Clinical manifestations of hydropic ear disease (Menière’s)
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Hydropic ear disease, initially described by and named after Prosper Menière, is one of the most frequent vertigo disorders and one of the most frequent inner ear disorders. It is the syndrome of endolymphatic hydrops which until 2007 could be diagnostically confirmed only by post-mortem histology. In the past, various attempts to formulate clinical diagnostic criteria have been undertaken but were hampered by the inability to ascertain the diagnosis in living patients. With the milestone achievement of endolymphatic hydrops imaging, today the pathology can be ascertained. In this study, we have performed a detailed analysis of the clinical features of hydropic ear disease for the first time by examining a large cohort of patients with morphologically confirmed endolymphatic hydrops using a detailed physician-administered neurotologic face-to-face interview.
During a hydropic vertigo attack, the patients report nausea, vomiting, sweating, urge to defecate, urge to urinate, phosphenes, headache, photophobia, phonophobia and even transient loss of consciousness. A third of the patients does not experience auditory symptoms during the vertigo attacks. Vertigo attacks last less than 20 min in more than one-fourth of the patients. Audiometric hearing loss has its greatest diagnostic value at the frequencies of 1 kHz and below. Cochleovestibular symptom onset simultaneity is associated with a high frequency of drop-attacks. Migraine and autoimmune disorders are not associated with hydropic ear disease.
This study marks the beginning of the clinical characterization of hydropic ear disease. The findings have important implications for the future formulation of clinical diagnostic criteria.
KeywordsHydropic ear disease Endolymphatic hydrops Diagnostic criteria Menière’s disease Vertigo Dizziness Hearing loss Tinnitus
This study was funded by the German Ministry of Research and Education (Grant number 01EO1401).
Compliance with ethical standards
Conflict of interest
All authors declare that he/she has no conflict of interest.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
- 2.Barber HO et al (1972) Meniere’s disease: criteria for diagnosis and evaluation of therapy for reporting. Trans Am Acad Ophthalmol Otolaryngol 76(6):1462–1464Google Scholar
- 5.Kitahara M (1990) Concepts and diagnostic criteria of Menière’s disease, in Menière’s Disease. Springer, Tokyo, 3–12Google Scholar
- 6.AAO-HNS (1995) Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere’s disease. Otolaryngol Head Neck Surg 113(3):181–185 (American Academy of Otolaryngology-Head and Neck Foundation, Inc.) Google Scholar
- 7.Lopez-Escamez JA et al (2015) Diagnostic criteria for Meniere’s disease. J Vestib Res 25(1):1–7Google Scholar
- 9.Hallpike CS, Cairns H (1938) Observations on the pathology of Menière’s syndrome. Proc R Soc Med 31:1317–1336Google Scholar
- 10.Yamakawa K (1938) Über die pathologische Veränderung bei einem Menière-Kranken. J Otolaryngol Soc Jpn 4:2310–2312 (Proceedings of 42nd Annual Meeting Oto-Rhino-Laryngol Soc Japan) Google Scholar
- 11.Gibson WP, Prasher DK (1983) Electrocochleography and its role in the diagnosis and understanding of Meniere’s disease. Otolaryngol Clin North Am 16(1):59–68Google Scholar
- 18.Jerin C et al (2015) Endolymphatic hydrops in a patient with a small vestibular schwannoma suggests a peripheral origin of vertigo. Austin J Radiol 2015(6):1033Google Scholar
- 23.Gurkov R et al (2012) In vivo visualized endolymphatic hydrops and inner ear functions in patients with electrocochleographically confirmed Meniere’s disease. Otol Neurotol 33(6):1040-1045Google Scholar
- 31.Bisdorff A et al (2009) Classification of vestibular symptoms: towards an international classification of vestibular disorders. J Vestib Res 19(1–2):1–13Google Scholar
- 34.Kirsch V et al (2018) High-resolution MRI of the inner ear enables syndrome differentiation and specific treatment of cerebellar downbeat nystagmus and secondary endolymphatic hydrops in a postoperative ELST patient. J NeurolGoogle Scholar
- 45.Gopen Q, Viirre E, Anderson J (2009) Epidemiologic study to explore links between Meniere syndrome and migraine headache. Ear Nose Throat J 88(11):1200–1204Google Scholar
- 53.Schubel J et al (2017) Latent Hypothyroidism in Adults. Dtsch Arztebl Int 114(25):430–438Google Scholar