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Vestibular evoked myogenic potentials in patients with idiopathic bilateral vestibulopathy. Report of three cases.

Idiopathic bilateral vestibulopathy (IBV) is an acquired bilateral peripheral vestibular disorder of unknown cause. Three patients diagnosed as IBV by neuro-otological examination were reported. They underwent vestibular evoked myogenic potential (VEMP) testing which reflects the functionality of the sacculo-collic pathway. As a result, 2 of the 3 patients showed bilateral absence of VEMPs and one showed unilateral absence. The VEMPs of the 3 patients revealed that IBV affects not only the superior but also the inferior vestibular nerve systems. As previously reported in the cases of vestibular neuritis, VEMP could be useful for classifying IBV according to the function of the inferior vestibular nerve.

Aged↗

Improvements in trunk sway observed for stance and gait tasks during recovery from an acute unilateral peripheral vestibular deficit.

OBJECTIVES: Our aim was to track improvements in postural control during recovery from an acute unilateral peripheral vestibular deficit (UVL), presumably due to vestibular neuritis, and to determine if recovery rates were different for stance and gait tasks. Postural control was quantified using simple measurements of trunk sway: amplitudes of trunk sway angle and angular velocity, in the roll and pitch directions as well as task duration, were examined for a battery of stance and gait tasks. These measures were collected at the onset of the deficit and then 3 weeks and 3 months later. STUDY DESIGN: A repeated-measures design was used for UVL subjects and age-matched healthy controls. Stance tasks involved standing on 1 or 2 legs with eyes open or closed. Gait tasks consisted of tandem gait, walking normally with eyes closed, or with the head rotating or head pitching, walking up and down stairs and walking over a series of low barriers. Stance and tandem gait tasks were repeated using a foam support surface instead of a normal floor. PATIENTS: Twenty-eight patients with acute UVL were examined. MAIN OUTCOME MEASURES: The range of trunk sway angular displacement and angular velocity in the pitch and roll directions was measured for each task in addition to task duration. The measures were compared with those of normal subjects. RESULTS: The amplitudes of pitch trunk sway for 2-legged stance tasks with eyes closed underwent the greatest reduction 3 weeks after UVL onset. At 3 months, trunk sway was almost normal for all 2-legged stance tasks. One-legged stance tasks with eyes open showed a similar but slower improvement. Stance time without a fall showed a very rapid improvement for 1-legged tasks but was still shorter than that of normal subjects at 3 months. Trunk sway for the simple gait tasks was within normal range at 3 months; however, task duration was still longer than normal. More complex gait tasks, such as walking 8 tandem steps on foam or walking up and down stairs, showed no improvement in trunk roll sway at 3 months. A mix of variables from mainly gait tasks best identified a balance deficit due to UVL, with complex gait tasks becoming more important for identification purposes as compensation progressed. The accuracy of UVL identification with durations alone was 75% of the accuracy with combined trunk sway and duration measures. CONCLUSIONS: These data suggest that recovery of normal trunk control during the compensation process for unilateral vestibular hypofunction is more rapid for stance tasks than gait tasks. Even at 3 months, trunk sway for complex gait tasks was not normal. Thus, trunk sway for gait tasks provides a better insight into remaining deficits in balance control of vestibular-loss patients than the sway of stance tasks.

Acute Disease↗

Do predictive mechanisms improve the angular vestibulo-ocular reflex in vestibular neuritis?

Recovery from vestibular neuritis (VN) is often incomplete which leads to persistent vestibular imbalance during rapid head movements. Patients with unilateral vestibular lesions have a larger gain of the horizontal vestibulo-ocular reflex during active compared to passive head movements. To test whether this gain increase is related to predictive mechanisms we studied 15 patients with VN and 14 control subjects during predictable and unpredictable passive horizontal head impulses in the light and darkness. The vestibulo-ocular reflex showed a significantly shorter latency and higher gain in the light for predictable head impulses towards the ipsilesional side. However, this effect is small and might contribute but cannot exclusively account for the gain increase during active head movements.

Adult↗

Production of diagnostic rules from a neurotologic database with decision trees.

A decision tree is an artificial intelligence program that is adaptive and is closely related to a neural network, but can handle missing or nondecisive data in decision-making. Data on patients with Meniere's disease, vestibular schwannoma, traumatic vertigo, sudden deafness, benign paroxysmal positional vertigo, and vestibular neuritis were retrieved from the database of the otoneurologic expert system ONE for the development and testing of the accuracy of decision trees in the diagnostic workup. Decision trees were constructed separately for each disease. The accuracies of the best decision trees were 94%, 95%, 99%, 99%, 100%, and 100% for the respective diseases. The most important questions concerned the presence of vertigo, hearing loss, and tinnitus; duration of vertigo; frequency of vertigo attacks; severity of rotational vertigo; onset and type of hearing loss; and occurrence of head injury in relation to the timing of onset of vertigo. Meniere's disease was the most difficult to classify correctly. The validity and structure of the decision trees are easily comprehended and can be used outside the expert system.

Databases, Factual↗

Audiovestibular evolution in a patient with multiple sclerosis.

Multiple sclerosis is characterized by the presence of multiple plaques within the central nervous system, manifesting as remission and exacerbation of neurologic dysfunction over variable time courses. We present the case of a 20-year-old woman. Before treatment, her auditory brain stem response (ABR) test revealed bilateral prolongation. A caloric test showed canal paresis of the right ear and a normal response on the left. A vestibular evoked myogenic potential (VEMP) test displayed an absent response in the right ear and a delayed response in the left. A magnetic resonance imaging (MRI) scan demonstrated multiple diffuse high signal lesions in the hemispheres, brain stem, and cerebellum. Six months after treatment, the demyelinating plaques were shown to have resolved spontaneously on MRI. Recovery of caloric responses was anticipated. Bilateral prolongation of ABRs remained, but the VEMP test disclosed a normal response in the right ear and a delayed response in the left. Accordingly, in addition to MRI, caloric tests and ABR and VEMP tests are useful in monitoring the evolution of audiovestibular function in patients with multiple sclerosis.

Adult↗

Prognosis of inner ear periphery and central vestibular plasticity in sudden deafness with vertigo.

OBJECTIVES: We sought to elucidate the clinical problems and otopathology of patients with sudden deafness with vertigo (SDV). METHODS: In 24 patients with SDV who had significant canal paresis (CP) at their first visit to our hospital between 1997 and 2001, we examined pure tone audiograms, caloric tests, and several questionnaires twice, at the first visit within 5 days after the onset and around 2 years after steroid therapy. RESULTS: These examinations revealed that improvements of auditory and vestibular function in patients with SDV tended to be correlated with one another. Sixteen of the 24 patients (66.7%) still had CP. This rate in SDV was significantly worse than that reported previously for vestibular neuritis (VN). On the other hand, patients with SDV with long-lasting CP had a faster reduction of head-shaking afternystagmus and of handicaps in their everyday life due to dizziness than did patients with VN and CP. CONCLUSIONS: These findings suggest that SDV may deteriorate the inner ear function more severely but accelerate the central vestibular compensation more effectively than VN after the lesion. It is well known that vestibular neurectomy causes much more severe motion-induced dizziness after surgery than does labyrinthectomy. Taken together, these findings suggest different regions of damage in SDV (mainly the labyrinth, as in labyrinthectomy) and VN (mainly the ganglion, as in vestibular neurectomy).

Adult↗

Transient evoked otoacoustic emissions in vestibular neuritis.

OBJECTIVES: Partial or total degeneration of the vestibulocochlear anastomosis at its takeoff from the saccular ganglion and regenerating efferent neural buds under the cochlear outer hair cells (OHCs) have been found in the temporal bones of human patients with a history of vestibular neuritis (VN). We sought to test whether VN has any functional impact on the ipsilateral OHCs by means of transient evoked otoacoustic emission (TEOAE) testing. METHODS: We retrospectively analyzed prospectively collected TEOAE data of 28 patients (19 female, 9 male; age range, 21 to 60 years; median age, 42.5 years). The pure tone air conduction hearing thresholds at each standard audiometric frequency (0.125 to 8 kHz) and the amplitudes of the TEOAEs at the 1-, 2-, 3-, and 4-kHz frequency bands were compared between the lesional and contralateral sides by use of the nonparametric sign test for 2 paired samples. RESULTS: No significant difference was found in the pure tone air conduction hearing thresholds between the lesional and contralateral ears at any of the 11 standard tested frequencies. No significant difference was found between the TEOAE amplitudes of the lesional and contralateral sides. CONCLUSIONS: Subclinical OHC functional impairment is not present in VN. Moreover, no apparent significant changes in medial olivocochlear bundle resting activity accompany VN.

Adult↗

Steroid effects on vestibular compensation in human.

Vestibular neuritis (VN) rapidly damages unilateral vestibular periphery, inducing severe balance disorders. In most cases, such vestibular imbalance is gradually restored to within the normal level after clinical therapies. This successive clinical recovery occurs due to regeneration of vestibular periphery and/or accomplishment of central vestibular compensation. We experienced 36 patients with VN treated at our hospital, including cases in our previous preliminary report. To elucidate effects of steroid therapy both on the recovery of peripheral function and on the adaptation of central vestibular compensation, we examined caloric test and several questionnaires with two randomly divided groups, 18 steroid-treated and 18 nonsteroid-treated patients, over two years after the onset. These examinations revealed that steroid-treated patients had a tendency of better canal improvements (13/18, 72%) than nonsteroid-treated ones (10/18, 55.6%). However, there was no significant difference between these two groups. In cases with persistent canal paresis, steroid-treated patients (n = 5) reduced handicaps in their everyday life due to the dizziness induced by head and/or body movements and the disturbance of their mood, more effectively than those with nonsteroid therapy (n = 8). These findings suggest that steroid therapy with VN could be effective on not only vestibular periphery but central vestibular system, to restore the balance.

Activities of Daily Living↗

Inferior vestibular neuritis: 3 cases with clinical features of acute vestibular neuritis, normal calorics but indications of saccular failure.

BACKGROUND: Vestibular neuritis (VN) is commonly diagnosed by demonstration of unilateral vestibular failure, as unilateral loss of caloric response. As this test reflects the function of the superior part of the vestibular nerve only, cases of pure inferior nerve neuritis will be lost. CASE PRESENTATIONS: We describe three patients with symptoms suggestive of VN, but normal calorics. All 3 had unilateral loss of vestibular evoked myogenic potential. A slight, asymptomatic position dependent nystagmus, with the pathological ear down, was observed. CONCLUSION: We believe that these patients suffer from pure inferior nerve vestibular neuritis.

Aged↗

Vestibular responses to sound.

Research into vestibular responses to sound has evolved in four stages. The first, largely the work of Tullio in the 1920s, involved inspection of the eye, head, and postural responses to sound of alert animals with surgical fenestrae into various parts of the bony labyrinth. The second, begun in 1964 by Bickford and his group and continued by our group and then by others in the last 10 years, involves the measurement of evoked myogenic potentials to air-conducted and bone-conducted clicks and tones in normal humans. The third, begun by Mikaelian at about the same time as Bickford and continued by McCue, our group, and others, involves electrophysiological recordings of primary vestibular afferent neuron responses to sound in anesthetized animals. The fourth involves measurements of vestibulo-ocular responses to sound in humans with the Tullio phenomenon. It was begun by Minor and his group in 1998 with the observation that sound-induced nystagmus in humans, the Tullio phenomenon, aligned with the rotation axis of the superior semicircular canal. They then showed a defect in the temporal bone between the apex of the superior semicircular canal and the middle cranial fossa, which was the cause of most, if not all, cases of sound-induced nystagmus. Here some of the key observations made in each of these four stages are reviewed.

Acoustic Stimulation↗

Canavan's leukodystrophy is associated with defects in cochlear neurodevelopment and deafness.

The authors present the temporal bone histopathology of two siblings (4 months old and 6 months old at autopsy) with Canavan's disease, an autosomal recessive leukodystrophy that is variably associated with sensorineural hearing loss. The histopathology demonstrated bilateral absence of the organ of Corti throughout the apical and basal cochlea and mild secondary atrophy of the spiral ganglia neurons. The vestibular end organs and ganglia were normal. These findings implicate a role of aminoacylase II in the neurodevelopment of the organ of Corti.

Atrophy↗

Migrainous vertigo presenting as episodic positional vertigo.

Migraine can cause vestibular symptoms including positional vertigo. Of 362 consecutive patients presenting with positional vertigo, 10 with migrainous vertigo mimicking benign paroxysmal positional vertigo (BPPV) were identified. The following factors help to distinguish migrainous positional vertigo from BPPV: short-duration symptomatic episodes and frequent recurrences, manifestation early in life, migrainous symptoms during episodes with positional vertigo, and atypical positional nystagmus.

Age of Onset↗

Cerebellar infarction presenting isolated vertigo: frequency and vascular topographical patterns.

OBJECTIVE: To determine the frequency of cerebellar infarction mimicking vestibular neuritis (VN), the pattern of clinical presentation, and the territory of the cerebellar infarction when it simulates VN. METHODS: We studied 240 consecutive cases of isolated cerebellar infarction in the territories of the cerebellar arteries diagnosed by brain MRI from the acute stroke registry at the Keimyung University Dongsan Medical Center. RESULTS: We identified 25 patients (10.4%) with isolated cerebellar infarction who had clinical features suggesting VN. Two types of cerebellar infarction simulating VN were found: isolated spontaneous prolonged vertigo with imbalance as a sole manifestation of cerebellar infarction (n = 24) and isolated spontaneous prolonged vertigo with imbalance as an initial manifestation of cerebellar infarction (n = 1) followed by delayed neurologic deficits 2 days after the onset. The cerebellar infarction territory most commonly involved was the medial branch of the posterior inferior cerebellar artery territory (24/25: 96%), followed by the anterior inferior cerebellar artery territory (1/25: 4%). None of patients with infarcts in the territory of the superior cerebellar artery or multiple cerebellar arteries showed isolated spontaneous prolonged vertigo. CONCLUSIONS: Cerebellar infarction simulating vestibular neuritis is more common than previously thought. Early recognition of the pseudo-vestibular neuritis of vascular cause may allow specific management.

Adult↗

Low recurrence rate of vestibular neuritis: a long-term follow-up.

We examined 103 patients with vestibular neuritis (VN) in a follow-up study (5.7 to 20.5 years, mean 9.8 years). Two patients (1.9%) had developed a second occurrence of VN 29 to 39 months after the first. VN affected the contralateral ear in both and caused less severe distressing vertigo and postural imbalance. Unlike Bell's palsy and sudden hearing loss, a relapse in the same ear did not occur.

Adult↗