Materiały źródłowe

• #1 Meniere Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK536955/

  Meniere disease is an inner ear disorder characterized by tinnitus, vertigo, and hearing loss. This is thought to occur due to the accumulation of endolymphatic fluid in the cochlea and the vestibular organ. […] Studies of the temporal bone revealed endolymphatic accumulation (hydrops) in the cochlea and the vestibular organ in patients with Meniere disease. Current research links endolymphatic hydrops to a hearing loss of 40dB. Vertigo may or may not be associated. […] The precise pathophysiology of Meniere disease is unknown. The most consistent histologic abnormality is endolymphatic hydrops, though not everyone with documented hydrops develops MD. Endolymphatic hydrops describes the expansion of the volume of the endolymphatic spaces and dilatation of Reissner’s membrane. The exact pathophysiology of endolymphatic hydrops is likewise unknown, though the most common theories postulate an imbalance between the secretion and resorption of endolymph in the cochlea.

• #2 Meniere Disease (Idiopathic Endolymphatic Hydrops): Background, Anatomy, Pathophysiology

  https://emedicine.medscape.com/article/1159069-overview

  The exact pathophysiology of Mnire disease is controversial. The underlying mechanism is believed to be distortion of the membranous labyrinth resulting from overaccumulation of endolymph. […] Some authors have questioned whether endolymphatic hydrops is actually a marker of disease rather than a cause. […] Attacks of hydrops probably are caused by an increase in endolymphatic pressure, which, in turn, causes a break in the membrane that separates the perilymph (potassium-poor extracellular fluid) from the endolymph (potassium-rich intracellular fluid). […] The physical distention caused by increased endolymphatic pressure also leads to a mechanical disturbance of the auditory and otolithic organs. […] This physical distention causes mechanical disturbance of the organ of Corti as well. […] Various extrinsic mechanisms are thought to contribute to the development of endolymphatic hydrops, including infection, trauma, and allergens.

• #2 Meniere’s disease: Pathogenesis, treatments, and emerging approaches for an idiopathic bioenvironmental disorder – PubMed

  https://pubmed.ncbi.nlm.nih.gov/37648189/

  Meniere’s disease (MD) is a severe inner ear condition known by debilitating symptoms, including spontaneous vertigo, fluctuating and progressive hearing loss, tinnitus, and aural fullness or pressure within the affected ear. […] Nevertheless, researchers have identified a key histopathological feature called Endolymphatic Hydrops (ELH), which refers to the excessive buildup of endolymph fluid in the membranous labyrinth of the inner ear. The exact root of ELH is not fully understood. Still, it is believed to involve several biological and bioenvironmental etiological factors such as genetics, autoimmunity, infection, trauma, allergy, and new theories, such as saccular otoconia blocking the endolymphatic duct and sac. […] To make significant advancements in addressing MD, it is crucial to gain a fundamental understanding of the disease process, laying the groundwork for more effective therapeutic approaches. This paper provides a comprehensive review of the pathophysiology of MD with a focus on old and recent theories.

• #3 Meniere disease: Evaluation, diagnosis, and management – UpToDate

  https://www.uptodate.com/contents/meniere-disease-evaluation-diagnosis-and-management/print

  Meniere disease (MD), named for French physician Prosper Ménière, is a condition characterized by episodic vertigo, tinnitus, and hearing loss. […] Meniere disease – The classic triad of symptoms (episodic vertigo, tinnitus, and hearing loss), likely caused by endolymphatic hydrops of the labyrinthine system of the inner ear.

• #4 Meniere’s Disease

  https://dizziness-and-balance.com/disorders/menieres/menieres.html

  The origin of Meniere’s disease is presently controversial. While in the past, it was felt that plumbing problems (hydrops) in the ear were responsible for the disease, the most current opinion is that hydrops is a marker for the Meniere’s disease, rather than necessarily being responsible for the symptoms. […] The most prevalent opinion is that an acute attack of Meniere’s disease results from fluctuating pressure of the fluid within the inner ear. A system of membranes, called the membranous labyrinth, contains a fluid called endolymph. The membranes can become dilated like a balloon when pressure increases. This is called „hydrops”. One way for this to happen is when the drainage system, called the endolymphatic duct or sac is blocked. […] On the other hand, hydrops is not found in all persons with Meniere’s disease, and hydrops is also commonly found (6%) on autopsy studies of persons who had no Meniere’s type symptoms.

• #4 Meniere’s Disease

  https://dizziness-and-balance.com/disorders/menieres/menieres.html

  Attention has also been focused on the immunologic function of the endolymphatic sac — immune disease may contribute to a substantial percentage of Meniere’s disease. […] The bottom line is that in most patients with Meniere’s disease, the underlying cause of Meniere’s disease is unknown. It is most often attributed to viral infections of the inner ear or a hereditary predisposition.

• #5 Pathophysiology of Meniere’s Disease | IntechOpen

  https://www.intechopen.com/chapters/52996

  Meniere’s disease, with its characteristic symptom triad of vertigo, balance and hearing disorders has yet to have its pathophysiology outlined conclusively. […] While the central dogma revolves around endolymphatic hydrops, this theory is not without flaws, such as its inability to explain all the physiological changes seen in patients, or the often absence of symptoms. […] The exact mechanism of this hydrops is unknown and can range from overproduction, decreased absorption, to mechanical obstruction. […] The chapter intends to outline mechanisms for the hydrops; however, this theory is plagued with controversy as shown in a study of cadaveric temporal bone specimens wherein 100% of the diagnosed MD patients had evidence of hydrops, contrasted by findings of hydrops in 51/79 patients without an MD diagnosis, highlighting gaps in this theory.

• #5 Pathophysiology of Meniere’s Disease | IntechOpen

  https://www.intechopen.com/chapters/52996

  In simple terms, the central hypothesis of Meniere’s disease pathophysiology is endolymphatic hydrops, due to a varied etiology (autoimmune, infectious, endocrine, allergic, vascular, autonomic, dietary, genetic, idiopathic, etc.) and is responsible for the symptoms of MD. […] This correlation, however, does not imply causality, for if hydrops was causative then not only would every patient with Meniere’s disease have hydrops but also the reverse; every case of hydrops would have symptoms of Meniere’s disease, a statement we know does not stand true. […] The theory regards that excess endolymph is due to overproduction or reduced resorption, either idiopathic or due to various etiologies, most likely being obstruction at the level of the duct or sac, which results in EH. […] The acuteness of attacks can be explained by the increased pressures within the scala media which result in a rupture of the membranous labyrinth.

• #5 Pathophysiology of Meniere’s Disease | IntechOpen

  https://www.intechopen.com/chapters/52996

  This potassium is excitotoxic when exposed to CN VIII and hair cells as it causes depolarization of the nerve cells and subsequent inactivation. […] While other pathological findings in hydrops include membrane ruptures, periductal sclerosis, damage to hair cells and spiral ganglion cells, studies also highlight other differences observed such as abnormal glycoprotein metabolism in the endolymphatic sac. […] A combination of the mechanical and chemical factors is likely to be in play in the pathophysiology of MD. […] The central theory has not only dominated the pathophysiological dogma of MD but also influenced the design of various tests such as glycerol test or potential ratios (AP/SP; explained later) used to diagnose the hydrops and by extension MD. […] Specific causes of hydrops include infectious (viruses and syphilis), allergic, genetic, trauma, autoimmune, otoconia or otoliths and low cerebrospinal fluid (CSF) pressures.

• #5 Pathophysiology of Meniere’s Disease | IntechOpen

  https://www.intechopen.com/chapters/52996

  The problem in MD is thought to be malabsorption of endolymph, mainly in the duct or sac. […] The pathophysiology of Meniere’s disease is tied to the physiology of the hydrops, which can be induced in experimental models by obliteration or blockage of the duct and/or sac in animals at a success rate approaching 100% in guinea pigs but with more variability in other species. […] Despite logistical and technical difficulties in obtaining tissues and fluid (as composition is usually disturbed during surgical procedures), some human and animal studies show a change in the sodium-potassium levels of the cochlear and vestibular endolymphatic fluids in MD and experimental hydrops, respectively. […] The cerebrospinal fluid pressure dictates the inner ear hydrostatic pressure through the cochlear aqueduct. […] The pathophysiology of MD remains elusive despite intense research. It is likely that hydrops may not be the cause of MD symptoms, rather an epiphenomenon.

• #6 Meniere’s disease – Notes, Pdf, Lecture, Definition, Diet

  https://www.entlecture.com/menieres-disease/

  Menieres disease is an idiopathic disorder affecting the membranous labyrinth of the inner ear. The condition is characterized by an abnormal accumulation of fluid (endolymph) within the labyrinth, leading to increased hydraulic pressure in the endolymphatic system. […] Menieres disease is idiopathic in nature, meaning its exact cause is unknown. […] The aetiology of Menieres disease remains idiopathic. However, Menieres syndrome can arise secondary to various disorders, including: […] The underlying pathology in Menieres disease involves the excessive accumulation of endolymph within the membranous labyrinth, leading to distention and rupture of the membranous labyrinth. This results in a mixing of endolymph and perilymph, which disrupts the ionic composition and affects the function of the inner ear. The mechanisms contributing to this excessive fluid accumulation include: Increased Production of Endolymph: Endolymph is produced by the stria vascularis, and its overproduction can lead to hydrops. […] Reduced Absorption of Endolymph: Normally, endolymph is absorbed in the endolymphatic sac. Any disruption in this process can contribute to fluid build-up. […] Combination of Both: In some cases, both increased production and reduced absorption may occur, exacerbating the condition.

• #7 Meniere’s Disease: Diagnosis and Management | Ento Key

  https://entokey.com/menieres-disease-diagnosis-and-management/

  Although a comprehensive discussion regarding the pathogenesis and pathophysiology of Menieres disease is beyond the scope of this chapter, we will discuss the basic theories. The etiology of Menieres disease is unknown, but the common histopathologic correlate is hydrops (first confirmed by Hallpike in 1938) of the endolymph-containing spaces of the inner ear. Animal studies where the endolymphatic sac was destroyed or blocked produced a similar endolymphatic hydropic state that is seen in temporal bone studies of patients with Menieres disease. This begs the question of whether this hydropic state is the result of overproduction of endolymph or inadequate resorption. Hebbar and colleagues suggest that endolymphatic hydrops results from inadequate resorption of endolymph from their anatomical studies, which showed that patients with Menieres disease had reductions in tubular specializations of the lining of the endolymphatic duct and sac. Shambaugh and Gussen have postulated that hydrops resulted from inadequate resorption of endolymph as a result of vascular insufficiency of the endolymphatic sac. Others have theorized that saccular damage results from an autoimmune or infectious phenomenon resulting in perisaccular inflammation, which ultimately results in perisaccular fibrosis with consequent reduced endolymph resorption. This theory is based on the presence of immune complexes and viral particles within the serum and the endolymph of patients with Menieres disease. Yazawa and Kitahara have corroborated these hypotheses with temporal bone studies showing perisaccular fibrosis and ischemic changes. Wackym and Sando, however, challenge the presence of perisaccular fibrosis in their review of 18 temporal bones from patients with premortem Menieres disease as compared with 19 normal temporal bones.

• #7 Meniere’s Disease: Diagnosis and Management | Ento Key

  https://entokey.com/menieres-disease-diagnosis-and-management/

  Regardless of the cause of the hydropic state, it is postulated that this distention of the endolymphatic space ultimately results in membrane rupture within the inner ear. What results is endolymph mixing with perilymph. This has been corroborated by temporal bone studies. Vertigo then ensues, and damage to the cochlear apparatus progressively impairs hearing. Because endolymphatic hydrops has been found in those without symptoms of Menieres disease, it is likely that the membrane rupture is what differentiates those with asymptomatic hydrops from those with Menieres syndrome. At this time, there is no absolute diagnostic test to confirm hydrops; however, there is a promise of newer imaging methods yet to come. Furthermore, although the theories forwarded by previous work are attractive, it must be clear that the true pathophysiology of Menieres disease is far from clear.

• #8 Meniere’s Disease: Nonsurgical Treatment | IntechOpen

  https://www.intechopen.com/chapters/69749

  Most common histopathologic finding is endolymphatic hydrops but the exact pathology is unknown. […] Till this day, an effective treatment protocol is not established, because the pathogenesis of Menieres disease is not clear. […] It is hypothesized that the ruptures of membranous labyrinth and cicatrization in healing process could cause the drain blockage that leads to endolymphatic hydrops. […] Schuknect explained that, the ruptures of membranous labyrinth cause the leakage of potassium enriched endolymph to perilymph depolarize the nerve cells. […] Since the Menieres disease takes its course through lifetime, the effects on the inner ear is irreversible at some point, so the hearing loss is permanent and vertigo attacks subdue. […] However, this theory is not accepted by all; some authors suggested that ruptures are occurred rarely and not adequate to explain all the symptoms.

• #9 Morphological and functional changes in a new animal model of Ménière’s disease | Laboratory Investigation

  https://www.nature.com/articles/labinvest201391

  In this investigation, we successfully developed a new clinically relevant animal model for MD, in which desmopressin (a VP type 2 receptor agonist) was administered after electrocauterization of the ES. […] We evaluated both morphological and behavioral alterations of the vestibular system in this animal model to elucidate the role of VP in the pathogenesis of MD. […] We assume that the pathogenesis of vertiginous attacks is as follows: activation of the VPAQP2 system by the V2 effect may yield overproduction of endolymph, with an increase in the influx of water from the perilymph into the endolymph, which causes sudden changes in endolymphatic pressure. […] We speculate that under chronic endolymphatic dysfunction, it may reduce absorption of endolymph and chemical mechanisms can concomitantly cause Menieres symptoms. […] These previous studies suggest that water homeostasis in the inner ear is partly regulated via the VPAQP2 system.

• #9 Morphological and functional changes in a new animal model of Ménière’s disease | Laboratory Investigation

  https://www.nature.com/articles/labinvest201391

  This observation indicates that malabsorption of endolymph in the ES is one of the possible mechanisms underlying the development of EH. […] Water homeostasis of the inner ear is essential for maintaining the functions of hearing and balance. EH is considered to be the result of disruption of inner ear water homeostasis, which involves excessive production of endolymph and/or reduced absorption of endolymph. […] Since the discovery of aquaporin (AQP) water channels, it has been proposed that precise regulation of water reabsorption largely depends on the regulation of AQP2 channels and that water permeability can change rapidly in response to vasopressin (VP). […] Recently, it has come to light that this mechanism has a crucial role not only in the kidney but also in the inner ear. […] Such accumulated evidence has led to the assumption that production of endolymph is controlled by VPAQP2 system.

• #9 Morphological and functional changes in a new animal model of Ménière’s disease | Laboratory Investigation

  https://www.nature.com/articles/labinvest201391

  The purpose of this study was to clarify the underlying mechanism of vertiginous attacks in Mnires disease (MD) while obtaining insight into water homeostasis in the inner ear using a new animal model. […] Our animal model induced severe endolymphatic hydrops in the cochlea and the saccule, and showed episodes of balance disorder along with spontaneous nystagmus. […] These findings suggest that administration of desmopressin can exacerbate endolymphatic hydrops because of acute V2 (vasopressin type 2 receptor)-mediated effects, and, when combined with endolymphatic sac dysfunction, can cause temporary vestibular abnormalities that are similar to the vertiginous attacks in patients with MD. […] Endolymphatic hydrops (EH) has been considered as the histopathological origin of MD, as characteristic morphological changes were reported to be produced by surgical obstruction of the endolymphatic sac (ES) in guinea-pig.

• #10 Ménière’s disease – Wikipedia

  https://en.wikipedia.org/wiki/M%C3%A9ni%C3%A8re%27s_disease

  Mnire’s disease is characterized by potentially severe and incapacitating episodes of vertigo, tinnitus, hearing loss, and a feeling of fullness in the ear. […] The cause of Mnire’s disease is unclear, but likely involves both genetic and environmental factors. […] A number of theories exist for why it occurs, including constrictions in blood vessels, viral infections, and autoimmune reactions. […] Symptoms are believed to occur as the result of increased fluid buildup in the labyrinth of the inner ear. […] The initial triggers of Mnire’s disease are not fully understood, with a variety of potential inflammatory causes that lead to endolymphatic hydrops, a distension of the endolymphatic spaces in the inner ear. Endolymphatic hydrops (EH) is strongly associated with developing Mnire’s disease, but not everyone with EH develops Mnire’s disease: „The relationship between endolymphatic hydrops and Meniere’s disease is not a simple, ideal correlation.”

• #11 Ménière disease: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/meniere-disease/

  Ménière disease is thought to be related to abnormalities of the inner ear, which contains structures that are needed for normal hearing and balance. Episodes of vertigo, tinnitus, and hearing loss likely result from fluctuating amounts of fluid in the inner ear. These changes disrupt signals sent from the inner ear to the brain that are related to sound and the body’s position and movement. […] The cause of Ménière disease is unknown, although it probably results from a combination of environmental and genetic factors. […] Researchers are looking for additional environmental and genetic factors that are associated with this complex disorder.

• #12 The putative role of brain lymphatic system in ménière disease pathogenesis – MedCrave online

  https://medcraveonline.com/MOJI/the-putative-role-of-brain-lymphatic-system-in-meacuteniegravere-disease-pathogenesis.html

  Mnire’s disease (MD) is usually considered as a multifactorial disease, thus there are not yet evidences for a specific treatment. […] MD is characterized by impairment of this balance between the compartments causing the so called Endolymphatic Hydrops (EH), usually considered the pathogenesis of MD. […] It has been suggested that certain cases of MD may have an altered immunological background, which may be attributable to an autoimmune mechanism that depends on humoral and/or cellular responses leading to an altered blood-labyrinth barrier. […] The role of a GS disturbance has been taken in account also for Mnire Disease pathogenesis because the cochlear aqueduct and the internal auditory canal communicate with the subarachnoid space from where CSF is driven into the Virchow-Robin spaces.

• #12 The putative role of brain lymphatic system in ménière disease pathogenesis – MedCrave online

  https://medcraveonline.com/MOJI/the-putative-role-of-brain-lymphatic-system-in-meacuteniegravere-disease-pathogenesis.html

  In this eye disorder, even if path physiology remains uncertain, the lamina cribrosa seems to be the primary site of injury. […] The endolymphatic duct and endolymphatic sac play both an absorptive and secretory role, as well as phagocytic and immunodefensive functions: the endolymphatic sac is capable of antigen recognition and processing for initiation of an immune response. […] The case reported in this paper seems strictly connected with the experiments conducted in mice that showed how impairment of these lymph-nodes compromises CNS macromolecule clearance. […] The interconnection of brain lymphatics with both the nasal mucosa and the deep cervical lymph-nodes allows T-cells and viruses to easily circulate into the brain and therefore in the inner ear too. […] This connection between Brain Lymphatic System and deep cervical limph-nodes seems to support the action of allergic reactions, viruses and immunological complex directly arising form the upper airways on the inner ear.

• #13 Journal of Otolaryngology | Open Access Journals

  https://juniperpublishers.com/gjo/GJO.MS.ID.556197.php

  Menieres disease (MD) is a chronic inner ear disorder characterized by episodic vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural fullness, significantly impacting patients quality of life. […] Among the various hypotheses proposed to explain MDs pathogenesis, the autoimmune hypothesis suggests that an aberrant immune response targets inner ear antigens, leading to the characteristic symptoms of MD. […] The autoimmune hypothesis suggests that Menieres disease may result from an aberrant immune response wherein the bodys immune system mistakenly targets the inner ear. […] This hypothesis has garnered significant attention due to growing evidence of autoimmune activity in MD patients, such as elevated levels of specific autoantibodies and abnormal immune cell function.

• #13 Journal of Otolaryngology | Open Access Journals

  https://juniperpublishers.com/gjo/GJO.MS.ID.556197.php

  The characteristic symptoms of Menieres disease, including vertigo, hearing loss, tinnitus, and aural fullness, can be directly linked to these autoimmune mechanisms. […] Understanding the immunological biomarkers associated with Menieres disease and autoimmune disorders factors the interpretation of their complex interactions and may lead to the development of innovative treatments. […] The autoimmune pathophysiology of MD was first stipulated in 1958 by Lehnhardt about abrupt bilateral hearing loss, which he attributed to the production of anti-cochlear antibodies in the inner ear. […] Identifying the autoimmune component of the pathophysiology of MD may open doors to research on the role of immunotherapy and immunosuppressive biologics in MD treatment. […] The autoimmune hypothesis posits that an aberrant immune response against inner ear antigens could significantly affect MDs pathogenesis. […] Evidence supporting this hypothesis includes the presence of specific autoantibodies, elevated inflammatory cytokines, and a higher prevalence of autoimmune comorbidities in MD patients.

• #14 Meniere’s Disease Neuroscience Group

  https://kollinginstitute.org.au/menieres-disease-neuroscience-group

  Most Menieres disease patients are considered sporadic, and 20 per cent of patients show an abnormal immune response with persistent levels of pro-inflammatory cytokines (IL1, TNFa, IL-6), leading to a systemic chronic inflammation. […] These findings support the hypothesis that the interaction between hair cells stereocilia and the tectorial membrane in the organ of Corti or the otolithic membrane in the saccule play a central role in the pathogenesis of the disease. […] According to epidemiological and genetic data, the condition has a significant genetic contribution. Familial MD is observed in 6-10% of cases and rare mutations in OTOG, MYO7A and TECTA genes have been found in several unrelated families. […] Therefore, genetic factors and innate/adaptive immune responses play a central role in the pathophysiology of the condition.

• #15 Elevated G-CSF, IL8, and HGF in patients with definite Meniere’s disease may indicate the role of NET formation in triggering autoimmunity and autoinflammation | Scientific Reports

  https://www.nature.com/articles/s41598-022-20774-8

  The etiology and mechanism causing Menieres disease (MD) are not understood. […] The present study investigated the possible molecular mechanism of autoimmunity and autoinflammation associated with MD. […] We hypothesize that G-CSF, IL8, and HGF, which are involved in the development of neutrophil extracellular traps (NETs) and through various mechanisms influence the functions of macrophages, lymphocytes, and dendritic cells, among others, are key players in the development of EH and MD and could be useful in elucidating the pathophysiological mechanisms leading to MD. […] Biomarkers identified in the present study may suggest that both autoimmune and autoinflammatory mechanisms are involved in MD. […] We observed that various cytokines participating in autoimmunity and autoinflammation were up- and downregulated in MD.

• #15 Elevated G-CSF, IL8, and HGF in patients with definite Meniere’s disease may indicate the role of NET formation in triggering autoimmunity and autoinflammation | Scientific Reports

  https://www.nature.com/articles/s41598-022-20774-8

  The AUC analysis showed that G-CSF, IL8, HGF, CTACK, MIP1, TRAIL, and PDGFBB have diagnostic relevance, suggesting that these chemokines play an important role in MD. […] IL8, MIP1a and GCSF also had internal correlation greater than 0.7 indicting that they probably act in the same pathway and are activated by the same mechanisms. […] It was reported that G-CSF, IL8, and HGF are involved in the development of NETs through a mechanism by which G-CSF stimulates the production and release of neutrophils and primes neutrophil programming toward NET generation. […] Therefore, we hypothesize that NET formation might be involved in initiating the pathological process of MD as consequence of the enhanced production of specific chemokines. […] Linear regression demonstrated significant correlations between MIP1 and GCSF, between IL2R and GCSF, and between MIP1 and IL8.

• #15 Elevated G-CSF, IL8, and HGF in patients with definite Meniere’s disease may indicate the role of NET formation in triggering autoimmunity and autoinflammation | Scientific Reports

  https://www.nature.com/articles/s41598-022-20774-8

  These results are likely to indicate that MIP1 and IL2R might interact with GCSF signaling and that MIP1 might also interact with IL8 signaling in MD. […] The present study also showed that higher levels of CTACK, IP-10, and MIG were associated to comorbidity of MD and hypertension, and there were positive age-related correlations of CTACK in the MD group but not in the control group. […] The current study demonstrated that G-CSF, IL8, HGF, CTACK, and MIP1 have diagnostic relevance for MD. […] G-CSF, IL8, and HGF are involved in the development of NETs and influence the functions of macrophages, lymphocytes, and dendritic cells, among others, through various mechanisms involved in MD.

• #16

  https://www.omim.org/entry/156000

  Meniere disease is a chronic illness characterized by intermittent episodes of vertigo lasting from minutes to hours, with fluctuating sensorineural hearing loss, tinnitus, and aural pressure (Sajjadi and Paparella, 2008). […] In a review of 500 patients with Meniere disease, Paparella (1985) detected a 20% incidence of positive family history and suggested multifactorial etiology. […] The findings were consistent with a genetic predisposition for the development of Meniere disease. […] By whole-exome sequencing of a Spanish grandmother, mother, and daughter with Meniere disease, Requena et al. (2015) identified 2 novel heterozygous variants, one in the DTNA gene (601239) and the other in the FAM136A gene (616275); all 3 patients carried both variants. […] The authors suggested that the OTOG gene plays a relevant role in the pathophysiology of hearing and vestibular function in Meniere disease. […] Paparella (1985) postulated that the variation in these 2 genes contributed to the multifactorial etiology of Meniere disease in this family.

• #17

  https://link.springer.com/article/10.1007/s10238-023-01192-0

  The etiology of Mnires disease (MD) remains controversial. Allergies are potential extrinsic factors that, in conjunction with underlying intrinsic factors, may cause MD. […] Patients with MD tend to have a high risk of comorbid allergies or an allergy history, showing positive allergy immunology characteristics. […] Allergy mediators such as IgE may worsen the symptoms of MD. […] Deposits of IgE in the vestibular end organs indicate the ability of the inner ear to participate in immune reactions. […] Allergic challenges can induce vertigo in animals and humans. […] Anti-allergy therapy plays a positive role in patients with MD and animal models of endolymphatic hydrops.

• #18 Neck and Brain Venous Lesions in Meniere’s Disease

  https://www.heraldopenaccess.us/openaccess/neck-and-brain-venous-lesions-in-meniere-s-disease

  Chronic Cerebo-Spinal Venous Insufficiency (CCSVI) was demonstrated in 81.4% of patients with Menieres disease vs. 12.7% of the volunteers. […] Our results suggest that there is a high incidence of neck and brain veins in patients suffering of Menire’s disease. […] EH appears to be the pathological substrate necessary, but it is not responsible for the entire cochlear and vestibular symptomatology. […] At the moment Endolimpatic Hydrops is a symptom as vertigo, hearing loss, fulness, tinnitus. […] Multiple causes would work synergistically in determining a condition of cellulartoxicity on non-receptive cells of the inner ear, particularly those of the vascular stroma and of the endolimphatic sac, affected by the homeostatic mechanisms of the endolimpha. […] The slowing down of the venous outflow causes an increase in venous pressure associated with a wave reflected by the arterial system, with a further increase in intravascular pressure to the capillary venous system that worsen the damage of the endothelial cells leading to small endothelial layer breaks.

• #19 Meniere’s Disease and Fluid build up in the ears – Chronic cerebrospinal venous insufficiency –

  https://caringmedical.com/prolotherapy-news/menieres-disease-chronic-cerebrospinal-venous-insufficiency/

  The researchers further suggest that chronic cerebrospinal venous insufficiency could be considered a new ultrasound vascular pattern of the cerebrospinal venous system in patients affected by definite Meniere’s Disease. […] Chronic cerebrospinal venous insufficiency was diagnosed in 330/412 (80.1%) of Meniere’s Disease patients. […] The results of the present study confirm the close relationship between vascular disorders and Meniere’s Disease. […] The encouraging responses to vascular interventional therapy on Meniere’s Disease symptoms suggest that this may be a promising path for interpretation and treatment of this complex disease. […] So here a connection may be made between weak and damaged cervical spine ligaments and the onset and progression of Meniere’s Disease. […] Treating and stabilizing the cervical ligaments can alleviate these problems by preventing excessive abnormal vertebrae movement, the development or advancing of cervical osteoarthritis, and the myriad of problematic symptoms they cause including nerve, vein, and arterial compression.

• #19 Meniere’s Disease and Fluid build up in the ears – Chronic cerebrospinal venous insufficiency –

  https://caringmedical.com/prolotherapy-news/menieres-disease-chronic-cerebrospinal-venous-insufficiency/

  Meniere’s disease is a disorder of the inner ear that causes spontaneous episodes of vertigo – a sensation of a spinning motion – along with fluctuating hearing loss, ringing in the ear (tinnitus), and sometimes a feeling of fullness or pressure in the ear. […] A February 2022 review in the Journal of the American Medical Association otolaryngology, head & neck surgery found that “The most common reported causes of Meniere’s disease were autoimmune or immune-mediated, genetic, or structural dysfunction of the inner ear.” […] The cervical spine, temporomandibular joint, and Eustachian tube all are connected through the autonomic nervous system as well as peripheral nerves such as the trigeminal nerve. […] All of these symptoms can easily appear if some condition were causing fluid to accumulate in the inner ear because of Eustachian tube dysfunction.

• #19 Meniere’s Disease and Fluid build up in the ears – Chronic cerebrospinal venous insufficiency –

  https://caringmedical.com/prolotherapy-news/menieres-disease-chronic-cerebrospinal-venous-insufficiency/

  One study reported that 75% of patients with Meniere’s disease show a strong association with head and neck movements in the atlanto-occipital and atlantoaxial joints triggering attacks of vertigo. […] This study compares the frequency of signs and symptoms from the cervical spine in 24 patients diagnosed with Meniere’s disease and 24 control subjects. […] Most of the patients (75%) reported a strong association between head-neck movements in the atlanto-occipital and atlanto-axial joints and triggered attacks of vertigo. […] The study shows a much higher prevalence of signs and symptoms of cervical spine disorders in patients diagnosed with Meniere’s disease compared with control subjects from the general population. […] The researchers of a May 2017 study in the medical journal Acta Otolaryng suggest and confirm a correlation between chronic cerebrospinal venous insufficiency (CCSVI) diagnosis and Meniere’s Disease.

• #20 Optimal management of Ménière’s disease | TCRM

  https://www.dovepress.com/optimal-management-of-meacuteniegraverersquos-disease-peer-reviewed-fulltext-article-TCRM

  There is no longer a clear consensus on the mechanism by which hydrops results in attacks in this group, but treatments continue to focus on decompressing the hydropic ear either through medications or by surgical intervention. […] The other major group, the migraine group believed early on that migraine explains many cases of the disorder, perhaps through the mechanism of ischemia due to vasospasm. […] We have hypothesized that hydrops acts as an intermittent Starling resistor to lower perfusion pressure in the inner ear, which is additive with other causes of lowered perfusion such as cerebrovascular diseases. […] Improvement in Mnires disease has been hypothesized to occur when hydrops is due to inflammation or autoimmune factors. […] Steroids are used to reduce edema after ischemic stroke, so steroid-responsive Mnires disease also supports ischemia as a mechanism of attacks.

• #21 Meniere’s Disease | ENT & Allergy of Delaware

  https://entad.org/resources/patient-information-dr-teixido/endolymphatic-hydrops-menieres-disease/

  Endolymphatic hydrops and Menieres disease are disorders of the inner ear. Although the cause is unknown, it probably results from an abnormality of the fluids of the inner ear. […] The cause of Menieres disease is still unknown. Recent research suggests disorders of maintenance of the ion balance in the inner ear fluids may be the root cause of the ear symptoms in Menieres disease. […] We have recently discovered that the tiny blood vessels in the inner ear are innervated by branches of the same nerve that innervates the intracranial blood vessels severely affected in migraine attacks. Electrical stimulation of this trigeminal nerve has caused fluid changes in the inner ear which could affect it severely enough to cause a problem like Menieres disease. […] There is increasing evidence to support this migraine mechanism of Menieres disease.

• #22 Meniere’s Disease: A Challenging and Relentless Disorder | Ento Key

  https://entokey.com/menieres-disease-a-challenging-and-relentless-disorder-2/

  Work to identify an underlying pathophysiology of ELH has identified several possible gene products for the disorder. […] In fact, the relationship of ELH to the symptoms of MD has also been questioned, raising debate as to whether the observed ELH is the direct pathologic initiator of cochleovestibular dysfunction or epiphenomena of subtler biochemical perturbations. […] In an effort to identify ELH with more certainty in vivo, Nakishima et al. described visualization of ELH in MD patients using intratympanic gadolinium and delayed high-resolution (3T) MRI; however, intratympanic gadolinium administration is off-label; therefore an intravenous (IV) gadolinium protocol has been developed.

• #23 SciELO Brazil – The clinical features and image characteristics of Meniere’s disease patients with endolymphatic hydrops confirmed by enhanced magnetic resonance imaging The clinical features and image characteristics of Meniere’s disease patients with e

  https://www.scielo.br/j/bjorl/a/BL3XKtwSmTjCnjxKF8kbXSy/?lang=en

  The MRI results showed that compared with patients with unilateral EH, the symptoms of the first affected ear of patients with bilateral EH were more serious. There were no significant differences in the age, age of first onset, and course of disease between unilateral EH patients and bilateral EH patients. In addition, we found that the degree of EH was significantly positively correlated with the degree of hearing loss. The more severe the vestibular and cochlear EH, the more severe the hearing loss. […] The degree and distribution of EH may be related to the degree of hearing loss.

• #24 Meniere’s Disease Treatment in Joliet, New Lenox and Morris, Illinois

  https://www.entsurgicalillinois.com/menieres-disease.html

  Mnires disease, also called idiopathic endolymphatic hydrops, is a disorder of the inner ear. […] Although the cause is unknown, it probably results from an abnormality in the fluids of the inner ear. […] The theory is that too much inner ear fluid accumulates either due to excess production or inadequate absorption. […] In some individuals, especially those with involvement of both ears, allergies or autoimmune disorders may play a role in producing Mnires disease. […] Diuretics (water pills) reduce the body’s total sodium count and, with it, the amount of water in the body. […] Because fluids shift from compartment to compartment, loss of salt and water into the urine will shrink the amount of fluid in the body generally as well as in the inner ear. […] This approach makes sense because people with Mnires Disease have too much fluid in the inner ear.

• #24 Meniere’s Disease Treatment in Joliet, New Lenox and Morris, Illinois

  https://www.entsurgicalillinois.com/menieres-disease.html

  If medical therapy fails, surgical treatment may be indicated. […] One approach is directed toward increasing the absorption of endolymph (the fluid in the hearing and balance canals of the inner ear), since there is an excess of endolymph in patients with Mnires Disease. […] The other approach aims at decreasing the inner ear’s vestibular balance function in order to reduce symptoms of vertigo. […] In principle, endolymphatic sac surgery is a non-destructive, surgical manipulation of the endolymphatic sac aimed at increasing fluid drainage from the inner ear. […] This surgical technique decreases vestibular function to control symptoms of vertigo, either by denervation or destruction of the affected ear. […] In 95% of cases, control of vertigo is achieved. […] For unilateral cases, intratympanic gentamicin reduces vertigo by decreasing residual balance function on the affected side, but with a 30% risk of hearing loss. […] All destructive procedures result in decreased vestibular function on the treated side, which many patients consider a fair exchange once central compensation has stabilized their balance function.

• #25 Inflammatory Mechanism, Imaging Diagnostics, and Novel Treatment of Meniere’s Disease | Frontiers Research Topic

  https://www.frontiersin.org/research-topics/52977/inflammatory-mechanism-imaging-diagnostics-and-novel-treatment-of-menieres-disease/magazine

  Menieres disease (MD) is believed to originate in the inner ear, and the specific pathological changes of endolymphatic hydrops can be demonstrated in histological preparations or with MRI, although the etiology of the disease is unknown. […] The favorable outcome of intratympanic corticosteroid indicates that inflammatory reaction is involved in most MD patients. […] This Research Topic aims to promote a better understanding of the underlying inflammatory mechanisms of MD, to improve the accuracy of MRI detection of endolymphatic hydrops in MD, and to explore and test the novel therapeutic strategy of MD targeting the inflammatory reaction process.

• #26 Meniere Disease – Ear, Nose, and Throat Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/ear-nose-and-throat-disorders/inner-ear-disorders/meniere-disease

  In Meniere disease, pressure and volume changes in the labyrinthine endolymph affect inner ear function. The etiology of endolymphatic fluid buildup is unknown. […] Meniere disease tends to be self-limited. Treatment of an acute attack is aimed at symptom relief and done in a staged fashion; the least invasive measures are done first, and then if the measures are ineffective, ablative procedures are sometimes done. […] Although more invasive, endolymphatic sac decompression relieves vertigo in most patients, spares vestibular function, and poses minimal risk of hearing loss. Thus this procedure is still classified as a vestibular-sparing treatment. […] When vestibular-sparing treatments are ineffective, an ablative procedure is considered. Intratympanic gentamicin (chemical labyrinthectomytypically 0.5 mL of a 40 mg/mL concentration) is injected through the tympanic membrane. Follow-up with serial audiometry is recommended to check for hearing loss. […] Vestibular neurectomy (an intracranial procedure) relieves vertigo in about 95% of patients and usually preserves hearing. A surgical labyrinthectomy is done only if preexisting hearing loss is profound.

• #27 Ménière’s Disease – American Hearing Research Foundation

  https://www.american-hearing.org/disease/menieres-disease/

  Mnires disease (Menieres) is a disorder of the inner ear that sometimes seems to carry with it more questions than answers. […] Although it can come on at any age, most people with Mnires are 40 or older. […] Mnires is called a disease, but it really is a cluster of symptoms for which the underlying cause is unknown and for which there currently is no cure. […] The inability to specifically identify the pathology of what is prompting the symptoms of Mnires sets it apart as its own disorder. […] With Mnires disease, the bottom-line question is: What is the underlying cause of the fluid build-up that is triggering the symptoms? […] Unfortunately, to date, the scientific and medical community has been unable to definitively identify what causes the fluid build-up in Mnires or why people get it.

• #28 Otolaryngology Head and Neck Surgery Molecular Otology Research| University Hospitals l Cleveland, OH | University Hospitals

  https://www.uhhospitals.org/uh-research/department-research/otolaryngology-research/translational-basic-research/molecular-otology

  The goal for this project is to better understand the molecular biological changes that occur in the inner ear as a result of endolymphatic hydrops, a condition known to accompany Meniere’s disease, a disorder associated with hearing loss and vertigo. […] This will provide clues to better understand the exact biochemical pathways that are activated in this condition and possibly provide for new treatment strategies. […] It is expected that this project will lead to a larger grant in the future which will allow us to explore and define the entire cascade of biochemical changes which accompany Meniere-related endolymphatic hydrops and could lead to better treatment alternatives for patients with Meniere-related hearing loss.

• #29 Reflection for Meniere’s Disease in the Era of Modern Precision Medicine – SciTeMed Publishing Group

  https://www.scitemed.com/article/3063/Reflection-for-Meniere%E2%80%99s-Disease-in-the-Era-of-Modern-Precision-Medicine

  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. […] 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. […] 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. […] Hence, patients with “asymptomatic” endolymphatic hydrops are not necessarily the same as those with “symptomatic” MD.

• #30 Autoimmunity as a Candidate for the Etiopathogenesis of Meniere’s Disease: Detection of Autoimmune Reactions and Diagnostic Biomarker Candidate | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0111039

  Certain findings have provided evidence that autoimmunity may underlie the pathology of Meniere’s disease. The prevalence of systemic autoimmune diseases such as rheumatoid arthritis, ankylosing spondylitis, and SLE in patients with Meniere’s disease is 3- to 8-fold higher than in the general population. […] The results of this study can provide the basic information for the development of diagnostic biomarkers as well as the understanding of pathologic mechanisms of Meniere’s disease. […] The most commonly encountered proteins in the ES luminal fluid were immunoglobulins and their variants. This is expected, as the ES is a known site of immunologic responses in the inner ear. However, the only proteins that were detected exclusively in the patients with Meniere’s disease were immunoglobulins, their variants, and interferon gamma regulatory factor, suggesting that increased inflammatory reactions in the inner ear may contribute to the pathology of Meniere’s disease. […] Our results imply that multiple autoantibodies or antigens rather than a specific antibody or antigen can cause autoimmune reactions in the inner ear that result in Meniere’s disease.

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