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.
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Menière, P. Maladie de l'oreille interne offrant les symtômes de la congestion cérébrale apoplectiforme. Gaz. Med. Paris 16, 88–89 (in French) (1861).
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).
Minor, L. B., Schessel, D. A. & Carey, J. P. Meniere's disease. Curr. Opin. Neurol. 17, 9–16 (2004).
Sajjadi, H. & Paparella, M. M. Meniere's disease. Lancet 372, 406–414 (2008).
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.
Hallpike, C. S. & Cairns, H. Observations on the pathology of Ménière's syndrome. J. Laryngol. Otol. 53, 625–655 (1938).
Yamakawa, K. Über die patologische Veränderungen bei einem Menière-kranken. J. Otorhinolaryngol. Soc. Jpn 44, 2310–2312 (1938).
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.
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.
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).
Rauch, S. D. Clinical hints and precipitating factors in patients suffering from Meniere's disease. Otolaryngol. Clin. North Am. 43, 1011–1017 (2010).
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).
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.
Miyagawa, M. et al. Endolymphatic hydrops and therapeutic effects are visualized in ‘atypical’ Meniere's disease. Acta Otolaryngol. 129, 1326–1329 (2009).
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).
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).
Kato, M. et al. Endolymphatic hydrops revealed by magnetic resonance imaging in patients with atypical Meniere's disease. Acta Otolaryngol. 133, 123–129 (2013).
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).
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).
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).
Lopez-Escamez, J. A. et al. Diagnostic criteria for Meniere's disease. J. Vestib. Res. 25, 1–7 (2015).
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).
Harris, J. P. & Alexander, T. H. Current-day prevalence of Meniere's syndrome. Audiol. Neurootol. 15, 318–322 (2010).
Stahle, J., Stahle, C. & Arenberg, I. K. Incidence of Meniere's disease. Arch. Otolaryngol. 104, 99–102 (1978).
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).
Kotimaki, J., Sorri, M., Aantaa, E. & Nuutinen, J. Prevalence of Meniere disease in Finland. Laryngoscope 109, 748–753 (1999).
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).
Meyerhoff, W. L., Paparella, M. M. & Shea, D. Meniere's disease in children. Laryngoscope 88, 1504–1511 (1978).
Filipo, R. & Barbara, M. Juvenile Meniere's disease. J. Laryngol. Otol. 99, 193–196 (1985).
Hausler, R., Toupet, M., Guidetti, G., Basseres, F. & Montandon, P. Meniere's disease in children. Am. J. Otolaryngol. 8, 187–193 (1987).
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).
Brantberg, K., Duan, M. & Falahat, B. Meniere's disease in children aged 4–7 years. Acta Otolaryngol. 132, 505–509 (2012).
Thomas, K. & Harrison, M. S. Long-term follow up of 610 cases of Meniere's disease. Proc. R. Soc. Med. 64, 853–857 (1971).
Friberg, U., Stahle, J. & Svedberg, A. The natural course of Meniere's disease. Acta Otolaryngol. Suppl. 406, 72–77 (1984).
Havia, M. & Kentala, E. Progression of symptoms of dizziness in Meniere's disease. Arch. Otolaryngol. Head Neck Surg. 130, 431–435 (2004).
Huang, C. H. & Young, Y. H. Bilateral Meniere's disease assessed by an inner ear test battery. Acta Otolaryngol. 135, 233–238 (2015).
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).
Huppert, D., Strupp, M. & Brandt, T. Long-term course of Meniere's disease revisited. Acta Otolaryngol. 130, 644–651 (2010).
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).
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).
Morrison, A. W., Bailey, M. E. & Morrison, G. A. Familial Meniere's disease: clinical and genetic aspects. J. Laryngol. Otol. 123, 29–37 (2009).
Klockars, T. & Kentala, E. Inheritance of Meniere's disease in the Finnish population. Arch. Otolaryngol. Head Neck Surg. 133, 73–77 (2007).
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).
Arweiler-Harbeck, D., Horsthemke, B., Jahnke, K. & Hennies, H. C. Genetic aspects of familial Meniere's disease. Otol. Neurotol. 32, 695–700 (2011).
Derebery, M. J. & Berliner, K. I. Prevalence of allergy in Meniere's disease. Otolaryngol. Head Neck Surg. 123, 69–75 (2000).
Koyama, S., Mitsuishi, Y., Bibee, K., Watanabe, I. & Terasaki, P. I. HLA associations with Meniere's disease. Acta Otolaryngol. 113, 575–578 (1993).
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).
Lempert, T. & Neuhauser, H. Epidemiology of vertigo, migraine and vestibular migraine. J. Neurol. 256, 333–338 (2009).
Radtke, A. et al. Migraine and Meniere's disease: is there a link? Neurology 59, 1700–1704 (2002).
Nakada, T. et al. Endolymphatic space size in patients with vestibular migraine and Meniere's disease. J. Neurol. 261, 2079–2084 (2014).
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).
Paparella, M. M. & Djalilian, H. R. Etiology, pathophysiology of symptoms, and pathogenesis of Meniere's disease. Otolaryngol. Clin. North Am. 35, 529–545 (2002).
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).
Wackym, P. A. Histopathologic findings in Meniere's disease. Otolaryngol. Head Neck Surg. 112, 90–100 (1995).
Gibson, W. P. & Arenberg, I. K. Pathophysiologic theories in the etiology of Meniere's disease. Otolaryngol. Clin. North Am. 30, 961–967 (1997).
Schuknecht, H. F. Pathology of the Ear (Harvard Univ. Press, 1993).
Nadol, J. B. & Thornton, A. R. Ultrastructural findings in a case of Menière's disease. Ann. Otol. Rhinol. Laryngol. 96, 449–454 (1987).
Conlon, B. J. & Gibson, W. P. Electrocochleography in the diagnosis of Meniere's disease. Acta Otolaryngol. 120, 480–483 (2000).
Yamamoto, M. et al. Relationship between the degree of endolymphatic hydrops and electrocochleography. Audiol. Neurootol. 15, 254–260 (2010).
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.
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).
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).
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).
Salt, A. N. & Plontke, S. K. Endolymphatic hydrops: pathophysiology and experimental models. Otolaryngol. Clin. North Am. 43, 971–983 (2010).
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).
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).
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).
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).
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).
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).
Megerian, C. A. et al. A mouse model with postnatal endolymphatic hydrops and hearing loss. Hear. Res. 237, 90–105 (2008).
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).
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).
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).
Momin, S. R. et al. Hearing preservation in guinea pigs with long-standing endolymphatic hydrops. Otol. Neurotol. 32, 1583–1589 (2011).
Kitahara, T. et al. Meniere's attacks occur in the inner ear with excessive vasopressin type-2 receptors. J. Neuroendocrinol. 20, 1295–1300 (2008).
Morrison, A. W. & Johnson, K. J. Genetics (molecular biology) and Meniere's disease. Otolaryngol. Clin. North Am. 35, 497–516 (2002).
Vrabec, J. T. Genetic investigations of Meniere's disease. Otolaryngol. Clin. North Am. 43, 1121–1132 (2010).
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).
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).
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).
Uno, A. et al. Changes in endolymphatic hydrops after sac surgery examined by Gd-enhanced MRI. Acta Otolaryngol. 133, 924–929 (2013).
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).
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).
Békésy, G. Direct observation of the vibrations of the cochlear partition under a microscope. Acta Otolaryngol. 42, 197–201 (1952).
Robles, L. & Ruggero, M. A. Mechanics of the mammalian cochlea. Physiol. Rev. 81, 1305–1352 (2001).
Yamashita, T. & Schuknecht, H. F. Apical endolymphatic hydrops. Arch. Otolaryngol. 108, 463–466 (1982).
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).
Stahle, J. & Bergman, B. The caloric reaction in meniere's disease: an electronystagmographical study in 300 patients. Laryngoscope 77, 1629–1643 (1967).
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).
Lackner, J. R. Motion sickness: more than nausea and vomiting. Exp. Brain Res. 232, 2493–2510 (2014).
Schuknecht, H. F. Pathophysiology of endolymphatic hydrops. Arch. Otorhinolaryngol. 212, 253–262 (1976).
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).
Schuknecht, H. (ed.) Histopathology of Meniere's Disease (Kugler, 1990).
Tonndorf, J. Vestibular signs and symptoms in Meniere's disorder: mechanical considerations. Acta Otolaryngol. 95, 421–430 (1983).
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.
Yamane, H. et al. Blockage of reuniting duct in Meniere's disease. Acta Otolaryngol. 130, 233–239 (2010).
Manzari, L., Burgess, A. M. & Curthoys, I. S. Vestibular function in Lermoyez syndrome at attack. Eur. Arch. Otorhinolaryngol. 269, 685–691 (2012).
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).
Kentala, E., Havia, M. & Pyykko, I. Short-lasting drop attacks in Meniere's disease. Otolaryngol. Head Neck Surg. 124, 526–530 (2001).
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).
Baloh, R. W., Jacobson, K. & Winder, T. Drop attacks with Meniere's syndrome. Ann. Neurol. 28, 384–387 (1990).
Sakata, T. & Kato, T. Feeling of ear fullness in acute sensorineural hearing loss. Acta Otolaryngol. 126, 828–833 (2006).
Yoshida, T. et al. Endolymphatic hydrops in patients with tinnitus as the major symptom. Eur. Arch. Otorhinolaryngol. 270, 3043–3048 (2013).
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).
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.
Yazawa, Y. & Kitahara, M. Bilateral endolymphatic hydrops in Meniere's disease: review of temporal bone autopsies. Ann. Otol. Rhinol. Laryngol. 99, 524–528 (1990).
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).
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).
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).
Levo, H., Stephens, D., Poe, D., Kentala, E. & Pyykkö, I. Fatigue in Meniere's disease. Int. J. Rehabil. Res. 11, 191–197 (2013).
Havia, M., Kentala, E. & Pyykko, I. Hearing loss and tinnitus in Meniere's disease. Auris Nasus Larynx 29, 115–119 (2002).
Havia, M., Kentala, E. & Pyykko, I. Postural instability in Meniere's disease. J. Vestib. Res. 14, 37–46 (2004).
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).
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).
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).
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).
Young, Y. H. Potential application of ocular and cervical vestibular-evoked myogenic potentials in Meniere's disease: a review. Laryngoscope 123, 484–491 (2013).
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).
Katayama, N. et al. Relationship between endolymphatic hydrops and vestibular-evoked myogenic potential. Acta Otolaryngol. 130, 917–923 (2010).
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).
Levine, S., Margolis, R. H. & Daly, K. A. Use of electrocochleography in the diagnosis of Meniere's disease. Laryngoscope 108, 993–1000 (1998).
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).
Magliulo, G., Cuiuli, G., Gagliardi, M., Ciniglio-Appiani, G. & D'Amico, R. Vestibular evoked myogenic potentials and glycerol testing. Laryngoscope 114, 338–343 (2004).
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).
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).
Blodow, A. et al. Caloric stimulation and video-head impulse testing in Meniere's disease and vestibular migraine. Acta Otolaryngol. 134, 1239–1244 (2014).
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).
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).
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).
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).
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).
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).
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).
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.
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).
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).
Kato, M. et al. Association between endolymphatic hydrops as revealed by magnetic resonance imaging and caloric response. Otol. Neurotol. 32, 1480–1485 (2011).
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).
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).
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).
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).
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).
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).
Nakashima, T. et al. Grading of endolymphatic hydrops using magnetic resonance imaging. Acta Otolaryngol. Suppl. 560, 5–8 (2009).
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).
Liu, F. et al. Noninvasive evaluation of endolymphatic space in healthy volunteers using magnetic resonance imaging. Acta Otolaryngol. 131, 247–257 (2011).
Gürkov, R. et al. MR volumetric assessment of endolymphatic hydrops. Eur. Radiol. 25, 585–595 (2015).
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).
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).
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).
Gürkov, R. et al. Effect of standard-dose betahistine on endolymphatic hydrops: an MRI pilot study. Eur. Arch. Otorhinolaryngol. 270, 1231–1235 (2013).
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).
Sharon, J. D., Trevino, C., Schubert, M. C. & Carey, J. P. Treatment of Meniere's disease. Curr. Treat. Options Neurol. 17, 341 (2015).
Foster, C. A. & Breeze, R. E. The Meniere attack: an ischemia/reperfusion disorder of inner ear sensory tissues. Med. Hypotheses 81, 1108–1115 (2013).
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).
Weerts, A. P. et al. Space motion sickness countermeasures: a pharmacological double-blind, placebo-controlled study. Aviat. Space Environ. Med. 85, 638–644 (2014).
Soto, E. & Vega, R. Neuropharmacology of vestibular system disorders. Curr. Neuropharmacol. 8, 26–40 (2010).
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).
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).
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).
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).
Thirlwall, A. S. & Kundu, S. Diuretics for Meniere's disease or syndrome. Cochrane Database Syst. Rev. 3, CD003599 (2006).
Claes, J. & Van de Heyning, P. H. A review of medical treatment for Meniere's disease. Acta Otolaryngol. Suppl. 544, 34–39 (2000).
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).
James, A. L. & Burton, M. J. Betahistine for Menière's disease or syndrome. Cochrane Database Syst. Rev. 1, CD001873 (2001).
Nauta, J. J. Meta-analysis of clinical studies with betahistine in Meniere's disease and vestibular vertigo. Eur. Arch. Otorhinolaryngol. 271, 887–897 (2014).
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).
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).
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).
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).
Phillips, J. S. & Westerberg, B. Intratympanic steroids for Meniere's disease or syndrome. Cochrane Database Syst. Rev. 7, CD008514 (2011).
Silverstein, H., Smouha, E. & Jones, R. Natural history versus surgery for Meniere's disease. Otolaryngol. Head Neck Surg. 100, 6–16 (1989).
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).
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).
Pullens, B., Verschuur, H. P. & van Benthem, P. P. Surgery for Meniere's disease. Cochrane Database Syst. Rev. 2, CD005395 (2013).
Welling, D. B. & Nagaraja, H. N. Endolymphatic mastoid shunt: a reevaluation of efficacy. Otolaryngol. Head Neck Surg. 122, 340–345 (2000).
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).
Greenberg, S. L. & Nedzelski, J. M. Medical and noninvasive therapy for Meniere's disease. Otolaryngol. Clin. North Am. 43, 1081–1090 (2010).
Rizzi, M. D. & Hirose, K. Aminoglycoside ototoxicity. Curr. Opin. Otolaryngol. Head Neck Surg. 15, 352–357 (2007).
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).
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).
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).
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).
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).
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).
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).
Yardley, L., Dibb, B. & Osborne, G. Factors associated with quality of life in Meniere's disease. Clin. Otolaryngol. Allied Sci. 28, 436–441 (2003).
Anderson, J. P. & Harris, J. P. Impact of Meniere's disease on quality of life. Otol. Neurotol. 22, 888–894 (2001).
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).
Stephens, D. et al. Positive experiences and quality of life in Meniere's disorder. Int. J. Audiol. 49, 839–843 (2010).
Cohen, H., Ewell, L. R. & Jenkins, H. A. Disability in Meniere's disease. Arch. Otolaryngol. Head Neck Surg. 121, 29–33 (1995).
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).
Levo, H., Kentala, E., Rasku, J. & Pyykko, I. Aural fullness in Meniere's disease. Audiol. Neurootol. 19, 395–399 (2014).
Antonovsky, A. The structure and properties of the sense of coherence scale. Soc. Sci. Med. 36, 725–733 (1993).
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).
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).
Plontke, S. K. & Gurkov, R. [Meniere's disease]. Laryngorhinootologie 94, 530–554 (in German) (2015).
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).
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).
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).
Qu, J., Wang, D. & Grosskreutz, C. L. Mechanisms of retinal ganglion cell injury and defense in glaucoma. Exp. Eye Res. 91, 48–53 (2010).
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).
Strupp, M. & Brandt, T. Peripheral vestibular disorders. Curr. Opin. Neurol. 26, 81–89 (2013).
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).
Tagaya, M. et al. Endolymphatic hydrops and blood–labyrinth barrier in Meniere's disease. Acta Otolaryngol. 131, 474–479 (2011).
Nakashima, T. et al. Disorders of cochlear blood flow. Brain Res. Brain Res. Rev. 43, 17–28 (2003).
Shi, X. Physiopathology of the cochlear microcirculation. Hear. Res. 282, 10–24 (2011).
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).
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).
Nakagawa, T. et al. Audiometric outcomes of topical IGF1 treatment for sudden deafness refractory to systemic steroids. Otol. Neurotol. 33, 941–946 (2012).
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.
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).
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).
Nakashima, T. et al. Idiopathic sudden sensorineural hearing loss in Japan. Acta Otolaryngol. 134, 1158–1163 (2014).
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).
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).
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).
Leliever, W. C. & Barber, H. O. Recurrent vestibulopathy. Laryngoscope 91, 1–6 (1981).
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.
The authors declare no competing interests.
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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
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