Abstract
Introduction
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.
Results
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.
Conclusion
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.
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Change history
18 February 2019
In the original publication, Fig. 1 was incorrectly published with incomplete legends.
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Funding
This study was funded by the German Ministry of Research and Education (Grant number 01EO1401).
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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.
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405_2018_5157_MOESM1_ESM.docx
App. 1: Frequencies of comorbid diagnoses (number of affected patients in parentheses) in patients with Hydropic Ear Disease. Diagnoses in red font mark those that affected at least 5 patients (i.e. 2% of the study population). App. 2: Frequencies of medications used (in % prevalence) by patients with Hydropic Ear Disease. The left column displays the ATC classes, the middle column displays the specific drugs (DOCX 26 KB)
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Gürkov, R., Jerin, C., Flatz, W. et al. Clinical manifestations of hydropic ear disease (Menière’s). Eur Arch Otorhinolaryngol 276, 27–40 (2019). https://doi.org/10.1007/s00405-018-5157-3
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DOI: https://doi.org/10.1007/s00405-018-5157-3