Meniere's Disease Patients Research Articles

Cochlear implantation in patients with definite Meniere's disease.

The exact pathomechanism of deafening in Meniere's disease (MD) is still unknown; intoxication of hair cells and neural damage from endolymphatic hydrops is discussed. In the literature, there are only a few reports on hearing outcome of MD patients after treatment with cochlear implantation (CI) whereby especially the comparison of MD vs. non-MD patients with CI differs. In this retrospective study, results in speech understanding [Freiburger Einsilber (FES65) and Hochmair-Schulz-Moser test in quiet (HSM) and in noise (HSM+10dB)] of 27 implanted MD patients were collected and compared to a matched standard CI cohort. Alternative diagnoses were excluded as far as possible by re-analyzing neuroradiologic imaging. After first fitting, MD patients showed significantly better results in FES and HSM testing compared to controls. At 1-year refitting, this effect could not be seen anymore. To conclude, cochlear implantation is a safe and effective treatment for deafness in MD patients. Results in speech understanding are at least equal compared to general CI recipients. To the best of our knowledge, this retrospective study examined the largest collective of CI users deafened by MD so far.

Is gentamycin delivery via sustained-release vehicles a safe and effective treatment for refractory Meniere's disease? A critical analysis of published interventional studies.

The aim of this study is to review the literature on sustained-release vehicles delivering gentamycin in the inner ear of patients suffering from Meniere's disease (MD), and critically assess their respective clinical effectiveness and safety. Asystematic literature reviewwas conducted in Medline and other database sources until January 2016, along with critical analysis of pooled data. Overall, six prospective and four retrospective studies were systematically analyzed. The total number of treated patients was 320. A 2year patient follow up was only reported in 40% of studies. Inner ear gentamycin delivery using sustained-release vehicles is associated with improved vertigo control (strength of recommendation B), and quality of life (strength of recommendation B) in MD sufferers. In addition, dynamic-release devices seem to achieve high rates of improvement in the appearance of tinnitus (65.4%) and aural pressure (76.2%). By contrast, percentages of complete and partial hearing loss appear unacceptably high (31.08 and 23.38% of patients, respectively), compared to historical data involving simple intratympanic gentamycin injections. Sustained-release vehicles for gentamycin delivery may have a role in the management of MD patients who have previously failed intratympanic gentamycin injections, or those who have already lost serviceable hearing. Their use as first line treatment over single intratympanic injections for all MD patients, who do not respond to conservative treatment should be discouraged.

Triple semicircular canal plugging: a novel modality for the treatment of intractable Meniere’s disease

Conclusions: TSCP, which can reduce vertiginous symptoms in patients with intractable MD, represents an effective therapy for this disorder.Objective: To explore the long-term efficacy of triple semicircular canal plugging (TSCP) in the treatment of intractable Meniere’s disease (MD).Methods: Seventy-nine patients diagnosed with unilateral MD referred to a vertigo clinic of the hospital between December 2010 and December 2013 were included in this study for retrospective analysis. TSCP was performed in the affected ear for each patient. Vertigo control and auditory function were measured. Pure tone audiometry, caloric test, and cervical vestibular evoked myogenic potential (cVEMP) were performed in 2-year follow-up. Thirty-six MD patients, who accepted endolymphatic sac decompression (ESD) operation were selected as a comparison group.Results: The total control rate of vertigo in the TSCP group was 98.7% in the 2-year follow-up, with a complete control rate of 81.0% and substantial control rate of 17.7%. The rate of hearing preservation was 70.9%. The total control rate of vertigo in the ESD operation group was 72.2%. The vertigo control rate of TSCP was significantly higher than that of ESD operation. Twenty-four months after treatment, canal paresis was found in the operation side of all patients of TSCP by means of caloric test.

Meniett Therapy for Ménière's Disease: An Updated Meta-analysis.

To re-evaluate the efficacy of Meniett therapy for the treatment of Ménière's disease (MD). PubMed, Embase, Cochrane Library, Clinicaltrials.gov, ChiCTR, and the CNKI database were searched for articles in English and Chinese published before August 31, 2015. Included in this meta-analysis were studies that dealt with outcomes of Meniett therapy for the treatment of MD, including randomized controlled clinical trials, case-control studies, and prospective or retrospective cohort studies, with sample sizes of ≥ 10 subjects. Keywords included endolymphatic hydrops, Ménière's disease, pressure, Meniett, and transtympanic micropressure treatment. Fourteen studies were included, involving a total of 345 MD patients. Data were analyzed using the Meta package in R. Dichotomous outcomes were expressed as risk ratios with 95% confidence intervals, and weighted mean differences with 95% confidence intervals were used to present continuous outcomes. Heterogeneity of the included studies was quantitatively assessed by χ(2) and I tests. Fixed-effects models were used for I(2) <50%; otherwise, random-effects models were used. Funnel plots were constructed to test the publication bias. This study showed that Meniett therapy may prevent vertigo attacks and substantially reduce its frequency in MD patients. It may also alleviate the functional deficit. The impact of Meniett therapy on hearing remains uncertain. The optimal effect might maintain for approximately 18 months. This meta-analysis suggested that Meniett therapy may be a useful second-line therapy in the treatment of MD.

The change of hippocampal volume and its relevance with inner ear function in Meniere's disease patients.

To reveal the relationship between the disease progression and the hippocampal volume in Meniere's disease, which may indirectly reflect the degree of patients' chronic exposure to stress. Retrospective review of medical records and brain magnetic resonance image (MRI) of Meniere's disease patients and healthy controls from 2008 to 2013. Age- and sex-matched consecutive 38 definite Meniere's disease patients and 76 healthy controls where brain MRIs, hearing, and vestibular function tests were available. We divided the patients into normal and abnormal groups according to the results functional tests such as pure tone audiometry (PTA), vestibular evoked myogenic potential (VEMP), and bithermal caloric test. Absolute hippocampal volume of Meniere's disease patients was significantly smaller than that of controls (3.33±0.84cm(3) for left side vs. 3.82±0.37cm(3) for right side, p<0.001). Mean left hippocampal volume of patients with abnormal VEMP and bithermal caloric test on lesion side was significantly smaller than that of the patients with the normal test result (all p<0.05). Average hearing thresholds of the patients showed strong correlation with left hippocampal volumes (p-value<0.001). Those with decreased left hippocampal volume were also more likely to experience augmented emotional stress reactivity. Meniere's disease patients had significantly decreased hippocampal volume and the volume was significantly correlated with severity of hearing and vestibular function of affected side. This may represent that Meniere's disease patients are exposed to chronic stress for unpredictable vertigo and hearing fluctuation, which finally cause hippocampus atrophy.

Diagnostic value of vestibular-evoked myogenic potentials in Ménière's disease and vestibular migraine.

Overlaps can be seen between vestibular migraine (VM) Ménière's Disease (MD) and diagnosis is difficult if hearing is normal. We aimed to investigate the sacculo-collic pathway in VM patients, MD patients, and healthy controls to define the diagnostic role of cervical VEMP (cVEMP). VEMP testing in response to 500 Hz and 1000 Hz air-conducted tone burst (TB) stimulation was studied prospectively in 22 subjects with definite VM (according to Bárány nomenclature), 30 subjects with unilateral definite MD, and 18 volunteers matched healthy controls. In VM subjects, response rate, p13 and n23 latencies were similar to healthy controls, but peak-to-peak amplitudes were bilaterally reduced at 500 Hz TBs (p= 0.005). cVEMP differentiated MD patients from VM and healthy controls with asymmetrically reduced amplitudes on affected ears with low response rates at 500 Hz TBs, and alteration of frequency dependent responses at 500 and 1000 Hz TBs. These findings suggest that cVEMP can be used as a diagnostic test to differentiate MD from VM. On the other hand, VEMP responses are symmetrically reduced on both sides in VM patients, suggesting that otolith organs might be affected by migraine-induced ischemia.

Evaluation of the otolith function using c/oVEMPs in patients with Ménière's disease.

BackgroundCervical and ocular vestibular evoked myogenic potentials (c/oVEMPs) reflect otolith function. Up-to-date, there are no published reports on the systemic evaluation of otolith function in Ménière’s Disease (MD) nor are there any reports on the differences in VEMPs between patients with early and late stage MD. The aim of this study was to evaluate the difference in c/oVEMPs between patients with MD and normal controls, as well as between patients with early and late stage MD.MethodsThirty patients with unilateral MD and thirty healthy subjects (as normal controls) were prospectively enrolled. c/oVEMPs using 500 Hz tone-burst stimuli were performed. VEMP tests were repeated 3 times on each subject to ensure reliability and reproducibility of responses. VEMPs were defined as present or absent. Abnormal VEMP was defined by lack of VEMP response.ResultsIn the control group, abnormal cVEMPs and oVEMPs responses were detected in 6.67 and 3.34 % respectively. In MD patients (20 with early stage MD [ES-MD], 10 with late stage MD [LS-MD]), abnormal cVEMPs and oVEMPs responses were detected in 40 and 16.7 % respectively. More patients with MD showed abnormal responses in c/oVEMPs as compared to the control group (p < 0.05). cVEMPs was more often abnormal as compared to oVEMPs in MD patients (p < 0.05). There was a significant difference in abnormal cVEMP responses between ES-MD patients (25 %) and LS-MD patients (70 %) (p < 0.05). Difference in abnormal oVEMP responses (ES-MD, 5 %; LS-MD, 40 %) was significant (p < 0.05).ConclusionAn increased occurrence of abnormal c/oVEMP recordings appeared in MD patients, possibly as a result of hydrops of the otolith. cVEMPs were more often abnormal in MD patients as compared to oVEMPs, suggesting that saccular dysfunction may be more common than utricular dysfunction. Furthermore, o/cVEMP abnormalities in the LS-MD group were significantly higher than those in the ES-MD group, suggesting the trend that otolith damage is gradually increasing with the aggravation of cochlear injury in MD.

Treatment of Meniere’s disease with intratympanic dexamethazone plus high dosage of betahistine

PurposeThe aim of the present study was to assess if the combined therapy of intratympanic dexamethasone (ITD) and high dosage of betahistine (HDBH) is able to provide increased vertigo control compared to ITD alone in patients suffering from definite unilateral Meniere’s disease (MD). Materials and methodsConsecutive MD patients were enrolled and randomly divided in two groups, each comprising 33 cases. Group A received a combination of ITD and identical-appearing placebo pills while Group B received a combination of ITD and HDBH. ITD protocol consisted of three consecutive daily injections. HDBH comprised 144mg/day (48mg tid). The main outcome measures were: 1) vertigo class, pure tone average (PTA), speech discrimination score (SDS) and Functional Level Score (FLS) according to the American Academy of Otolaryngology-Head and Neck Surgery criteria; 2) complete and substantial vertigo control according to the Kaplan–Meier survival method. ResultsSixty two patients completed the 24-month follow-up. A complete vertigo control was achieved in 14 patients (44%) from Group A and in 22 patients (73.3%) from Group B, statistically significant (p=0.01). Complete vertigo relief is also significant according to the Kaplan–Meier method: p=0.027, log rank test. Substantial vertigo control was obtained in 21 patients (65.6%) in Group A and 27 patients (90%) in Group B. The difference is statistically significant, p=0.02. The difference is significant according to the Kaplan–Meier method: p=0.035, log rank test. No significant differences between hearing levels and tinnitus scores were demonstrated between the groups. ConclusionsOur preliminary results demonstrate that complete and substantial vertigo control is significantly higher in patients treated with a combination of HDBH and ITD.

Impact evaluation and association with EuroQol 5D health-related utility values in Ménière's disease.

The study was aimed at evaluating the validity of impact measures among patients with Ménière’s disease (MD) with outcome variables of EuroQol generic health-related quality of life (HRQoL) measures (i.e., EQ-5D) by using Visual Analogue Scale (VAS) and EQ-5D index values. 183 members (out of 200 contacted) of the Finish Ménière Association returned the questionnaires that they had filled out. Various open-ended and structured questionnaires focusing on diagnostic aspects of symptoms and impairment caused by the disease were used. For activity limitation and participation restriction, standardized questionnaires were used. Open-ended questions on impact of the disease were asked, and subsequently classified based on the WHO-ICF classification. The general HRQoL was evaluated with EQ-5D index value and EQ VAS instruments. Correlation and linear regression analyses were used to explore the association between HRQoL and other aspects. Based on the explanatory power of different models the disease specific semeionic model provides the most accurate prediction in EQ-5D index calculations (38 % of the variance explained). In EQ VAS scores, HRQoL is most accurately determined by participation restriction (53 % of the variance explained), but the worst prediction was in ICF-based limitations (8 % of the variance explained). Interestingly, attitude and personal trait explained the reduction of HRQoL somewhat better than ICF-based variables. Activity limitation and participation restrictions are significant components of MD, but are less frequently recognized as significant factors in self-evaluating the effect of MD on the quality of life. The current study results suggest that MD patients seem to have problem identifying factors causing activity limitation and participation restrictions and hence use the semiotic description focusing on complaints.

Prognostic implications of and audiometric evidence for hearing fluctuation in Meniere's disease.

1) To establish criteria for significant hearing fluctuation by assessing the range and occurrence of hearing fluctuations over the course of Meniere's disease; 2) to determine if audiometric evidence exists to support the notion that Meniere's disease is a pathophysiologic process involving the whole cochlea; and 3) to suggest prognostic implications for initial hearing fluctuation in patients with Meniere's disease. Retrospective case series review. A total of 488 patients diagnosed by 1995 American Academy of Otolaryngology-Head and Neck Surgery Meniere's disease criteria for whom audiometric data were prospectively collected (2 cohorts: 341 and 146 patients initially seen between April 2002 to July 2003 and between January to December 2010, respectively). Based on several definitions for significant hearing fluctuation, change in hearing was categorized as "same," "worse," or "better" between any two consecutive evaluations. The relationship of initial hearing fluctuation to future hearing fluctuation and future hearing loss was evaluated. Hearing fluctuation was evident in Meniere's disease patients with heterogeneous audiometric follow-up; and the characteristics of these hearing fluctuations, including the mean incidence, is described. Audiometric data suggests that there is a high congruence in Meniere's disease between changes in low- and high-frequency thresholds. Initial hearing fluctuation is associated with the occurrence of future and more frequent hearing fluctuations. Understanding the range of hearing fluctuations establishes a basis for determining audiometric thresholds used in evaluating future therapeutic trials aimed at the prevention of hearing loss in Meniere's disease. This knowledge will also inform the counseling directed toward patients diagnosed with Meniere's disease.

The correlation between symptoms of definite Meniere's disease and endolymphatic hydrops visualized by magnetic resonance imaging.

This study aimed at investigating the correlation between a battery of diagnostic symptoms of definite Meniere's disease (MD) and the degree of endolymphatic hydrops (EH) in the inner ear. Prospective study. Fifty-four patients diagnosed with unilateral definite MD were enrolled in the study. The hearing levels of all patients at the low, middle, and high frequencies were evaluated. Three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) and three-dimensional real inversion recovery (3D-real IR) magnetic resonance imaging (MRI) were performed 24 hours after bilateral intratympanic injection of gadolinium to assess the presence and grading of EH. Various degrees of EH were observed in the vestibule and/or each turn of the cochlea in the affected ears of all patients. The duration of MD disease and low-tone and middle-tone hearing thresholds were proportional to the extent of EH in the vestibule and cochlear. However, no significant correlation was demonstrated between EH and other aspects of symptoms such as high-tone hearing loss, tinnitus, and aural fullness. Of all subjects, 16.7% exhibited bilateral EH on MRI exam who were diagnosed with unilateral MD based on diagnostic criteria. EH in the inner ear of MD patients exhibits a progressive deteriorative trend over time. Low-tone and middle-tone hearing thresholds can indirectly reflect the severity of EH in the cochlea. EH may not be the primary cause of tinnitus and aural fullness in patients with MD. Patients diagnosed with unilateral MD should maintain long-term regular follow-ups for the possibility of developing bilateral EH. 4.

Migraine features in patients with Meniere's disease.

To better understand the features of migraine in Meniere's disease (MD). Retrospective review of prospectively obtained surveys in an outpatient clinic of a tertiary medical center. Detailed questionnaires on headaches and dizziness were given to consecutive patients presenting with dizziness. The responses were verified by the clinician with the patient. The data, in addition to the clinical history and audiogram, were used to diagnose patients with migraine headaches and MD using criteria set by the International Headache Society (IHS) and the American Academy of Otolaryngology-Head and Neck Surgery, respectively. The prevalence of migraine-like symptoms in those patients with MD, who did not fit the diagnostic criteria for migraine, was evaluated. Thirty-seven patients with definite MD were included. There was a predominance of females (female/male:26/11). Mean age of patients was 52 ± 14 years. Nineteen patients (51%) had migraine headaches. Fifteen patients fulfilled the criteria for definite vestibular migraine. Of those who did not fulfill the IHS migraine criteria, a majority had characteristics such as a family history of migraine, visual motion sensitivity, or lifelong motion sickness that were highly suggestive of a migraine disorder. A majority of patients with MD have migraine headaches as defined by the IHS. Sensitivity to visual motion, light and sound, head motion, smells, weather changes, or medication was present in 95% of all patients with definite MD and 82% of non-IHS migraine MD patients. This may suggest that MD may be an atypical variant of migraine.

Genetic aspects and clinical characteristics of familial Meniere's disease in a South Korean population.

This study was undertaken to investigate the prevalence, inheritance patterns, and clinical characteristics of familial Meniere's disease (MD) in a South Korean population. Direct interviews, telephone interviews, and reviews of the medical records of definite Meniere's disease patients and their families. Direct and telephone interviews were performed for 286 definite MD patients and their family members who were suspected of having MD. The diagnosis of MD in family members was made by obtaining a detailed history, performing basic neurotological examinations and reviewing hearing test results. The clinical characteristics as well as the prevalence and inheritance patterns of familial MD were analyzed. The prevalence of familial Meniere-like syndrome (at least one family member with definite MD and other members with probable MD) and definite familial MD (two or more family members with definite Meniere's disease) were 9.8% and 6.3%, respectively, and the most common inheritance pattern was autosomal dominant with incomplete penetrance. The significant clinical characteristics of familial cases were an early disease onset and a higher prevalence of migraines. This is the first report describing the genetic aspects of MD in a single large Asian population. The prevalence of definite familial MD was 6.3% with an incomplete autosomal dominant inheritance pattern in most cases. Early-onset age and a high prevalence of migraines were significant clinical features of familial MD in this South Korean population. These data could provide a basis for the analysis of the genetic mechanism of familial MD in Asian populations. 4.

Practical 3DCT imaging of the vestibular aqueduct for Meniere’s disease

Conclusion: Radiographic visualization of the vestibular aqueduct (VA) from a lateral inside view was effective in assessing patients with Meniere’s disease (MD). There were no VA shapes specific to MD on radiography, except for an obliterated VA. This technique could yield more accurate images and functional assessment of the VA for MD evaluation in a clinically useful and convenient manner, without requiring morphologic measurement. Objective: To visualize the detailed images of the VA using three-dimensional (3D) computed tomography (CT) and discuss its clinical utility in assessing MD. Subjects and methods: The VAs in 13 healthy subjects and 25 MD patients, who were definitely diagnosed according to criteria described by the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology, Head and Neck Surgery (AAO-HNS), were imaged from the medial and lateral sides using 3DCT and compared to conventional CT images. Results: Examination of the VA from both the lateral outside and inside views on 3DCT yielded more precise images than generated by conventional CT and could be useful to estimate the VA function. The estimated VA function in the MD ears was significantly abnormal compared to the function in healthy ears. An obliterated VA was characteristic of affected MD ears.

Effects of body tilt on multifrequency admittance tympanometry.

Hydrops and abnormalities of inner fluid pressure are involved in some otologic diseases such as Ménière's disease (MD). However, demonstrating abnormal perilymphatic or endolymphatic pressure is challenging. Multifrequency tympanometry studies in MD patients demonstrated an increase of the width of conductance tympanograms (outside an attack) compared with controls. To confirm that the increase in conductance width is caused by hyperpressure and not hypopressure in these patients tested outside an attack, we assessed the effect of changes in inner ear fluid pressure caused by body tilt on the results of multifrequency admittancemetry tympanograms. A multifrequency tympanometry including conductance (G) tympanogram at 2 kHz and resonance frequency measurements were performed in 20 volunteers (40 ears) free of otologic or neurologic disease. The measures were collected in three different positions: vertical, supine, and Trendelenburg positions. Changes in inner ear fluid pressure caused by body tilt induced an increase in the width of G tympanograms. In the vertical position, the mean value was 141.7 ± 56.5 daPa; in the supine position, it increased to 158 ± 58.3 daPa; and increased even more in the Trendelenburg position (20 degrees), with a mean of 184 ± 69.6 daPa (p < 0.01). Resonance frequency also increased in the Trendelenburg position.We conclude that the increased width of G tympanograms in MD patients outside an attack may be caused by an increase in inner ear fluid pressure.

Benign Paroxysmal Positional Vertigo Prevalence in Meniere’s Disease: Is Meniere's Disease a Predisposing Factor?

Article type: Original Article Background: Meniere’s disease (MD) is a chronic illness defined as an idiopathic syndrome of endolymphatic hydrops. Benign paroxysmal positional vertigo (BPPV) is a balance disorder and can be the sequel of diverse inner ear impairments. Objectives: The purpose of this study was to investigate the prevalence of BPPV in MD patients. Materials and Methods: All patients with definite or probable MD participated in this cross-sectional descriptive and analytic study between March 2013 and February 2014. We used the American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS) criteria plus electrocochleography (ECochG) test results to define the presence of MD and positional tests to determine the presence of BPPV. The frequency was reported. Independent sample t-test was also used in SPSS19. Results: A total of 39 patients (43 ears) with definite or probable MD (26 female, 13 male; mean age: 46.4 ± 9.2 years) were enrolled in this study. Six out of 39 cases (15.4%) had BPPV in the posterior semicircular canal. The mean duration of MD was 28.5 months in BPPV group and 13.2 months in the non-BPPV group. Regardless of the age of the patients, there was a significant difference (p = 0.02) between the duration of MD in the two groups. Conclusions: It seems that MD and BPPV are related and a longer duration of MD may result in a greater risk of developing BPPV.

Effect of hearing loss, age, and gender on the outcome of the cochlear hydrops analysis masking procedure.

The aims of the study are to investigate the effect of hearing loss, age, and gender on the outcome of the cochlear hydrops analysis masking procedure (CHAMP) and to assess the clinical utility of CHAMP for the diagnosis of Ménière's disease (MD). Prospective observational study. Otolaryngology department of a tertiary referral hospital. We recruited MD patients (MD, 12 subjects) and hearing loss patients (HL, 10 subjects). Control subjects (NC, 43 subjects) were matched for gender and age. CHAMP was performed in the patients and control subjects. The mean difference in latency between the wave V for the click alone response and wave V for the 0.5-kHz high-pass masking noise condition was compared among groups, and the effects of gender and age on the results were analyzed in the NC group. Both the MD group and the HL group had a smaller difference in latency compared to the NC group. The MD group and HL group showed no significant difference in latency. In the NC group, there were no significant differences in latency depending on gender and age. Hearing level seems to significantly affect the CHAMP results, making it unreliable to differentiate between MD patients and non-MD hearing loss patients. Therefore, the diagnostic value of CHAMP might be limited in MD.

Bilateral Meniere’s disease assessed by an inner ear test battery

Conclusion: An inner ear test battery may suggest bilateral involvement and aid in ‘grading’ of potential disease in a patient with actively symptomatic Meniere’s disease (MD). Objectives: This study applied an inner ear test battery in patients with bilateral MD to map the inner ear deficits in each ear. Methods: From 2009 to 2012, 100 (20%) of 498 MD patients were diagnosed with bilateral involvement, which was defined as established MD in one ear, and the opposite ear had inner ear symptoms combined with documented hearing loss. Each patient underwent audiometry, and ocular vestibular-evoked myogenic potential (oVEMP), cervical VEMP (cVEMP), and caloric tests. Results: Grading of inner ear deficits was based on the number of abnormal results in the inner ear test battery. Of 100 patients with bilateral MD, 54% had the same grade and 46% had different grades in their 2 ears. On the other hand, based on four-tone average, 79% had the same Meniere stage and 21% had different stages in their two ears. The difference between ears with asymmetric grades (46%) and ears with asymmetric stages (21%) accounts for 25% of bilateral MD patients, which may be caused by the saccular hydrops in the opposite ear.

Altered chromogranin A circulating levels in Meniere's disease.

Objectives. Meniere's disease (MD) is an inner ear disorder characterized by episodic vertigo, ear fullness, and hearing loss; usually vertigo attacks cluster in specific period. We studied in MD patients the circulating levels of chromogranin A (CgA) and vasostatin-1 (VS-1), secreted by the neuroendocrine system and involved in the regulation of the endothelial barrier function. Methods. Serum levels were assessed in 37 MD patients and 36 controls. The ratio between VS-1 and CgA was calculated. Results. CgA was increased in patients compared to controls (1.46 versus 0.67 nM, p = 0.01) while no difference was detected for VS-1 (0.41 versus 0.39, resp.). CgA levels in patients positively correlated with the frequency of vertigo spells in the previous four weeks (p = 0.008) and negatively with the time in days from the last vertigo attack (p = 0.018). Furthermore, the VS-1/CgA ratio negatively correlated with the frequency of vertigo spells (p = 0.029) and positively correlated with the time from the last attack (p = 0.003). Conclusion. The results indicate that variations of CgA levels, but not of VS-1, occur in the blood of patients with active MD, depending on the frequency of vertigo spells and the time from the last crisis.

4.メニエール病

Although the basis for both the sporadic and inherited forms of Meniere's disease (MD) remain undefined, it is likely to be multifactorial, one of the factors being a genetic predisposition. Several candidate genes, such as AQPs, COCH, and HLA, have been proposed for MD, many of them regulating directly or indirectly the ionic or water transport of the inner ear. Mutations/polymorphisms in KCNE potassium channel genes might play a causative role in MD, because KCNE potassium channels have been suggested to be involved in the transmembrane ion and water transport mechanisms of the inner ear. The SNPs analyses identified that 112G/A SNP in the KCNE1 gene and 198T/C SNP in the KCNE3 gene could determine an increased susceptibility to develop MD. Because of the familial clustering, the geographical and racial differences, no convincing evidence for an association with any genes in the development of MD exists. The phenotypic diversity of MD makes the selection of patients a challenge, and the late onset of the disease may complicate the selection of the controls. Finally, the predisposing factors for MD should differ between ethnic groups and even between families affected. In summary, existing candidate gene reports provide very little information on the etiology of MD at present. Future studies should emphasize the need to collect larger, well-defined control groups as well as sporadic/familial MD patients.