Chronic Electrical Stimulation of the Otolith Organ: Preliminary Results in Humans with Bilateral Vestibulopathy and Sensorineural Hearing Loss.

INTRODUCTION: Bilateral vestibulopathy is an important cause of imbalance that is misdiagnosed. The clinical management of patients with bilateral vestibular loss remains difficult as there is no clear evidence for an effective treatment. In this paper, we try to analyze the effect of chronic electrical stimulation and adaptation to electrical stimulation of the vestibular system in humans when stimulating the otolith organ with a constant pulse train to mitigate imbalance due to bilateral vestibular dysfunction (BVD). METHODS: We included 2 patients in our study with BVD according to Criteria Consensus of the Classification Committee of the Bárány Society. Both cases were implanted by using a full-band straight electrode to stimulate the otoliths organs and simultaneously for the cochlear stimulation we use a perimodiolar electrode. RESULTS: In both cases Vestibular and clinical test (video head impulse test, videonistagmography cervical vestibular evoked myogenic potentials, cVEMP and oVEMP), subjective visual vertical test, computerized dynamic posturography, dynamic gait index, Time UP and Go test and dizziness handicap index) were performed. Posture and gait metrics reveal important improvement if compare with preoperartive situation. Oscillopsia, unsteadiness, independence and quality of life improved to almost normal situation. DISCUSSION/CONCLUSION: Prosthetic implantation of the otolith organ in humans is technically feasible. Electrical stimulation might have potential effects on balance and this is stable after 1 year follow-up. This research provides new possibilities for the development of vestibular implants to improve gravito-inertial acceleration sensation, in this case by the otoliths stimulation.

Physiology, clinical evidence and diagnostic relevance of sound-induced and vibration-induced vestibular stimulation.

PURPOSE OF REVIEW: To examine the recent literature concerning the neural basis and clinical evidence for the response of the labyrinth to sound and vibration: vestibular-evoked myogenic potentials (VEMPs) and vibration-induced nystagmus (VIN). RECENT FINDINGS: There are two streams of information from each otolith - a sustained stream (afferents with regular resting activity, signalling gravity and low-frequency linear accelerations) and a transient stream (afferents with irregular resting activity) signalling onset of linear acceleration, and sound and vibration. These irregular neurons are synchronized to each cycle of the stimulus. Neurons in the transient stream are tested by presenting sounds or vibration (500 Hz) and using surface electrodes to measure myogenic potentials from muscles activated by otolithic stimuli (VEMPs). 100 Hz vibration activates irregular canal afferents and causes a stimulus-locked VIN in patients with asymmetric canal function. These new tests of the transient system have one big advantage over older tests of the sustained system - they reliably show the effect of long-term unilateral vestibular loss. SUMMARY: The new physiological and anatomical evidence shows how sound and vibration activate otolith and canal receptors and so provides the scientific foundation for VEMPs and VIN, which are important tools for diagnosing vestibular disorders. VIDEO ABSTRACT: http://links.lww.com/CONR/A47.

Altered processing of otolithic information in isolated lateral medullary infarction.

Ocular and cervical vestibular-evoked myogenic potentials (VEMPs) evaluate the function of otolithic pathways in central as well as peripheral vestibular disorders. This study aimed to determine the associations and dissociations of otolithic dysfunction in lateral medullary infarction (LMI), the most well-known disorder of central vestibulopathy. At the Dizziness Clinic of a referral-based University Hospital, 45 patients with isolated LMI (28 men, mean age = 55.6 ± 12.5) had evaluation of the ocular tilt reaction (OTR), tilt of the subjective visual vertical (SVV), and ocular and cervical VEMPs from Janurary 2011 to August 2015 during the acute phase, 1-11 days from the symptom onset (median = 2 days). Almost all (42/45, 93 %) patients showed at least one component of the OTR or SVV tilt that was invariably ipsiversive. In contrast, oVEMPs were abnormal only in 12 (27 %) and cVEMPs in 13 (29 %) patients. Only three patients showed abnormal results in all the tests of the OTR, SVV tilt, and ocular and cervical VEMPs. Abnormal oVEMPs were more common in patients with the OTR than those without (38 vs 6 %, Pearson X 2 test, p = 0.021). In contrast, abnormality of cVEMPs showed no correlation with the presence of OTR (28 vs 31 %, Pearson X 2 test, p = 0.795). In patients with LMI, ipsiversive OTR is invariable, but abnormalities of oVEMPs and cVEMPs were much less common and mostly dissociated even in the patients with abnormal results. This discrepancy in otolithic dysfunction suggests different anatomical substrates and/or dissimilar reciprocal modulation for processing of each otolithic signal in central vestibular structures located in the dorsolateral medulla.

Normal pressure hydrocephalus: Increase of utricular input in responders to spinal tap test.

OBJECTIVE: To investigate whether there is a change in ocular (oVEMP) and cervical (cVEMP) vestibular evoked myogenic potentials in patients with normal pressure hydrocephalus (NPH) before and after spinal tap test (STT). METHODS: In 25 patients (6 females, age 62-83years) c/oVEMP were measured before and after STT. Patients with an increase of >20% of walking velocity were classified as responders (n=10). VEMP were also measured in a control group of 13 non-NPH patients. RESULTS: All patients had reproducible oVEMP; 68% had cVEMP. There was a significant increase of the peak-to-peak (pp) oVEMP amplitude after STT in responders (8.5±2.7 to 18.9±7.5µV (p=0.010)). No significant changes were found in non-responders (13.4±7.6 to 15.3±8.6µV) or controls (12.4±7.6 to 12.5±6.8µV). There were no significant differences in cVEMP before and after spinal tap test (STT). CONCLUSION: One third of patients with suspected NPH had impaired otolith function. Responders to STT only had a significant increase of oVEMP and thereby utricular input, probably due to a decrease of pressure. SIGNIFICANCE: Both findings indicate that otolith dysfunction may contribute to imbalance in NPH and that increased utricular function after STT may be relevant for gait improvement.

Diagnostic value of vestibular-evoked myogenic potentials in Ménière's disease and vestibular migraine.

Overlaps can be seen between vestibular migraine (VM) Ménière's Disease (MD) and diagnosis is difficult if hearing is normal. We aimed to investigate the sacculo-collic pathway in VM patients, MD patients, and healthy controls to define the diagnostic role of cervical VEMP (cVEMP). VEMP testing in response to 500 Hz and 1000 Hz air-conducted tone burst (TB) stimulation was studied prospectively in 22 subjects with definite VM (according to Bárány nomenclature), 30 subjects with unilateral definite MD, and 18 volunteers matched healthy controls. In VM subjects, response rate, p13 and n23 latencies were similar to healthy controls, but peak-to-peak amplitudes were bilaterally reduced at 500 Hz TBs (p= 0.005). cVEMP differentiated MD patients from VM and healthy controls with asymmetrically reduced amplitudes on affected ears with low response rates at 500 Hz TBs, and alteration of frequency dependent responses at 500 and 1000 Hz TBs. These findings suggest that cVEMP can be used as a diagnostic test to differentiate MD from VM. On the other hand, VEMP responses are symmetrically reduced on both sides in VM patients, suggesting that otolith organs might be affected by migraine-induced ischemia.

Personality traits modulate subcortical and cortical vestibular and anxiety responses to sound-evoked otolithic receptor stimulation.

OBJECTIVE: Strong links between anxiety, space-motion perception, and vestibular symptoms have been recognized for decades. These connections may extend to anxiety-related personality traits. Psychophysical studies showed that high trait anxiety affected postural control and visual scanning strategies under stress. Neuroticism and introversion were identified as risk factors for chronic subjective dizziness (CSD), a common psychosomatic syndrome. This study examined possible relationships between personality traits and activity in brain vestibular networks for the first time using functional magnetic resonance imaging (fMRI). METHODS: Twenty-six right-handed healthy individuals underwent fMRI during sound-evoked vestibular stimulation. Regional brain activity and functional connectivity measures were correlated with personality traits of the Five Factor Model (neuroticism, extraversion-introversion, openness, agreeableness, consciousness). RESULTS: Neuroticism correlated positively with activity in the pons, vestibulo-cerebellum, and para-striate cortex, and negatively with activity in the supra-marginal gyrus. Neuroticism also correlated positively with connectivity between pons and amygdala, vestibulo-cerebellum and amygdala, inferior frontal gyrus and supra-marginal gyrus, and inferior frontal gyrus and para-striate cortex. Introversion correlated positively with amygdala activity and negatively with connectivity between amygdala and inferior frontal gyrus. CONCLUSIONS: Neuroticism and introversion correlated with activity and connectivity in cortical and subcortical vestibular, visual, and anxiety systems during vestibular stimulation. These personality-related changes in brain activity may represent neural correlates of threat sensitivity in posture and gaze control mechanisms in normal individuals. They also may reflect risk factors for anxiety-related morbidity in patients with vestibular disorders, including previously observed associations of neuroticism and introversion with CSD.

Danhong enhances recovery from residual dizziness after successful repositioning treatment in patients with benign paroxysmal positional vertigo.

OBJECTIVES: Although the repositioning maneuvers are usually very effective in patients with BPPV, some patients still complain residual dizziness. Danhong injection (DHI), a traditional Chinese medicine, can effectively dilate blood vessels and improve microcirculation, and has been proven to be effective in improving cervical vertigo and posterior circulation ischemic vertigo. The aim of this study was to evaluate the effects of DHI on residual dizziness after successful repositioning treatment in patients with BPPV. METHODS: Eighty-six patients with BPPV were randomized into two treatment groups, DHI group and non DHI group. The DHI group received the same repositioning treatment as the non-DHI group, with the addition of DHI therapy. The durations of residual dizziness of DHI group and non-DHI group were compared. In addition, the scores of the dizziness handicap inventory of these two groups were calculated. RESULTS: The durations of residual dizziness of DHI group were shorter than that of non-DHI group. There were no significant differences in the scores of dizziness handicap inventory in the first week between these two groups, and there were much significant differences in the second, the fourth, the sixth and eighth weeks. CONCLUSIONS: The results demonstrate that DHI can significantly improve the residual dizziness after successful repositioning treatment in patients with BPPV.

Augmented ocular vestibular evoked myogenic potentials to air-conducted sound in large vestibular aqueduct syndrome.

OBJECTIVE: To demonstrate the value of recording air-conducted ocular Vestibular Evoked Myogenic Potentials (oVEMP) in a patient with bilaterally enlarged vestibular aqueducts. DESIGN: Cervical VEMP and oVEMP were recorded from a patient presenting with bilateral hearing loss and imbalance, attributable to large vestibular aqueduct syndrome. The stimuli were air-conducted tone bursts at octave frequencies from 250 to 2000 Hz. Amplitudes and thresholds were measured and compared with the normal response range of 32 healthy control subjects. RESULTS: oVEMP reflexes demonstrated pathologically increased amplitudes and reduced thresholds for low-frequency tone bursts. Cervical VEMP amplitudes and thresholds were within normal limits for both ears across all frequencies of stimulation. CONCLUSIONS: This study is the first to describe the augmentation of AC oVEMPs in an adult with large vestibular aqueduct syndrome.

The vestibular evoked myogenic potential (VEMP): air- versus bone-conducted stimuli.

OBJECTIVE: Several studies have evaluated the effects of different stimulus and recording parameters on the cervical vestibular evoked myogenic potential (cVEMP); however, it is difficult to directly compare these studies as they have all used different recording methods, different sternocleidomastoid (SCM) muscle contraction/electromyography monitoring methods, and different stimulus parameters. DESIGN: : This study made a direct comparison of the cVEMP in response to air-conducted (AC) and bone-conducted (BC) stimuli in the same subjects, using the same stimulus/recording/electromyography monitoring methods. RESULTS: We found that the input/output (I/O) functions were more linear in response to AC stimuli, whereas cVEMPs in response to BC stimuli began to saturate at the highest level. In addition, cVEMP threshold was obtained at a lower stimulus level (i.e., at a lower sensation level) in response to BC stimuli as compared with AC stimuli, and cVEMPs in response to BC stimuli were larger than cVEMPs in response to AC stimuli, which is in agreement with what has been found in previous studies. In addition, this was one of the few studies to evaluate the repeatability of the cVEMP in response to BC stimuli. Interestingly, we found that cVEMP latency in response to BC stimuli was, in most cases, less variable than cVEMP latency obtained in response to AC stimuli, whereas the reverse was true for cVEMP amplitude. We also found that BC masking presented to the forehead affected response amplitude of the AC cVEMP regardless of the specific SCM muscle contraction/toneburst presentation condition. In addition, we found that the ratio of amplitude reduction was greater in the binaural stimulation/bilateral SCM muscle contraction condition as compared with the monaural stimulation/bilateral SCM muscle contraction condition. CONCLUSIONS: The present experiment provided a direct comparison of the cVEMP in response to AC versus BC 500 Hz short-duration toneburst stimuli in the same subjects. The results of the present experiment also provide insight into the laterality of the cVEMP response and reveal that the cVEMP may not be completely ipsilateral (i.e., there may be a form of bilateral interaction that occurs when both sides are stimulated simultaneously). Last, the results indicate that BC stimuli likely activates the saccule as well as the utricle, given that AC VEMPs can be masked by the administration of BC masking noise presented to the midline.

[Air conducted ocular VEMP: II. First clinical investigations]. / Luftleitungsinduzierte oculäre VEMP: II. Erste klinische Untersuchungen.

BACKGROUND: Vestibular-evoked myogenic potentials (VEMP) are widely used to assess vestibular function. Air conducted (AC) cervical VEMP (cVEMP) reflect sacculus and inferior vestibular nerve function. Ocular VEMP (oVEMP) however has been hardly examined up to now. In recent studies it has been assumed that AC oVEMP probably reflects superior vestibular nerve function. The aim of this pilot study was to evaluate clinical application of the AC oVEMP. MATERIAL AND METHODS: AC oVEMP were recorded in patients with peripheral vestibular disorders (n=21). In addition thermal irritation and head impulse test were performed and AC cVEMP were recorded. For intense AC-sound stimulation tone bursts (500 Hz) with 100 dB nHL were used. RESULTS: In peripheral vestibular disorders AC oVEMP and AC cVEMP could be classified into: • type 1 (inferior vestibular neuritis) with loss of AC oVEMP but normal AC cVEMP, • type 2, probable type of superior vestibular neuritis, showing present AC cVEMP but loss of AC oVEMP, • type 3, probable complete vestibular neuritis, without AC oVEMP and AC cVEMP. CONCLUSIONS: AC oVEMP may be used as an appropriate test for clinical investigation in patients with vestibular disorders. AC oVEMP is an additional, essential test for assessing otolith function beside AC cVEMP. Further vestibular test are necessary for precise clinical interpretation.

[Air conducted ocular VEMP: I. Determination of a method and application in normal patients]. / Luftleitungsinduzierte oculäre VEMP I. Methodik und Untersuchungen bei normalen Probanden.

BACKGROUND: Air conducted (AC) cervical vestibular evoked myogenic potentials (AC cVEMP) and air conducted ocular VEMP (AC oVEMP) may be used for measurement of otolith function. However AC oVEMP are few examined till now. The aim of this pilot study was to apply a method for use of AC oVEMP in clinical practice. PATIENTS AND METHODS: AC oVEMP were recorded in healthy voluntary people (n=20) using intense AC-sound stimulation (500 Hz tone bursts, 100 dB nHL). Thermal irrigation and AC cVEMP were normal as including criteria. Values were evaluated statistically. RESULTS: AC oVEMP were recorded in all healthy patients. Mean and standard deviation for the first negative peak was 11.35±1.00 ms and for the first negative peak 16.30±1.10 ms. The mean amplitudes were 7.70±4.50 µV. The stability of n10 and p15 component was the same. CONCLUSIONS: AC oVEMP can be easy and fast obtained. N10 and p15 latencies may used as parameter for clinical interpretation. Amplitude fluctuations are relatively large. Results can be used in further clinical investigation of AC oVEMP.

Subjective visual vertical before and after treatment of a BPPV episode.

OBJECTIVE: The study analyses the behavior of subjective visual vertical (SVV) in benign paroxysmal positional vertigo (BPPV) before and after treatment, and offers a clinical-pathogenic interpretation. METHODS: We studied 30 consecutive patients with BPPV of the posterior semicircular canal treated with the Epley repositioning maneuver. SVV was determined at three different stages: at the time of diagnosis (1st test), after the repositioning maneuver (2nd test), and then 7 days after the resolution of the clinical picture (3rd test). The main study parameter was represented by the mean of 6 consecutive measurements (SVV(0)) for each patient. SVV was also examined in 20 healthy subjects, who represented the control group. The comparison between mean values and standard deviations showed a statistical significance of p<0.05. RESULTS: During the first test, the degree of deviation of SVV was significantly higher in the patient group than in the control group. Tilting towards the affected side was observed in all cases. The 2nd test showed an inversion in the orientation of SVV in 16 patients, and as a result of the Epley maneuver there was a statistically significant variation in SVV(0) values in 20 patients with respect to the previous test (2nd test vs. 1st test). This involved 87% (23 patients) of those who then had a negative Dix-Hallpike test, and none of the ones in whom paroxysmal positional nystagmus persisted. Lastly, no differences emerged in the behavior of the patient group vs. the control group during the third test. CONCLUSIONS: SVV is often altered during active BPPV. The degree of otolithic dysfunction is never high and, in all cases, it is brief in duration. Tilting towards the dysfunctional side is essentially a constant in untreated BPPV. This could be due to a substantial loss of otoconia, with a decrease in the density and specific weight of the macula, and thus hypofunction of the receptor. The observation of a significant variation in SVV after therapeutic maneuvers has a favorable predictive value, as it probably reflects the migration of otoliths to the utricle, where saturation mechanisms can often have irritative effects leading to the inversion of SVV.

Enhanced otolithic function in semicircular canal dehiscence.

The enhanced sound- and vibration-induced vestibular evoked myogenic potentials (VEMPs) and their lower threshold in patients with a thinning of the bony wall of the superior semicircular canal (superior canal dehiscence, SCD) have been interpreted as being due to the dehiscence allowing sound and vibration to activate, unusually, the receptors of the dehiscent semicircular canal. We report a patient with bilateral SCD, as verified by high resolution CT scans, who had bilaterally decreased superior semicircular canal function, as shown by rotational tests of canal function. This patient also showed enhanced VEMPs and reduced thresholds. We conclude that in this patient the enhanced VEMP responses are thus probably due to enhanced otolithic stimulation by sound and vibration after dehiscence.

Auditory-pupillary responses in deaf subjects.

Pupillary dilation in response to sound stimuli is well established and is generally considered to represent a startle reflex to sound. We believe that the auditory-pupillary response represents not only a simple startle reflex to sound stimuli but also represents a reaction to stimulation of other sense organs, such as otolith organs. Eight young healthy volunteers without a history of hearing and equilibrium problems and 12 subjects with bilateral deafness participated in this study. Computer pupillography was used to analyze the auditory-pupillary responses of both eyes in all subjects. We found that auditory-pupillary responses occurred even in subjects with bilateral deafness and that this response was comparable to those of normal subjects. We propose that the auditory-pupillary response also relates to vestibular function. Thus, assessing the auditory-pupillary response may be useful for evaluating the vestibulo-autonomic response in patients with peripheral disequilibrium.

Evaluation of guinea pig model for ocular and cervical vestibular-evoked myogenic potentials for vestibular function test.

OBJECTIVES/HYPOTHESIS: This study used air-conducted sound (ACS) and bone-conducted vibration (BCV) stimuli in eliciting ocular vestibular-evoked myogenic potential (oVEMP) and cervical VEMP (cVEMP) in guinea pigs. STUDY DESIGN: Prospective study. METHODS: Ten guinea pigs were treated with gentamicin (4 mg) on the left ear, whereas the right ear served as a control. One week after treatment, each animal underwent oVEMP and cVEMP tests using ACS and BCV modes in a randomized order, and was sacrificed for morphological study. RESULTS: Using ACS mode, oVEMPs were absent in all 10 (100%) animals despite the stimulus intensity increased up to 120 dB pe SPL. Conversely, using BCV mode, oVEMPs were present on the left (lesion) eye, and absent on the right (control) eye in all (100%) animals. For the cVEMPs via ACS mode, all right (control) necks had clear cVEMPs, and all (100%) left (lesion) necks revealed absent cVEMPs. However, via BCV mode, all right (control) necks and six (60%) left (lesion) necks showed clear cVEMPs. Morphological study demonstrated substantial loss of hair cells in the utricular and saccular macula. CONCLUSIONS: The cVEMP test via ACS mode is specific for investigating the saccular disorder, whereas the oVEMP test via BCV mode is preferable for investigating the utricular disorders in humans. The guinea pig model is consistent with the findings of humans. Restated, appropriate animal models for cVEMP and oVEMP in guinea pigs are via ACS and BCV modes, respectively.

[Ocular tilt reaction in thalamic infarct]. / Reacción ocular de inclinación en infarto talámico.

Ocular tilt reaction (OTR) includes skew deviation, eye torsion and head tilt. It is usually accompanied by a tilt in the subjective visual vertical. OTR seems to reflect an otolithic dysfunction. This case report shows an OTR of central origin as a result of simultaneous paramedial thalamic and mesencephalon rostral infarcts.

Vestibular-evoked myogenic potentials and caloric tests in infants with congenital rubella.

UNLABELLED: Vestibular-evoked myogenic potentials and caloric tests in infants with congenital rubella. OBJECTIVE: Congenital rubella is rare due to maternal vaccination, but vestibular function can be markedly impaired in affected infants. Currently, vestibular testing is not routinely performed in infants. This study evaluated inner ear function in infants with congenital rubella. METHODOLOGY: The study included 54 3-month-old infants: 40 healthy controls and 14 with congenital rubella that was serologically confirmed in mothers and infants. There were two subgroups of infants with congenital rubella: 6 infants with clinical symptoms of intrauterine rubella infection present at birth and 8 asymptomatic infants. Caloric testing and vestibular-evoked myogenic potential (VEMP) testing were performed on all of the infants. RESULTS: In congenital rubella there was no reaction to caloric stimulation in 43% of the ears, and no VEMPs were recorded in 64%. The hearing thresholds were below 80 dB hearing level in 21% of the ears and greater than 80 dB hearing level in 43% of the ears. CONCLUSIONS: The degree of vestibular organ impairment in infants with congenital rubella is higher in individuals with profound hearing loss. VEMPs were absent more frequently than were caloric responses. Vestibular testing should be routinely performed in all infants with congenital rubella.

Vestibular drop attack secondary to Meniere's disease results from unstable otolithic function.

CONCLUSION: The otolithic organs of patients with vestibular drop attack (VDA) secondary to Meniere's disease were damaged but the damage was not complete. In other words, the otolithic functions of patients with VDA were unstable. OBJECTIVE: To evaluate otolithic function using vestibular evoked myogenic potential (VEMP) in patients with VDA secondary to Meniere's disease. PATIENTS AND METHODS: Clinical records of three patients with VDA secondary to Meniere's disease were reviewed with special reference to VEMP testing. RESULTS: The three patients were classified as stage II or III Meniere's disease. A long-term follow-up of VEMP in two patients showed reversible changes of VEMP reflexes, and VEMP testing with glycerol administration in two patients revealed the recovery of VEMP responses after taking glycerol, and the existence of saccular endolymphatic hydrops.

The effects of logon versus click on vestibular evoked myogenic potentials.

CONCLUSION: Logon is superior to click to trigger larger and more consistent vestibular evoked myogenic potentials (VEMPs). OBJECTIVES: To record and compare the parameters of VEMPs evoked by bone- and air-conducted logon (l-VEMPs) and click (c-VEMPs). SUBJECTS AND METHODS: Air- and bone-conducted l-VEMPs and c-VEMPs were recorded in 28 normal ears with an Amplaid MK12 (Amplaid, Milan) equipment. RESULTS: VEMPs response rate was 100% with both air-conducted logon and click, while l-VEMPs showed a higher response rate (79%) in comparison with c-VEMPs (21%) with bone-conducted stimuli. A significant (p<0.05) increase of P1, N1 and P1-N1 amplitude and augmented P1 and N1 latencies were noticed in l-VEMPs with respect to c-VEMPs.