Bilateral Vestibulopathy

1. A D E W I J A Y A , M D – D E C E M B E R 2 0 2 0
Bilateral Vestibulopathy

2. Outline:
 Definition
 Epidemiology
 Etiology
 Natural Courses
 Pathophysiology
 Diagnostic Criteria
 Differential Diagnosis
 Management
 Prognosis
 Summary

3. Definition
 Chronic vestibular syndrome which is characterized
by unsteadiness when walking or standing, which
worsen in darkness and/or on uneven ground, or
during head motion.
 Additionally, patients may describe head or body
movement-induced blurred vision or oscillopsia.
There are typically no symptoms while sitting or
lying down under static conditions.
Strupp M, Kim JS, Murofushi T, Straumann D, Jen JC, Rosengren SM, Della Santina CC, Kingma H. Bilateral vestibulopathy: Diagnostic criteria Consensus document of the
Classification Committee of the Bárány Society. Journal of Vestibular Research. 2017 Jan 1;27(4):177-89.

4. Epidemiology
 Prevalence (US): 28 per 100.000
 Incidence: 4-7 %
 Mean age: 50-60 years
Strupp M, Kim JS, Murofushi T, Straumann D, Jen JC, Rosengren SM, Della Santina CC, Kingma H. Bilateral vestibulopathy: Diagnostic criteria Consensus document of the
Classification Committee of the Bárány Society. Journal of Vestibular Research. 2017 Jan 1;27(4):177-89.

5. Etiology
 Mostly unidentified
 Ototoxic drugs
 Bilateral Menière’s disease
 Meningitis
 Genetic mutations
 Association with cerebellar disorders
 etc
Rinne T. , Bronstein A.M. , Rudge P. , Gresty M.A. and Luxon L.M. , Bilateral loss of vestibular function: Clinical findings in 53 patients, J Neurol 245(6-7) (1998), 314–321.
Zingler V.C. , Cnyrim C. , Jahn K. , Weintz E. , Fernbacher J. , Frenzel C. , et al., Causative factors and epidemiology of bilateral vestibulopathy in 255 patients, Ann
Neurol 61(6) (2007), 524–532.

6. Natural Course
 Slowly progressive in 60 % patients
 Causes an impairment of the health-related quality
of life in 90% of the patients
Zingler V.C. , Cnyrim C. , Jahn K. , Weintz E. , Fernbacher J. , Frenzel C. , et al., Causative factors and epidemiology of bilateral vestibulopathy in 255 patients, Ann
Neurol 61(6) (2007), 524–532.
Guinand N. , Boselie F. , Guyot J.P. and Kingma H. , Quality of life of patients with bilateral vestibulopathy, Ann Otol Rhinol Laryngol 121(7) (2012), 471–477.
Zingler V.C. , Weintz E. , Jahn K. , Huppert D. , Cnyrim C. , Brandt T. , et al., Causative factors, epidemiology, and follow-up of bilateral vestibulopathy, Ann N Y Acad Sci
1164 (2009), 505–508.

7. Pathophysiology
 Bilateral impairment or loss of peripheral vestibular input
causes deficits of vestibulo-ocular and vestibulo-spinal
reflexes, orientation, navigation, and spatial memory:
1. The visual world cannot be stabilized on the retina during
high-acceleration head movements, which leads to head-
movement induced oscillopsia and reduced dynamic visual
acuity
2. Balance during standing and locomotion is impeded
3. Lose their sense of earth-verticality and become disoriented
4. Spatial learning performance is delayed as a consequence of
anatomical and functional changes in the hippocampal
formation
Strupp M, Kim JS, Murofushi T, Straumann D, Jen JC, Rosengren SM, Della Santina CC, Kingma H. Bilateral vestibulopathy: Diagnostic criteria Consensus document of the
Classification Committee of the Bárány Society. Journal of Vestibular Research. 2017 Jan 1;27(4):177-89.

8. Diagnostic Criteria
A. Chronic vestibular syndrome with the following symptoms
1. Unsteadiness when walking or standing plus at least one of 2 or 3
2. Movement-induced blurred vision or oscillopsia during walking or quick head/body
movements and/or
3. Worsening of unsteadiness in darkness and/or on uneven ground
B. No symptoms while sitting or lying down under static conditions
C. Bilaterally reduced or absent angular VOR function documented by
– bilaterally pathological horizontal angular VOR gain <0.6, measured by the video-
HIT or scleral-coil technique and/or
– reduced caloric response (sum of bithermal max. peak SPV on each side <6°/sec7)
and/or
– reduced horizontal angular VOR gain <0.1 upon sinusoidal stimulation on a rotatory
chair (0.1Hz, Vmax=50°/sec) and a phase lead >68 degrees (time constant <5sec).
D. Not better accounted for by another disease
Strupp M, Kim JS, Murofushi T, Straumann D, Jen JC, Rosengren SM, Della Santina CC, Kingma H. Bilateral vestibulopathy: Diagnostic criteria Consensus document of the
Classification Committee of the Bárány Society. Journal of Vestibular Research. 2017 Jan 1;27(4):177-89.

9. Diagnostic Criteria (Probable)
A. Chronic vestibular syndrome with the following symptoms
1. Unsteadiness when walking or standing plus at least one of 2 or 3
2. Movement-induced blurred vision or oscillopsia during walking or
quick head/body movements and/or
3. Worsening of unsteadiness in darkness and/or on uneven ground
B. No symptoms while sitting or lying down under static conditions
C. Bilaterally pathological horizontal bedside head impulse test
D. Not better accounted for by another disease
Strupp M, Kim JS, Murofushi T, Straumann D, Jen JC, Rosengren SM, Della Santina CC, Kingma H. Bilateral vestibulopathy: Diagnostic criteria Consensus document of the
Classification Committee of the Bárány Society. Journal of Vestibular Research. 2017 Jan 1;27(4):177-89.

10. Differential Diagnosis
 Cerebellar ataxias without bilateral vestibulopathy
 Downbeat nystagmus syndrome
 Functional dizziness: persistent postural-perceptual dizziness,
phobic postural dizziness, visual induced dizziness
 Unilateral vestibular deficit
 Intoxications
 Vestibular suppressant medications
 Orthostatic tremor
 Visual disorders (if oscillopsia is prominent)
 Peripheral neuropathies
 Movement disorders: Parkinson’s disease, atypical Parkinson’s
syndromes, multiple system atrophies
 Central gait disorders due to normal pressure hydrocephalus,
frontal gait disorders, lower-body Parkinson, subcortical vascular
encephalopathy or multiple sclerosis
Strupp M, Kim JS, Murofushi T, Straumann D, Jen JC, Rosengren SM, Della Santina CC, Kingma H. Bilateral vestibulopathy: Diagnostic criteria Consensus document of the
Classification Committee of the Bárány Society. Journal of Vestibular Research. 2017 Jan 1;27(4):177-89.

11. Management
 Treating the cause (unfortunately the causes is rarely
identified)
 Avoidance of vestibular suppresants and ototoxins
 Vestibular rehabilitation
Herdman SJ, Hall CD, Schubert MC, Das VE, Tusa RJ. Recovery of dynamic visual acuity in bilateral vestibular hypofunction. Arch Otolaryngol HNS 2007;133:383-389
Krebs, D.E., et al., Double-blind, placebo-controlled trial of rehabilitation for bilateral vestibular hypofunction: preliminary report. Otolaryngol Head Neck Surg, 1993.
109(4): p. 735-41.

12. Prognosis
 Often causes some permanent impairment adn
disability
 After 4-5 years, further improvement is unlikely
Lucieer, F., et al. (2018). "Full Spectrum of Reported Symptoms of Bilateral Vestibulopathy Needs Further Investigation-A Systematic Review." Front Neurol 9: 352.

13. Summary
 Chronic vestibular syndrome
 Unsteadiness when walking or standing
 Worsen in darkness and/or on uneven ground, or
during head motion
 Affect health-related quality of life in 90 % patients
 Treating the cause (unfortunately the causes is rarely
identified)
 Avoidance of vestibular suppresants and ototoxins
 Vestibular rehabilitation

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