Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Primer
  • Published:

Meniere's disease

Abstract

Meniere's disease (MD) is a disorder of the inner ear that causes vertigo attacks, fluctuating hearing loss, tinnitus and aural fullness. The aetiology of MD is multifactorial. A characteristic sign of MD is endolymphatic hydrops (EH), a disorder in which excessive endolymph accumulates in the inner ear and causes damage to the ganglion cells. In most patients, the clinical symptoms of MD present after considerable accumulation of endolymph has occurred. However, some patients develop symptoms in the early stages of EH. The reason for the variability in the symptomatology is unknown and the relationship between EH and the clinical symptoms of MD requires further study. The diagnosis of MD is based on clinical symptoms but can be complemented with functional inner ear tests, including audiometry, vestibular-evoked myogenic potential testing, caloric testing, electrocochleography or head impulse tests. MRI has been optimized to directly visualize EH in the cochlea, vestibule and semicircular canals, and its use is shifting from the research setting to the clinic. The management of MD is mainly aimed at the relief of acute attacks of vertigo and the prevention of recurrent attacks. Therapeutic options are based on empirical evidence and include the management of risk factors and a conservative approach as the first line of treatment. When medical treatment is unable to suppress vertigo attacks, intratympanic gentamicin therapy or endolymphatic sac decompression surgery is usually considered. This Primer covers the pathophysiology, symptomatology, diagnosis, management, quality of life and prevention of MD.

This is a preview of subscription content, access via your institution

Access options

Buy this article

Prices may be subject to local taxes which are calculated during checkout

Figure 1: Clinical symptoms associated with Meniere's disease.
Figure 2: Diagram of the inner ear.
Figure 3: Cumulative age distribution of onset of symptoms in patients with Meniere's disease.
Figure 4: Endolymphatic hydrops.
Figure 5: Mouse model of endolymphatic hydrops.
Figure 6: Electrocochleography in a patient with left Meniere's disease.
Figure 7: Vestibular-evoked myogenic potential in a patient with right Meniere's disease.
Figure 8: Visualization of endolymphatic hydrops using MRI and intratympanic gadolinium-based contrast agent.
Figure 9: Visualization of endolymphatic hydrops using MRI and intravenous gadolinium-based contrast agent.
Figure 10: New proposed classification of Meniere's disease with the use of endolymphatic hydrops imaging.

Similar content being viewed by others

References

  1. Menière, P. Maladie de l'oreille interne offrant les symtômes de la congestion cérébrale apoplectiforme. Gaz. Med. Paris 16, 88–89 (in French) (1861).

    Google Scholar 

  2. Menière, P. Mémoire sur les lésions de l'oreille interne donnant lieu à des symtômes de congestion cérébrale apoplectiforme. Gaz. Med. Paris 16, 597–601 (in French) (1861).

    Google Scholar 

  3. Minor, L. B., Schessel, D. A. & Carey, J. P. Meniere's disease. Curr. Opin. Neurol. 17, 9–16 (2004).

    Article  PubMed  Google Scholar 

  4. Sajjadi, H. & Paparella, M. M. Meniere's disease. Lancet 372, 406–414 (2008).

    Article  PubMed  Google Scholar 

  5. Pyykkö, I., Nakashima, T., Yoshida, T., Zou, J. & Naganawa, S. Meniere's disease: a reappraisal supported by a variable latency of symptoms and the MRI visualisation of endolymphatic hydrops. BMJ Open 3, e001555 (2013). MRI frequently reveals EH in the monosymptomatic ear.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Hallpike, C. S. & Cairns, H. Observations on the pathology of Ménière's syndrome. J. Laryngol. Otol. 53, 625–655 (1938).

    Article  Google Scholar 

  7. Yamakawa, K. Über die patologische Veränderungen bei einem Menière-kranken. J. Otorhinolaryngol. Soc. Jpn 44, 2310–2312 (1938).

    Google Scholar 

  8. Merchant, S. N., Adams, J. C. & Nadol, J. B. Pathophysiology of Meniere's syndrome: are symptoms caused by endolymphatic hydrops? Otol. Neurotol. 26, 74–81 (2005). EH is regarded as a histological marker of MD based on an observational study of histopathological specimens of temporal bone.

    Article  PubMed  Google Scholar 

  9. Foster, C. A. & Breeze, R. E. Endolymphatic hydrops in Meniere's disease: cause, consequence, or epiphenomenon? Otol. Neurotol. 34, 1210–1214 (2013). EH alone is insufficient to cause MD, but one or more additional cofactors are required to turn asymptomatic EH into symptomatic MD.

    Article  PubMed  Google Scholar 

  10. Cha, Y. H., Brodsky, J., Ishiyama, G., Sabatti, C. & Baloh, R. W. The relevance of migraine in patients with Meniere's disease. Acta Otolaryngol. 127, 1241–1245 (2007).

    Article  CAS  PubMed  Google Scholar 

  11. Rauch, S. D. Clinical hints and precipitating factors in patients suffering from Meniere's disease. Otolaryngol. Clin. North Am. 43, 1011–1017 (2010).

    Article  PubMed  Google Scholar 

  12. Committee on Hearing and Equilibrium. Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere's disease. Otolaryngol. Head Neck Surg. 113, 181–185 (1995).

    Article  Google Scholar 

  13. Nakashima, T. et al. Visualization of endolymphatic hydrops in patients with Meniere's disease. Laryngoscope 117, 415–420 (2007). This study includes the first visualization of EH in patients with MD with use of 3D fluid-attenuated inversion recovery MRI.

    Article  PubMed  Google Scholar 

  14. Miyagawa, M. et al. Endolymphatic hydrops and therapeutic effects are visualized in ‘atypical’ Meniere's disease. Acta Otolaryngol. 129, 1326–1329 (2009).

    Article  PubMed  Google Scholar 

  15. Fiorino, F., Pizzini, F. B., Beltramello, A. & Barbieri, F. MRI performed after intratympanic gadolinium administration in patients with Meniere's disease: correlation with symptoms and signs. Eur. Arch. Otorhinolaryngol. 268, 181–187 (2011).

    Article  PubMed  Google Scholar 

  16. Gürkov, R. et al. In vivo visualized endolymphatic hydrops and inner ear functions in patients with electrocochleographically confirmed Meniere's disease. Otol. Neurotol. 33, 1040–1045 (2012).

    Article  PubMed  Google Scholar 

  17. Kato, M. et al. Endolymphatic hydrops revealed by magnetic resonance imaging in patients with atypical Meniere's disease. Acta Otolaryngol. 133, 123–129 (2013).

    Article  PubMed  Google Scholar 

  18. Le, C. H., Truong, A. Q. & Diaz, R. C. Novel techniques for the diagnosis of Meniere's disease. Curr. Opin. Otolaryngol. Head Neck Surg. 21, 492–496 (2013).

    Article  PubMed  Google Scholar 

  19. Liu, F. et al. Noninvasive evaluation of the effect of endolymphatic sac decompression in Meniere's disease using magnetic resonance imaging. Acta Otolaryngol. 134, 666–671 (2014).

    Article  PubMed  Google Scholar 

  20. Committee on Hearing and Equilibrium. Report of subcommittee on equilibrium and its measurement. Meniere's disease: criteria for diagnosis and evaluation of therapy for reporting. Trans. Am. Acad. Ophthalmol. Otolaryngol. 76, 1462–1464 (1972).

    Google Scholar 

  21. Lopez-Escamez, J. A. et al. Diagnostic criteria for Meniere's disease. J. Vestib. Res. 25, 1–7 (2015).

    PubMed  Google Scholar 

  22. Tyrrell, J. S., Whinney, D. J., Ukoumunne, O. C., Fleming, L. E. & Osborne, N. J. Prevalence, associated factors, and comorbid conditions for Meniere's disease. Ear Hear. 35, e162–e169 (2014).

    Article  PubMed  Google Scholar 

  23. Harris, J. P. & Alexander, T. H. Current-day prevalence of Meniere's syndrome. Audiol. Neurootol. 15, 318–322 (2010).

    Article  PubMed  Google Scholar 

  24. Stahle, J., Stahle, C. & Arenberg, I. K. Incidence of Meniere's disease. Arch. Otolaryngol. 104, 99–102 (1978).

    Article  CAS  PubMed  Google Scholar 

  25. Wladislavosky-Waserman, P., Facer, G. W., Mokri, B. & Kurland, L. T. Meniere's disease: a 30-year epidemiologic and clinical study in Rochester, Mn, 1951–1980. Laryngoscope 94, 1098–1102 (1984).

    Article  CAS  PubMed  Google Scholar 

  26. Kotimaki, J., Sorri, M., Aantaa, E. & Nuutinen, J. Prevalence of Meniere disease in Finland. Laryngoscope 109, 748–753 (1999).

    Article  CAS  PubMed  Google Scholar 

  27. Havia, M., Kentala, E. & Pyykko, I. Prevalence of Meniere's disease in general population of Southern Finland. Otolaryngol. Head Neck Surg. 133, 762–768 (2005).

    Article  PubMed  Google Scholar 

  28. Meyerhoff, W. L., Paparella, M. M. & Shea, D. Meniere's disease in children. Laryngoscope 88, 1504–1511 (1978).

    Article  CAS  PubMed  Google Scholar 

  29. Filipo, R. & Barbara, M. Juvenile Meniere's disease. J. Laryngol. Otol. 99, 193–196 (1985).

    Article  CAS  PubMed  Google Scholar 

  30. Hausler, R., Toupet, M., Guidetti, G., Basseres, F. & Montandon, P. Meniere's disease in children. Am. J. Otolaryngol. 8, 187–193 (1987).

    Article  CAS  PubMed  Google Scholar 

  31. Choung, Y. H., Park, K., Kim, C. H., Kim, H. J. & Kim, K. Rare cases of Meniere's disease in children. J. Laryngol. Otol. 120, 343–352 (2006).

    Article  CAS  PubMed  Google Scholar 

  32. Brantberg, K., Duan, M. & Falahat, B. Meniere's disease in children aged 4–7 years. Acta Otolaryngol. 132, 505–509 (2012).

    Article  PubMed  Google Scholar 

  33. Thomas, K. & Harrison, M. S. Long-term follow up of 610 cases of Meniere's disease. Proc. R. Soc. Med. 64, 853–857 (1971).

    CAS  PubMed  PubMed Central  Google Scholar 

  34. Friberg, U., Stahle, J. & Svedberg, A. The natural course of Meniere's disease. Acta Otolaryngol. Suppl. 406, 72–77 (1984).

    CAS  PubMed  Google Scholar 

  35. Havia, M. & Kentala, E. Progression of symptoms of dizziness in Meniere's disease. Arch. Otolaryngol. Head Neck Surg. 130, 431–435 (2004).

    Article  PubMed  Google Scholar 

  36. Huang, C. H. & Young, Y. H. Bilateral Meniere's disease assessed by an inner ear test battery. Acta Otolaryngol. 135, 233–238 (2015).

    Article  PubMed  Google Scholar 

  37. House, J. W., Doherty, J. K., Fisher, L. M., Derebery, M. J. & Berliner, K. I. Meniere's disease: prevalence of contralateral ear involvement. Otol. Neurotol. 27, 355–361 (2006).

    Article  PubMed  Google Scholar 

  38. Huppert, D., Strupp, M. & Brandt, T. Long-term course of Meniere's disease revisited. Acta Otolaryngol. 130, 644–651 (2010).

    Article  PubMed  Google Scholar 

  39. Shojaku, H. et al. Epidemiologic characteristics of definite Meniere's disease in Japan. A long-term survey of Toyama and Niigata prefectures. ORL J. Otorhinolaryngol Relat. Spec. 67, 305–309 (2005).

    Article  PubMed  Google Scholar 

  40. Shojaku, H. et al. Changes in the characteristics of definite Meniere's disease over time in Japan: a long-term survey by the Peripheral Vestibular Disorder Research Committee of Japan, formerly the Meniere's Disease Research Committee of Japan. Acta Otolaryngol. 129, 155–160 (2009).

    Article  PubMed  Google Scholar 

  41. Morrison, A. W., Bailey, M. E. & Morrison, G. A. Familial Meniere's disease: clinical and genetic aspects. J. Laryngol. Otol. 123, 29–37 (2009).

    Article  CAS  PubMed  Google Scholar 

  42. Klockars, T. & Kentala, E. Inheritance of Meniere's disease in the Finnish population. Arch. Otolaryngol. Head Neck Surg. 133, 73–77 (2007).

    Article  PubMed  Google Scholar 

  43. Cha, Y. H., Kane, M. J. & Baloh, R. W. Familial clustering of migraine, episodic vertigo, and Meniere's disease. Otol. Neurotol. 29, 93–96 (2008).

    Article  PubMed  PubMed Central  Google Scholar 

  44. Arweiler-Harbeck, D., Horsthemke, B., Jahnke, K. & Hennies, H. C. Genetic aspects of familial Meniere's disease. Otol. Neurotol. 32, 695–700 (2011).

    Article  PubMed  Google Scholar 

  45. Derebery, M. J. & Berliner, K. I. Prevalence of allergy in Meniere's disease. Otolaryngol. Head Neck Surg. 123, 69–75 (2000).

    Article  CAS  PubMed  Google Scholar 

  46. Koyama, S., Mitsuishi, Y., Bibee, K., Watanabe, I. & Terasaki, P. I. HLA associations with Meniere's disease. Acta Otolaryngol. 113, 575–578 (1993).

    Article  CAS  PubMed  Google Scholar 

  47. Yeo, S. W. et al. Influence of human leukocyte antigen in the pathogenesis of Meniere's disease in the South Korean population. Acta Otolaryngol. 122, 851–856 (2002).

    CAS  PubMed  Google Scholar 

  48. Lempert, T. & Neuhauser, H. Epidemiology of vertigo, migraine and vestibular migraine. J. Neurol. 256, 333–338 (2009).

    Article  PubMed  Google Scholar 

  49. Radtke, A. et al. Migraine and Meniere's disease: is there a link? Neurology 59, 1700–1704 (2002).

    Article  CAS  PubMed  Google Scholar 

  50. Nakada, T. et al. Endolymphatic space size in patients with vestibular migraine and Meniere's disease. J. Neurol. 261, 2079–2084 (2014).

    Article  PubMed  Google Scholar 

  51. Okuno, T. & Sando, I. Localization, frequency, and severity of endolymphatic hydrops and the pathology of the labyrinthine membrane in Meniere's disease. Ann. Otol. Rhinol. Laryngol. 96, 438–445 (1987).

    Article  CAS  PubMed  Google Scholar 

  52. Paparella, M. M. & Djalilian, H. R. Etiology, pathophysiology of symptoms, and pathogenesis of Meniere's disease. Otolaryngol. Clin. North Am. 35, 529–545 (2002).

    Article  PubMed  Google Scholar 

  53. Ikeda, M. & Sando, I. Endolymphatic duct and sac in patients with Meniere's disease. A temporal bone histopathological study. Ann. Otol. Rhinol. Laryngol. 93, 540–546 (1984).

    Article  CAS  PubMed  Google Scholar 

  54. Wackym, P. A. Histopathologic findings in Meniere's disease. Otolaryngol. Head Neck Surg. 112, 90–100 (1995).

    Article  CAS  PubMed  Google Scholar 

  55. Gibson, W. P. & Arenberg, I. K. Pathophysiologic theories in the etiology of Meniere's disease. Otolaryngol. Clin. North Am. 30, 961–967 (1997).

    CAS  PubMed  Google Scholar 

  56. Schuknecht, H. F. Pathology of the Ear (Harvard Univ. Press, 1993).

    Google Scholar 

  57. Nadol, J. B. & Thornton, A. R. Ultrastructural findings in a case of Menière's disease. Ann. Otol. Rhinol. Laryngol. 96, 449–454 (1987).

    Article  PubMed  Google Scholar 

  58. Conlon, B. J. & Gibson, W. P. Electrocochleography in the diagnosis of Meniere's disease. Acta Otolaryngol. 120, 480–483 (2000).

    Article  CAS  PubMed  Google Scholar 

  59. Yamamoto, M. et al. Relationship between the degree of endolymphatic hydrops and electrocochleography. Audiol. Neurootol. 15, 254–260 (2010).

    Article  PubMed  Google Scholar 

  60. Kimura, R. S. & Schuknecht, H. F. Membranous hydrops in the inner ear of the guinea pig after obliteration of the endolymphatic sac. Pract. Otolaryngol. 27, 343–354 (1965). This paper shows the formation of EH in guinea pigs by obliteration of the endolymphatic duct and sac.

    Google Scholar 

  61. Koskas, H. J., Linthicum, F. H. Jr & House, W. F. Membranous ruptures in Meniere's disease: existence, location, and incidence. Otolaryngol. Head Neck Surg. 91, 61–67 (1983).

    Article  CAS  PubMed  Google Scholar 

  62. Semaan, M. T., Alagramam, K. N. & Megerian, C. A. The basic science of Meniere's disease and endolymphatic hydrops. Curr. Opin. Otolaryngol. Head Neck Surg. 13, 301–307 (2005).

    Article  PubMed  Google Scholar 

  63. Marshall, A. F. et al. Magnetic resonance imaging of guinea pig cochlea after vasopressin-induced or surgically induced endolymphatic hydrops. Otolaryngol. Head Neck Surg. 142, 260–265 (2010).

    Article  PubMed  Google Scholar 

  64. Salt, A. N. & Plontke, S. K. Endolymphatic hydrops: pathophysiology and experimental models. Otolaryngol. Clin. North Am. 43, 971–983 (2010).

    Article  PubMed  PubMed Central  Google Scholar 

  65. Harris, J. P. & Ryan, A. F. Effect of a middle ear immune response on inner ear antibody levels. Ann. Otol. Rhinol. Laryngol. 94, 202–206 (1985).

    Article  CAS  PubMed  Google Scholar 

  66. Gloddek, B., Lamm, K. & Haslov, K. Influence of middle ear immune response on the immunological state and function of the inner ear. Laryngoscope 102, 177–181 (1992).

    Article  CAS  PubMed  Google Scholar 

  67. Seki, M., Miyasaka, H., Edamatsu, H. & Watanabe, K. Changes in permeability of strial vessels following vibration given to auditory ossicle by drill. Ann. Otol. Rhinol. Laryngol. 110, 122–126 (2001).

    Article  CAS  PubMed  Google Scholar 

  68. Zou, J., Bretlau, P., Pyykkö, I., Starck, J. & Toppila, E. Sensorineural hearing loss after vibration: an animal model for evaluating prevention and treatment of inner ear hearing loss. Acta Otolaryngol. 121, 143–148 (2001).

    Article  CAS  PubMed  Google Scholar 

  69. Megerian, C. A. Diameter of the cochlear nerve in endolymphatic hydrops: implications for the etiology of hearing loss in Meniere's disease. Laryngoscope 115, 1525–1535 (2005).

    Article  PubMed  Google Scholar 

  70. Bixenstine, P. J., Maniglia, M. P., Vasanji, A., Alagramam, K. N. & Megerian, C. A. Spiral ganglion degeneration patterns in endolymphatic hydrops. Laryngoscope 118, 1217–1223 (2008).

    Article  PubMed  Google Scholar 

  71. Megerian, C. A. et al. A mouse model with postnatal endolymphatic hydrops and hearing loss. Hear. Res. 237, 90–105 (2008).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  72. Melki, S. J. et al. A mouse model validates the utility of electrocochleography in verifying endolymphatic hydrops. J. Assoc. Res. Otolaryngol. 15, 413–421 (2014).

    Article  PubMed  PubMed Central  Google Scholar 

  73. Sheykholeslami, K., Megerian, C. A. & Zheng, Q. Y. Vestibular evoked myogenic potentials in normal mice and Phex mice with spontaneous endolymphatic hydrops. Otol. Neurotol. 30, 535–544 (2009).

    Article  PubMed  PubMed Central  Google Scholar 

  74. Semaan, M. T. et al. Characterization of neuronal cell death in the spiral ganglia of a mouse model of endolymphatic hydrops. Otol. Neurotol. 34, 559–569 (2013).

    Article  PubMed  PubMed Central  Google Scholar 

  75. Momin, S. R. et al. Hearing preservation in guinea pigs with long-standing endolymphatic hydrops. Otol. Neurotol. 32, 1583–1589 (2011).

    Article  PubMed  PubMed Central  Google Scholar 

  76. Kitahara, T. et al. Meniere's attacks occur in the inner ear with excessive vasopressin type-2 receptors. J. Neuroendocrinol. 20, 1295–1300 (2008).

    Article  CAS  PubMed  Google Scholar 

  77. Morrison, A. W. & Johnson, K. J. Genetics (molecular biology) and Meniere's disease. Otolaryngol. Clin. North Am. 35, 497–516 (2002).

    Article  PubMed  Google Scholar 

  78. Vrabec, J. T. Genetic investigations of Meniere's disease. Otolaryngol. Clin. North Am. 43, 1121–1132 (2010).

    Article  PubMed  Google Scholar 

  79. Hietikko, E., Sorri, M., Mannikko, M. & Kotimaki, J. Higher prevalence of autoimmune diseases and longer spells of vertigo in patients affected with familial Meniere's disease: a clinical comparison of familial and sporadic Meniere's disease. Am. J. Audiol. 23, 232–237 (2014).

    Article  PubMed  Google Scholar 

  80. Hietikko, E., Kotimaki, J., Okuloff, A., Sorri, M. & Mannikko, M. A replication study on proposed candidate genes in Meniere's disease, and a review of the current status of genetic studies. Int. J. Audiol. 51, 841–845 (2012).

    Article  PubMed  Google Scholar 

  81. Requena, T. et al. Identification of two novel mutations in FAM136A and DTNA genes in autosomal-dominant familial Meniere's disease. Hum. Mol. Genet. 24, 1119–1126 (2015).

    Article  CAS  PubMed  Google Scholar 

  82. Uno, A. et al. Changes in endolymphatic hydrops after sac surgery examined by Gd-enhanced MRI. Acta Otolaryngol. 133, 924–929 (2013).

    Article  CAS  PubMed  Google Scholar 

  83. Suga, K. et al. Changes in endolymphatic hydrops in patients with Meniere's disease treated conservatively for more than 1 year. Acta Otolaryngol. 135, 866–870 (2015).

    Article  PubMed  Google Scholar 

  84. Sepahdari, A. R., Vorasubin, N., Ishiyama, G. & Ishiyama, A. Endolymphatic hydrops reversal following acetazolamide therapy: demonstration with delayed intravenous contrast-enhanced 3D-FLAIR MRI. AJNR Am. J. Neuroradiol. 37, 151–154 (2016).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  85. Békésy, G. Direct observation of the vibrations of the cochlear partition under a microscope. Acta Otolaryngol. 42, 197–201 (1952).

    Article  PubMed  Google Scholar 

  86. Robles, L. & Ruggero, M. A. Mechanics of the mammalian cochlea. Physiol. Rev. 81, 1305–1352 (2001).

    Article  CAS  PubMed  Google Scholar 

  87. Yamashita, T. & Schuknecht, H. F. Apical endolymphatic hydrops. Arch. Otolaryngol. 108, 463–466 (1982).

    Article  CAS  PubMed  Google Scholar 

  88. Wu, Q., Dai, C., Zhao, M. & Sha, Y. The correlation between symptoms of definite Meniere's disease and endolymphatic hydrops visualized by magnetic resonance imaging. Laryngoscope 126, 974–979 (2016).

    Article  PubMed  Google Scholar 

  89. Stahle, J. & Bergman, B. The caloric reaction in meniere's disease: an electronystagmographical study in 300 patients. Laryngoscope 77, 1629–1643 (1967).

    Article  CAS  PubMed  Google Scholar 

  90. Bance, M., Mai, M., Tomlinson, D. & Rutka, J. The changing direction of nystagmus in acute Meniere's disease: pathophysiological implications. Laryngoscope 101, 197–201 (1991).

    Article  CAS  PubMed  Google Scholar 

  91. Lackner, J. R. Motion sickness: more than nausea and vomiting. Exp. Brain Res. 232, 2493–2510 (2014).

    Article  PubMed  PubMed Central  Google Scholar 

  92. Schuknecht, H. F. Pathophysiology of endolymphatic hydrops. Arch. Otorhinolaryngol. 212, 253–262 (1976).

    Article  CAS  PubMed  Google Scholar 

  93. Kingma, C. M. & Wit, H. P. The effect of changes in perilymphatic K+ on the vestibular evoked potential in the guinea pig. Eur. Arch. Otorhinolaryngol. 267, 1679–1684 (2010).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  94. Schuknecht, H. (ed.) Histopathology of Meniere's Disease (Kugler, 1990).

    Google Scholar 

  95. Tonndorf, J. Vestibular signs and symptoms in Meniere's disorder: mechanical considerations. Acta Otolaryngol. 95, 421–430 (1983).

    Article  CAS  PubMed  Google Scholar 

  96. Gibson, W. P. Hypothetical mechanism for vertigo in Meniere's disease. Otolaryngol. Clin. North Am. 43, 1019–1027 (2010). This paper suggests that abrupt flow or a change in volume inside the endolymphatic space causes the vertigo attacks associated with MD.

    Article  PubMed  Google Scholar 

  97. Yamane, H. et al. Blockage of reuniting duct in Meniere's disease. Acta Otolaryngol. 130, 233–239 (2010).

    Article  PubMed  Google Scholar 

  98. Manzari, L., Burgess, A. M. & Curthoys, I. S. Vestibular function in Lermoyez syndrome at attack. Eur. Arch. Otorhinolaryngol. 269, 685–691 (2012).

    Article  PubMed  Google Scholar 

  99. Viana, L. M., Bahmad, F. Jr & Rauch, S. D. Intratympanic gentamicin as a treatment for drop attacks in patients with Meniere's disease. Laryngoscope 124, 2151–2154 (2014).

    Article  CAS  PubMed  Google Scholar 

  100. Kentala, E., Havia, M. & Pyykko, I. Short-lasting drop attacks in Meniere's disease. Otolaryngol. Head Neck Surg. 124, 526–530 (2001).

    Article  CAS  PubMed  Google Scholar 

  101. Ozeki, H., Iwasaki, S. & Murofushi, T. Vestibular drop attack secondary to Meniere's disease results from unstable otolithic function. Acta Otolaryngol. 128, 887–891 (2008).

    Article  PubMed  Google Scholar 

  102. Baloh, R. W., Jacobson, K. & Winder, T. Drop attacks with Meniere's syndrome. Ann. Neurol. 28, 384–387 (1990).

    Article  CAS  PubMed  Google Scholar 

  103. Sakata, T. & Kato, T. Feeling of ear fullness in acute sensorineural hearing loss. Acta Otolaryngol. 126, 828–833 (2006).

    Article  PubMed  Google Scholar 

  104. Yoshida, T. et al. Endolymphatic hydrops in patients with tinnitus as the major symptom. Eur. Arch. Otorhinolaryngol. 270, 3043–3048 (2013).

    Article  PubMed  Google Scholar 

  105. Yoshida, T. K. et al. MRI evaluation of the bilateral endolymphatic space in patients with unilateral Meniere's disease. Equilib. Res. 72, 247–253 (2013).

    Article  Google Scholar 

  106. Gu, X. et al. Diagnostic advantages of intratympanically gadolinium contrast-enhanced magnetic resonance imaging in patients with bilateral Meniere's disease. Am. J. Otolaryngol. 36, 67–73 (2015). This paper reports the MRI evaluation of the size of the endolymphatic space in both ears in patients with bilateral and unilateral MD, which revealed a profound relationship between the affected ear and the presence of EH, but some patients with unilateral MD also had EH in the unaffected side.

    Article  PubMed  Google Scholar 

  107. Yazawa, Y. & Kitahara, M. Bilateral endolymphatic hydrops in Meniere's disease: review of temporal bone autopsies. Ann. Otol. Rhinol. Laryngol. 99, 524–528 (1990).

    Article  CAS  PubMed  Google Scholar 

  108. Jerin, C., Krause, E., Ertl-Wagner, B. & Gürkov, R. Longitudinal assessment of endolymphatic hydrops with contrast-enhanced magnetic resonance imaging of the labyrinth. Otol. Neurotol. 35, 880–883 (2014).

    Article  PubMed  Google Scholar 

  109. Gürkov, R. et al. Herniation of the membranous labyrinth into the horizontal semicircular canal is correlated with impaired caloric response in Meniere's disease. Otol. Neurotol. 33, 1375–1379 (2012).

    Article  PubMed  Google Scholar 

  110. Levo, H., Stephens, D., Poe, D., Kentala, E. & Pyykko, I. Use of ICF in assessing the effects of Meniere's disorder on life. Ann. Otol. Rhinol. Laryngol. 119, 583–589 (2010).

    Article  PubMed  Google Scholar 

  111. Levo, H., Stephens, D., Poe, D., Kentala, E. & Pyykkö, I. Fatigue in Meniere's disease. Int. J. Rehabil. Res. 11, 191–197 (2013).

    Google Scholar 

  112. Havia, M., Kentala, E. & Pyykko, I. Hearing loss and tinnitus in Meniere's disease. Auris Nasus Larynx 29, 115–119 (2002).

    Article  CAS  PubMed  Google Scholar 

  113. Havia, M., Kentala, E. & Pyykko, I. Postural instability in Meniere's disease. J. Vestib. Res. 14, 37–46 (2004).

    CAS  PubMed  Google Scholar 

  114. Grill, E., Strupp, M., Muller, M. & Jahn, K. Health services utilization of patients with vertigo in primary care: a retrospective cohort study. J. Neurol. 261, 1492–1498 (2014).

    Article  PubMed  Google Scholar 

  115. de Waele, C., Huy, P. T., Diard, J. P., Freyss, G. & Vidal, P. P. Saccular dysfunction in Meniere's disease. Am. J. Otol. 20, 223–232 (1999).

    Article  CAS  PubMed  Google Scholar 

  116. Young, Y. H., Huang, T. W. & Cheng, P. W. Assessing the stage of Meniere's disease using vestibular evoked myogenic potentials. Arch. Otolaryngol. Head Neck Surg. 129, 815–818 (2003).

    Article  PubMed  Google Scholar 

  117. Rauch, S. D. et al. Vestibular evoked myogenic potentials versus vestibular test battery in patients with Meniere's disease. Otol. Neurotol. 25, 981–986 (2004).

    Article  PubMed  Google Scholar 

  118. Young, Y. H. Potential application of ocular and cervical vestibular-evoked myogenic potentials in Meniere's disease: a review. Laryngoscope 123, 484–491 (2013).

    Article  PubMed  Google Scholar 

  119. Huang, C. H., Wang, S. J. & Young, Y. H. Localization and prevalence of hydrops formation in Meniere's disease using a test battery. Audiol. Neurootol. 16, 41–48 (2011).

    Article  PubMed  Google Scholar 

  120. Katayama, N. et al. Relationship between endolymphatic hydrops and vestibular-evoked myogenic potential. Acta Otolaryngol. 130, 917–923 (2010).

    Article  PubMed  Google Scholar 

  121. Yamasoba, T., Kikuchi, S., Sugasawa, M., Yagi, M. & Harada, T. Acute low-tone sensorineural hearing loss without vertigo. Arch. Otolaryngol. Head Neck Surg. 120, 532–535 (1994).

    Article  CAS  PubMed  Google Scholar 

  122. Levine, S., Margolis, R. H. & Daly, K. A. Use of electrocochleography in the diagnosis of Meniere's disease. Laryngoscope 108, 993–1000 (1998).

    Article  CAS  PubMed  Google Scholar 

  123. Young, Y. H., Wu, C. C. & Wu, C. H. Augmentation of vestibular evoked myogenic potentials: an indication for distended saccular hydrops. Laryngoscope 112, 509–512 (2002).

    Article  PubMed  Google Scholar 

  124. Magliulo, G., Cuiuli, G., Gagliardi, M., Ciniglio-Appiani, G. & D'Amico, R. Vestibular evoked myogenic potentials and glycerol testing. Laryngoscope 114, 338–343 (2004).

    Article  PubMed  Google Scholar 

  125. Curthoys, I. S. A critical review of the neurophysiological evidence underlying clinical vestibular testing using sound, vibration and galvanic stimuli. Clin. Neurophysiol. 121, 132–144 (2010).

    Article  PubMed  Google Scholar 

  126. Wen, M. H., Cheng, P. W. & Young, Y. H. Augmentation of ocular vestibular-evoked myogenic potentials via bone-conducted vibration stimuli in Meniere disease. Otolaryngol. Head Neck Surg. 146, 797–803 (2012).

    Article  PubMed  Google Scholar 

  127. Blodow, A. et al. Caloric stimulation and video-head impulse testing in Meniere's disease and vestibular migraine. Acta Otolaryngol. 134, 1239–1244 (2014).

    Article  PubMed  Google Scholar 

  128. McGarvie, L. A., Curthoys, I. S., MacDougall, H. G. & Halmagyi, G. M. What does the head impulse test versus caloric dissociation reveal about vestibular dysfunction in Meniere's disease? Ann. NY Acad. Sci. 1343, 58–62 (2015).

    Article  PubMed  Google Scholar 

  129. Pyykkö, I., Zou, J., Poe, D., Nakashima, T. & Naganawa, S. Magnetic resonance imaging of the inner ear in Meniere's disease. Otolaryngol. Clin. North Am. 43, 1059–1080 (2010).

    Article  PubMed  Google Scholar 

  130. Naganawa, S. & Nakashima, T. Visualization of endolymphatic hydrops with MR imaging in patients with Meniere's disease and related pathologies: current status of its methods and clinical significance. Jpn J. Radiol. 32, 191–204 (2014).

    Article  PubMed  Google Scholar 

  131. Niyazov, D. M., Andrews, J. C., Strelioff, D., Sinha, S. & Lufkin, R. Diagnosis of endolymphatic hydrops in vivo with magnetic resonance imaging. Otol. Neurotol. 22, 813–817 (2001).

    Article  CAS  PubMed  Google Scholar 

  132. Zou, J., Pyykko, I., Bretlau, P., Klason, T. & Bjelke, B. In vivo visualization of endolymphatic hydrops in guinea pigs: magnetic resonance imaging evaluation at 4.7 Tesla. Ann. Otol. Rhinol. Laryngol. 112, 1059–1065 (2003).

    Article  PubMed  Google Scholar 

  133. Naganawa, S. et al. Separate visualization of endolymphatic space, perilymphatic space and bone by a single pulse sequence; 3D-inversion recovery imaging utilizing real reconstruction after intratympanic Gd-DTPA administration at 3 Tesla. Eur. Radiol. 18, 920–924 (2008).

    Article  PubMed  Google Scholar 

  134. Naganawa, S. et al. Imaging of endolymphatic and perilymphatic fluid at 3T after intratympanic administration of gadolinium-diethylene-triamine pentaacetic acid. AJNR Am. J. Neuroradiol. 29, 724–726 (2008).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  135. Naganawa, S. et al. Semi-quantification of endolymphatic size on MR imaging after intravenous injection of single-dose gadodiamide: comparison between two types of processing strategies. Magn. Reson. Med. Sci. 12, 261–269 (2013). This paper reports the development of HYDROPS to obtain a considerably better MRI image for the evaluation of EH after intravenous injection of an ordinary dose of gadolinium.

    Article  CAS  PubMed  Google Scholar 

  136. Fukuoka, H. et al. Comparison of the diagnostic value of 3 T MRI after intratympanic injection of GBCA, electrocochleography, and the glycerol test in patients with Meniere's disease. Acta Otolaryngol. 132, 141–145 (2012).

    Article  PubMed  Google Scholar 

  137. Gürkov, R., Flatz, W., Louza, J., Strupp, M. & Krause, E. In vivo visualization of endolyphatic hydrops in patients with Meniere's disease: correlation with audiovestibular function. Eur. Arch. Otorhinolaryngol. 268, 1743–1748 (2011).

    Article  PubMed  Google Scholar 

  138. Kato, M. et al. Association between endolymphatic hydrops as revealed by magnetic resonance imaging and caloric response. Otol. Neurotol. 32, 1480–1485 (2011).

    Article  PubMed  Google Scholar 

  139. Yoshioka, M. et al. Individual differences in the permeability of the round window: evaluating the movement of intratympanic gadolinium into the inner ear. Otol. Neurotol. 30, 645–648 (2009).

    Article  PubMed  Google Scholar 

  140. Zou, J. & Pyykkö, I. Enhanced oval window and blocked round window passages for middle-inner ear transportation of gadolinium in guinea pigs with a perforated round window membrane. Eur. Arch. Otorhinolaryngol. 272, 303–309 (2015).

    Article  PubMed  Google Scholar 

  141. Attye, A. et al. Recurrent peripheral vestibulopathy: is MRI useful for the diagnosis of endolymphatic hydrops in clinical practice? Eur. Radiol. 25, 3043–3049 (2015).

    Article  PubMed  Google Scholar 

  142. Homann, G. et al. HR 3 Tesla MRI for the diagnosis of endolymphatic hydrops and differential diagnosis of inner ear tumors — demonstrated by two cases with similar symptoms. Rofo 186, 225–229 (2014).

    Article  CAS  PubMed  Google Scholar 

  143. Homann, G. et al. Semi-quantitative versus volumetric determination of endolymphatic space in Meniere's disease using endolymphatic hydrops 3T-HR-MRI after intravenous gadolinium injection. PLoS ONE 10, e0120357 (2015).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  144. Tanigawa, T. et al. Visualization of endolymphatic hydrops after administration of a standard dose of an intravenous gadolinium-based contrast agent. Acta Otolaryngol. 131, 596–601 (2011).

    Article  CAS  PubMed  Google Scholar 

  145. Nakashima, T. et al. Grading of endolymphatic hydrops using magnetic resonance imaging. Acta Otolaryngol. Suppl. 560, 5–8 (2009).

    Article  Google Scholar 

  146. Fang, Z. M. et al. A new magnetic resonance imaging scoring system for perilymphatic space appearance after intratympanic gadolinium injection, and its clinical application. J. Laryngol. Otol. 126, 454–459 (2012).

    Article  PubMed  Google Scholar 

  147. Liu, F. et al. Noninvasive evaluation of endolymphatic space in healthy volunteers using magnetic resonance imaging. Acta Otolaryngol. 131, 247–257 (2011).

    Article  PubMed  Google Scholar 

  148. Gürkov, R. et al. MR volumetric assessment of endolymphatic hydrops. Eur. Radiol. 25, 585–595 (2015).

    Article  PubMed  Google Scholar 

  149. Naganawa, S. et al. Volume quantification of endolymph after intravenous administration of a single dose of gadolinium contrast agent: comparison of 18- versus 8-minute imaging protocols. Magn. Reson. Med. Sci. 14, 257–262 (2015).

    Article  CAS  PubMed  Google Scholar 

  150. Naganawa, S., Suzuki, K., Yamazaki, M., Sakurai, Y. & Ikeda, M. Time course for measuring endolymphatic size in healthy volunteers following intravenous administration of gadoteridol. Magn. Reson. Med. Sci. 13, 73–80 (2014).

    Article  CAS  PubMed  Google Scholar 

  151. Gürkov, R., Flatz, W., Ertl-Wagner, B. & Krause, E. Endolymphatic hydrops in the horizontal semicircular canal: a morphologic correlate for canal paresis in Ménière's disease. Laryngoscope 123, 503–506 (2013).

    Article  PubMed  Google Scholar 

  152. Gürkov, R. et al. Effect of standard-dose betahistine on endolymphatic hydrops: an MRI pilot study. Eur. Arch. Otorhinolaryngol. 270, 1231–1235 (2013).

    Article  PubMed  Google Scholar 

  153. Fiorino, F., Pizzini, F. B., Barbieri, F. & Beltramello, A. Magnetic resonance imaging fails to show evidence of reduced endolymphatic hydrops in gentamicin treatment of Meniere's disease. Otol. Neurotol. 33, 629–633 (2012).

    Article  PubMed  Google Scholar 

  154. Sharon, J. D., Trevino, C., Schubert, M. C. & Carey, J. P. Treatment of Meniere's disease. Curr. Treat. Options Neurol. 17, 341 (2015).

    Article  PubMed  Google Scholar 

  155. Foster, C. A. & Breeze, R. E. The Meniere attack: an ischemia/reperfusion disorder of inner ear sensory tissues. Med. Hypotheses 81, 1108–1115 (2013).

    Article  CAS  PubMed  Google Scholar 

  156. Lauter, J. L., Lynch, O., Wood, S. B. & Schoeffler, L. Physiological and behavioral effects of an antivertigo antihistamine in adults. Percept. Mot. Skills 88, 707–732 (1999).

    Article  CAS  PubMed  Google Scholar 

  157. Weerts, A. P. et al. Space motion sickness countermeasures: a pharmacological double-blind, placebo-controlled study. Aviat. Space Environ. Med. 85, 638–644 (2014).

    Article  CAS  PubMed  Google Scholar 

  158. Soto, E. & Vega, R. Neuropharmacology of vestibular system disorders. Curr. Neuropharmacol. 8, 26–40 (2010).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  159. Teggi, R., Fabiano, B., Recanati, P., Limardo, P. & Bussi, M. Case reports on two patients with episodic vertigo, fluctuating hearing loss and migraine responding to prophylactic drugs for migraine. Meniere's disease or migraine-associated vertigo? Acta Otorhinolaryngol. Ital. 30, 217 (2010).

    CAS  PubMed  PubMed Central  Google Scholar 

  160. Lassen, L. F., Hirsch, B. E. & Kamerer, D. B. Use of nimodipine in the medical treatment of Meniere's disease: clinical experience. Am. J. Otol. 17, 577–580 (1996).

    CAS  PubMed  Google Scholar 

  161. Monzani, D. et al. Effect of a fixed combination of nimodipine and betahistine versus betahistine as monotherapy in the long-term treatment of Meniere's disease: a 10-year experience. Acta Otorhinolaryngol. Ital. 32, 393–403 (2012).

    CAS  PubMed  PubMed Central  Google Scholar 

  162. Nakayama, M. et al. A pilot study on the efficacy of continuous positive airway pressure (CPAP) on the manifestations of Meniere's disease in patients with concomitant obstructive sleep apnea syndrome (OSAS). J. Clin. Sleep Med. 11, 1101–1107 (2015).

    Article  PubMed  PubMed Central  Google Scholar 

  163. Thirlwall, A. S. & Kundu, S. Diuretics for Meniere's disease or syndrome. Cochrane Database Syst. Rev. 3, CD003599 (2006).

    Google Scholar 

  164. Claes, J. & Van de Heyning, P. H. A review of medical treatment for Meniere's disease. Acta Otolaryngol. Suppl. 544, 34–39 (2000).

    Article  CAS  PubMed  Google Scholar 

  165. van Deelen, G. W. & Huizing, E. H. Use of a diuretic (Dyazide) in the treatment of Meniere's disease. A double-blind cross-over placebo-controlled study. RL J. Otorhinolaryngol Relat. Spec. 48, 287–292 (1986).

    Article  CAS  Google Scholar 

  166. James, A. L. & Burton, M. J. Betahistine for Menière's disease or syndrome. Cochrane Database Syst. Rev. 1, CD001873 (2001).

    Google Scholar 

  167. Nauta, J. J. Meta-analysis of clinical studies with betahistine in Meniere's disease and vestibular vertigo. Eur. Arch. Otorhinolaryngol. 271, 887–897 (2014).

    Article  PubMed  Google Scholar 

  168. Moller, M. N., Kirkeby, S., Vikesa, J., Nielsen, F. C. & Caye-Thomasen, P. Expression of histamine receptors in the human endolymphatic sac: the molecular rationale for betahistine use in Menieres disease. Eur. Arch. Otorhinolaryngol.http://dx.doi.org/10.1007/s00405-015-3731-5 (2015).

  169. Strupp, M., Zwergal, A., Feil, K., Bremova, T. & Brandt, T. Pharmacotherapy of vestibular and cerebellar disorders and downbeat nystagmus: translational and back-translational research. Ann. NY Acad. Sci. 1343, 27–36 (2015).

    Article  CAS  PubMed  Google Scholar 

  170. Ihler, F. et al. Betahistine exerts a dose-dependent effect on cochlear stria vascularis blood flow in guinea pigs in vivo. PLoS ONE 7, e39086 (2012).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  171. Garduno-Anaya, M. A., Couthino De Toledo, H., Hinojosa-Gonzalez, R., Pane-Pianese, C. & Rios-Castaneda, L. C. Dexamethasone inner ear perfusion by intratympanic injection in unilateral Meniere's disease: a two-year prospective, placebo controlled, double-blind, randomized trial. Otolaryngol. Head Neck Surg. 133, 285–294 (2005).

    Article  PubMed  Google Scholar 

  172. Phillips, J. S. & Westerberg, B. Intratympanic steroids for Meniere's disease or syndrome. Cochrane Database Syst. Rev. 7, CD008514 (2011).

    Google Scholar 

  173. Silverstein, H., Smouha, E. & Jones, R. Natural history versus surgery for Meniere's disease. Otolaryngol. Head Neck Surg. 100, 6–16 (1989).

    Article  CAS  PubMed  Google Scholar 

  174. Adunka, O. et al. Labyrinth anesthesia — a forgotten but practical treatment option in Meniere's disease. ORL J. Otorhinolaryngol. Relat. Spec. 65, 84–90 (2003).

    Article  PubMed  Google Scholar 

  175. Thomsen, J., Bonding, P., Becker, B., Stage, J. & Tos, M. The non-specific effect of endolymphatic sac surgery in treatment of Meniere's disease: a prospective, randomized controlled study comparing “classic” endolymphatic sac surgery with the insertion of a ventilating tube in the tympanic membrane. Acta Otolaryngol. 118, 769–773 (1998).

    Article  CAS  PubMed  Google Scholar 

  176. Pullens, B., Verschuur, H. P. & van Benthem, P. P. Surgery for Meniere's disease. Cochrane Database Syst. Rev. 2, CD005395 (2013).

    Google Scholar 

  177. Welling, D. B. & Nagaraja, H. N. Endolymphatic mastoid shunt: a reevaluation of efficacy. Otolaryngol. Head Neck Surg. 122, 340–345 (2000).

    Article  CAS  PubMed  Google Scholar 

  178. Sood, A. J., Lambert, P. R., Nguyen, S. A. & Meyer, T. A. Endolymphatic sac surgery for Meniere's disease: a systematic review and meta-analysis. Otol. Neurotol. 35, 1033–1045 (2014).

    Article  PubMed  Google Scholar 

  179. Greenberg, S. L. & Nedzelski, J. M. Medical and noninvasive therapy for Meniere's disease. Otolaryngol. Clin. North Am. 43, 1081–1090 (2010).

    Article  PubMed  Google Scholar 

  180. Rizzi, M. D. & Hirose, K. Aminoglycoside ototoxicity. Curr. Opin. Otolaryngol. Head Neck Surg. 15, 352–357 (2007).

    Article  PubMed  Google Scholar 

  181. Chia, S. H., Gamst, A. C., Anderson, J. P. & Harris, J. P. Intratympanic gentamicin therapy for Meniere's disease: a meta-analysis. Otol. Neurotol. 25, 544–552 (2004).

    Article  PubMed  Google Scholar 

  182. Helling, K., Schonfeld, U. & Clarke, A. H. Treatment of Meniere's disease by low-dosage intratympanic gentamicin application: effect on otolith function. Laryngoscope 117, 2244–2250 (2007).

    Article  CAS  PubMed  Google Scholar 

  183. Calzada, A. P., Lopez, I. A., Ishiyama, G. & Ishiyama, A. Otolithic membrane damage in patients with endolymphatic hydrops and drop attacks. Otol. Neurotol. 33, 1593–1598 (2012).

    Article  PubMed  PubMed Central  Google Scholar 

  184. Ishiyama, G., Lopez, I., Baloh, R. W. & Ishiyama, A. Histopathology of the vestibular end organs after intratympanic gentamicin failure for Meniere's disease. Acta Otolaryngol. 127, 34–40 (2007).

    Article  PubMed  Google Scholar 

  185. Lacour, M. & Bernard-Demanze, L. Interaction between vestibular compensation mechanisms and vestibular rehabilitation therapy: 10 recommendations for optimal functional recovery. Front. Neurol. 5, 285 (2014).

    PubMed  Google Scholar 

  186. Soderman, A. C., Bagger-Sjoback, D., Bergenius, J. & Langius, A. Factors influencing quality of life in patients with Meniere's disease, identified by a multidimensional approach. Otol. Neurotol. 23, 941–948 (2002).

    Article  PubMed  Google Scholar 

  187. Tyrrell, J. et al. Mental health and subjective well-being of individuals with Meniere's: cross-sectional analysis in the UK Biobank. Otol. Neurotol. 36, 854–861 (2015).

    Article  PubMed  Google Scholar 

  188. Yardley, L., Dibb, B. & Osborne, G. Factors associated with quality of life in Meniere's disease. Clin. Otolaryngol. Allied Sci. 28, 436–441 (2003).

    Article  CAS  PubMed  Google Scholar 

  189. Anderson, J. P. & Harris, J. P. Impact of Meniere's disease on quality of life. Otol. Neurotol. 22, 888–894 (2001).

    Article  CAS  PubMed  Google Scholar 

  190. Levo, H. et al. EuroQol 5D quality of life in Meniere's disorder can be explained with symptoms and disabilities. Int. J. Rehabil. Res. 35, 197–202 (2012).

    Article  PubMed  Google Scholar 

  191. Stephens, D. et al. Positive experiences and quality of life in Meniere's disorder. Int. J. Audiol. 49, 839–843 (2010).

    Article  PubMed  Google Scholar 

  192. Cohen, H., Ewell, L. R. & Jenkins, H. A. Disability in Meniere's disease. Arch. Otolaryngol. Head Neck Surg. 121, 29–33 (1995).

    Article  CAS  PubMed  Google Scholar 

  193. Mira, E. Improving the quality of life in patients with vestibular disorders: the role of medical treatments and physical rehabilitation. Int. J. Clin. Pract. 62, 109–114 (2008).

    Article  CAS  PubMed  Google Scholar 

  194. Levo, H., Kentala, E., Rasku, J. & Pyykko, I. Aural fullness in Meniere's disease. Audiol. Neurootol. 19, 395–399 (2014).

    Article  PubMed  Google Scholar 

  195. Antonovsky, A. The structure and properties of the sense of coherence scale. Soc. Sci. Med. 36, 725–733 (1993).

    Article  CAS  PubMed  Google Scholar 

  196. Eriksson, M. & Lindstrom, B. Antonovsky's sense of coherence scale and the relation with health: a systematic review. J. Epidemiol. Commun. Health 60, 376–381 (2006).

    Article  Google Scholar 

  197. Arroll, M., Dancey, C. P., Attree, E. A., Smith, S. & James, T. People with symptoms of Meniere's disease: the relationship between illness intrusiveness, illness uncertainty, dizziness handicap, and depression. Otol. Neurotol. 33, 816–823 (2012).

    Article  PubMed  Google Scholar 

  198. Plontke, S. K. & Gurkov, R. [Meniere's disease]. Laryngorhinootologie 94, 530–554 (in German) (2015).

    Article  CAS  PubMed  Google Scholar 

  199. Shimono, M. et al. Endolymphatic hydrops revealed by magnetic resonance imaging in patients with acute low-tone sensorineural hearing loss. Otol. Neurotol. 34, 1241–1246 (2013).

    Article  PubMed  Google Scholar 

  200. Liu, Y. P. et al. Endolymphatic hydrops detected by 3-dimensional fluid-attenuated inversion recovery MRI following intratympanic injection of gadolinium in the asymtomatic contralateral ears of patients with unilateral Meniere's disease. Med. Sci. Monitor 21, 701–707 (2015).

    Article  Google Scholar 

  201. Barnett, E. M., Zhang, X., Maxwell, D., Chang, Q. & Piwnica-Worms, D. Single-cell imaging of retinal ganglion cell apoptosis with a cell-penetrating, activatable peptide probe in an in vivo glaucoma model. Proc. Natl Acad. Sci. USA 106, 9391–9396 (2009).

    Article  PubMed  PubMed Central  Google Scholar 

  202. Qu, J., Wang, D. & Grosskreutz, C. L. Mechanisms of retinal ganglion cell injury and defense in glaucoma. Exp. Eye Res. 91, 48–53 (2010).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  203. Nakashima, T. et al. A perspective from magnetic resonance imaging findings of the inner ear: relationships among cerebrospinal, ocular and inner ear fluids. Auris Nasus Larynx 39, 345–355 (2012).

    Article  PubMed  Google Scholar 

  204. Strupp, M. & Brandt, T. Peripheral vestibular disorders. Curr. Opin. Neurol. 26, 81–89 (2013).

    Article  PubMed  Google Scholar 

  205. McGarvie, L. A., Curthoys, I. S., MacDougall, H. G. & Halmagyi, G. M. What does the dissociation between the results of video head impulse versus caloric testing reveal about the vestibular dysfunction in Meniere's disease? Acta Otolaryngol. 135, 859–865 (2015).

    Article  PubMed  Google Scholar 

  206. Tagaya, M. et al. Endolymphatic hydrops and blood–labyrinth barrier in Meniere's disease. Acta Otolaryngol. 131, 474–479 (2011).

    Article  CAS  PubMed  Google Scholar 

  207. Nakashima, T. et al. Disorders of cochlear blood flow. Brain Res. Brain Res. Rev. 43, 17–28 (2003).

    Article  PubMed  Google Scholar 

  208. Shi, X. Physiopathology of the cochlear microcirculation. Hear. Res. 282, 10–24 (2011).

    Article  PubMed  PubMed Central  Google Scholar 

  209. Diamond, C., O'Connell, D. A., Hornig, J. D. & Liu, R. Systematic review of intratympanic gentamicin in Meniere's disease. J. Otolaryngol. 32, 351–361 (2003).

    Article  PubMed  Google Scholar 

  210. Seggas, I., Koltsidopoulos, P., Bibas, A., Tzonou, A. & Sismanis, A. Intratympanic steroid therapy for sudden hearing loss: a review of the literature. Otol. Neurotol. 32, 29–35 (2011).

    Article  PubMed  Google Scholar 

  211. Nakagawa, T. et al. Audiometric outcomes of topical IGF1 treatment for sudden deafness refractory to systemic steroids. Otol. Neurotol. 33, 941–946 (2012).

    PubMed  Google Scholar 

  212. Ahsan, S. F., Standring, R. & Wang, Y. Systematic review and meta-analysis of Meniett therapy for Meniere's disease. Laryngoscope 125, 203–208 (2015). This paper suggests that Meniett therapy (local pressure therapy to the ear) might be beneficial to reduce the frequency of vertigo in MD, but further studies need to be carried.

    Article  PubMed  Google Scholar 

  213. van Sonsbeek, S., Pullens, B. & van Benthem, P. P. Positive pressure therapy for Meniere's disease or syndrome. Cochrane Database Syst. Rev. 3, CD008419 (2015).

    Google Scholar 

  214. Pyykkö, I., Zou, J., Zhang, W. & Zhang, Y. Nanoparticle-based delivery for the treatment of inner ear disorders. Curr. Opin. Otolaryngol. Head Neck Surg. 19, 388–396 (2011).

    Article  PubMed  Google Scholar 

  215. Nakashima, T. et al. Idiopathic sudden sensorineural hearing loss in Japan. Acta Otolaryngol. 134, 1158–1163 (2014).

    Article  PubMed  PubMed Central  Google Scholar 

  216. Schuknecht, H. F., Suzuka, Y. & Zimmermann, C. Delayed endolymphatic hydrops and its relationship to Meniere's disease. Ann. Otol. Rhinol. Laryngol. 99, 843–853 (1990).

    Article  CAS  PubMed  Google Scholar 

  217. Huygen, P. L. & Admiraal, R. J. Audiovestibular sequelae of congenital cytomegalovirus infection in 3 children presumably representing 3 symptomatically different types of delayed endolymphatic hydrops. Int. J. Pediatr. Otorhinolaryngol. 35, 143–154 (1996).

    Article  CAS  PubMed  Google Scholar 

  218. Shojaku, H. et al. Clinical characteristics of delayed endolymphatic hydrops in Japan: a nationwide survey by the Peripheral Vestibular Disorder Research Committee of Japan. Acta Otolaryngol. 130, 1135–1140 (2010).

    Article  PubMed  Google Scholar 

  219. Leliever, W. C. & Barber, H. O. Recurrent vestibulopathy. Laryngoscope 91, 1–6 (1981).

    Article  CAS  PubMed  Google Scholar 

  220. Jerin, C., Berman, A., Krause, E., Ertl-Wagner, B. & Gürkov, R. Ocular vestibular evoked myogenic potential frequency tuning in certain Meniere's disease. Hear. Res. 310, 54–59 (2014). This paper proposes a new definition of certain MD that requires confirmation of EH on MRI.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Contributions

Introduction (T.N.); Epidemiology (M.L.C.); Mechanisms/pathophysiology (C.A.M., N.F.M. and I.P.); Diagnosis, screening and prevention (Y.-H.Y. and S.N.); Management (C.A.F.); Quality of life (M.A.A.); Outlook (T.N. and I.P.); Overview of Primer (T.N. and I.P.).

Corresponding author

Correspondence to Tsutomu Nakashima.

Ethics declarations

Competing interests

The authors declare no competing interests.

PowerPoint slides

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Nakashima, T., Pyykkö, I., Arroll, M. et al. Meniere's disease. Nat Rev Dis Primers 2, 16028 (2016). https://doi.org/10.1038/nrdp.2016.28

Download citation

  • Published:

  • DOI: https://doi.org/10.1038/nrdp.2016.28

This article is cited by

Search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing