Objectives Endolymphatic sac decompression surgery (ESDS) is one of the surgical methods for intractable Menière’s disease (MD), and it is known as a relatively safe treatment that does not cause hearing loss. However, the effectiveness and the degree of vertigo control rate of ESDS are still controversial. In this study, we aimed to evaluate the clinical outcomes of ESDS in intractable MD.
Methods We retrospectively reviewed 33 patients who underwent ESDS for intractable MD from January 2002 to March 2022. Clinical characteristics of patients, pure tone threshold, medical treatment method, and number of vertigo attacks before and after surgery were assessed. The improvement of hearing and vertigo was evaluated according to the 1995 American Academy of Otolaryngology-Head and Neck Surgery criteria.
Results Of the 33 patients, the average follow-up period was 21.2 months, with immediate follow-up within 2 months (28 patients), short term between 2 and 6 months (27 patients), and long term at 12 months or later (29 patients). In the immediate hearing threshold, both air conduction and bone conduction showed slight deterioration, but there was no significant change in the long-term hearing threshold. At long-term follow-up, 12 patients (41.4%) were able to live without medication, and 18 patients (62.1%) showed improvement in their vertigo symptoms. In addition, patients who showed improvement in hearing also showed improvement in vertigo at the same time.
Conclusions ESDS in intractable MD is a relatively safe and effective treatment method for reducing vertigo attack without worsening hearing threshold.
The absence of a temporal bone overlying the superior semicircular canal causes superior semicircular canal dehiscence (SSCD). The vestibular symptom of SSCD syndrome (SCDS) is vertigo and audiologic symptoms include autophony, hyperacusis, and ear fullness. A 52-year-old man presented with left-sided unilateral hearing loss, aural fullness, and recurrent spinning-type vertigo. He had positive Hennebert sign and mixed-type hearing loss, with a prominent low-frequency air-bone gap. These symptoms reminded us of SCDS, and computed tomography (CT) revealed SSCD. However, the patient had not experienced vertigo until 1 week prior to the visit. In addition, the audiogram revealed fluctuation of hearing, which was aggravated when the vestibular symptoms manifested. Vertigo might be due to Menière’s disease rather than SCDS and SSCD was incidentally detected on CT. According to reviews, this is no reported case of SCDS manifested as Menière’s disease, so we report this case with a brief review of the literature.
Objectives The aim of this paper was to determine if a correlation exists between video head impulse test (vHIT) and electronystagmography with caloric test (ENG). More specifically, comparison of covert and overt value from vHIT test with cold and warm stimulation value from ENG test.
Methods Retrospective study of our single institue from the period of January 2015 to January 2017 enrolled 91 patients. Patients were divided into 3 groups by their diagnosis of either vestibular neuritis (VN), Meniere disease, or sudden sensorineural loss with vertigo accordingly. Each of the patients’ both ENG and vHIT data were recorded and parameters were evaluated.
Results VN group was the only group to show a significant correlation between canal paresis (CP) with covert and overt saccades. Further analysis was done in the VN group and result showed covert saccade showing a larger area under the receiver operation characteristic curve value (0.77) compared to overt saccades (0.70), implying that covert saccade is a more accurate parameter for the prediction of the CP value. Furthermore, a positive correlation was seen between the gain value and the cold caloric stimulation value as well as between warm caloric stimulation value.
Conclusions The value of our study lies in the fact that we have attempted to find a correlation between different parameters of 2 different vestibular tests. We concluded that the evaluation of overt nystagmus by the bedside head thrust test is inappropriate for predicting CP, and a vHIT is required to accurately evaluate vestibular function.
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A Comparative Analysis of the Vestibulocochlear Function in Patients with Isolated Semicircular Canal Hypofunction Using a Video Head Impulse Test Yu Jung Park, Min Young Lee, Ji Eun Choi, Jae Yun Jung, Jung Hwa Bahng Research in Vestibular Science.2023; 22(2): 34. CrossRef
Tumarkin otolithic crisis is an abrupt attack of falling without loss of consciousness because of peripheral vestibular disorders. It occurs without warning in patients with late stage or end-stage of Meniere’s disease. It is an otologic emergency due to the risk of fall down injury, and has been treated with labyrinthectomy, vestibular neurectomy, and intratympanic gentamicin injection. Many reports have reported the efficacy of intratympanic injection of gentamicin (ITIG) as chemical ablation. We report a 58-year-old man with Tumarkin otolothic crisis from Meniere’s disease who presented with sudden drop attack while driving a taxi. He has suffered from recurrent rotatory vertigo accompanied by fluctuating hearing loss and tinnitus in the right ear for years. Despite medical treatment over the next 2 months, he experienced three more sudden drop attacks. He got chemical ablation with ITIG and remains without sudden drop attack until fourteen months later. Tumarkin otolithic crisis is a life-threatening otologic emergency. What we have experienced in this case is that for the safety and quality of life of the patient who suffers from Tumarkin otolithic crisis, aggressive vestibular function ablation with high dose ITIG is necessary.
Objectives Ménière disease is a clinical syndrome characterized by the four major symptoms of episodic vertigo, sensorineural hearing loss, tinnitus, and aural fullness. Sensorineural hearing loss, especially low frequency, is the characteristic type of audiogram in Ménière's disease. However, it is difficult to distinguish idiopathic sudden sensorineural hearing loss (ISSNHL) with vertigo from the first attack of Ménière disease. The purpose of this study was to investigate the incidence of progression into Ménière Disease from low frequency ISSNHL.
Methods Two hundred eighty-three patients were included in this study. We classified the patients with ISSNHL according to the hearing loss in audiogram and analyzed how many of them actually progressed to Ménière disease based on diagnosis criteria.
Results Among the 240 patients, 37.1% (89 patients) were confirmed low frequency ISSNHL and 14.6% (13 patients) of them were diagnosed with Meniere disease.
Conclusions This study showed that the progression from low frequency ISSNHL to Ménière disease was higher than other frequency ISSNHL, as in other studies.
Objectives The purpose of this study is to investigate the hyper-responsiveness of cervical vestibular-evoked myogenic potential (cVEMP) in patients with Meniere disease (MD), and to compare the result of cVEMP between probable and definite MD group.
Methods A total of 110 patients satisfied with probable MD and definite MD criteria, which is recently formulated by the Classification Committee of the Bárány Society, were included. An interpeak amplitude and interaural amplitude difference (IAD) ratio of both ears was measured. The abnormal response of ipsi-lesional cVEMP was categorized into 2 groups; hyper-response and hypo-response. Chi-square test and Mann-Whitney U-test were used for statistical analysis.
Results In the probable MD and definite MD group, the mean IAD was 25.24%±17.79% and 53.82%±34.98%, respectively (p<0.01). The abnormal response of cVEMP at the affected ear was more frequent in the definite MD group, compared to the probable MD group (32/40 vs. 13/36, p<0.01). However, hyper-response was more frequently observed in the patients with probable MD, compared to the patients with definite MD (13/36 vs. 3/40, p<0.01).
Conclusion Hyper-response of cVEMP was more frequently observed in the early probable MD patients. It might be an early sign of MD, related with the saccular hydrops, which can help the early detection and treatment.
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Superior Semicircular Canal Dehiscence Syndrome Manifested as Menière’s Disease: A Case Report Byeong Jin Kim, Yun Na Yang, Chan Mi Lee, Eun Jung Lee Research in Vestibular Science.2021; 20(3): 108. CrossRef
Characteristics of Nystagmus during Attack of Vestibular Migraine Soyeon Yoon, Mi Joo Kim, Minbum Kim Research in Vestibular Science.2019; 18(2): 38. CrossRef
Vestibular schwannoma (VS) can have various symptoms and almost half of the patients have balance problems. Some patients with VS can suffer from recurrent vertigo mimicking Meniere’s disease. We present the case of a 61-year-old male patient who was suffering from recurrent vertigo due to VS while symptoms improved by chemical labyrinthectomy with gentamicin. We consider that chemical labyrinthectomy can be an option in the treatment of patients with recurrent vertigo for VS.
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Diagnosis and Management of Vestibular Schwannoma: Focus on Dizziness Sung Il Nam Research in Vestibular Science.2021; 20(4): 119. CrossRef
Ménièreʼs 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 Ménièreʼs 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 Ménièreʼs disease have been published, but there is no clear evidence that Ménièreʼs 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.
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 Ménièreʼs disease since endolymphatic hydrops is a main pathological finding of the disease. In this review, we discussed about the possible pathological mechanism of Ménièreʼs 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.
Endolymphatic hydrops is a representing pathologic finding of Ménière's disease. For the induction of endolymphatic hydrops in an animal model, surgical ablation of endolymphatic sac has been used. Although traditional model with the blockage of endolymphatic sac induced severe hydrops, it has several limitations for the study of pathophysiology of Ménière's disease. Recently, modified experimental models have been introduced, in which additional procedure was performed to induce the acute aggravation of hydrops after the surgical ablation. These new models could be helpful to elucidate the mechanism and develop a new treatment of Ménière's disease. In this review, we introduce the characteristics of animal models using surgical ablation of endolymphatic sac from the classical model to novel modified models.
Periodic alternating nystagmus (PAN) is a spontaneous horizontal jerky nystagmus that reverses its direction periodically with a quiescent interval. PAN has been reported in acquired and congenital forms. The main lesion site of the acquired form of PAN has been attributed to the caudal brainstem or cerebellum. Herein we report a 63-year-old male patient with Meniere’s disease, who presented PAN during a vertigo attack. The patient demonstrated no abnormality on neurologic evaluation and brain imaging, which is different feature compared to the central or congenital form of PAN. It should be kept in mind that peripheral vestibular disorders such as Meniere’s disease can produce PAN.
It is known that endolymphatic hydrops is a pathophysiologic mechanism of Meniere’s disease, while the detailed role is not fully clarified. Study for endolymphatic hydrops mainly represents a pathologic histologic finding found only in the post-mortem examination of temporal bone, so endolymphatic hydrops animal models have played important role in terms of understanding how hydrops affects the function of the inner ear. Numerous challenges with diverse animals and techniques have been tried to reveal the possible pathophysiology of endolymphatic hydrops and develop an effective treatment. This review shows historical background and technical advances in experimental animal model for endolymphatic hydrops and Meniere’s disease.
Objective: Mèniére's disease is one of the most common causes of episodic vestibular syndrome that shows symptom complexes of recurrent vertigo, hearing loss, tinnitus and ear fullness. Isosorbide (Isobide) is a osmotic diuretics which has been used for Meniere's disease to reduce the endolymphatic pressure. The purpose of this study was to evaluate the efficacy of isosorbide in the treatment of Mèniére's disease by comparing the combined therapy of isosorbide and betahistine (Meniace) to betahistine only.
Methods Among 220 patients enrolled with Mèniére's disease from 9 centers, 187 patients completed this clinical study. Patients were randomly subjected either to betahistine alone therapy at dose of 6 mg three times a day (n=97) or to combined therapy with isosorbide (dose of 30 mL three times a day) and betahistine (n=90) for 12 weeks. Two groups were compared at 4 and 12 weeks after treatment on frequency of vertigo, hearing level (pure tone audiometry, speech audiometry), electocochleography (ECoG), tinnitus (Tinnitus Handicap Inventory, THI) and quality of life (Korean functional level scale, Korean dizziness handicap inventory).
Results During first 4 weeks after treatment, the frequency of vertigo was not reduced in either betahistine alone therapy group or combined therapy group. However, between 8 and 12 weeks, the frequency of vertigo was significantly reduced in either group, and in the combined group frequency of vertigo was more significantly reduced than in the betahistine alone therapy group (p=0.041). The hearing level, ECoG, tinnitus and quality of life was not significantly different between two groups.
Conclusion Isosorbide and betahistine combined therapy were more effective for vertigo control than betahistine alone therapy. Isosorbide is an effective diuretic in vertigo control in definite Mèniére's disease.