Idiopathic endolymphatic hydrops (Meniere's disease)

There continues to be considerable confusion concerning the diagnosis and treatment of perilymphatic fistulas (PLFs) and Meniere's disease. This paper reports an 8-year experience with patients whose symptoms were compatible with Meniere's disease but who had some other element that raised the possibility of their having a PLF. This review is a retrospective report on 64 patients who had fluctuating hearing loss, vertigo, tinnitus, and aural fullness typical of Meniere's disease. However, they also had a positive fistula test and/or symptoms beginning immediately after head trauma. They all underwent at least a unilateral PLF repair, and some also underwent an endolymphatic sac-mastoid shunt operation. This report provides a literature review to help put this series' results into perspective. Of those 40 patients who underwent an initial PLF repair, 58% had a successful outcome. Of the 10 patients who underwent an initial PLF repair plus an endolymphatic sac-mastoid shunt, 70% had a successful outcome. Since it is often difficult to distinguish patients with PLFs and secondary endolymphatic hydrops (Meniere's syndrome) from patients with Meniere's disease (idiopathic endolymphatic hydrops), PLF repair is a reasonable first operation, reserving an endolymphatic sac-mastoid shunt operation as a secondary procedure if needed. The decision to consider a PLF repair is guided by a positive fistula test or an immediate onset of Meniere's disease symptom complex after head or ear trauma.

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IntroductionMeniere's disease is associated with impaired hearing, tinnitus, vertigo, and aural fullness. Many anatomical studies have suggested idiopathic endolymphatic hydrops as the pathological basis of Meniere's disease, which now can be visualized by using gadolinium -enhanced magnetic resonance imaging of the inner ear. ObjectiveTo investigate the development of endolymphatic hydrops in Meniere's disease by monitoring the vestibules and cochleae of affected patients. MethodsInner ears of 178 patients with definite unilateral Meniere's disease diagnosis were visualized by 3-dimensional fluid-attenuated inversion recovery and three-dimensional real inversion recovery magnetic resonance imaging following bilateral gadolinium intratympanic injection. The scans were used to evaluate the presence and degree of endolymphatic hydrops in the vestibules and cochlear structures, including the cochlear apical turn, the cochlear middle turn, and the cochlear basal turn. The correlation of endolymphatic hydrops occurrence between the various parts of the inner ear was determined. ResultsSymptomatic endolymphatic hydrops was detected on the affected side in all patients, whereas asymptomatic endolymphatic hydrops was detected on the unaffected contra-lateral side in 32 patients (18.0%). On the affected side, the cochlear apical turn and the cochlear middle turn demonstrated significantly higher rates of endolymphatic hydrops than the cochlear basal turn and the vestibule. The severity of endolymphatic hydrops gradually decreased from the cochlear apical turn to the cochlear basal turn. On the contra lateral side, the incidence and degree of the detected asymptomatic endolymphatic hydrops were significantly greater in the cochleae than in the vestibules (p<0.05), with no significant difference detected between the cochlear turns. ConclusionProgression of endolymphatic hydrops appears to be directional, initiated in the cochlea. The order of endolymphatic hydrops severity gradually decreases from the cochlear apical turn to the cochlear basal turn and then to the vestibule. Endolymphatic hydrops in the vestibule is associated with symptomatic Meniere's disease.

A clinical diagnosis of Ménière's disease (MD) is made based on medical history and audiometry findings. The 1995 American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) guidelines requires histopathological confirmation of endolymphatic hydrops (EH) for a diagnosis of “certain” MD. Symptoms such as dizziness and ear fullness are important diagnostic features; however, the descriptions provided by patients are frequently vague and non-specific. A recently developed magnetic resonance imaging (MRI) protocol to document EH is, therefore, useful for the evaluation of inner ear status in patients with MD. In this study, patients with MD were assessed using MRI and the HYDROPS (HYbriD of Reversed image Of Positive endolymph signal and native image of positive perilymph Signal) protocol to investigate the effectiveness of MRI for visualization of the endolymphatic space in the diagnosis of MD by correlating clinical laboratory parameters with the grade of EH. Of the 123 patients with MD recruited in this study, 80 had definite MD, 11 had probable MD, and 32 had possible MD based on the 1995 AAO-HNS guidelines. The EH grade based on HYDROPS MRI was determined independently by two otorhinolaryngologists and compared with several clinical parameters, including the diagnostic scale of MD (1995 AAO-HNS guidelines), pure tone average (PTA), low tone average (LTA), canal paresis (CP) on the caloric test, and disease duration. Cochlear hydrops and vestibular hydrops were detected in 58 and 80% of 80 definite MD ears, in 33 and 58% of 12 probable MD ears, and in 5 and 27% of 37 possible MD ears, respectively. The proportion of higher hydrops grades increased significantly with grade according to the MD diagnostic scale (p < 0.0001). Both PTA and LTA were significantly higher in patients with hydrops grade 2 than hydrops grade 0 in both the cochlea and the vestibule. CP was significantly higher in patients with grade 2 than grade 0 vestibular hydrops. Disease duration was not associated with hydrops grade. Radiological evaluation of MD using the HYDROPS protocol is useful for evaluation of the extent and severity of EH in the diagnosis of MD based on its pathophysiological mechanism.

Meniere's disease is a heterogeneous disease of the audiovestibular system, etiologically caused by endolymphatic hydrops, accompanied by both vestibular and auditory symptoms. Various evaluation methods can be used in the diagnosis of the disease. Some of these are; Pure tone audiometry, otoacoustic emissions, Electrocochleography (EcochG), vestibular evoked myogenic potentials and magnetic resonance imaging. Electrocochleography is a testing method that allows the assessment of cochlear electrical potentials. The primary condition diagnosed by ECochG and available for neuromonitoring intraoperatively is Meniere's disease. In Meniere's disease, the magnitude of the summation potential (SP) increases with the impairment of the transduction process, while the action potential (AP) will remain relatively constant. This condition causes an increased SP/AP ratio and is pathophysiologically associated with endolymphatic hydrops. The aim of our study is to investigate the role and worth of EcochG in the clinical diagnosis of Meniere's disease according to the studies conducted in the current literature. Pubmed and Web of Science database was used for our review study. The search words used to examine the studies in between 2014-2024 were determined as ''ECochG, Meniere's disease''. In this context, a total of 126 studies were reached. 9 research articles and 4 systematic reviews were included, including the role of Electrocochleography in the diagnosis of Meniere's disease and its evaluation results. It is thought that the EcochG test method alone will not be sufficient in the diagnosis of Meniere's disease and that examining it together with other evaluation methods will guide clinicians to a more accurate and reliable diagnosis.

It is widely accepted that Meniere’s is an idiopathic disease involving the membranous inner ear. Disease which cause secondary endolymphatic hydrops (SEH) are differentiated from Meniere’s disease. Theoretically this idea is acceptable, but it is quite difficult to distinguish SEH from idiopathic endolymphatic hydrops (IEH). In other words, when symptoms similar to those of Meniere’s disease coexist with another definitive disease, we find difficulty in determining whether the symptoms are caused by the disease itself or whether they are truly those of Meniere’s disease. In this paper, we will discuss this controversial problem in diagnosing Meniere’s disease.

The aim of this study was to evaluate endolymphatic hydrops using the 3T temporal bone magnetic resonance imaging (MRI), performed according to the chosen protocol, and determine whether it could be applied as an objective diagnostic tool for Menière's disease. 105 participants diagnosed with probable (n = 50) and definite (n = 55) Menière's disease were included in this prospective study at Vilnius University Hospital, Santaros Clinics. Audiometry, vestibular function tests, videonystagmography, and computer posturography were performed before MRI. The 3T MRI with gadolinium contrast was performed to evaluate the endolymphatic hydrops. Imaging protocol consisted of 3D-FLAIR and 3D T2DRIVE sequences. Vestibular endolymphatic sac was interpreted as enlarged if occupied more than 50% of the vestibular area. 78.1% of subjects had abnormal MRI findings other than hydrops, and it was more than 90% (50/55) of patients in the definite MD group (p < 0.001). Changes in caloric test were observed in 63.8% of subjects in general, and in 76.4% of patients with a definite Menière's disease. The side of the endolymphatic hydrops observed on MR imaging corresponded to the clinical diagnosis of the Menière's disease based on the results of audiometry (p < 0.001) and unilateral weakness (p < 0.001). Endolymphatic hydrops on MRI and directional preponderance in caloric test were two independent predictors of the definite Menière's disease. Temporal bone 3T MRI with gadolinium contrast is clinically superior to confirm the diagnosis of Menière's disease. Grade II endolymphatic hydrops on MRI, directional preponderance, and unilateral weakness on caloric test were independent predictors for the definite Menière's disease.

Click-evoked otoacoustic emissions were recorded from 18 patients with Meniere's disease (MD), most of whom had low-frequency hearing loss (LFHL). The main frequencies obtained from patients with MD, where the maximum peak power of the emissions existed on the frequency spectra, were examined and compared with those obtained from patients with LFHL without MD and from subjects with normal hearing.The main frequencies of the emissions in patients with MD, ranged mainly from 0.8kHz to 1.1kHz, -somewhat lower than those in subjects with normal hearing (mainly from 1.0kHz to 1.4kHz). And in MD patients with LFHL, the main frequencies were lower than in patients with LFHL without MD. Furthermore, in 2 patients with MD, the main frequencies became higher when the osmotic diuretics, glycerol or isosorbide were administrated.These findings suggest that in ears with MD, low-frequency dominant emissions are elicited because of a modification of the oscillations of the basilar membrane in endolymphatic hydrops, which is considered to be the main pathological finding in MD. It is possible that the main frequencies of the emissions might rise when endolymphatic hydrops is reduced by treatment with osmotic diuretics.

We compared the signal intensity ratio (SIR) of the cochlear basal turn between Meniere's disease and healthy controls to investigate potential damage of the blood-labyrinth barrier in Meniere's disease. Thirty patients diagnosed with unilateral definite Meniere's disease and 24 healthy controls were enrolled. 3D-FLAIR scan was conducted to assess the grades of endolymphatic hydrops in Meniere's patients while measuring the SIR of cochlear basal turns in both groups. The differences of bilateral SIR between Meniere's disease and healthy control were compared, and the correlation between the SIR on affected ear in Meniere's disease and the grades of cochlear and vestibular hydrops were analyzed. SIR of affected ear in Meniere's disease exhibited significant increase compared to that of unaffected ear. No significant difference was observed in SIR between the two ears in the healthy control. Furthermore, the SIR of unaffected side in Meniere's disease was higher than that of both ears in healthy controls. The SIR in affected ear of Meniere's disease exhibited positive correlation with hydrops in both cochlea and vestibula. The permeability of blood-labyrinth barrier is increased in Meniere's disease, in combination with the typical criteria of Meniere's disease it may be a good biological marker. Destruction of blood-labyrinth barrier may be one of the causes of endolymphatic hydrops in Meniere's disease.

Menière's disease (MD) is associated with endolymphatic hydrops (EH), which is an accumulation of excessive endolymphatic fluid in the inner ear. However, using temporal bone CT, lower visualization rates of the vestibular aqueduct (VA) in these patients have also been reported. In this retrospective single-center imaging study, we have included 25 healthy subjects and 47 patients having a definite, probable, or possible clinical diagnosis of MD that underwent temporal bone CT and inner ear MRI performed 4 h after contrast media administration. Two radiologists independently ranked the morphology of the VA in healthy subjects and in MD patients, using a three-level score (completely visible, discontinuous, and not visible). Each subject was then graded, based on both the VA's appearance and on EH presence. In healthy subjects, the VA was normal (grade 0) in 22/25 (88%) ears and discontinuous (grade I) in 3 healthy ears (12%). In the symptomatic ears of MD patients, we found 17/56 ears (30.3%) with VA grade 0, 15/56 ears (26.8%) with grade I, and 24/56 ears (42.8%) with grade II (p < 0.001). In MD patients, EH was observed in 46/94 ears (48.9%). A VA of grade 0 would eliminate the presence of EH with a negative predictive value of 88.6%, while a VA grade II would predict the presence of saccular hydrops with a positive predictive value of 93.1%. The evaluation by temporal bone CT of the VA can predict the presence of EH on MRI with a high positive predictive value. • The evaluation by temporal bone CT of the vestibular aqueduct can predict the presence of EH on MRI. • A vestibular aqueduct of grade 0 would eliminate the presence of EH on MRI with a negative predictive value of 88.6%. • A vestibular aqueduct grade II would predict the presence of endolymphatic hydrops on MRI with a positive predictive value of 93.1%.

Meniere disease (MD) is characterized by the classic triad of symptoms (episodic vertigo, tinnitus, and hearing loss) is likely caused by endolymphatic hydrops of the labyrinthine system of the inner ear.The course of MD is variable.Some patients experience progressive hearing loss with infrequent vestibular symptoms; some have severe and frequent vertigo with only mild auditory symptoms; and some manifest both auditory and vestibular symptoms in equal measure. Most patients tend to cycle from active symptoms to prolonged remissions. A clinical diagnosis of MD is made based upon the following criteria: Two or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours Audiometrically documented low- to mid-frequency sensorineural hearing loss in the affected ear Fluctuating aural symptoms (reduced or distorted hearing, tinnitus, or fullness) in the affected ear Symptoms not better accounted for by another vestibular diagnosis Although audiometric testing is a required part of the diagnostic evaluation, there is no specific diagnostic test for MD. A variety of other conditions can present with symptoms similar to MD and are often considered in the differential diagnosis.The conditions include vestibular migraine, vestibular schwannoma, multiple sclerosis (MS), transient ischemic attacks (TIAs), benign paroxysmal positional vertigo, and Cogan syndrome. Dietary and lifestyle modifications for all patients – As initial therapy for all patients with MD, (Grade 2C).If other triggers are identified (eg, nicotine, stress, monosodium glutamate [MSG]), these should also be avoided. Dietary and lifestyle modifications should be continued indefinitely Vestibular rehabilitation for persistent disequilibrium – For patients with MD and persistent disequilibrium symptoms between attacks, we suggest referral for vestibular rehabilitation therapy (Grade 2C).Although vestibular rehabilitation does not reduce the frequency of vertigo attacks, the exercise activities maximize balance and central nervous system (CNS) compensation for disequilibrium symptoms.Vestibular rehabilitation has no role in the treatment of acute vertigo due to MD. Pharmacotherapy for refractory symptoms – For all patients with MD with refractory symptoms and poor quality of life despite dietary and lifestyle interventions,we suggest the use of pharmacotherapy rather than no pharmacotherapy (Grade 2C). Betahistine and diuretics are the two options for pharmacologic therapy to reduce the severity and intensity of MD attacks. We suggest treatment with betahistine rather than diuretics, when available (Grade 2C). Acute episodes of vertigo should be managed with vestibular suppressants and antiemetics if necessary. Glucocorticoid therapy (systemic or intratympanic) for persistent symptoms – Among all patients with refractory symptoms severe enough to require further treatment beyond dietary changes, lifestyle adjustment, and first-line pharmacotherapy, there is no widely accepted agreement upon which treatment is preferred. However, we suggest treatment with glucocorticoids rather than other therapies for these patients (Grade 2C). For the majority of patients with MD and refractory, disabling vertigo symptoms despite first-line treatments, we treat with a limited course of oral glucocorticoids. For patients with MD with disabling vertigo symptoms despite first-line treatments, and in whom oral glucocorticoid therapy is contraindicated, or who through shared decision-making prefer intratympanic therapy with intratympanic glucocorticoids. Additional treatment options for patients refractory to glucocorticoid therapy – For patients with refractory MD symptoms and continued poor quality of life despite treatment with glucocorticoids (systemic or intratympanic),additional treatments are offered depending on the degree of labyrinthine function (severity of vertigo attacks and the degree of disequilibrium between attacks) and the level of hearing loss to determine the most appropriate management for an individual patient. For MD patients with preserved hearing,treatment with endolymphatic sac procedures (including decompression and/or shunting) or sacculotomy is offered; if this is unsuccessful,we typically then offer treatment with intratympanic gentamycin. For patients with MD with complete hearing loss in the affected ear, we suggest treatment with IT gentamycin rather than labyrinthectomy (Grade 2C). Labyrinthectomy is generally reserved for those patients who have disabling symptoms that persist despite treatment with intratytmpanic gentamicin.

Endolymphatic hydrops is a characteristic pathological manifestation of Meniere's disease (MD) that has been previously associated with autoimmunity. Interest in the circulating immune complex (CIC) has increased due to its reported role in the occurrence of MD. The present study aimed to investigate the potential value of serum CIC concentration in the diagnosis of MD and the therapeutic potential of cyclophosphamide (CTX) for the treatment of MD. In the present study, guinea pigs were immunized with isologous crude inner ear antigens to establish an autoimmune MD model. Pure tone audiometry, Vestibular-evoked myogenic potential test, electrocochleography test and auditory brainstem response was applied in this study for assessing the severity of MD in guinea pigs. ELISA was applied to measure CIC, tumor necrosis factor α (TNF-α) and heat shock protein 70 (HSP70) expression levels in the serum samples of different groups of patients. Western blotting was applied to detect the protein expression of HSP70 in inner ear tissues in guinea pigs. Hematoxylin and eosin staining was applied to visualize the spiral ganglions in spiral ganglions models. CIC expression in the inner ear was detected by immunohistochemistry. In vivo experiments were performed to confirm the therapeutic effects of CTX in MD. Serum concentrations of CIC, TNF-α and HSP70 were found to be significantly higher in patients with MD, which were also associated with increases in hearing classification and the severity of endolymphatic hydrops. Using a guinea pig MD model, ELISA results revealed significantly increased serum CIC, TNF-α and HSP70 concentrations compared with those in the control group. ABR results showed that the thresholds in the CTX group were notably decreased compared with that in the dexamethasone group, whereas CIC concentrations in the serum were reduced following dexamethasone and CTX treatments compared with those after saline treatment. In the inner ear tissues, the CIC concentration in CTX group was lower than that in the dexamethasone group. Similarly, reductions in HSP70 and TNF-α concentrations was also observed in a similar manner. Immunohistochemistry staining found notably lower CIC deposition in the inner ear tissues following CTX treatment than that in dexamethasone group. Taken together, higher CIC expression can be used as a biomarker for MD diagnosis. The efficacy of CTX in MD was found to be higher compared with that in dexamethasone, which may be associated with the effective inhibition of CIC, HSP70 and TNF-α generation.

ObjectiveThe diagnosis of Ménière's disease (MD) and delayed endolymphatic hydrops (DEH) is challenging due to overlapping symptoms. This study aimed to retrospectively evaluate the diagnostic performance of gadolinium-enhanced delayed inner ear magnetic resonance imaging (MRI) for visualizing endolymphatic hydrops (EH) in patients with MD and DEH.MethodsThis retrospective study included 191 patients with unilateral MD (n = 164) or DEH (n = 27), stratified into definite MD (DMD, n = 129), probable MD (PMD, n = 35), and DEH subgroups. All patients underwent intratympanic administration of gadolinium followed by 3.0-T MRI using 3D-FLAIR and 3D-real-IR sequences. Clinical data, including pure-tone audiometry (PTA), speech recognition score (SRS), electrocochleography (ECochG), and caloric testing, were analyzed to assess correlations between EH and auditory-vestibular function.ResultsEH was detected in 85.2% (23/27) of clinically diagnosed DEH patients. Detection rates were 96.1% in DMD cases and 28.6% in PMD cases. There was no significant difference in cochlear or vestibular EH between the DMD and DEH groups. However, the DMD group exhibited a significantly higher incidence and severity of EH than the PMD group (P < 0.05). In DMD patients, the degree of EH was positively correlated with PTA, clinical stage, and disease course (P < 0.05), and negatively correlated with SRS (P < 0.05). The degree of vestibular EH was also positively correlated with caloric test abnormalities (Spearman's ρ = 0.350, P < 0.001).ConclusionGadolinium-enhanced delayed MRI of the inner ear provides objective evidence of EH and improves the diagnostic accuracy for MD and DEH. Its incorporation into standardized diagnostic protocols may further enhance clinical decision-making.

Meniere’s disease is nearly invariably associated with endolymphatic hydrops (the net accumulation of water in the inner ear endolymphatic space). Vestibular maculae utriculi were acquired from patients undergoing surgery for Meniere’s disease and acoustic neuroma and from autopsy (subjects with normal hearing and balance). Quantitative immunostaining was conducted with antibodies against aquaporins (AQPs) 1, 4, and 6, Na+K+ATPase, Na+K+2Cl co-transporter (NKCC1), and α-syntrophin. mRNA was extracted from the surgically acquired utricles from subjects with Meniere’s disease and acoustic neuroma to conduct quantitative real-time reverse transcription with polymerase chain reaction for AQP1, AQP4, and AQP6. AQP1 immunoreactivity (−IR) was located in blood vessels and fibrocytes in the underlying stroma, without any apparent alteration in Meniere’s specimens when compared with acoustic neuroma and autopsy specimens. AQP4-IR localized to the epithelial basolateral supporting cells in Meniere’s disease, acoustic neuroma, and autopsy. In specimens from subjects with Meniere’s disease, AQP4-IR was significantly decreased compared with autopsy and acoustic neuroma specimens. AQP6-IR occurred in the sub-apical vestibular supporting cells in acoustic neuroma and autopsy samples. However, in Meniere’s disease specimens, AQP6-IR was significantly increased and diffusely redistributed throughout the supporting cell cytoplasm. Na+K+ATPase, NKCC1, and α-syntrophin were expressed within sensory epithelia and were unaltered in Meniere’s disease specimens. Expression of AQP1, AQP4, or AQP6 mRNA did not differ in vestibular endorgans from patients with Meniere’s disease. Changes in AQP4 (decreased) and AQP6 (increased) expression in Meniere’s disease specimens suggest that the supporting cell might be a cellular target.

Serum antidiuretic hormone (ADH) levels were determined with a radioimmunoassay using a reversed-phase C18 silica column in 200 patients with vertigo, dizziness and/or deafness; 85 of them had a diagnosis of Meniere's disease. The serum ADH level was significantly elevated in patients with Meniere's disease and in others with endolymphatic hydrops; e. g. cochlear Meniere's disease or delayed hydrops. On the other hand, the serum ADH level was rarely elevated in patients without endolymphatic hydrops. The increase of serum ADH in Meniere's disease showed no differences in relation to sex, duration of disease or laterality, but it was closely linked to vertigo attacks. The serum ADH level was very high during the week after an attack of vertigo and dizziness (acute phase), but it fell when the attack ended (remission). These results suggest that disorders of ADH-control in the inner ear are part of the mechanism underlying vertigo attacks in Meniere's disease.