Delayed endolymphatic hydrops （DEH） is a rare disease that causes vertigo and is often misdiagnosed as other vertigo diseases. This article reports on a patient with vertigo who was easily misdiagnosed. The patient was a middle ear cholesteatoma complicated by labyrinthine fistula （LF）; however, his vertigo was episodic vertigo, which could not be explained solely by LF causing labyrinthitis. The possibility of endolymphatic hydrops was suspected, which was confirmed by inner ear magnetic resonance gadolinium imaging. This is the first reported case of middle ear cholesteatoma complicated by LF and DEH. The patient underwent surgical resection of the cholesteatoma and three semicircular canal obstructions at the same time. During two years postoperative follow-up, the patient did not experience a recurrence of vertigo. When diagnosing vertigo diseases, a careful history of vertigo is of utmost importance.
Humans ; Endolymphatic Hydrops/diagnosis* ; Cholesteatoma, Middle Ear/complications* ; Vertigo/complications* ; Labyrinth Diseases/complications* ; Magnetic Resonance Imaging/adverse effects* ; Semicircular Canals

Objective:To investigate the characteristics of wideband acoustic immittance（WAI） measurements in patients with unilateral Ménière's disease（MD） and evaluate the clinical value of WAI in diagnosis of MD. Methods:WAI was performed in 30 patients with unilateral MD（30 ears for symptomatic and 30 ears for asymptomatic） and in 26 healthy individuals（52 ears）（control group）. The WAI measurements, including the frequency first appearing two peaks in energy absorbance（EA） tympanogram, resonance frequency（RF）, the peak value of absorbance（PVA）, the integral area of absorbance（IAA）, EA curve at peak pressure, were analyzed. Results:①The occurrence of two peaks in EA tympanogram in both the MD symptomatic and asymptomatic ear was observed in 27 ears（84.4%）, and 38 ears（70.4%） in the control group, with no significant difference in the frequency of first appearing in two peaks onset between the groups（all P>0.05）. ②The RF of the MD symptomatic ears was significantly lower than that of the asymptomatic ears（t=-3.544, P=0.001） and that of the control subjects（t=2.084, P=0.041）; there was no difference of RF between the MD asymptomatic ears and the control group（P>0.05）. ③The PVA were significantly lower in both MD symptomatic（t=4.240, P<0.01） and asymptomatic ears（t=4.202, P=0.001） than in controls. ④The IAA in MD symptomatic（t=3.295, P=0.001） and asymptomatic ears（t=3.193, P=0.003） was significantly lower than in the control group. ⑤Comparison of the EA curve at peak pressure of the three groups: the EAs of MD symptomatic ears were lower than those of the control group at the range of 1 059-2 911 Hz（all P<0.05）; the EAs of MD symptomatic ears were lower than those of MD asymptomatic ears within 1 000 Hz and 1 834-2 119 Hz（all P<0.05）; the EAs of MD asymptomatic ears were lower than those of the control group at the range of 515-2 748 Hz（all P<0.05）. Conclusion:Symptomatic ears in unilateral MD patients show alterations in some WAI measurements compared to asymptomatic ears and/or controls, suggesting that middle ear mechanical fuction of the affected side may be modified due to the endolymphatic hydrops. The clinical significance of WAI needs to be further explored in the context of evaluating MD.
Humans ; Meniere Disease/diagnosis* ; Endolymphatic Hydrops/diagnosis* ; Ear ; Hearing Tests ; Acoustics

Objective: To investigate the frequency characteristics and the pathological characteristics of the horizontal crista ampullaris in patients with Meniere's disease,and to analyse its structural basis. Methods: Between March, 2019 and November, 2019, seventy-two patients diagnosed as Meniere's disease (27 males and 45 females, aged from 13 to 74 years, with a course of disease ranging from 4 months to 32 years)in Shandong Provincial ENT Hospital were included.Caloric test, sinusoidal harmonic acceleration test (SHA), video-head impulse test (v-HIT), Gadolinium-enhanced inner-ear 3D-FLAIR MRI and pure tone audiometry were conducted in the patients. The function of the horizontal semicircular canal in these patients were analysed as well as its relationship with the degree of endolymphatic hydrops,clinical stage and duration. Light microscopy and transmission electron microscopy were used to observe the ultrastructure of horizontal semicircular canal crista ampullaris from six patients with refractory Meniere's disease who underwent labyrinthectomy. The number of type Ⅰ and type Ⅱ vestibular hair cells, the common pathophysiological changes of horizontal semicircular canal crista ampullaris were investigated in these patients. Statistical analysis was performed using SPSS 19.0. Results: With the increase of detection frequency, the abnormal rate decreased gradually. The abnormal rate of caloric test was 69.4% (50/72), SHA 51.4% (37/72), V-HIT 36.1% (26/72), comparation of the positive rate among the three tests showed statistically significant differences(P<0.05).Neither caloric test nor SHA had correlation with the degree of hydrops(P>0.05), but v-HIT(r=0.434,P<0.01).There was correlation with clinical stage to SHA and v-HIT(r=0.338,0.462,P<0.01), except caloric test(P>0.05).No significant relation was found with caloric test, SHA, v-HIT and course of disease(P>0.05).Morphological observation found abnormal monolayer epithelialization of the horizontal semicircular canal crista ampullaris significantly decreased number of type Ⅱ hair cells compared with type Ⅰhair cells. Hair cells showed perinuclear vacuolization, cytoplasmic vacuoles, mitochondrial electron density increasement and loss of stereocilia. Conclusions: The horizontal semicircular canal damage in the patients with Meniere's disease has a frequency-dependent characteristic, mainly occurres in low frequency area. With progress of the disease, the high frequency area of ampulla will be impaired gradually, and it is related to the degree of endolymphatic hydrops and hearing level. Hair cell injury would be observed,the frequency characteristics may be more associated with the disorder of type Ⅱ hair cells.
Caloric Tests ; Endolymphatic Hydrops ; Female ; Humans ; Male ; Meniere Disease ; Semicircular Canals ; Semicircular Ducts

Objective: To analyze the clinical characteristics of vestibular syncope (VS) associated with drop attacks (DA) in delayed endolymphatic hydrops (DEH). Methods: DEH cases with complete data were retrospectively analyzed, including three DEH cases with DA and VS (VS group), and six DEH cases without DA or VS (control group). The clinical profile, the results of neurotological examinations [such as pure tone audiometry, electrocochleography (EcochG), caloric test, vestibular evoked myogenic potentials (VEMP), and video head impulse test (vHIT)] and treatment outcomes were analyzed. Results: (1) In the VS group, there were three cases of ipsilateral DEH; in the control group, there were six cases of ipsilateral type. One case in each group had a history of migraine. (2) The prevalence of abnormal results in caloric test, vHIT, cervical VEMP, and ocular VEMP in the VS group was 3/3, 1/3, 2/2, and 2/2, respectively, and in the control group was 3/6, 0/3, 1/6, and 4/6, respectively. Two cases in each group underwent EcochG, and no identifiable waveform was elicited on the affected side, and-SP/AP ratio of unaffected side was less than 0.4. (3) Patients in both groups were initially treated with conservative medication. Two cases in the VS group subsequently received intratympanic injections of dexamethasone. No DA or VS occurred during a follow-up period lasting over one year. All patients achieved good control of vertigo during the follow-up period. Conclusions: VS may occur in the patients with DEH. The differential diagnosis of syncope in patients with otogenic vertiginous disease can help improve clinical diagnosis and treatment.
Endolymphatic Hydrops/diagnosis* ; Humans ; Retrospective Studies ; Syncope ; Vestibular Evoked Myogenic Potentials ; Vestibule, Labyrinth

Low frequency sensorineural deafness is a common subtype of idiopathic sudden deafness. Certain patients suffered recurrent attacks without vertigo, much alike Meniere's disease. Few of them developed into definite Meniere's disease during long-term follow-up in many clinical studies. Although the pathophysiology of recurrent low frequency deafness is yet unknown, the desease is considered associated with early state of endolymphatic hydrops or migraine. Otologists shall be aware of its clinical course and careful explanation is necessary at time of initial informed consent.
Endolymphatic Hydrops ; complications ; Hearing Loss, Sensorineural ; complications ; diagnosis ; Hearing Loss, Sudden ; Humans ; Meniere Disease ; complications ; Vertigo

BACKGROUND AND OBJECTIVES: Despite the extensive research for the Meniere's disease (MD), it's pathophysiology still remains uncertain and questionable among scientists. Clinical symptoms and audiometric tests form the basis for the diagnosis. Nevertheless the differential diagnosis can be extremely challenging, due to subjective and not specific results. Incorrect diagnosis is most likely and for this reason there is a great demand for objective and reliable tests. SUBJECTS AND METHODS: The presence of endolymphatic hydrops is necessary condition but non enough for the diagnosis. In this study we analyze retrospectively the summating potentials (SP)/action protentials (AP) ratio from peritympanic electrocochleography in 34 patients,divided in two groups (13 patients classified as defined MD and 21 patients classified as probable MD). The purpose was to identify the utility of the exam so to be able to achieve an early defined diagnosis. Furthermore our intention was to obtain an objective test, besides the clinical criteria currently in use for the diagnosis and the classification of the MD. RESULTS: The analysis of the SP/AP results shows high predictability for the MD (positive in six out of seven cases with a sensitivity greater than 80%). CONCLUSIONS: Our study demonstrates a satisfactory sensibility percentage in recurrent defined MD presentation (>80%). Early identification of MD can lead to an early treatment and control of the progression of the disease. Furthermore it could be a valuable instrument for the follow up and evaluation of the patients classified as probable MD in accordance with the criteria of the American Academy of Otolaryngology-Head and Neck Surgery (1995).
Audiometry, Evoked Response* ; Classification ; Diagnosis ; Diagnosis, Differential ; Early Diagnosis* ; Endolymphatic Hydrops ; Follow-Up Studies ; Humans ; Intention ; Meniere Disease* ; Neck ; Retrospective Studies

The Best clinical approaches for Meniere's disease starts from the proper diagnosis which is based on the clinical symptoms, including the characteristic features of vertigo and auditory symptoms. After first criteria has been made in 1972 by the American Academy of Otolaryngology-Head and Neck Surgery, it has been revised two times in 1985 and 1995. Japanese criteria have been made in 1974 and revised 2008. Most recent criteria of the Barany Society has been established in 2015. The basic notion is almost similar among these criteria, but detailed descriptions has been gradually changed in characteristics of vertigo and auditory symptoms based on the updated research data and consensus. Understanding of how the diagnostic criteria of Meniere's disease has been evolved is very important in terms of good clinical practice because they contain the development of knowledge about the disease. This article reviewed all the diagnostic criteria, and compared the differences.
Asian Continental Ancestry Group ; Consensus ; Diagnosis* ; Endolymphatic Hydrops ; Humans ; Meniere Disease* ; Neck ; Vertigo

OBJECTIVE: There were few recent study concern about usefulness of cochlear hydrops analysis masking procedure (CHAMP) in progression of Ménière's disease. The purpose of this study is to analyze changes in the CHAMP as advancement of Ménière's disease. METHODS: We studied 19 cases of ‘ Definite’ group of Ménière's disease. We assumed progression of Ménière's disease would be checked by pure tone threshold by four tone average (4PTA), low tone average (LPTA). We also compared electrocochleography (ECoG), dizziness handicap inventory (DHI), tinnitus handicap inventory (THI) as parameters for progression of Ménière's disease to CHAMP latency delay and amplitude ratio. Chi-square test was used as a statistical method. RESULTS: In the group of patients had abnormal amplitude ratio, ECoG value were not improved, but there was not statistically significant (odds ratio [OR]=5.727, p>0.05). Better DHI and THI score were not necessarily construed as the improvement value in the CHAMP (p>0.05). In amplitude ratio abnormal group, 4PTA was aggravated, but not statistically significant (OR=1.5, p>0.05). In the group of patient had abnormality in both latency delay and amplitude ratio, LPTA was relatively aggravated (OR in latency delay: 2, OR in amplitude ratio: 10); however, reveals no statistically significance between them (p>0.05). Change of ECoG, hearing threshold including 4PTA and LPTA with progression of Ménière's disease were not correlated significantly with latency delay or amplitude ratio of CHAMP. CONCLUSION: We conclude that CHAMP does not reflect clinical features with progression of Ménière's disease.
Audiometry, Evoked Response ; Dizziness ; Edema ; Endolymphatic Hydrops ; Hearing ; Hearing Loss ; Humans ; Masks ; Meniere Disease ; Methods ; Tinnitus ; Vertigo

Inner ear is composed of cochlea, vestibule, and endolymphatic sac which are enclosed by thin layer of epithelial cells. The enclosed space is filled with fluid named as endolymph where the [K⁺] is high and [Na⁺] is low. This unique ion composition is very important in maintaining normal hearing and balance function by providing K⁺ ions into sensory hair cells, which finally depolarize hair cells to facilitate the transport of sound and acceleration stimulation to central nervous system. The ion composition of inner ear is maintained by various ion transport through ion channels, transporters, and exchangers in the inner ear sensory and extra-sensory epithelium. The disruption of normal endolymphatic ion composition by the deterioration of the function of those ion channels can cause dysfunction of sensory epithelium, which consequently results in hearing and balance disorders. One of the possible pathology from the disruption of inner ear ion homeostasis is endolymphatic hydrops which is a phenomenon of excessive fluid accumulation of inner ear. The dysfunction of ion channels in inner ear epithelium can be an etiology of Ménière's disease since endolymphatic hydrops is a main pathological finding of the disease. In this review, we discussed about the possible pathological mechanism of Ménière's disease as a perspective of channelopathy as well as the role of various ion channels in the regulation of inner ear fluid volume based on the findings revealed by electrophysiological studies.
Acceleration ; Central Nervous System ; Channelopathies ; Cochlea ; Ear, Inner ; Electrophysiology ; Endolymph ; Endolymphatic Hydrops ; Endolymphatic Sac ; Epithelial Cells ; Epithelium ; Hair ; Hearing ; Homeostasis ; Ion Channels ; Ion Transport ; Ions ; Meniere Disease ; Pathology

Ménière's disease is a poorly understood disorder of the inner ear characterized by intermittent episodic vertigo, fluctuating hearing loss, ear fullness and tinnitus. In this paper, we present a review of the histopathology, cytochemistry, gene, blood-labyrinthine barrier and imaging of Ménière's disease. Histopathology is significant for neuroepithelial damage with hair cell loss, basement membrane thickening, perivascular damage and microvascular damage. Cytochemical alterations are significant for altered AQP4 and AQP6 expression in the supporting cell, and altered cochlin and mitochondrial protein expression. Since the discovery of aquaporin water channels (AQP1, AQP2, AQP3, AQP4, AQP5, AQP6, AQP7 and AQP9), it has become clear that these channels play a crucial role in inner ear fluid homeostasis. Several gene studies related to Ménière's disease have been published, but there is no clear evidence that Ménière's disease is associated with a special gene. Currently, imaging techniques to determine the extent and presence of endolymphatic hydrops are being studied, and further studies are needed to correlate the visualization of the endolymphatic hydrops with clinical symptoms.
Aquaporins ; Basement Membrane ; Ear ; Ear, Inner ; Endolymphatic Hydrops ; Hair ; Hearing Loss ; Histocytochemistry ; Homeostasis ; Magnetic Resonance Imaging ; Meniere Disease ; Mitochondrial Proteins ; Tinnitus ; Vertigo