Bilateral Vestibular Loss Research Articles

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

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. 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. 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. 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. 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. 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.

Functional impact of bilateral vestibular loss and the unexplained complaint of oscillopsia.

The vestibulo-ocular reflex (VOR) stabilizes vision during head movements. VOR disorders lead to symptoms such as imbalance, dizziness, and oscillopsia. Despite similar VOR dysfunction, patients display diverse complaints. This study analyses saccades, balance, and spatial orientation in chronic peripheral and central VOR disorders, specifically examining the impact of oscillopsia. Participants involved 15 patients with peripheral bilateral vestibular loss (pBVL), 21 patients with clinically and genetically confirmed Machado-Joseph disease (MJD) who also have bilateral vestibular deficit, and 22 healthy controls. All pBVL and MJD participants were tested at least 9 months after the onset of symptoms and underwent a detailed clinical neuro-otological evaluation at the Dizziness and Eye Movements Clinic of the Meir Medical Center. Among the 15 patients with pBVL and 21 patients with MJD, only 5 patients with pBVL complained of chronic oscillopsia while none of the patients with MJD reported this complaint. Comparison between groups exhibited significant differences in vestibular, eye movements, balance, and spatial orientation. When comparing oscillopsia with no-oscillopsia subjects, significant differences were found in the dynamic visual acuity test, the saccade latency of eye movements, and the triangle completion test. Even though there is a significant VOR gain impairment in MJD with some subjects having less VOR gain than pBVL with reported oscillopsia, no individuals with MJD reported experiencing oscillopsia. This study further supports that subjects experiencing oscillopsia present a real impairment to stabilize the image on the retina, whereas those without oscillopsia may utilize saccade strategies to cope with it and may also rely on visual information for spatial orientation. Finding objective differences will help to understand the causes of the oscillopsia experience and develop coping strategies to overcome it.

The effect of increased listening effort on the balance performance of patients with compensated vestibular lesion

ObjectivesThis study investigated the effects of listening effort (LE) on balance in patients with compensated vestibular deficits compared to healthy peers. MethodsThe subjects included two main groups: a control group of 15 healthy subjects and a study group of 19 patients with compensated vestibular pathology. The computerized dynamic posturography test (CDP) was conducted without the speech-in-noise task as a baseline, then the participant was subjected to a dual task in which the auditory task (speech-in-noise sentences) was given as the primary task, and the balance function test was the secondary task. Results: within-group analysisThe study group showed statistically significantly worse values of all body balance parameters under dual-task than the baseline in all conditions. These differences were much higher under the compliant platform conditions. However, these findings were not statistically significant in the control group. Between-group analysisThe study group showed a statistically significant decline in body balance reactions compared to the control group under dual-task with increased listening effort and the compliant platform. Study subgroup analysis revealed statistically significant differences between patients with unilateral vestibular loss (UVL) and those with bilateral vestibular loss (BVL) in the unstable platform condition. ConclusionOur study regarding implementing a dual-tasking paradigm as a measure of LE during the evaluation of chronic vestibular patients with CDP demonstrated how dual-tasking with increased LE affects postural stability. Because of this, patients will probably be more prone to tripping and falling in multitasking situations, as found in real-world settings. This fact should be taken into consideration while testing patients with chronic vertigo and compensated states at VNG. A dual-task paradigm helps uncover the unrevealed pathology.

Electrical vestibular stimulation as a tool for treatment of bilateral vestibular loss. Literature review

The review of the literature on RSCI and PubMed databases presents methods of electrical vestibular stimulation to improve vestibular function in patients with bilateral vestibulopathy. The variants of stimulation of peripheral vestibular structures, such as vestibular implantation, noise galvanic vestibular stimulation are described. The perspectives of development of this direction, advantages of application of electrical stimulation in the future, as well as current limitations that do not currently allow to use these methods in clinical practice are shown.

Vestibular contributions to linear motion perception.

Vestibular contributions to linear motion (i.e., translation) perception mediated by the otoliths have yet to be fully characterized. To quantify the maximal extent that non-vestibular cues can contribute to translation perception, we assessed vestibular perceptual thresholds in two patients with complete bilateral vestibular ablation to compare to our data in 12 young (< 40years), healthy controls. Vestibular thresholds were assessed for naso-occipital ("x-translation"), inter-aural ("y-translation"), and superior-inferior ("z-translation") translations in three body orientations (upright, supine, side-lying). Overall, in our patients with bilateral complete vestibular loss, thresholds were elevated ~ 2-45 times relative to healthy controls. No systematic differences in vestibular perceptual thresholds were noted between motions that differed only with respect to their orientation relative to the head (i.e., otoliths) in patients with bilateral vestibular loss. In addition, bilateral loss patients tended to show a larger impairment in the perception of earth-vertical translations (i.e., motion parallel to gravity) relative to earth-horizontal translations, which suggests increased contribution of the vestibular system for earth-vertical motions. However, differences were also noted between the two patients. Finally, with the exception of side-lying x-translations, no consistent effects of body orientation in our bilateral loss patients were seen independent from those resulting from changes in the plane of translation relative to gravity. Overall, our data confirm predominant vestibular contributions to whole-body direction-recognition translation tasks and provide fundamental insights into vestibular contributions to translation motion perception.

Repetitive Low-Intensity Vestibular Noise Stimulation Partly Reverses Behavioral and Brain Activity Changes following Bilateral Vestibular Loss in Rats.

Low-intensity noisy galvanic vestibular stimulation (nGVS) can improve static and dynamic postural deficits in patients with bilateral vestibular loss (BVL). In this study, we aimed to explore the neurophysiological and neuroanatomical substrates underlying nGVS treatment effects in a rat model of BVL. Regional brain activation patterns and behavioral responses to a repeated 30 min nGVS treatment in comparison to sham stimulation were investigated by serial whole-brain 18F-FDG-PET measurements and quantitative locomotor assessments before and at nine consecutive time points up to 60 days after the chemical bilateral labyrinthectomy (BL). The 18F-FDG-PET revealed a broad nGVS-induced modulation on regional brain activation patterns encompassing biologically plausible brain networks in the brainstem, cerebellum, multisensory cortex, and basal ganglia during the entire observation period post-BL. nGVS broadly reversed brain activity adaptions occurring in the natural course post-BL. The parallel behavioral locomotor assessment demonstrated a beneficial treatment effect of nGVS on sensory-ataxic gait alterations, particularly in the early stage of post-BL recovery. Stimulation-induced locomotor improvements were finally linked to nGVS brain activity responses in the brainstem, hemispheric motor, and limbic networks. In conclusion, combined 18F-FDG-PET and locomotor analysis discloses the potential neurophysiological and neuroanatomical substrates that mediate previously observed therapeutic nGVS effects on postural deficits in patients with BVL.

Identification of vestibular loss in children with sensorineural hearing loss using the balance subset of the BOT-2 test

BackgroundVestibular loss in children with sensorineural hearing loss (SNHL) is quite high. Despite the high prevalence of vestibular loss and balance impairment in children with SNHL, they are rarely assessed by clinicians and therefore are commonly undiagnosed. The purpose of the study was to evaluate the balance subset of the Bruininks-Oseretsky Test (BOT-2) as a tool to identify vestibular loss in children with SNHL and to determine its predictive values for vestibular loss.MethodsThe study included 210 children allocated into 4 groups: group 1 (control healthy children), group II (children with SNHL but without vestibular loss), group III (children with SNHL and unilateral vestibular loss), and group IV (children with SNHL and bilateral vestibular loss). Caloric test, video head impulse test (vHIT), and cervical vestibular evoked myogenic potential (c-VEMP) test were used for vestibular assessment in children with SNHL and to allocate them accordingly into one of the aforementioned groups. Scores of the balance subset of the BOT-2 were compared among the three groups and compared to the control healthy children.Results21.4% of children with SNHL but without vestibular loss have balance deficit revealed by the balance subset of the BOT-2, reflecting its better sensitivity for detecting balance deficit than the physiologic vestibular tests. Children with unilateral or bilateral vestibular loss (groups III and IV) showed a more significant balance deficit than children without vestibular loss (group II). The worst balance score was found in children with bilateral vestibular loss. The positive predictive value of the BOT-2 for peripheral vestibular loss in children with SNHL was 88%.ConclusionThe balance subset of the BOT-2 has particularly good predictive values for vestibular loss in children with SNHL. The test is a simple, easy, fast office test which does not require any costly equipment.

Speech, Gait, and Vestibular Function in Cerebellar Ataxia with Neuropathy and Vestibular Areflexia Syndrome.

(1) Background: Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is characterized by late-onset cerebellar ataxia, bilateral vestibulopathy, and sensory neuronopathy mostly due to biallelic RFC1 expansion. (2) Objectives: The aim of this case series is to describe vestibular, gait, and speech alterations in CANVAS via a systematic approach. (3) Methods: All patients (n = 5) underwent a standardized clinical-instrumental examination, including the perceptual and acoustic analysis of speech, instrumental gait, and balance analysis (posturographic data were acquired using a force plate [Kistler, Winterthur, Switzerland] while 3D gait analysis, inclusive of surface electromyography, was acquired using a motion capture system [SMART DX, BTS Bioengineering, Milan, Italy], a wireless electromyograph [FreeEMG, BTS Bioengineering, Milan, Italy]), and vestibular assessment with video-oculography. (4) Results: Five patients were included in the analysis: three females (patients A, B, C) and two males (patients D and E) with a mean age at evaluation of 62 years (SD ± 15.16, range 36-74). The mean age of symptoms' onset was 55.6 years (SD ± 15.04, range 30-68), and patients were clinically and instrumentally evaluated with a mean disease duration of 6.4 years (SD ± 0.54, range 6-7). Video-Frenzel examination documented spontaneous downbeat nystagmus enhanced on bilateral gaze in all patients, except for one presenting with slight downbeat nystagmus in the supine position. All patients exhibited different degrees of symmetrically reduced VOR gain for allsix semicircular canals on the video-head impulse test and an unexpectedly normal ("false negative") VOR suppression, consistent with combined cerebellar dysfunction and bilateral vestibular loss. Posturographic indices were outside their age-matched normative ranges in all patients, while 3D gait analysis highlighted a reduction in ankle dorsiflexion (limited forward rotation of the tibia over the stance foot during the stance phase of gait and fatigue of the dorsiflexor muscles) and variable out-of-phase activity of plantar flexors during the swing phase. Finally, perceptual-acoustic evaluation of speech showed ataxic dysarthria in three patients. Dysdiadochokinesis, rhythm instability, and irregularity were observed in the oral diadochokinesis task. (5) Conclusions: CANVAS is a recently discovered syndrome that is gaining more and more relevance within late-onset ataxias. In this paper, we aimed to contribute to a detailed description of its phenotype.

Vestibular Preservation in Pediatric Cochlear Implantation.

Evaluate the rate of preserved vestibular function in pediatric cochlear implant surgery. Retrospective case review. Pre- and post-operative vestibular tests were compared in children who underwent cochlear implantation at a tertiary level pediatric hospital over a 4-year period. Data from 59 implanted ears in 44 children was included. Median age was 2.8 years at initial testing (range 7 months - 21 years) with 1:1 male/female ratio. Implant surgeries were 26 unilateral, 13 bilateral simultaneous, and 5 bilateral sequential. The majority were implanted with slim, non-styletted electrodes (86.4%) via a round window approach (91.5%). Normal pre-operative results were preserved post-operatively on rotary chair testing in 75% (21/28) of patients, cervical vestibular evoked myogenic potential testing in (75%) 30/40 of ears tested, ocular vestibular evoked myogenic potential testing in 85.7% (6/7) of ears tested, video head impulse testing in 100% (9/9) of ears tested, and computerized dynamic posturography in 100% (5/5) of patients tested. Overall, 62.5% of patients had no new deficits on any vestibular test performed post-operatively. Preservation rates of vestibular function following cochlear implant surgery were higher in this cohort than what has been reported in many earlier studies. Contemporary, less traumatic electrodes and insertion techniques may be a significant factor. The risk of causing a new, severe bilateral vestibular loss with long-term functional impacts appears to be low. Further study is warranted on the impacts of different cochlear implant electrode designs and insertion approaches on post-operative vestibular preservation. 4, Case Series Laryngoscope, 134:1913-1918, 2024.

Cortical facilitation of somatosensory inputs using gravity-related tactile information in humans with vestibular hypofunction.

A few years after their bilateral vestibular loss, patients usually show a motor repertoire that is almost back to normal. This recovery is thought to involve an upregulation of the visual and proprioceptive information that compensates for the lack of vestibular information. Here, we investigated whether plantar tactile inputs, which provide body information relative to the ground and to the Earth vertical, contribute to this compensation. More specifically, we tested the hypothesis that somatosensory cortex response to electric stimulation of the plantar sole in standing adults will be greater in humans (n = 10) with bilateral vestibular hypofunction (VH) than in an age-matched healthy group (n = 10). Showing significantly greater somatosensory evoked potentials (i.e., P1N1) in VH than in control subjects, the electroencephalographic recordings supported this hypothesis. Furthermore, we found evidence that increasing the differential pressure between both feet, by adding a 1-kg mass at each pendant wrist, enhanced the internal representation of body orientation and motion relative to a gravitational reference frame. The large decrease in alpha power in the right posterior parietal cortex (and not in the left) is in line with this assumption. Finally, behavioral analyses showed that trunk oscillations were smaller than head oscillations in VH and showed a reverse pattern for healthy participants. These findings are consistent with a tactile-based postural control strategy in the absence of vestibular input and a vestibular-based control strategy in healthy participants where the head serves as a reference for balance control.NEW & NOTEWORTHY Somatosensory cortex excitability is greater in participants with bilateral vestibular hypofunction than in age-matched healthy humans. To control balance, healthy humans "locked" the head whereas participants with vestibular hypofunction "locked" their pelvis. For participants with vestibular hypofunction, increasing loading/unloading of the feet enhances the internal representation of body state in the posterior parietal cortex.

A Paradoxical Clinical Coincidence: Benign Paroxysmal Positional Vertigo and Bilateral Vestibulopathy.

Benign paroxysmal positional vertigo (BPPV) and bilateral vestibulopathy (BVL) are two completely different forms of vestibular disorder that occasionally occur in the same patient. We conducted a retrospective review searching for that coincidence in our database of the patients seen over a 15-year period and found this disorder in 23 patients, that is 0.4%. More frequently they occurred sequentially (10/23) and BPPV was diagnosed first. Simultaneous presentation occurred in 9/23 patients. It was subsequently studied, but in a prospective manner, in patients with BPPV on all of whom a video head impulse test was performed to search for bilateral vestibular loss; we found it was slightly more frequent (6/405). Both disorders were treated accordingly, and it was found that the results follow the general trend in patients with only one of those disorders.

Development of a new clinical tool to evaluate the balance abilities of children with bilateral vestibular loss: The Geneva Balance Test.

Vestibular deficits are considered rare in children, but the lack of systematic screening leads to underdiagnosis. It has been demonstrated that chronic vestibular dysfunction impacts the normal psychomotor development of children. Early identification is needed to allow for clinical management, ensuring better global development. For this purpose, our research group has developed the Geneva Balance Test (GBT), aiming to objectively quantify the balance capacity of children over a broad age range, to screen for bilateral vestibulopathy (BV), and to quantify the improvement of balance abilities in children. To determine the capacity of the GBT to quantify the balance capacity of children with BV, we conducted an observational prospective study with three populations: 11 children with BV, and two age-matched control groups composed of (1) 15 healthy subjects without the vestibular or auditory disorder (HS) and (2) 11 pediatric cochlear implant recipients (CIs) without vestibular disorders. Results of the three populations have been compared in three different age sub- groups (3-5, 6-9, and ≥10 years), and with results of a short, modified version of the Bruininks-Oseretsky test of Motor proficiency Ed. 2 (mBOT-2). Statistical analyses demonstrated significant differences in the scores of the GBT between children aged 3-5, 6-9, and ≥10 years with BV and in both control populations (HS and CI). BV scores reflected poorer balance capacities at all ages. Children in the youngest CI sub-group (3-5 years) showed intermediate GBT scores but reached HS scores at 6-9 years, reflecting an improvement in their balance capacities. All the results of the GBT were significantly correlated with mBOT-2 results, although only a few BV completed the entire mBOT-2. In this study, the GBT allowed quantifying balance deficits in children with BV. The BOT-2 test is not validated for children <4.5 years of age, and the GBT seems to be better tolerated in all populations than the mBOT-2. Furthermore, mBOT-2 results saturated, reaching maximum values by 6-9 years whereas the GBT did not, suggesting that the GBT could be a useful tool for monitoring the development of balance capacities with age and could be used in the follow-up of children with severe vestibular disorders.

Cognitive functions in episodic vestibular disorders: Meniere's disease and vestibular migraine.

Cognitive deficits have been defined in patients with bilateral and unilateral vestibular loss. To investigate cognitive functions in patients with episodic vestibular disorders. Nineteen patients with Meniere's disease (MD), 19 patients with vestibular migraine (VM) and 21 age and education matched healthy controls were studied. Mini Mental State Examination assessing global mental status, Reading Span Test and the Stroop Test evaluating working memory, cognitive processing, reading comprehension and attention, Trail Making Test and Benton's Judgment of Line Orientation Test investigating visual processing, visuospatial skills, processing speed were used. Beck depression and anxiety inventories were given to evaluate the emotional status. Cognitive test results of the MD and VM patients were not significantly different from the healthy controls (p > 0.05) as well as Beck depression scores (p = 0.14). Beck anxiety scores showed significant difference (p = 0.003). VM patients had significantly higher scores than the healthy controls (p = 0.002) on pairwise comparisons. The scores of the MD patients did not reach statistical significance (p = 0.15). Episodic vestibular disorders like MD and VM without inter-ictal vestibular deficits do not seem to be associated with cognitive impairment. Patients with VM have significantly higher anxiety scores than the healthy controls and MD patients.

Vestibular Contributions to Primate Neck Postural Muscle Activity during Natural Motion.

To maintain stable posture of the head and body during our everyday activities, the brain integrates information across multiple sensory systems. Here, we examined how the primate vestibular system, independently and in combination with visual sensory input, contributes to the sensorimotor control of head posture across the range of dynamic motion experienced during daily life. We recorded activity of single motor units in the splenius capitis and sternocleidomastoid muscles in rhesus monkeys during yaw rotations spanning the physiological range of self-motion (up to 20 Hz) in darkness. Splenius capitis motor unit responses continued to increase with frequency up to 16 Hz in normal animals, and were strikingly absent following bilateral peripheral vestibular loss. To determine whether visual information modulated these vestibular-driven neck muscle responses, we experimentally controlled the correspondence between visual and vestibular cues of self-motion. Surprisingly, visual information did not influence motor unit responses in normal animals, nor did it substitute for absent vestibular feedback following bilateral peripheral vestibular loss. A comparison of muscle activity evoked by broadband versus sinusoidal head motion further revealed that low-frequency responses were attenuated when low- and high-frequency self-motion were experienced concurrently. Finally, we found that vestibular-evoked responses were enhanced by increased autonomic arousal, quantified via pupil size. Together, our findings directly establish the vestibular system's contribution to the sensorimotor control of head posture across the dynamic motion range experienced during everyday activities, as well as how vestibular, visual, and autonomic inputs are integrated for postural control.SIGNIFICANCE STATEMENT Our sensory systems enable us to maintain control of our posture and balance as we move through the world. Notably, the vestibular system senses motion of the head and sends motor commands, via vestibulospinal pathways, to axial and limb muscles to stabilize posture. By recording the activity of single motor units, here we show, for the first time, that the vestibular system contributes to the sensorimotor control of head posture across the dynamic motion range experienced during everyday activities. Our results further establish how vestibular, autonomic, and visual inputs are integrated for postural control. This information is essential for understanding both the mechanisms underlying the control of posture and balance, and the impact of the loss of sensory function.

Common neural correlates of vestibular stimulation and fear learning: an fMRI meta-analysis

BackgroundA bidirectional functional link between vestibular and fear-related disorders has been previously suggested.ObjectiveTo test a potential overlap of vestibular and fear systems with regard to their brain imaging representation maps.MethodsBy use of voxel-based mapping permutation of subject images, we conducted a meta-analysis of earlier functional magnetic resonance imaging (fMRI) studies applying vestibular stimulation and fear conditioning in healthy volunteers.ResultsCommon clusters of concordance of vestibular stimulation and fear conditioning were found in the bilateral anterior insula cortex, ventrolateral prefrontal cortex and the right temporal pole, bilaterally in the adjacent ventrolateral prefrontal cortex, cingulate gyrus, secondary somatosensory cortex, superior temporal and intraparietal lobe, supplementary motor area and premotor cortex, as well as subcortical areas, such as the bilateral thalamus, mesencephalic brainstem including the collicular complex, pons, cerebellar vermis and bilateral cerebellar hemispheres. Peak areas of high concordance for activations during vestibular stimulation but deactivations during fear conditioning were centered on the posterior insula and S2.ConclusionsThe structural overlap of both networks allows the following functional interpretations: first, the amygdala, superior colliculi, and antero-medial thalamus might represent a release of preprogramed sensorimotor patterns of approach or avoidance. Second, the activation (vestibular system) and deactivation (fear system) of the bilateral posterior insula is compatible with the view that downregulation of the fear network by acute vestibular disorders or unfamiliar vestibular stimulation makes unpleasant perceived body accelerations less distressing. This also fits the clinical observation that patients with bilateral vestibular loss suffer from less vertigo-related anxiety.

Role of Videonystagmography in Patients of BPPV

Videonystagmography (VNG) is a complete diagnostic system for recording, analyzing, and reporting eye movements using video imaging technology, in which hi-tech video goggles with infrared cameras are used. VNG includes a series of tests used to determine whether a vestibular disease may be causing a balance or dizziness problem; VNG can differentiate between a central and a peripheral vestibular lesion, and, if peripheral, it can decipher between unilateral and bilateral vestibular loss. VNG addresses the functionality of each ear

Visuospatial Cognitive Dysfunction in Patients with Vestibular Loss.

To characterize visuospatial and nonvisuospatial cognitive domains affected by vestibular loss and determine whether patient-reported outcomes measures (PROMs) correlate with performance on neuropsychological tests. Cross-sectional study. University-based tertiary medical center. Sixty-nine age-matched subjects: 25 patients with bilateral vestibular loss (BVL), 14 patients with unilateral vestibular loss (UVL), and 30 normal controls (NC). Neuropsychological tests used to assess visuospatial and auditory short-term and working memory, number magnitude representation, executive function, and attention. Validated PROMs used to evaluate quality of life and subjective cognitive impairment. Performance on neuropsychological tests and scores on PROM surveys. BVL and UVL patients performed significantly worse than NC subjects on tasks requiring visuospatial representation compared with NC subjects ( p < 0.01). BVL patients demonstrated decreased performance on spatial representation tasks compared with UVL and NC subjects ( p < 0.05 and p < 0.05, respectively). All subject groups performed similarly on tasks assessing nonvisuospatial cognitive domains, such as auditory short-term and working memory, executive function, and attention. PROMs did not seem to correlate with performance on neuropsychological tasks. Patients with vestibular loss exhibit impairments in tasks requiring visuospatial representation but perform similarly to NC subjects in tasks of auditory working memory, executive function, or attention. Currently available questionnaires may be insufficient to screen patients for cognitive deficits.

Progressive loss of hearing and balance in superficial siderosis due to occult spinal dural defects

PurposeSuperficial siderosis, a progressive, debilitating, neurological disease, often presents with bilateral impairment of auditory and vestibular function. We highlight that superficial siderosis is often due to a repairable spinal dural defect of the type that can also cause spontaneous intracranial hypotension.MethodsRetrospective chart review of five patients presenting with moderate to severe, progressive bilateral sensorineural hearing loss as well as vestibular loss. All patients had developed superficial siderosis from spinal dural defects: three after trauma, one after spinal surgery and one from a thoracic discogenic microspur.ResultsThe diagnosis was made late in all five patients; despite surgical repair in four, hearing and vestibular loss failed to improve.ConclusionsIn patients presenting with progressive bilateral sensorineural hearing loss, superficial siderosis should be considered as a possible cause. If these patients also have bilateral vestibular loss, cerebellar impairment and anosmia, then the diagnosis is likely and the inevitable disease progress might be halted by finding and repairing the spinal dural defect.

Correlation of SVINT and Sensory Organization Test in Children with Hearing Loss.

Objective: The skull vibration-induced-nystagmus test (SVINT) is a noninvasive and effective screening tool for the function of the otolith and canal structures in children. It can instantaneously assess vestibular asymmetry. This study aimed to analyze the SVINT results of healthy children vs. children with hearing loss (HL) and to correlate it with sensory organization test (SOT) results as a functional balance evaluation tool. Design: This case-controlled study compared the results of SVINT to the results of the SOT of the computerized dynamic posturography (CDP) in a control group of 120 healthy normal-hearing children (i.e., NH group) vs. hearing loss (HL) group of 60 children, including 30 children with hearing aids (HAs) and 30 children with a unilateral cochlear implant (CI). The SVINT results were compared to the caloric test (CaT) and video head impulse test (vHIT) and associated with SOT scores. Results: Thirty-one children in the HL group had normal SVINT and normal SOT results. A total of 21 children in the HL group had SVINT-negative and abnormal results in the SOT (possibly due to bilateral vestibular loss (BVL)). Eight children in the HL group had positive SVINT and abnormal SOT results. However, none of the children had only positive SVINT with normal SOT findings. Moreover, 52% of children had a normal result on both the SOT and CaT, whereas 27% had abnormal results on both tests (17% bilateral weakness and 10% unilateral), and 22% had the only result of the SOT suggesting a functional abnormality. Similarly, when associating the result to vHIT, 51% had normal results on both tests, and 25% had abnormal results (13% bilateral and 12% unilateral weakness). Conclusions: SVINT findings can be correlated with SOT findings in the case of the unilateral vestibular lesion (UVL), which adds a diagnostic value in these pediatric cases but may differ in the case of the bilateral vestibular lesion (BVL). However, SVINT findings need to be cautiously interpreted in light of other test findings such as the SOT, CaT, and vHIT.

Does vestibular loss result in cognitive deficits in children with cochlear implants?

In adults, vestibular loss is associated with cognitive deficits; however, similar relationships have not been studied in children. Evaluate the effect of vestibular loss on working memory and executive function in children with a cochlear implant (CCI) compared to children with normal hearing (CNH). Vestibular evoked myogenic potential, video head impulse, rotary chair, and balance testing; and the following clinical measures: vision, hearing, speech perception, language, executive function, and working memory. Thirty-eight CNH and 37 CCI participated (26 with normal vestibular function, 5 with unilateral vestibular loss, 6 with bilateral vestibular loss). Children with vestibular loss demonstrated the poorest balance performance. There was no significant reduction in working memory or executive function performance for either CCI group with vestibular loss; however, multivariate regression analysis suggested balance performance was a significant predictor for several working memory subtests and video head impulse gain was a significant predictor for one executive function outcome. CCI with vestibular loss did not have significantly reduced working memory or executive function; however, balance performance was a significant predictor for several working memory subtests. Degree of hearing loss should be considered, and larger sample sizes are needed.