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Orphanet Journal of Rare Diseases
Published in: Orphanet Journal of Rare Diseases 1/2019

Open Access 01-12-2019 | Bilateral Vestibulopathy | Review

Recent advances in idiopathic bilateral vestibulopathy: a literature review

Authors: Chisato Fujimoto, Masato Yagi, Toshihisa Murofushi

Published in: Orphanet Journal of Rare Diseases | Issue 1/2019

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Abstract

Background

Idiopathic bilateral vestibulopathy (IBV) is an acquired bilateral peripheral vestibular dysfunction of unknown etiology, with persistent unsteadiness but without sensorineural hearing loss (SNHL) other than age-related hearing loss (ARHL).

Main text

The prevalence of IBV is unknown. The most common symptom is persistent unsteadiness, particularly in darkness and/or on uneven ground. The other main symptom is oscillopsia during head and body movements. IBV is neither associated with SNHL, except for ARHL, nor any other neurological dysfunction that causes balance disorders. The clinical time course of IBV can generally be divided into two main types: progressive type and sequential type. The progressive type involves gradually progressive persistent unsteadiness without episodes of vertigo. The sequential type involves recurrent vertigo attacks accompanied by persistent unsteadiness. Originally, IBV was found to exhibit bilateral dysfunction in the lateral semicircular canals (LSCCs) and the superior vestibular nerve (SVN) system. However, recently, with the development of more sophisticated vestibular function tests of the otolith organs and vertical semicircular canals, it has been revealed that IBV involves peripheral vestibular lesions other than those already identified in the LSCC and the SVN system. Furthermore, novel subtypes of IBV that do not involve bilateral dysfunction of the LSCC and/or the SVN system have been proposed. Therapeutically, exercise-based vestibular rehabilitation in adult bilateral vestibulopathy (BVP) patients has resulted in improved gaze and postural stability moderately. There are several technical approaches for the treatment of BVP such as vestibular implants, sensory substitution devices and noisy galvanic vestibular stimulation.

Conclusions

Combined use of various vestibular function tests, including recently developed tests, revealed the diversity of lesion sites in IBV. Further studies are required to determine the therapeutic effects of the technical approaches on IBV.
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Literature
1.
Baloh RW, Jacobson K, Honrubia V. Idiopathic bilateral vestibulopathy. Neurology. 1989;39:272–5.CrossRef
2.
Vibert D, Liard P, Hausler R. Bilateral idiopathic loss of peripheral vestibular function with normal hearing. Acta Otolaryngol. 1995;115:611–5.CrossRef
3.
Sargent EW, Goebel JA, Hanson JM, Beck DL. Idiopathic bilateral vestibular loss. Otolaryngol Head Neck Surg. 1997;116:157–62.CrossRef
4.
Priesol AJ, Valko Y, Merfeld DM, Lewis RF. Motion perception in patients with idiopathic bilateral vestibular hypofunction. Otolaryngol Head Neck Surg. 2014;150:1040–2.CrossRef
5.
Kapoula Z, Gaertner C, Yang Q, Denise P, Toupet M. Vergence and standing balance in subjects with idiopathic bilateral loss of vestibular function. PLoS One. 2013;8:e66652.CrossRef
6.
Colebatch JG, Halmagyi GM, Skuse NF. Myogenic potentials generated by a click-evoked vestibulocollic reflex. J Neurol Neurosurg Psychiatry. 1994;57:190–7.CrossRef
7.
Iwasaki S, Chihara Y, Smulders YE, Burgess AM, Halmagyi GM, Curthoys IS, Murofushi T. The role of the superior vestibular nerve in generating ocular vestibular-evoked myogenic potentials to bone conducted vibration at Fz. Clin Neurophysiol. 2009;120:588–93.CrossRef
8.
Murofushi T. Clinical application of vestibular evoked myogenic potential (VEMP). Auris Nasus Larynx. 2016;43:367–76.CrossRef
9.
Murofushi T, Halmagyi GM, Yavor RA, Colebatch JG. Absent vestibular evoked myogenic potentials in vestibular neurolabyrinthitis. An indicator of inferior vestibular nerve involvement? Arch Otolaryngol Head Neck Surg. 1996;122:845–8.CrossRef
10.
MacDougall HG, Weber KP, McGarvie LA, Halmagyi GM, Curthoys IS. The video head impulse test: diagnostic accuracy in peripheral vestibulopathy. Neurology. 2009;73:1134–41.CrossRef
11.
Tarnutzer AA, Bockisch CJ, Buffone E, Weber KP. Hierarchical cluster analysis of Semicircular Canal and otolith deficits in bilateral Vestibulopathy. Front Neurol. 2018;9:244.CrossRef
12.
Matsuzaki M, Murofushi T. Vestibular evoked myogenic potentials in patients with idiopathic bilateral vestibulopathy. Report of three cases. ORL J Otorhinolaryngol Relat Spec. 2001;63:349–52.CrossRef
13.
Fujimoto C, Murofushi T, Sugasawa K, Chihara Y, Ushio M, Yamasoba T, Iwasaki S. Bilateral vestibulopathy with dissociated deficits in the superior and inferior vestibular systems. Ann Otol Rhinol Laryngol. 2012;121:383–8.CrossRef
14.
Fujimoto C, Murofushi T, Chihara Y, Ushio M, Suzuki M, Yamaguchi T, Yamasoba T, Iwasaki S. Effect of severity of vestibular dysfunction on postural instability in idiopathic bilateral vestibulopathy. Acta Otolaryngol. 2013;133:454–61.CrossRef
15.
Fujimoto C, Murofushi T, Chihara Y, Suzuki M, Yamasoba T, Iwasaki S. Novel subtype of idiopathic bilateral vestibulopathy: bilateral absence of vestibular evoked myogenic potentials in the presence of normal caloric responses. J Neurol. 2009;256:1488–92.CrossRef
16.
Fujimoto C, Kinoshita M, Kamogashira T, Egami N, Sugasawa K, Yamasoba T, Iwasaki S. Characteristics of vertigo and the affected vestibular nerve systems in idiopathic bilateral vestibulopathy. Acta Otolaryngol. 2016;136:43–7.CrossRef
17.
Fujimoto C, Iwasaki S, Matsuzaki M, Murofushi T. Lesion site in idiopathic bilateral vestibulopathy: a galvanic vestibular-evoked myogenic potential study. Acta Otolaryngol. 2005;125:430–2.CrossRef
18.
Ward BK, Agrawal Y, Hoffman HJ, Carey JP, Della Santina CC. Prevalence and impact of bilateral vestibular hypofunction: results from the 2008 US National Health Interview Survey. JAMA Otolaryngol Head Neck Surg. 2013;139:803–10.CrossRef
19.
Kim S, Oh YM, Koo JW, Kim JS. Bilateral vestibulopathy: clinical characteristics and diagnostic criteria. Otol Neurotol. 2011;32:812–7.CrossRef
20.
Lucieer F, Vonk P, Guinand N, Stokroos R, Kingma H, van de Berg R. Bilateral vestibular hypofunction: insights in etiologies, clinical subtypes, and diagnostics. Front Neurol. 2016;7:26.CrossRef
21.
Rinne T, Bronstein AM, Rudge P, Gresty MA, Luxon LM. Bilateral loss of vestibular function: clinical findings in 53 patients. J Neurol. 1998;245:314–21.CrossRef
22.
Tarnutzer AA, Bockisch CJ, Buffone E, Weiler S, Bachmann LM, Weber KP. Disease-specific sparing of the anterior semicircular canals in bilateral vestibulopathy. Clin Neurophysiol. 2016;127:2791–801.CrossRef
23.
Zingler VC, Cnyrim C, Jahn K, Weintz E, Fernbacher J, Frenzel C, Brandt T, Strupp M. Causative factors and epidemiology of bilateral vestibulopathy in 255 patients. Ann Neurol. 2007;61:524–32.CrossRef
24.
Arbusow V, Strupp M, Dieterich M, Stocker W, Naumann A, Schulz P, Brandt T. Serum antibodies against membranous labyrinth in patients with “idiopathic” bilateral vestibulopathy. J Neurol. 1998;245:132–6.CrossRef
25.
Elstner M, Schmidt C, Zingler VC, Prokisch H, Bettecken T, Elson JL, Rudolph G, Bender A, Halmagyi GM, Brandt T, et al. Mitochondrial 12S rRNA susceptibility mutations in aminoglycoside-associated and idiopathic bilateral vestibulopathy. Biochem Biophys Res Commun. 2008;377:379–83.CrossRef
26.
Udut EV, Shchetinin PP, Rudenko TV, Lucieer F, Kingma H, Shchetinina AP, Pleshkov MO, Demkin VP, Udut VV. Pathogenetic role of endothelial dysfunction in idiopathic Vestibulopathy. Bull Exp Biol Med. 2018;164:726–9.CrossRef
27.
Baloh RW, Ishyama A, Wackym PA, Honrubia V. Vestibular neuritis: clinical-pathologic correlation. Otolaryngol Head Neck Surg. 1996;114:586–92.CrossRef
28.
Schuknecht HF, Kitamura K. Second Louis H. Clerf Lecture. Vestibular neuritis. Ann Otol Rhinol Laryngol Suppl. 1981;90:1–19.CrossRef
29.
Hemenway WG, Lindsay JR. Postural vertigo due to unilateral sudden partial loss of vestibular function. Ann Otol Rhinol Laryngol. 1956;65:692–706.CrossRef
30.
Bumm P, Schlimok G. lymphocyte subpopulations and HLA-DR determinations in diseases of the inner ear and Bell’s palsy. HNO. 1986;34:525–7.PubMed
31.
Nadol JB Jr. Vestibular neuritis. Otolaryngol Head Neck Surg. 1995;112:162–72.CrossRef
32.
Ogata Y, Sekitani T, Shimogori H, Ikeda T. Bilateral vestibular neuronitis. Acta Otolaryngol Suppl. 1993;503:57–60.CrossRef
33.
Schuknecht HF, Witt RL. Acute bilateral sequential vestibular neuritis. Am J Otolaryngol. 1985;6:255–7.CrossRef
34.
Ruehl RM, Guerkov R. Amiodarone-induced gait unsteadiness is revealed to be bilateral vestibulopathy. Eur J Neurol. 2017;24:e7–8.CrossRef
35.
Gurkov R, Manzari L, Blodow A, Wenzel A, Pavlovic D, Luis L. Amiodarone-associated bilateral vestibulopathy. Eur Arch Otorhinolaryngol. 2018;275:823–5.CrossRef
36.
Gurkov R. Amiodarone: a newly discovered association with bilateral Vestibulopathy. Front Neurol. 2018;9:119.CrossRef
37.
Fife TD, Robb MJA, Steenerson KK, Saha KC. Bilateral vestibular dysfunction associated with chronic exposure to military jet propellant type-eight jet fuel. Front Neurol. 2018;9:351.CrossRef
38.
Acierno MD, Trobe JD, Shepard NT, Cornblath WT, Disher MJ. Two types of oscillopsia in a patient with idiopathic vestibulopathy. J Neuroophthalmol. 1997;17:92–4.PubMed
39.
Murofushi T. Vestibular neuropathy and vestibular evoked myogenic potential. In: Kaga K, Starr A, editors. Neuropathies of the auditory and vestibular eighth cranial nerves. Tokyo: Springer Japan; 2009. p. 85–92.CrossRef
40.
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 Barany Society. J Vestib Res. 2017;27:177–89.CrossRef
41.
Toupet M, Van Nechel C, Hautefort C, Duquesne U, Heuschen S, Bozorg Grayeli A. Subjective visual vertical in idiopathic bilateral vestibular hypofunction: enhanced role of vision, neck, and body proprioception. Otol Neurotol. 2017;38:1010–6.CrossRef
42.
Fujimoto C, Yamamoto Y, Kamogashira T, Kinoshita M, Egami N, Uemura Y, Togo F, Yamasoba T, Iwasaki S. Noisy galvanic vestibular stimulation induces a sustained improvement in body balance in elderly adults. Sci Rep. 2016;6:37575.CrossRef
43.
van Dooren TS, Lucieer FMP, Duijn S, Janssen AML, Guinand N, Perez Fornos A, Van Rompaey V, Kingma H, Ramat S, van de Berg R. The functional head impulse test to assess Oscillopsia in bilateral Vestibulopathy. Front Neurol. 2019;10:365.CrossRef
44.
Guinand N, Pijnenburg M, Janssen M, Kingma H. Visual acuity while walking and oscillopsia severity in healthy subjects and patients with unilateral and bilateral vestibular function loss. Arch Otolaryngol Head Neck Surg. 2012;138:301–6.CrossRef
45.
Kremmyda O, Hufner K, Flanagin VL, Hamilton DA, Linn J, Strupp M, Jahn K, Brandt T. Beyond dizziness: virtual navigation, Spatial Anxiety and Hippocampal Volume in Bilateral Vestibulopathy. Front Hum Neurosci. 2016;10:139.CrossRef
46.
Dobbels B, Peetermans O, Boon B, Mertens G, Van de Heyning P, Van Rompaey V. Impact of bilateral Vestibulopathy on spatial and nonspatial cognition: a systematic review. Ear Hear. 2019;40:757–65.CrossRef
47.
Porciuncula F, Johnson CC, Glickman LB. The effect of vestibular rehabilitation on adults with bilateral vestibular hypofunction: a systematic review. J Vestib Res. 2012;22:283–98.PubMed
48.
Guinand N, Guyot JP, Kingma H, Kos I, Pelizzone M. Vestibular implants: the first steps in humans. Conf Proc IEEE Eng Med Biol Soc. 2011;2011:2262–4.PubMed
49.
Merfeld DM, Lewis RF. Replacing semicircular canal function with a vestibular implant. Curr Opin Otolaryngol Head Neck Surg. 2012;20:386–92.CrossRef
50.
van de Berg R, Guinand N, Nguyen TA, Ranieri M, Cavuscens S, Guyot JP, Stokroos R, Kingma H, Perez-Fornos A. The vestibular implant: frequency-dependency of the electrically evoked vestibulo-ocular reflex in humans. Front Syst Neurosci. 2014;8:255.PubMed
51.
Barros CG, Bittar RS, Danilov Y. Effects of electrotactile vestibular substitution on rehabilitation of patients with bilateral vestibular loss. Neurosci Lett. 2010;476:123–6.CrossRef
52.
Danilov YP, Tyler ME, Skinner KL, Bach-y-Rita P: Efficacy of electrotactile vestibular substitution in patients with bilateral vestibular and central balance loss. Conf Proc IEEE Eng Med Biol Soc. 2006;Suppl:6605–6609.
53.
Dozza M, Chiari L, Horak FB. Audio-biofeedback improves balance in patients with bilateral vestibular loss. Arch Phys Med Rehabil. 2005;86:1401–3.CrossRef
54.
Robinson BS, Cook JL, Richburg CM, Price SE. Use of an electrotactile vestibular substitution system to facilitate balance and gait of an individual with gentamicin-induced bilateral vestibular hypofunction and bilateral transtibial amputation. J Neurol Phys Ther. 2009;33:150–9.CrossRef
55.
Iwasaki S, Yamamoto Y, Togo F, Kinoshita M, Yoshifuji Y, Fujimoto C, Yamasoba T. Noisy vestibular stimulation improves body balance in bilateral vestibulopathy. Neurology. 2014;82:969–75.CrossRef
56.
Pan W, Soma R, Kwak S, Yamamoto Y. Improvement of motor functions by noisy vestibular stimulation in central neurodegenerative disorders. J Neurol. 2008;255:1657–61.CrossRef
57.
Soma R, Nozaki D, Kwak S, Yamamoto Y. 1/f noise outperforms white noise in sensitizing baroreflex function in the human brain. Phys Rev Lett. 2003;91:078101.CrossRef
58.
Yamamoto Y, Struzik ZR, Soma R, Ohashi K, Kwak S. Noisy vestibular stimulation improves autonomic and motor responsiveness in central neurodegenerative disorders. Ann Neurol. 2005;58:175–81.CrossRef
59.
Iwasaki S, Fujimoto C, Egami N, Kinoshita M, Togo F, Yamamoto Y, Yamasoba T. Noisy vestibular stimulation increases gait speed in normals and in bilateral vestibulopathy. Brain Stimul. 2018;11:709–15.CrossRef
60.
Wuehr M, Nusser E, Decker J, Krafczyk S, Straube A, Brandt T, Jahn K, Schniepp R. Noisy vestibular stimulation improves dynamic walking stability in bilateral vestibulopathy. Neurology. 2016;86:2196–202.CrossRef
61.
McDonnell MD, Ward LM. The benefits of noise in neural systems: bridging theory and experiment. Nat Rev Neurosci. 2011;12:415–26.CrossRef
62.
Wuehr M, Boerner JC, Pradhan C, Decker J, Jahn K, Brandt T, Schniepp R. Stochastic resonance in the human vestibular system - noise-induced facilitation of vestibulospinal reflexes. Brain Stimul. 2018;11:261–3.CrossRef
63.
Fujimoto C, Egami N, Kawahara T, Uemura Y, Yamamoto Y, Yamasoba T, Iwasaki S. Noisy galvanic vestibular stimulation sustainably improves posture in bilateral Vestibulopathy. Front Neurol. 2018;9:900.CrossRef
Metadata
Title
Recent advances in idiopathic bilateral vestibulopathy: a literature review
Authors
Chisato Fujimoto
Masato Yagi
Toshihisa Murofushi
Publication date
01-12-2019
Publisher
BioMed Central
Published in
Orphanet Journal of Rare Diseases / Issue 1/2019
Electronic ISSN: 1750-1172
DOI
https://doi.org/10.1186/s13023-019-1180-8

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