Vestibular Deficits Research Articles

Evidence of vestibular dysfunction in children with enlarged vestibular aqueduct

ObjectiveTo investigate the occurrence and characteristics of balance and vestibular deficits in pediatric patients with enlarged vestibular aqueduct (EVA). Materials and methodsRetrospective review of 53 children with EVA who underwent a comprehensive vestibular evaluation in our pediatric balance and vestibular program. Laboratory testing included videonystagmography (VNG), rotary chair, video head impulse testing (vHIT), vestibular evoked myogenic potential (VEMP), subjective visual vertical (SVV) and Sensory Organization Test (SOT) in posturography. ResultsThe mean age of these children, 31 girls and 22 boys, was 7.1 years (SD = 4.8). Among these 53 children, 16 had unilateral EVA (7 on the left side and 9 on the right side) and 37 had bilateral EVA, in which genetic testing confirmed 5 cases of Pendred syndrome. Abnormal testing results were found in 58% (11/19) on SOT, 67% (32/48) on rotary chair, 55% (48/88 of ears) on VEMP, 30% (8/27) on vHIT, 39% (7/18) on SVV, and 8% (4/53) on VNG. ConclusionsVestibular dysfunction may be a common finding in children with EVA. Clinicians who provide medical care for children with EVA need to be familiar with signs of potential balance and vestibular impairments. Although performing vestibular evaluation on young children with EVA can be difficult, objective testing is important in order to identify any potential vestibular deficit in these pediatric patients so that proper vestibular rehabilitation and balance retraining can be provided.

Predictors of persistent postural-perceptual dizziness (PPPD) and similar forms of chronic dizziness precipitated by peripheral vestibular disorders: a systematic review

BackgroundThe literature on predictors of persistent postural-perceptual dizziness (PPPD) following peripheral vestibular insults has not been systematically reviewed.MethodsWe systematically reviewed studies on predictors of PPPD and its four predecessors (phobic...

Durable improvement in participant-reported measures of disability and objective posturography after computerized vestibular retraining.

Unilateral vestibular deficits are associated with postural instability and loss of quality of life. Common treatments frequently fail to achieve satisfactory outcomes. To assess the durability of changes in participant-reported disability and objective posturography after computerized vestibular retraining. This was a single-group study. Individuals with persistent symptoms of an objectively determined unilateral vestibular deficit completed questionnaires and posturography assessments before and after twelve sessions of computerized retraining, and 4-6 months and 10-12 months after treatment. 13 participants completed the post-treatment assessments; 9 completed the follow up. Mean improvements in perceived disability at 4-6 months after retraining were: DHI 14.3 points (95% confidence interval 4.0 to 24.5), ABC scale 14.9 points (4.3 to 25.6), FES-I 11.6 points (-3.2 to 26.5).The SOT composite score increased by 11.4 points (95% CI 1.9 to 20.9; p = 0.0175) immediately after treatment, 8.9 points (-2.9 to 20.7; p = 0.1528) at 4-6 months, and 10.6 points (2.2 to 19.0; p = 0.0162) after 10-12 months. At the 10-12 month time point, the areas of the functional stability region increased significantly for both endpoint excursion (p = 0.0086) and maximum excursion (p = 0.0025). Computerized vestibular retraining was associated with improved participant reported disability and objective measures of postural stability.

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.

Intratumoral hemorrhage in vestibular schwannomas after stereotactic radiosurgery.

Vestibular schwannomas (VSs) are benign tumors of the cerebellopontine angle that are typically managed with stereotactic radiosurgery (SRS). Intratumoral hemorrhage (ITH) of VSs is a rare occurrence that results in worsening vestibular and new cranial nerve deficits. Few reports have described the management and outcomes of this entity after SRS. To further delineate the incidence and impact of this event, the authors performed a retrospective review of their VS SRS patients at a single center. Between 1987 and 2022, 2058 patients with VSs underwent Gamma Knife radiosurgery (GKRS) at the University of Pittsburgh Medical Center. The authors performed a review of the prospectively maintained VS database at their center to identify patients with ITH. The presentation, management, and clinical and imaging outcomes of the patients are reported. A total of 1902 VS patients had sufficient clinical and imaging follow-up data. Five Koos grade III (n = 1) and IV (n = 4) VS patients developed ITH after GKRS, resulting in a cumulative incidence rate of 0.26%. The age at presentation ranged from 62 to 79 years, and 3 patients were male. The time from VS diagnosis to GKRS ranged from 1 to 13 months, and the time from GKRS to ITH ranged from 2 to 130 months. Three patients had bleeding risk factors. One patient required urgent surgical intervention due to the ITH volume, while the other 4 patients were initially observed. Three patients remained stable and required no delayed intervention; 1 patient required delayed resection because of symptom progression and hemorrhagic expansion. Histopathological analysis revealed multiple fragments of S-100-positive cells, hemorrhage, and hemosiderin-laden macrophages. At last follow-up, 4 patients had clinically improved and 1 patient remained stable. ITH after VS radiosurgery is a rare phenomenon with a cumulative incidence rate of 0.26% in this series. Patient-tailored management in the form of observation or resection is based on patient presentation, acuity, and ITH size.

The role of vestibular cold caloric tests in the presence of spontaneous nystagmus.

The bithermal caloric test is commonly used to detect a canal paresis. However, in case of spontaneous nystagmus, this procedure can provide results of non-univocal interpretation. On the other hand, confirming the presence of a unilateral vestibular deficit can help to differentiate between central and a peripheral vestibular involvement. We studied 78 patients suffering from acute vertigo and showing spontaneous horizontal unidirectional nystagmus. All patients were submitted to bithermal caloric tests, and the results were compared with those obtained using a monothermal (cold) caloric test. We demonstrate the congruence between the bithermal and monothermal (cold) caloric test through mathematical analysis of the results of both tests in patients with acute vertigo and spontaneous nystagmus. We propose to perform the caloric test in the presence of a spontaneous nystagmus using a monothermal cold assuming that the prevalence of the response to the cold irrigation on the side towards which the nystagmus beats is a sign of the presence of pathological unilateral weakness and therefore more likely peripheral in its origin.

A cross-sectional survey of non-specialist Australian audio-vestibular clinical practice for traumatic brain injury and rehabilitation.

This study explored non-specialist audiological clinical practice in the context of traumatic brain injury (TBI), and whether such practices incorporated considerations of TBI-related complexities pertaining to identification, diagnosis and management of associated auditory and vestibular disturbances. A cross-sectional online survey exploring clinical practice, TBI-related training and information provision was distributed to audiologists across Australia via Audiology Australia and social media. Fifty audiologists, 80% female and 20% male, participated in this study. Years of professional practice ranged from new graduate to more than 20 years of experience. Clear gaps of accuracy in knowledge and practice across all survey domains relating to the identification, diagnosis and management of patients with auditory and/or vestibular deficits following TBI were evident. Further, of the surveyed audiologists working in auditory and vestibular settings, 91% and 86%, respectively, reported not receiving professional development for the diagnosis and management of post-traumatic audio-vestibular deficits. Inadequate resources, equipment availability and TBI-related training may have contributed to the gaps in service provision, influencing audiological management of patients with TBI. A tailored TBI approach to identification, diagnosis and management of post-traumatic auditory and vestibular disturbances is needed.

Can vigilance predict the status of safe functional gait and risk of falls in patients with peripheral vestibular disorders? A cross-sectional study

ObjectivesPeripheral vestibular disorders except from reflexes dysfunction correspond also to cognitive decline. The objectives of this cross-sectional study were to a) identify correlations among variables of functional gait, cognitive function, and perceived dizziness and b) explore variables that could be used as prognostic factors of functional gait in people with peripheral vestibular deficits. MethodsWe recruited 154 people with peripheral vestibular deficits. The participants presented with moderate disability in terms of the Dizziness Handicap Inventory questionnaire (mean: 48.00, 95% confidence interval: 45.24–50.75), deficits in the Functional Gait Assessment test (mean: 22.75, 95% confidence interval: 22.13–23.40) and indication of mild cognitive impairment based on Montreal Cognitive Assessment tool (mean: 25.18, 95% confidence interval: 24.75–25.60). ResultsStatistically significant correlations found among functional gait and gender, age, educational level, perceived level of disability and the total score of the Montreal Cognitive Assessment tool. Several components of the cognitive screening test (executive function, vigilance, language skills, verbal fluency) also correlated statistically significant with functional gait. Linear regression models revealed that age, perceived level of disability and vigilance significantly predicted functional gait variability (R2 = 0.350; p < 0.001) as well as high risk of falling, as indicated by a score on Functional Gait Assessment test <22/30 (R2 = 0.380). ConclusionsCognitive impairments affect functional gait in people with peripheral vestibular disorders. Thus, the integration of cognitive functional assessment must be considered as a prerequisite for functional assessment and designing rehabilitation programs that will include dual task training.

Vestibulo-ocular reflex gain improvements at peak head acceleration and velocity following onset of unilateral vestibular neuritis: Insights into neural compensation mechanisms.

An acute unilateral peripheral vestibular deficit (aUPVD) due to vestibular neuritis causes deficient yaw axis vestibular ocular reflex (VOR) gains. Using video head impulse tests (vHITs), we examined phasic and tonic velocity gains of the VOR over time to determine if these differed at onset and during subsequent improvement. The VOR responses of 61 patients were examined within 5 days of aUPVD onset, and 3 and 7 weeks later using vHIT with mean peak yaw angular velocities of 177°/s (sd 45°/s) and mean peak accelerations of 3660°/s2 (sd 1300°/s2). The phasic velocity or acceleration gain (aG) was computed as the ratio of eye to head velocity around peak head acceleration, and the tonic velocity gain (vG) was calculated as the same ratio around peak head velocity. aG increased ipsi-deficit from 0.45 at onset to 0.67 at 3 weeks and 7 weeks later, and vG increased ipsi-deficit from 0.29 to 0.51 and 0.53, respectively, yielding a significant time effect (p < 0.001). Deficit side aG was significantly greater (p < 0.001) than vG at all time points. Deficit side gain improvements in aG and vG were similar. Contra-deficit aG increased from 0.86 to 0.95 and 0.94 at 3 weeks and 7 weeks, and vG contra-deficit increased from 0.84, to 0.89 and 0.87, respectively, also yielding a significant time effect (p = 0.004). Contra-deficit aG and vG were normal at 3 weeks. Mean canal paresis values improved from 91% to 67% over the 7 weeks. Acceleration and velocity VOR gains on the deficit side are reduced by aUPVD and improve most in the first 3 weeks after aUPVD onset. Deficit side aG is consistently higher than deficit side vG following an aUPVD, suggesting that acceleration rather than velocity sensitive compensatory neural mechanisms are predominant during the compensation process for aUPVD.

Shox2 is required for vestibular statoacoustic neuron development.

Homeobox genes act at the top of genetic hierarchies to regulate cell specification and differentiation during embryonic development. We identified the short stature homeobox domain 2 (shox2) transcription factor that is required for vestibular neuron development. shox2 transcripts are initially localized to the otic placode of the developing inner ear where neurosensory progenitors reside. To study shox2 function, we generated CRISPR-mediated mutant shox2 fish. Mutant embryos display behaviors associated with vestibular deficits and showed reduced number of anterior statoacoustic ganglion neurons that innervate the utricle, the vestibular organ in zebrafish. Moreover, a shox2-reporter fish showed labeling of developing statoacoustic ganglion neurons in the anterior macula of the otic vesicle. Single cell RNA-sequencing of cells from the developing otic vesicle of shox2 mutants revealed altered otic progenitor profiles, while single molecule in situ assays showed deregulated levels of transcripts in developing neurons. This study implicates a role for shox2 in development of vestibular but not auditory statoacoustic ganglion neurons.

Oxytocin Disturbs Vestibular Compensation and Modifies Behavioral Strategies in a Rodent Model of Acute Vestibulopathy

Unilateral inner ear injury is followed by behavioral recovery due to central vestibular compensation. The therapeutic effect of oxytocin (OT) on vestibular compensation was investigated by behavioral testing in a rat model of unilateral vestibular neurectomy (UVN). Animals in the oxytocin group (UVN-OT) exhibited delayed vestibular compensation on the qualitative scale of vestibular deficits and aggravated static postural deficits (bearing surface) compared to animals in the NaCl group (UVN-NaCl). Surprisingly, oxytocin-treated animals adopt a different postural strategy than untreated animals. Instead of shifting their weight to the ipsilesional paws (left front and hind paws), they shift their weight to the front paws (right and left) without modification along the lateral axis. Furthermore, some locomotor strategies of the animals to compensate for the vestibular loss are also altered by oxytocin treatment. UVN-OT animals do not induce an increase in the distance traveled, their mean velocity is lower than that in the control group, and the ipsilesional body rotations do not increase from 7 to 30 days after UVN. This study reveals that oxytocin treatment hinders the restoration of some postural and locomotor deficits while improving others following vestibular lesions. The mechanisms of the action of oxytocin that support these behavioral changes remain to be elucidated.

Automatic ML-based vestibular gait classification: examining the effects of IMU placement and gait task selection

BackgroundVestibular deficits can impair an individual’s ability to maintain postural and/or gaze stability. Characterizing gait abnormalities among individuals affected by vestibular deficits could help identify patients at high risk of falling and inform rehabilitation programs. Commonly used gait assessment tools rely on simple measures such as timing and visual observations of path deviations by clinicians. These simple measures may not capture subtle changes in gait kinematics. Therefore, we investigated the use of wearable inertial measurement units (IMUs) and machine learning (ML) approaches to automatically discriminate between gait patterns of individuals with vestibular deficits and age-matched controls. The goal of this study was to examine the effects of IMU placement and gait task selection on the performance of automatic vestibular gait classifiers.MethodsThirty study participants (15 with vestibular deficits and 15 age-matched controls) participated in a single-session gait study during which they performed seven gait tasks while donning a full-body set of IMUs. Classification performance was reported in terms of area under the receiver operating characteristic curve (AUROC) scores for Random Forest models trained on data from each IMU placement for each gait task.ResultsSeveral models were able to classify vestibular gait better than random (AUROC > 0.5), but their performance varied according to IMU placement and gait task selection. Results indicated that a single IMU placed on the left arm when walking with eyes closed resulted in the highest AUROC score for a single IMU (AUROC = 0.88 [0.84, 0.89]). Feature permutation results indicated that participants with vestibular deficits reduced their arm swing compared to age-matched controls while they walked with eyes closed.ConclusionsThese findings highlighted differences in upper extremity kinematics during walking with eyes closed that were characteristic of vestibular deficits and showed evidence of the discriminative ability of IMU-based automated screening for vestibular deficits. Further research should explore the mechanisms driving arm swing differences in the vestibular population.

Can hyperventilation test and duration of spontaneous nystagmus help differentiate between vascular and inflammatory aetiology of acute unilateral vestibular deficit?

To relate clinically the duration of spontaneous nystagmus and hyperventilation-induced nystagmus (HVIN) to vascular or inflammatory aetiology of acute unilateral vestibulopathy observed in a very early stage. This is a retrospective study on 198 patients with acute unilateral vestibulopathy. In the short-lasting nystagmus group (spontaneous nystagmus < 48 h), mean age and cardiovascular risk were significantly higher; the rates of negative HVIN and paretic HVIN were 41.7% and 58.3%, respectively. In the long-lasting nystagmus group (spontaneous nystagmus > 48 h), mean age and vascular risk were lower; HVIN was absent in 12.6% of the cases, HVIN excitatory patterns were observed in 40.3% of cases and a paretic pattern in 47.1%. A vascular aetiology should be considered the most likely in patients with spontaneous nystagmus < 48 hours: all patients were > 60 years old, cardiovascular risk was higher and HVIN was always absent or paretic. In the group with nystagmus > 48 hours, similarly, data indicate a higher incidence of paretic HVIN in older patients and higher vascular risk, even if the data does not allow us to lean clearly towards one of the two aetiological hypotheses.

Modern methods of investigating the vestibular function in children

ABSTRACT. Worldwide, there are about 466 million people (6.1%) with hearing loss and approximately 34 million of them (7%) are children, two-thirds of whom live in developing countries. In the course of research, it has been proven that in 60% of cases, sensorineural deafness is combined with vestibular disorders, which can be both obvious (rarely in children) and hidden. Timely detection of vestibular disorders in young children, especially hidden ones, prevents delay in the child’s motor development and significantly reduces the risk of vestibular dizziness development in older age. The aim of the article was to study modern instrumental methods of balance function research in children based on scientific analysis of contemporary literary data. Materials and methods: A comprehensive search of relevant literature sources was conducted in December 2021 using electronic databases RubMed, EMBASE, MEDLINE, U.S. National Library of Medicine Clinical Trials, Research Gate and Cocharane Library. The results: Despite highly developed technological diagnostic capabilities of present-day medicine, the study of etiology, prevention and correction of various balance and hearing disorders in children of different ages is one of the most acute issues to be solved by modern medical community. Up-to-date improvements in the diagnostic methods of the auditory and vestibular analyzer expand the possibilities of interpreting their results and allow a deeper understanding of the degree of the stato-acoustic analyzer damage. To investigate the vestibular function, vestibulometry and otoneurological examinations are usually carried out, which are the same tests that are used in adults. However, such examinations require more patience from the child and they should be conducted in a playful manner. Detection of hidden vestibular dysfunctions ought to be comprehensive, in particular, using instrumental methods, one of which is static computer stabilometry, that creates an opportunity to determine translational and motor disorders. The conclusion. Long-term problems with balance in children can lead to a delay of motor functions development, which can further slow down the child's achievement of age-appropriate psychomotor stages and have a negative impact on school performance, and in general, on social, speech and intellectual development. Therefore, detection of vestibular dysfunction at an early age is of great medical and social importance. Acute otitis media is currently a curable disease in most cases, if undesirable consequences are prevented during the period of examination and treatment. Vestibular function can be adequately assessed using reliable instrumental tests. In this sense, static stabilometry is a very useful method for the objective determination of vestibular deficits in children, regardless of the presence or absence of any subjective complaints.

Pediatric Vestibular Assessment: Clinical Framework.

Although vestibular deficits can have severe repercussions on the early motor development in children, vestibular assessment in young children has not yet been routinely integrated in clinical practice and clear diagnostic criteria to detect early vestibular deficits are lacking. In young children, specific adjustments of the test protocol are needed, and normative data are age-dependent as the vestibular pathways mature through childhood. Therefore, this study aims to demonstrate the feasibility of an extensive age-dependent vestibular test battery, to provide pediatric normative data with the concurrent age trends, and to offer a clinical framework for pediatric vestibular testing. This normative study included 133 healthy children below the age of 4 years (mean: 22 mo, standard deviation: 12.3 mo, range: 5-47 mo) without history of hearing loss or vestibular symptoms. Children were divided into four age categories: 38 children younger than 1 year old, 37 one-year olds, 33 two-year olds, and 25 three-year olds. Children younger than 3 years of age were examined with the video Head Impulse Test (vHIT) of the horizontal semicircular canals, cervical vestibular evoked myogenic potentials (cVEMP) with bone conduction stimuli, and the rotatory test at 0.16, 0.04, and 0.01 Hz. In 3-year old children, the vHIT of the vertical semicircular canals and ocular vestibular evoked myogenic potentials (oVEMP) using a minishaker were added to the protocol. The horizontal vHIT appeared to be the most feasible test across age categories, except for children younger than 1-year old in which the success rate was the highest for the cVEMP. Success rates of the rotatory test varied the most across age categories. Age trends were found for the vHIT as the mean vestibulo-ocular reflex (VOR) gain increased significantly with age (r = 0.446, p < 0.001). Concerning the cVEMP, a significant increase with age was found for latency P1 (r = 0.420, p < 0.001), rectified interpeak amplitude P1-N1 (r = 0.574, p < 0.001), and averaged electromyographic (EMG) activity (r = 0.430, p < 0.001), whereas age trends for the latency N1 were less pronounced (r = 0.264, p = 0.004). Overall, the response parameters of the rotatory test did not show significant age effects ( p > 0.01), except for the phase at 0.01 Hz (r = 0.578, p < 0.001). Based on the reported success rates and age-dependent normative vestibular data, straightforward cutoff criteria were proposed (vHIT VOR gain < 0.7, cVEMP rectified interpeak amplitude < 1.3, oVEMP interpeak amplitude < 10 µV) with accompanying clinical recommendations to diagnose early vestibular impairment. In this large cohort of typically developing children below the age of 4 years, the vHIT and cVEMP were the most feasible vestibular tests. Moreover, the age-dependent normative vestibular data could specify age trends in this group of young children. Finally, based on the current results and clinical experience of more than ten years at the Ghent University Hospital (Belgium), a clinical framework to diagnose early vestibular deficits in young patients is proposed.

RADT-33. INTRATUMORAL HEMORRHAGE IN VESTIBULAR SCHWANNOMAS FOLLOWING GAMMA KNIFE STEREOTACTIC RADIOSURGERY

Abstract INTRODUCTION Intratumoral hemorrhage (ITH) is a rare occurrence in vestibular schwannomas (VS) resulting in worsening vestibular and cranial nerve deficits. Scarce data described the occurrence, management, and outcomes of ITH after stereotactic radiosurgery (SRS). OBJECTIVE The aim of this study is to delineate the incidence, presentation, management, and outcomes of ITH in VS patients following SRS. METHODS A total of 2058 patients with VS underwent Gamma Knife radiosurgery (GKRS) at the University of Pittsburgh Medical Center between 1987 and 2022. The authors identified patients with ITH. Their presentation, management, clinical, and imaging outcomes are reported and analyzed. RESULTS A total of 1902 VS patients had sufficient clinical and imaging follow-up data. Five Koos III (n = 1) and IV (n = 4) VS patients developed ITH after GKRS, resulting in a cumulative incidence rate of 0.26%. The age at presentation ranged from 62 to 79 years, and 3 of the patients were males. The time from VS diagnosis to GKRS ranged from 1-13 months, and the time from GKRS to ITH ranged from 2-130 months. Three patients had bleeding risk factors. One patient required urgent surgical intervention due to the ITH volume, while the other four patients initially were observed. Three remained stable and required no delayed intervention. Histopathological analysis revealed multiple fragments of S-100–positive cells, hemorrhage, and hemosiderin-laden macrophages. At last follow-up, 4 patients had clinically improved, and one remained stable.ConclusionVS ITH following SRS is a rare complication with a cumulative incidence rate of 0.26%. Patient-tailored management is vital based on patient presentation, acuity, and ITH size and characteristics.

A study on incidence of vestibular deficits in children with sensory neural hearing loss by using cervical Vestibular Evoked Myogenic Potentials (cVEMP)

This study was conducted from an Audiology &amp; rehabilitation clinic in Kerala- India from January 2022 to August 2022.In both evolutionary and embryonic perspective, the organs responsible for hearing and balance (auditory &amp; vestibular system) have a common origin and developmental pattern. Sensory neural hearing loss (SNHL) is a common congenital sensory deficit occurring in large number of children and it is extensively investigated and has well defined management protocols such as hearing aids or cochlear implants and speech-language rehabilitation. But in comparison with the case of SNHL, very little effort has been done to investigate and treat the vestibular deficits which can co-occur along with SNHL. This study is an attempt to throw some light about the incidence of vestibular deficits in hearing impaired children. It was found that abnormal/absent VEMP responses were found in 57% of children with SNHL. VEMP responses were completely absent in 65% of ears with severe to profound hearing loss. Vestibular dysfunction was also present in 19% of children with moderate to moderately severe hearing loss. Incidence of vestibular deficits increases in higher degree of hearing loss such as severe to profound category. Motor delay was also reported in 10 out of 26 participants in this study which could be attributed to lack of visual motor development in children with vestibular deficits. However a large number of vestibular deficits in children with SNHL still remain under reported and uninvestigated which points to the need for conducting vestibular assessment in children diagnosed with SNHL.

A study on incidence of vestibular deficits in children with sensory neural hearing loss by using cervical Vestibular Evoked Myogenic Potentials (cVEMP)

A study on incidence of vestibular deficits in children with sensory neural hearing loss by using cervical Vestibular Evoked Myogenic Potentials (cVEMP)

Vestibular Infant Screening-Flanders: What is the Most Appropriate Vestibular Screening Tool in Hearing-Impaired Children?

As children with sensorineural hearing loss have an increased risk for vestibular impairment, the Vestibular Infant Screening-Flanders project implemented a vestibular screening by means of cervical vestibular evoked myogenic potentials (cVEMP) at the age of 6 months for each child with hearing loss in Flanders (Belgium). Given that vestibular deficits can affect the child's development, this vestibular screening should allow early detection and intervention. However, less is currently known about which screening tool would be the most ideal and how vestibular impairment can evolve. Therefore, this study aimed to determine the most appropriate tool to screen for vestibular deficits, to assess the necessity of vestibular follow-up, and to set clinical guidelines for vestibular screening in children with hearing loss. In total, 71 children with congenital or early-onset sensorineural hearing loss were enrolled (mean age at first appointment = 6.7 months). Follow-up was provided at 6 months, 1, 2, and 3 years of age. Below three years of age, the video Head Impulse Test (vHIT) of the horizontal semicircular canals (SCC), the cVEMP, and the rotatory test at 0.16, 0.04, and 0.01 Hz were applied. At 3 years of age, the vHIT of the vertical SCC and ocular vestibular evoked myogenic potentials (oVEMP) were added. To evaluate early motor development, the Alberta Infant Motor Scale (AIMS) results at 6 months and 1-year old were included. At 6 months of age, the highest success rate was obtained with the cVEMP (90.0%) compared to the vHIT (70.0%) and the rotatory test (34.3-72.9%). Overall, vestibular deficits were found in 20.0% of the children, consisting of 13.9% with both SCC and otolith deficits (bilateral: 9.3%, unilateral: 4.6%), and 6.1% with unilateral isolated SCC (4.6%) or otolith (1.5%) deficits. Thus, vestibular deficits would not have been detected in 4.6% of the children by only using the cVEMP, whereas 1.5% would have been missed when only using the vHIT. Although vestibular deficits were more frequently found in severe to profound hearing loss (28.6%), characteristics of vestibular function were highly dependent on the underlying etiology. The AIMS results showed significantly weaker early motor development in children with bilateral vestibular deficits ( p = 0.001), but could not differentiate children with bilateral normal vestibular function from those with unilateral vestibular deficits ( p > 0.05). Progressive or delayed-onset vestibular dysfunction was only found in a few cases (age range: 12-36 months), in which the hearing loss was mainly caused by congenital cytomegalovirus (cCMV). The cVEMP is the most feasible screening tool to assess vestibular function in 6-months-old children with hearing loss. Although the majority of children with vestibular deficits are detected with the cVEMP, the vHIT seems even more sensitive as isolated SCC deficits are associated with specific etiologies of hearing loss. As a result, the cVEMP is an appropriate vestibular screening tool, which is advised at least in severe to profound hearing loss, but certain etiologies require the addition of the vHIT (i.e., cCMV, meningitis, cochleovestibular anomalies with or without syndromic hearing loss).

Pathogenic Variants in the COCH Gene That Lead to Hearing Loss and Vestibular Deficit: Update on DFNA9 and Introducing DFNB110

Pathogenic Variants in the COCH Gene That Lead to Hearing Loss and Vestibular Deficit: Update on DFNA9 and Introducing DFNB110