Many diseases have been renamed on the basis of their anatomical sites and pathophysiological findings, as understanding of their underlying mechanisms has improved over time. One of them is Meniere’s disease (MD), a well-known vertiginous disease named after the French doctor, Prosper Meniere, who had suggested in 1861 that its symptoms came from the inner ear and not the brain, as was the prevailing belief at that time. This symptom-based diagnosis for MD was against its pathological findings and raised one important issue: whether MD was a specific inner ear disease or was it just a general description for an inner ear disease with deafness and vertigo? The prevailing inclusion criteria for MD were not specific and diagnostic practices tended to restrict MD as a specific inner ear disease by excluding known inner ear and/or brain etiologies. This exclusion criteria were against Dr. Meniere’s original concept, who had emphasized the fact of vertigo could be caused by inner ear problem. Thus, it was believed that MD was a general description for an inner ear disease with deafness and vertigo. In the era of modern precision medicine, it is recommended that doctors inform patients with deafness and vertigo about the diseased site and the related pathological changes instead of a diagnosis of MD.
As understanding of the underlying mechanisms of diseases has improved, many diseases have been renamed over the years on the basis of their anatomical sites and/or pathophysiological findings while building on the contributions of previous researchers and/or doctors. Meniere’s disease (MD) is a well-known traditional disease that was named after Dr. Prosper Meniere in 1872 . In an article published in 1861, Dr. Meniere had reported a type of deafness with vertigo and suggested that, in addition to brain disorders, vertigo could also originate from inner ear problems. Hence deafness and vertigo that originated from the inner ear still carries Dr. Meniere’s name in recognition of his contribution. However, the underlying mechanisms of MD were unclear at that time. In 1938, research papers published by Yamakawa et al. and Hallpike and Cairns found that endolymphatic hydrops might account for MD . As understanding of the disease improved, it was increasingly understood that MD was a kind of deafness and vertigo which could be caused by endolymphatic hydrops in the inner ear. Thereafter, it has been defined by symptomatic criteria since 1972. Further, the symptomatic criteria for the disease have been revised several times in 1985, 1995, and 2015 .
The symptom-based diagnosis goes somewhat against pathological findings. It raised an important issue: whether MD was a specific inner ear disease or was it just a general description for an inner ear disease with deafness and vertigo? The prevailing symptomatic inclusion criteria were not very specific, as some other inner ear and/or brain diseases could also be diagnosed as MD, such as transient ischemic attack (TIA) , vestibular migraine (VM), cochleovestibular migraine (CVM) , and other similar conditions. Hence, the symptomatic criteria tended to exclude all known inner ear and/or brain etiologies, restricting MD as a unique form of inner ear disease. Unless the etiology and/or pathological changes underlying deafness and vertigo became clear, the diagnostic philosophy continued to define MD as simply a collection of inner ear disease with deafness and vertigo of unknown etiology. Most importantly, the exclusion criteria for the disease were against Dr. Meniere’s concept, which had emphasized that vertigo could be caused by inner ear problem. Thus, we believed that MD was just a general description for an inner ear disease with deafness and vertigo. However, many subsequent studies had found that MD was often comorbid in patients with arthritis, psoriasis, migraine, gastroesophageal reflux disease, irritable bowel syndrome, and chronic kidney disease . Thus, MD was also a mixed-type inner ear disease of deafness and vertigo, as was commonly believed, and the exclusion of known etiology during diagnosis was not justified.
The pathophysiology of endolymphatic hydrops and its association with MD remains elusive . The symptoms of most patients with MD become noticeable only after a considerable accumulation of endolymph. However, the diagnosis of MD based on symptomatic inclusion and exclusion criteria does not reflect the existence of endolymphatic hydrops either by electrocochleography (ECOG)  or by magnetic resonance imaging (MRI) . It was observed that the ratio of summation potential versus action potential (SP/AP ratio) was not elevated but matched for MD in 43.9% (29/66) of the MD patients based on symptomatic criteria . The inversion of the saccule to utricle area ratio (SURI) on the axial 3D-FLAIR MRI was found only in 50% (15/30) patients . On the other hand, asymptomatic endolymphatic hydrops might be found on the unaffected side of patients with unilateral MD [4,5]. Hence, patients with “asymptomatic” endolymphatic hydrops are not necessarily the same as those with “symptomatic” MD. Thus, it may be difficult to attribute the etiology of the patients with MD solely to endolymphatic hydrops. Meanwhile, the condition of endolymphatic hydrops is not restricted to patients with typical MD but can also be present in other conditions such as atypical MD, large vestibular aqueduct syndrome, superior canal dehiscence syndrome, low cerebrospinal fluid pressure syndrome and perilymphatic fistula .
Based on above discussion, it was believed that MD was just a general description for an inner ear disease with deafness and vertigo. In the era of modern precision medicine, we would encourage doctors to inform their patients with deafness and vertigo about the diseased sites and the related pathological changes instead of a general diagnosis of MD only.
Received date: November 02, 2021
Accepted date: December 06, 2021
Published date: December 14, 2021
The study is in accordance with the ethical standards of the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
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© 2021 The Author. This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 International License (CC-BY).
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Menière disease is by definition associated with symptoms. However, the symptoms of most patients with Menière disease present after a considerable accumulation of endolymph has occurred. This fact suggests that endolymphatic hydrops may be symptomatic or asymptomatic. So, it is pertinent to note that patients with "asymptomatic" endolymphatic hydrops are not necessarily the same as those with "symptomatic" Menière disease. The term Menière disease, rather than endolymphatic hydrops, refers to a broad variety of inner ear disorders related to imbalance.
Response Thank you very much for your expert comment. Now, we have added this concept with its related references in our revised manuscripts.
It has been shown in recent MRI studies that patients with Menière disease -- both typically and atypically -- have endolymphatic hydrops. Nevertheless, some studies have also found that patients with unilateral Menière disease do occasionally have asymptomatic endolymphatic hydrops on the unaffected side. Since no definitive association has been demonstrated between endolymphatic hydrops and Menière disease, it is unlikely that endolymphatic hydrops can be attributed solely to the cause of Menière disease. Therefore, the term Menière disease should not be replaced with the term endolymphatic hydrops since it is not an exact synonym.
ResponseThank you very much for your expert comment. Now, we have added the findings as you mentioned. And, we have also dropped our suggestion about replacing the term Menière disease with endolymphatic hydrops.
The pathophysiology of endolymphatic hydrops and its association with Menière disease remain elusive. In one respect, endolymphatic hydrops may not be associated with Menière disease, since the development of endolymphatic hydrops occurs before the onset of Menière disease. Additionally, it should be noted that endolymphatic hydrops does not occur exclusively in Menière disease, and that its detection is not essential for the classification of Menière disease, in spite of the fact that the disease is frequently accompanied by endolymphatic hydrops. On the basis of the above facts, I question whether it makes logical sense to replace Menière disease with endolymphatic hydrops.
ResponseThank you very much for your expert comment. We very agreed with your concept and have added this information in the revised manuscript. And, we have dropped our suggestion about replacing the term Menière disease with endolymphatic hydrops.
It is common to observe endolymphatic hydrops in patients with Menière's disease, a characteristic feature of the disease. It is unclear, however, what causes endolymphatic hydrops in patients with Menière disease. There is also considerable uncertainty regarding the relationship between hemolymphatic hydrops and Menière's disease. It should be noted that endolymphatic hydrops are not exclusively associated with Menière disease but can also be seen in patients with atypical Menière disease. Additionally, endolymphatic hydrops may occur concurrently with diseases such as large vestibular aqueduct syndrome, superior canal dehiscence syndrome, low cerebrospinal fluid pressure syndrome, and perilymphatic fistula. As far as I'm concerned, the rationale is not clear for replacing Menière disease with endolymphatic hydrops. As an example, patients who suffer from superior canal dehiscence syndrome may experience vertigo that is not necessarily related to Menière disease.
ResponseThank you very much for your expert comment. Now, we have added the concept as you mentioned. And, we have also dropped our suggestion about replacing the term Menière disease with endolymphatic hydrops.