Pelin KOÇDOR, Michael PAPARELLA, Meredith ADAMS, Sebahattin CÜREOĞLU

Pathogenesis and pathophysiology of Meniere’s disease: An update

Meniere’s disease is known with certain clinical symptoms such as fluctuating hearing loss, episodic vertigo, and tinnitus and characterized by endolymphatic hydrops found on post-mortem examination. The pathophysiology of Meniere’s disease is still questionable, and it has not been fully understood despite almost a century of research. Many determinants are effective in the occurrence of endolymphatic hydrops and in the pathogenesis of relevant cochleovestibular dysfunction. This review discusses research studies conducted in recent years concerning the pathogenesis and pathophysiology of Meniere’s disease. Histopathological research studies conducted in recent years on patients with Meniere’s disease have often focused on certain subjects as follows: aquaporins, oxidative stress, genetics, cochlear lateral wall changes, longitudinal flow blockage, intraskeletal channels of the otic capsule, hydropic and cellular changes in Reissner’s membrane, cochlear hair cells and spiral ganglion cells, round window thickness, basement membrane pathology, and otolithic membrane damages. Recent studies conducted on pathogenesis mechanisms of Meniere’s disease would provide an insight for the improvement of diagnosis and treatment of this weakening disease.

Keywords:

Endolymphatic hydrops, Meniere’s disease, pathogenesis,

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Hallpike CS, Cairns H. Observations on the pathology of Meniere’s syndrome. J Laryngol Otol 1938;53:625-55.
Yamakawa K. Uber die pathologisch Veranderung bei einem Meniere-Kranken. J Otorhinolaryngol Soc Jpn 1938;4:2310-2.
Paparella MM, Djalilian HR. Etiology, pathophysiology of symptoms, and pathogenesis of Meniere’s disease. Otolaryngol Clin North Am 2002;35:529-45.
Schuknecht HF, Rüther A. Blockage of longitudinal flow in endolymphatic hydrops. Eur Arch Otorhinolaryngol 1991;248:209-17.
Yamane H, Takayama M, Sunami K, Sakamoto H, Imoto T, Anniko M. Blockage of reuniting duct in Meniere's disease. Acta Otolaryngol 2010;130:233-9.
Shimizu S, Cureoglu S, Yoda S, Suzuki M, Paparella MM. Blockage of longitudinal flow in Meniere’s disease: A human temporal bone study. Acta Otolaryngol 2011;131:263-8.
Merchant SN, Adams JC, Nadol JB Jr. Pathophysiology of Meniere’s syndrome: are symptoms caused by endolymphatic hydrops? Otol Neurotol 2005;26:74-81.
Michaels L, Soucek S, Linthicum F. The intravestibular source of the vestibular aqueduct. II: its structure and function clarified by a developmental study of the intra-skeletal channels of the otic capsule. Acta Otolaryngol 2010;130:420-8.
Michaels L, Soucek S. The intravestibular source of the vestibular aqueduct. III: Osseous pathology of Ménière’s disease, clarified by a developmental study of the intraskeletal channels of the otic capsule. Acta Otolaryngol 2010;130:793-8.
Yoda S, Cureoglu S, Shimizu S, Morita N, Fukushima H, Sato T, et al. Round window membrane in Ménière’s disease: a human temporal bone study. Otol Neurotol 2011;32:147-51.
De Beer L, Stokroos R, Kingma H. Intratympanic gentamicin therapy for intractable Ménière’s disease. Acta Otolaryngol 2007;127:605-12.
Herraiz C, Plaza G, Aparicio JM, Gallego I, Marcos S, Ruiz C. Transtympanic steroids for Ménière’s disease. Otol Neurotol 2010;31:162-7.
Calzada AP, Lopez IA, Ishiyama G, Ishiyama A. Otolithic membrane damage in patients with endolymphatic hydrops and drop attacks. Otol Neurotol 2012;33:1593-8.
Cureoglu S, Schachern PA, Paul S, Paparella MM, Singh RK. Cellular changes of Reissner's membrane in Meniere’s disease: human temporal bone study. Otolaryngol Head Neck Surg 2004;130:113-9.
Kariya S, Cureoglu S, Fukushima H, Kusunoki T, Schachern PA, Nishizaki K, et al. Histopathologic changes of contralateral human temporal bone in unilateral Ménière’s disease. Otol Neurotol 2007;28:1063-8.
Ishiyama G, Tokita J, Lopez I, Tang Y, Ishiyama A. Unbiased stereological estimation of the spiral ligament and stria vascularis volumes in aging and Ménière’s disease using archival human temporal bones. J Assoc Res Otolaryngol 2007;8:8-17.
Kariya S, Cureoglu S, Fukushima H, Nomiya S, Nomiya R, Schachern PA, et al. Vascular findings in the stria vascularis of patients with unilateral or bilateral Ménière’s disease: a histopathologic temporal bone study. Otol Neurotol 2009;30:1006-12.
McCall AA, Ishiyama GP, Lopez IA, Bhuta S, Vetter S, Ishiyama A. Histopathological and ultrastructural analysis of vestibular endorgans in Meniere’s disease reveals basement membrane pathology. BMC Ear Nose Throat Disord 2009;9:4.
Vrabec JT. Genetic investigations of Meniere’s disease. Otolaryngol Clin North Am 2010;43:1121-32.
Mhatre AN, Jero J, Chiappini I, Bolasco G, Barbara M, Lalwani AK. Aquaporin-2 expression in the mammalian cochlea and investigation of its role in Meniere’s disease. Hear Res 2002;170:59-69.
Doi K, Sato T, Kuramasu T, Hibino H, Kitahara T, Horii A, et al. Ménière’s disease is associated with single nucleotide polymorphisms in the human potassium channel genes, KCNE1 and KCNE3. ORL J Otorhinolaryngol Relat Spec 2005;67:289-93.
Teggi R, Lanzani C, Zagato L, Delli Carpini S, Manunta P, Bianchi G, et al. Gly460Trp alpha-adducin mutation as a possible mechanism leading to endolymphatic hydrops in Ménière’s syndrome. Otol Neurotol 2008;29:824-8.
Fransen E, Verstreken M, Verhagen WI, Wuyts FL, Huygen PL, D’Haese P, et al. High prevalence of symptoms of Menière’s disease in three families with a mutation in the COCH gene. Hum Mol Genet 1999;8:1425-9.
Vrabec JT, Liu L, Li B, Leal SM. Sequence variants in host cell factor C1 are associated with Ménière’s disease. Otol Neurotol 2008;29:561-6.
Kawaguchi S, Hagiwara A, Suzuki M. Polymorphic analysis of the heat-shock protein 70 gene (HSPA1A) in Ménière’s disease. Acta Otolaryngol 2008;128:1173-7.
Lopez-Escamez JA, Saenz-Lopez P, Acosta L, Moreno A, Gazquez I, Perez-Garrigues H, et al. Association of a functional polymorphism of PTPN22 encoding a lymphoid protein phosphatase in bilateral Meniere’s disease. Laryngoscope 2010;120:103-7.
Furuta T, Teranishi M, Uchida Y, Nishio N, Kato K, Otake H, et al. Association of interleukin-1 gene polymorphisms with sudden sensorineural hearing loss and Ménière’s disease. Int J Immunogenet 2011;38:249-54.
Gazquez I, Moreno A, Aran I, Soto-Varela A, Santos S, Perez-Garrigues H, et al. MICA-STR A.4 is associated with slower hearing loss progression in patients with Ménière’s disease. Otol Neurotol 2012;33:223-9.
Gazquez I, Soto-Varela A, Aran I, Santos S, Batuecas A, Trinidad G, et al. High prevalence of systemic autoimmune diseases in patients with Menière’s disease. PLoS One 2011;6:e26759.
Ishiyama G, López IA, Ishiyama A. Aquaporins and Meniere’s disease. Curr Opin Otolaryngol Head Neck Surg 2006;14:332-6.
Beitz E, Kumagami H, Krippeit-Drews P, Ruppersberg JP, Schultz JE. Expression pattern of aquaporin water channels in the inner ear of the rat. The molecular basis for a water regulation system in the endolymphatic sac. Hear Res 1999;132:76-84.
Takeda T, Sawada S, Takeda S, Kitano H, Suzuki M, Kakigi A, et al. The effects of V2 antagonist (OPC-31260) on endolymphatic hydrops. Hear Res 2003;182:9-18.
Fukushima K, Takeda T, Kakigi A, Takeda S, Sawada S, Nishioka R, et al. Effects of lithium on endolymph homeostasis and experimentally induced endolymphatic hydrops. ORL J Otorhinolaryngol Relat Spec 2005;67:282-8.
Sawada S, Takeda T, Kitano H, Takeuchi S, Kakigi A, Azuma H. Aquaporin-2 regulation by vasopressin in the rat inner ear. Neuroreport 2002;13:1127-9.
Kawedia JD, Nieman ML, Boivin GP, Melvin JE, Kikuchi K, Hand AR, et al. Interaction between transcellular and paracellular water transport pathways through Aquaporin 5 and the tight junction complex. Proc Natl Acad Sci U S A 2007;104:3621-6.
Mhatre AN, Steinbach S, Hribar K, Hoque AT, Lalwani AK. Identification of aquaporin 5 (AQP5) within the cochlea: cDNA cloning and in situ localization. Biochem Biophys Res Commun 1999;264:157-62.
Nishio N, Teranishi M, Uchida Y, Sugiura S, Ando F, Shimokata H, et al. Polymorphisms in genes encoding aquaporins 4 and 5 and estrogen receptor a in patients with Ménière’s disease and sudden sensorineural hearing loss. Life Sci 2013;92:541-6.
Fukushima M, Kitahara T, Fuse Y, Uno Y, Doi K, Kubo T. Changes in aquaporin expression in the inner ear of the rat after i.p. injection of steroids. Acta Otolaryngol Suppl 2004;553:13-8.
Kitahara T, Doi K, Maekawa C, Kizawa K, Horii A, Kubo T, et al. Meniere’s attacks occur in the inner ear with excessive vasopressin type-2 receptors. J Neuroendocrinol 2008;20:1295-300.
Calabrese V, Cornelius C, Maiolino L, Luca M, Chiaramonte R, Toscano MA, et al. Oxidative stress, redox homeostasis and cellular stress response in Ménière’s disease: role of vitagenes. Neurochem Res 2010;35:2208-17.
Gazquez I, Lopez-Escamez JA, Moreno A, Campbell CA, Meyer NC, Carey JP, et al. Functional variants in NOS1 and NOS2A are not associated with progressive hearing loss in Ménière’s disease in a European Caucasian population. DNA Cell Biol 2011;30:699-708.
Labbé D, Teranishi MA, Hess A, Bloch W, Michel O. Activation of caspase-3 is associated with oxidative stress in the hydropic guinea pig cochlea. Hear Res 2005;202:21-7.