities of postural control. To date, few studies have focused on visuo-oculomotor and vestibulo-ocular functions at early-stage AIS. METHODS: Fifty-three adolescent girls were diagnosed with AIS (mean age: 11.6 ± 2.1 yr) on clinical and radiological criteria (mean Cobb angle: 14.8° ± 5.0°). Visuo-oculomotor and vestibulo-ocular functions were studied with video-oculography, including saccades, smooth pursuit, caloric test, and pendular rotation, with visual vestibular ocular reflex and vestibulo-ocular reflex sequences. Two patient groups were defined according to the mean Cobb angle: group 1 included 29 patients with a Cobb angle from 5° to 14° and group 2 included 24 patients with a Cobb angle from 15° to 25°. RESULTS: The group 2 showed different saccade characteristics than group 1: higher latencies for saccade sequences characterized by temporal uncertainty and predictive direction; lower velocity regardless of the type of the saccades. No difference was observed for saccadic accuracy and smooth-pursuit gain. For the visual vestibular ocular reflex, group 2 showed lower total maximal slow-phase velocity than group 1, whereas the vestibulo-ocular reflex (tested in dark) did not differ between groups. No difference was observed concerning the caloric vestibular test. CONCLUSION: Patients with a Cobb angle of 15° or more presented normal vestibulo-ocular responses but altered visuo-oculomotor functions, especially for the saccadic latency and velocity. This could be the result of a dysfunction of oculomotor pathways at cerebellar and/or brainstem level. These central disorders may be incriminated in the development of AIS.

Cochleovestibular impairment in pediatric Cogan's syndrome.

Cogan's syndrome is a rare, chronic inflammatory disorder that typically targets the eyes and vestibuloauditory apparatus, but it may also involve other organs. Three pediatric cases of Cogan's syndrome (ages 5, 13, and 18 years) are reported with long-term follow-up and complete and regular cochleovestibular functional evaluation and ophthalmologic and neurologic examinations. One case was a typical form (characterized by an interstitial keratitis and cochleovestibular impairment), whereas the other 2 cases were atypical forms with uveitis and polyarthritis. In all 3 cases, the first clinical sign was nonspecific eye redness misdiagnosed as a banal conjunctivitis, initially or secondarily associated with bilateral endocochlear sensorineural hearing loss and complete bilateral peripheral vestibular deficit. During the acute phase, early steroid treatment (prednisone, 1 mg/kg/day) was effective in treating the ocular lesions (3 of 3 cases) and improving hearing (2 of 3 cases) but less effective for the vestibular loss (2 of 3 cases). Adverse effects and dependence on the steroid occurred in 2 cases, and immunosuppressive drugs were necessary to avoid recurrences in 1 case. Over the long-term, the disease was controlled in 2 cases but continued to progress in the other. Cogan's syndrome in childhood should be suspected in cases of conjunctivitis associated with inner-ear symptoms; a prompt steroid treatment can avoid progressive impairment of multiple sensorineural functions (vision, balance, hearing). Long-term management involves limiting disease recurrences by adaptive therapies, screening for complications (aortitis in particular), and planning rehabilitation for the sensorial deficits.