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At least 19 recordsLinked to original sources
PubMed1996

Bilateral vestibulopathy revisited.

Bilateral vestibular failure (BVF) is an often undetected disorder of the peripheral labyrinths or the eighth nerves. Key symptoms are oscillopsia during locomotion or head movements and unsteadiness, particularly in the dark. Diagnosis is made by a bedside test for defective vestibulo-ocular reflex and the absence of nystagmic reaction to both caloric and rotatory pendular testing. Most frequent etiologies include ototoxicity, cerebellar degeneration, meningitis, neuropathies, sequential vestibular neuritis, autoimmune disorders, tumors, and miscellaneous otological diseases. Idiopathic BVF is found in more than twenty percent of the patients. Recovery is possible but mostly incomplete. Somatosensory and visual input largely substitute the vestibular deficit for spatial orientation, postural balance and ocular motor control.

Diagnosis, Differential↗
PubMed2004

Suggestive linkage to chromosome 6q in families with bilateral vestibulopathy.

BACKGROUND: Of the more than 40 genetically defined dominantly inherited hearing loss syndromes, only a few are associated with bilateral vestibulopathy. No genetic mutations have been identified in families with bilateral vestibulopathy and normal hearing. OBJECTIVE: To perform a genome-wide scan for linkage in four families with dominantly inherited bilateral vestibulopathy. METHODS: Patients in four families reported brief episodes of vertigo followed by imbalance and oscillopsia. Bilateral vestibulopathy was documented with quantitative rotational testing. Most patients with bilateral vestibulopathy also had migraine. A 10 cM genome-wide screen was conducted using 423 microsatellite markers to identify linkage with vestibulopathy. RESULTS: The authors identified a 24 cM region on chromosome 6q suggestive of linkage to vestibulopathy in these four families (maximum lod score of 2.9 at marker D6S1556). A small fifth family with a different phenotype was not linked to this region on chromosome 6q. CONCLUSIONS: This is the first report of linkage in families with dominantly inherited vestibulopathy and normal hearing. Genetic heterogeneity is likely with inherited vestibulopathy.

Chromosomes, Human, Pair 6↗
PubMed2005

Lesion site in idiopathic bilateral vestibulopathy: a galvanic vestibular-evoked myogenic potential study.

CONCLUSION: The result suggests that patients with idiopathic bilateral vestibulopathy may have nerve lesions when the inferior nerve system is affected, while the inferior vestibular nerve system may be spared. OBJECTIVE: To clarify the lesion site in idiopathic bilateral vestibulopathy, an acquired bilateral vestibulopathy of unknown cause. MATERIAL AND METHODS: Two 75-year-old males diagnosed with idiopathic bilateral vestibulopathy were enrolled. Both showed absent or highly decreased responses on the caloric test on both sides. They underwent vestibular-evoked myogenic potential (VEMP) testing by means of acoustical and electrical stimulation. As acoustic stimulation, 95 dB nHL clicks and short tone bursts (500 Hz) were presented, while 3 mA (1 ms) short-duration galvanic stimuli were presented as electrical stimulation. Responses were recorded on the sternocleidomastoid muscles. RESULTS: Both patients showed unilateral absence of VEMPs with both acoustic and short-duration galvanic stimuli.

Acoustic Stimulation↗
PubMedDate not supplied

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↗
PubMed1989

Idiopathic bilateral vestibulopathy.

We report the clinical features of 22 patients with acquired bilateral vestibulopathy of unknown cause. All had either absent or markedly decreased responses to both caloric and rotational testing. They presented with dysequilibrium and imbalance, worse at night; most reported oscillopsia but none had associated hearing loss or other neurologic symptoms. Nine reported prior prolonged episodes of vertigo consistent with the diagnosis of bilateral sequential vestibular neuritis. Of the remaining 13, none had exposure to known ototoxins or a positive family history. Idiopathic bilateral vestibulopathy is an important cause of progressive imbalance in adults and should be considered even though hearing is normal.

Adult↗
PubMed1998

Serum antibodies against membranous labyrinth in patients with "idiopathic" bilateral vestibulopathy.

To investigate the possibility of an autoimmune mechanism in idiopathic bilateral vestibulopathy (IBV), we screened patients' sera for antibodies against inner ear structures. IgG antibodies against membranous labyrinth (ampulla, semicircular canals, saccule and utricle) were detected in 8 of 12 patients by immunofluorescence on rat inner ear cryosections. All but one serum of 22 healthy controls and the sera of 6 patients with known autoimmune disorders showed only background staining. Low-titre anti-nuclear IgM antibodies were present in three control sera and one IBV serum. High-titre anti-nuclear IgM was found in a patient with lupus erythematosus and in one with scleroderma. Anti-nuclear IgM was not organ-specific. No human serum used contained detectable anti-vascular preformed antibodies. Cross-reactivity to sections of liver, kidney, cornea, brain and skeletal muscle was absent. Double-staining for IgG and F-actin, the primary constituent of hair cell cilia, did not show predominant Ig-coating of sensory hair cells. Immunosuppressive therapy in 3 IBV patients did not improve the disorder, probably owing to irreversible loss of sensory and neural structures. These data suggest that the bulk of anti-labyrinthine autoantibodies may be an epiphenomenon, yet a small subgroup of organ-specific autoantibodies may synergize with a cellular response in the development of vestibular lesions.

Autoantibodies↗
PubMed2001

Vestibular function in severe bilateral vestibulopathy.

OBJECTIVES: To assess residual vestibular function in patients with severe bilateral vestibulopathy comparing low frequency sinusoidal rotation with the novel technique of random, high acceleration rotation of the whole body. METHODS: Eye movements were recorded by electro-oculography in darkness during passive, whole body sinusoidal yaw rotations at frequencies between 0.05 and 1.6 Hz in four patients who had absent caloric vestibular responses. These were compared with recordings using magnetic search coils during the first 100 ms after onset of whole body yaw rotation at peak accelerations of 2800 degrees /s(2). Off centre rotations added novel information about otolithic function. RESULTS: Sinusoidal yaw rotations at 0.05 Hz, peak velocity 240 degrees/s yielded minimal responses, with gain (eye velocity/head velocity)<0.02, but gain increased and phase decreased at frequencies between 0.2 and 1.6 Hz in a manner resembling the vestibulo-ocular reflex. By contrast, the patients had profoundly attenuated responses to both centred and eccentric high acceleration transients, representing virtually absent responses to this powerful vestibular stimulus. CONCLUSION: The analysis of the early ocular response to random, high acceleration rotation of the whole body disclosed a profound deficit of semicircular canal and otolith function in patients for whom higher frequency sinusoidal testing was only modestly abnormal. This suggests that the high frequency responses during sinusoidal rotation were of extravestibular origin. Contributions from the somatosensory or central predictor mechanisms, might account for the generation of these responses. Random, transient rotation is better suited than steady state rotation for quantifying vestibular function in vestibulopathic patients.

Adult↗
PubMed2003

Cerebellar ataxia with bilateral vestibulopathy: description of a syndrome and its characteristic clinical sign.

We report four patients with the syndrome of cerebellar ataxia with bilateral vestibulopathy (CABV) and, using search coil oculography, we validate its characteristic clinical sign, namely impairment of the visually enhanced vestibulo-ocular reflex (VVOR) or doll's head reflex. In our four patients, CABV began in the sixth decade of life; they are still ambulant and self-caring 8-20 years after onset. The cause of CABV in our four patients is unknown. None has a family history of cerebellar or vestibular disease; spinocerebellar ataxia (SCA) 1, 2, 3, 6, 7 and Friedreich's ataxia were excluded by genetic testing. Three of the four have a sensory peripheral neuropathy but none has extrapyramidal or significant autonomic problems, and none has gluten sensitivity. We measured eye rotations in response to head-on-trunk head rotations and in response to head-and-trunk (en bloc) rotations. Horizontal smooth pursuit (SP), vestibulo-ocular reflex (VOR) and VVOR gains were measured in response to head rotations at 0.1, 0.3, 0.6 and 1.0 Hz. The optokinetic reflex (OKR) was tested by measuring optokinetic nystagmus slow phase velocity during constant 50 degrees /s rotation of the subject in light. The results showed that CABV patients had impairment of all three compensatory eye movement reflexes, the VOR, the OKR and SP. During VVOR testing, as the frequency of head rotation increased from 0.1 to 1.0 Hz, eye velocity failed to match head velocity, gaze velocity increased, and gaze position errors developed, which were corrected with bursts of saccades, the basis of the clinical sign of an impaired VVOR.

Adult↗
PubMed2005

Immunosuppressive treatment in bilateral vestibulopathy with inner ear antibodies.

CONCLUSIONS: Although vestibular recovery was observed after steroid treatment, it remains uncertain whether this improvement was spontaneous or due to medication. These data do not allow us to generally recommend corticosteroid treatment in patients with BVF and inner ear antibodies. OBJECTIVE: A retrospective study was performed based on the observation of two patients with suspected autoimmune bilateral vestibular failure (BVF) with normal hearing and antilabyrinthine or nervous tissue-specific serum antibodies who showed vestibular recovery after corticosteroid treatment. MATERIAL AND METHODS: Twelve patients with BVF and serum inner ear antibodies who had received imuunosuppressive treatment with corticosteroids were evaluated in terms of medical history, repetitive caloric irrigation and repetitive determination of inner ear antibodies. BVF was complete in four patients and incomplete in eight. RESULTS: After immunosuppressive therapy, four of the 12 patients showed a moderate recovery of the peak slow-phase velocity of horizontal nystagmus induced by bithermal caloric stimulation, which was only transient in two of them.

Adult↗
PubMed2000

Effects of vestibular and cerebellar deficits on gaze and torso stability during ambulation.

We measured gaze, head, and torso stability during ambulation to determine how vestibulo-ocular reflex dysfunction caused by unilateral vestibulopathy, bilateral vestibulopathy, and cerebellar dysfunction might affect image stabilization on the retina. Subjects were tested during standing, walking, and running on a treadmill. Gaze velocity, vestibulo-ocular reflex gain, and head velocities were calculated from angular positions of the eye and head, as well as linear positions of the head and trunk. Mean gaze velocity with a visible, distant target was below 4 degrees /second for all measurement conditions in control and vestibulopathic subjects. The performance of unilaterally vestibulopathic subjects was indistinguishable from that of control subjects except that the former had less vertical translation during walking. Bilaterally vestibulopathic subjects demonstrated less head translation than control subjects but had higher gaze velocity. In subjects with cerebellar dysfunction, gaze velocity was elevated by pathologic nystagmus, but head movements were similar to those of control subjects.

Adult↗
PubMed2001

Age-related changes in vestibular function: a longitudinal study.

The objective of this study was to identify age-related changes in the vestibulo-ocular reflex (VOR) in normal older subjects and patients complaining of disequilibrium followed serially over time. Cross-sectional studies have found decreases in vestibular function in older subjects compared to younger subjects, but no prior longitudinal studies have been made following the same older subjects over time. We measured VOR responses using step and sinusoidal stimuli in 110 normal subjects and 51 patients with disequilibrium of unknown cause. This report focuses on 57 normal subjects who were able to return for five yearly examinations and 7 patients who were found to have severe bilateral vestibulopathy on the initial examination. In the normal subjects, there was a significant shortening of the VOR time constant to step changes in angular velocity and an amplitude-dependent decrease in gain and increase in phase lead of low-frequency sinusoidal responses over the five examinations. There was also a decrease in the gain of visual-vestibular responses at low frequencies of sinusoidal stimulation over the five examinations. The patients with bilateral vestibulopathy showed relatively little change in vestibular function over the five years of follow-up. This is the first longitudinal study to show age-related decreases in vestibular responses in normal older subjects. The VOR changes identified were not associated with symptoms or signs of disequilibrium. A small subgroup of patients with disequilibrium of unknown cause had profound bilateral vestibulopathy that remained stable during follow-up. These patients likely had an underlying disease even though in most cases the disease process could not be identified.

Aged↗
PubMed2006

Pharmacological advances in the treatment of neuro-otological and eye movement disorders.

PURPOSE OF REVIEW: First, to describe the current pharmacological treatment options for peripheral and central vestibular, cerebellar, and ocular motor disorders. Second, to identify vestibular and ocular motor disorders in which treatment trials are warranted. RECENT FINDINGS: Peripheral vestibular disorders: In vestibular neuritis recovery of the peripheral vestibular function can be improved by treatment with oral corticosteroids. In Ménière's disease treatment strategies range from low-salt diet, diuretics, and betahistine, to intratympanic injection of corticosteroids or gentamicin. Unfortunately most of the trials on Ménière's disease do not have an up-to-date design. In bilateral vestibulopathy steroids do not seem to improve vestibular function.Central vestibular, cerebellar, and ocular motor disorders: The use of aminopyridines introduced a new therapeutic principle in the treatment of downbeat and upbeat nystagmus and episodic ataxia type 2 (EA2). These potassium channel blockers presumably increase the activity and excitability of cerebellar Purkinje cells, thereby augmenting the inhibitory influence of these cells on vestibular and cerebellar nuclei. A few studies showed that baclofen improves periodic alternating nystagmus, and gabapentin and memantine, pendular nystagmus. Many other eye movement disorders, however, such as ocular flutter, opsoclonus, central positioning, or see-saw nystagmus are still difficult to treat. SUMMARY: Although progress has been made in the treatment of vestibular neuritis, downbeat and upbeat nystagmus, as well as EA2, state-of-the-art trials must still be performed on many vestibular and ocular motor disorders, namely Ménière's disease, bilateral vestibulopathy, vestibular paroxysmia, vestibular migraine, and many forms of central eye movement disorders.

Animals↗
PubMed1997

Two types of oscillopsia in a patient with idiopathic vestibulopathy.

A patient with an idiopathic bilateral vestibulopathy described two types of oscillopsia, one induced by head movement, the other induced by changing pressure in the right external auditory canal. This is the first report of both types of oscillopsia occurring in the same individual and illustrates their different mechanisms and symptomatology.

Adult↗
PubMed1994

Familial vestibulopathy: a new dominantly inherited syndrome.

Three patients who presented with episodic vertigo followed by gait imbalance and oscillopsia had profound bilateral vestibular loss despite normal hearing. All had a parent with similar findings. The patients, their affected parent, and multiple other family members had a history of migraine headaches, although several of the latter had normal vestibular function. Acetazolamide stopped or markedly decreased the frequency of vertigo attacks in the three patients treated but had little effect on the chronic vestibular loss. This is the first report of a dominantly inherited bilateral vestibulopathy associated with normal hearing.

Acetazolamide↗
PubMed1996

Vestibular dysfunction in chronic inflammatory demyelinating polyneuropathy.

Chronic inflammatory demyelinating polyneuropathy (CIDP) has occasionally been associated with clinical or laboratory evidence (magnetic resonance imaging,[MRI], visual evoked response, and brainstem auditory evoked response [BAER] of cranial neuropathy. In most cases, the relationship of cranial nerve involvement to CIDP remains unclear. A 45-year-old woman noted foot numbness, limb weakness, gait and postural instability, and oscillopsia. An IgG kappa monoclonal gammopathy of undetermined significance was found. Bilateral vestibulopathy was documented by clinical examination, bithermal calorics, rotary chair testing, BAERs, and dynamic posturography. MRI with gadolinium demonstrated enhancement of cranial nerve VIII bilaterally. Over the next 6 years, the patients's relapsing and remitting course of CIDP and vestibulopathy was assessed by quantitative muscle and vestibular function testing (clinically and neurophysiologically), and dynamic visual acuity. There was a striking synchronization between her CIDP and vestibulopathy with respect to clinical course including relapses and responses to immune therapy. The response to therapy, and evidence derived from clinical and laboratory investigations, suggest that the vestibular dysfunction was immune mediated.

Chronic Disease↗