Incidence of virus infection as a cause of Meniere’s disease or endolymphatic hydrops assessed by electrocochleography

Menieres disease (MD) may follow viral infection such as by neurotropic viruses known to invade the endolymphatic sac (ES) and provoke endolymphatic hydrops (EH). The objective of this study was to investigate whether neurotropic viruses may cause infection of the inner ear and provoke EH. Antiviral immunoglobulin (IgG) assay against herpes simplex 1 (HSV1), herpes simplex 2 (HSV2), adenovirus (ADV), varicella zoster virus (VZV) and cytomegalovirus (CMV) were performed in 109 patients with an advanced stage of MD and compared with those obtained from 26 patients operated on because of vestibular schwannoma (VS), who served as a control group, to evaluate whether there is an association between the IgG levels and the ECoGs summating potential/action potential ratio (SP/AP ratio) in the MD group. In MD patients, the IgG titre against VZV and ADV were significantly higher than in the control (schwannoma) group. However, no correlation was found between the IgG levels against ADV and VZV with the SP/AP ratio. Neurotropic viruses such VZV and ADV may play a role in the pathogenesis of MD, despite the absence of association between the levels of IgG titres and the SP/AP ratio     

Evidence of surgical treatments for intractable Meniere’s disease

Meniere’s disease is an inner ear disease, characterized by recurrent rotatory vertigo, sensorineural hearing loss and tinnitus. There are some with frequent vertigo attacks, progressive hearing loss and persistent annoying tinnitus even through the continuous standard medical treatments. These cases are thought to account for 10%–20% of all cases of Meniere’s disease. In this review article, we would like to demonstrate the evidences for surgical treatments according to the previous papers, and consider the next therapeutic strategies including surgical options according to the international guidelines.     

The theory of autoimmunity in Meniere’s disease is lacking evidence

The role of immunological factors in the pathophysiology of Meniere’s disease (MD) has been hypothesized. In order to evaluate the current level of evidence on autoimmunity in MD, original articles relevant to the matter (1970–2016) were reviewed. The following has been considered to support the theory of autoimmunity in MD (1) the increased prevalence of autoimmune diseases among MD patients, (2) the elevated levels of antibodies and immunocomplexes in MD patients, (3) the association of MD with HLA-types and genetic polymorphisms and (4) the positive corticosteroid-responsiveness detected in some MD patients. However, all studies have been small and lack positive replication. Studies concerning antibodies, HLA types and genetic polymorphisms have produced conflicting results and no single antibody, HLA type or polymorphism has been found in all or even in a significant subpopulation of MD patients. No convincing basic research evidence of autoimmunity in MD exists hence the field needs further study.     

Diagnostic value of refixation saccades in the Video Head Impulse Test (vHIT) in unilateral definite Meniere’s disease

Abstract

Introduction: We currently interpret the video Head Impulse Test (vHIT) results mainly based on the gain value.

Aim: The purpose of this study is to evaluate vHIT results for both gain and re-fixation saccades on unilateral definite Meniere's disease (MD) subjects in comparison with normal healthy subjects.

Materials and Methods: Forty unilateral definite MD subjects and age-matched healthy subjects were recruited. Pure tone audiometry, the caloric test, and the vHIT test were performed on MD subjects. The vHIT test was performed on healthy subjects.

Results: The velocity regression gain (VRG) of the affected ear in patients with MD is significantly lower than of those in healthy subjects. The total percentage of refixation saccades is significantly higher in patients with MD when compared to healthy subjects. VRG values were not well-correlated with the percentage of refixation saccades. VRG asymmetry values are also not well-correlated with the percentage of unilateral canal weakness. A moderately stronger correlation between the percentage of refixation saccades and percentage of unilateral canal weakness, with an r² of 0.474.

Conclusions: The present study suggests that while VRGs are still a diagnostic parameter of detecting MD, the presence of refixation saccades can also have diagnostic value, especially with normal VRGs, in detecting MD.     

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.     

Concomitant diseases and their effect on disease prognosis in Meniere’s disease: diabetes mellitus identified as a negative prognostic factor

Objective: To study comorbidities and their effect on the disease progression in Meniere’s disease (MD).

Methods: Retrospective study on 350 definite MD patients diagnosed according to AAO-HNS 1995 criteria using hospital records and postal questionnaire.

Results: The prevalence of migraine, hypothyroidism, allergies, coronary heart disease and autoimmune diseases was more common in MD patients than reported in the general population of Finland. Diabetes mellitus was associated with both more severe hearing impairment (p?=?.033) and more frequent vertigo (p?=?.028) in MD patients. The number of concomitant diseases was associated with more frequent vertigo (p?=?.021).

Conclusions: A patient’s concomitant diseases, especially diabetes, should be treated effectively because they might affect the progression of MD. Further studies on the effects of concomitant diseases on MD prognosis are needed.     

Inter-frequency amplitude ratio of oVEMP for differentiating Meniere’s disease from BPPV: clinical validation using a double-blind approach

Objective: The study aimed at examining the usefulness of inter-frequency amplitude ratio (IFAR) of ocular vestibular evoked myogenic potential (oVEMP) in identifying Meniere’s disease (MD) and differentiating it from benign paroxysmal positional vertigo (BPPV).

Design: A case-control design was used with a double blind approach. Phase 1 included 70 healthy individuals and 36 individuals each with MD and BPPV and Phase 2 included 20 individuals each with MD and BPPV. The age range of the participants in both phases was 15–50 years. All participants underwent oVEMP testing using 500 and 1000?Hz tone bursts and IFAR was obtained.

Results: The results in phase 1 revealed significantly higher IFARs in Meniere’s disease than BPPV and healthy individuals (p?Conclusions: IFAR of oVEMP appears highly sensitive and specific parameter for identifying MD and a clinically valid tool for differentiating MD from BPPV.     

Preliminary results of a new delivery system for gentamicin to the inner ear in patients with Meniere’s disease

Intratympanic gentamicin therapy has gained some clinical popularity in the treatment of vertigo associated with Meniere’s disease. This therapeutic modality offers some advantages over traditional surgical treatment. The vestibulotoxic effect of gentamicin is well documented, but there is no general agreement about the dose needed to control vertigo attacks without affecting hearing. In the current preliminary study 27 patients with Meniere’s disease refractory to medical management were treated by small doses of gentamicin delivered via microcatheter in the round window niche and administered by an electronic micropump. The patients received a total dose of 0.24–90 mg. The effect on vestibular symptoms resulted in the cessation of vertigo in the 22 patients, control of drop attacks in 4 of 6, and release of aural pressure and fullness in 2 of 4. Significant hearing loss (anacusis) occurred in six patients, slightly related to the flow rate in the pump setting. Different explanations for the loss of hearing are presented. The new delivery system for gentamicin appears to be effective in controlling vertigo, but with an unacceptable negative effect on hearing. The effectiveness and the safety of this new delivery system must be investigated further in controlled studies. However, it opens up the possibility of future novel ways of treating inner ear diseases, such as sudden deafness and tinnitus, as well as for the protection, repair, and regeneration of inner ear sensory cell function in damage due to aging, noise, toxic substances, immune reactions, etc. Received: 8 September 1999 / Accepted in revised form: 29 May 2000     

Intratympanic gentamicin treatment ‘as needed’ for Meniere’s disease. Long-term analysis using the Kaplan–Meier method

In recent years, several titration or on-demand protocols using low-dose repeated intratympanic (IT) gentamicin injections have been adopted for the vertigo control in unilateral medical refractory Menière’s disease (MD). Because of the frequent recurrence and the need to treat the patients several times, it is difficult to strictly follow the 1995 AAO-HNS criteria to classify the results. The Kaplan–Meier analysis provides an effective and simpler method to address these concerns. We report the results of a long-term study (7 years) on a large population of MD patients (174) treated with on-request low-dose delayed IT gentamicin injections analysed using the Kaplan–Meier survival method. Effective vertigo control was obtained with a single injection in 40.2 % of the patients (excellent responders) and with repeated injections (2–9) in 43.7 % of the patients (moderate responders). Only six patients (3.5 %) needed to be submitted to vestibular neurectomy because of the persistence of vertigo attacks (non-responders). A subgroup of 22 patients (12.6 %) reporting a late recurrence of vertigo attacks after an initial vertigo-free interval lasting more than 2 years (short-term responders) were successfully treated with a further cycle of injections. In no cases, we observed significant signs of cochlear or vestibular toxicity. Kaplan–Meier survival analysis provided an excellent method for reporting treatment success or failure in patients followed for variable length of time with our kind of protocol.     

Impacts of different methylprednisolone administration routes in patients with sudden hearing loss or Meniere’s disease

BackgroundEvidence suggests that glucocorticoids are important in the treatment of sudden hearing loss (SHL) and Meniere’s disease (MD). However, different glucocorticoid administration methods may have a significant impact on treatment outcomes.ObjectiveThis study aimed to investigate effects of different glucocorticoid administration methods on sudden hearing loss and Meniere’s disease.MethodsIn this study, glucocorticoids were administered orally in 18 patients, by retroauricular injection in 15 patients and by intratympanic injection in 15 patients. White blood cell (WBC) count, serum K⁺, fasting plasma glucose (FPG), body temperature, heart rate and blood pressure were used to evaluate effects of glucocorticoids on patients with hearing loss. Visual analog scale (VAS) of pain and sleep disorders were also surveyed, and pure tone audiometry (PTA) results were compared among groups to evaluate efficacy of different glucocorticoids administration methods.ResultWBC count, heart rate and blood pressure were higher in patients taking oral glucocorticoids, while body temperature, serum K⁺ and FPG levels did not change in all three groups. However, patients who received intratympanic injection of glucocorticoids experienced more pain, while those taking oral glucocorticoids reported more sleep impairment. Treatment efficacy on hearing loss was not significantly different among the three groups.ConclusionThese findings suggest that systemic glucocorticoid administration can result in greater whole body responses than local administration, but with similar hearing treatment efficacy.     

Ramsay-Hunt’s综合征合并多脑神经损伤1例报告并文献复习

目的探讨Ramsay-Hunt’s综合征合并多脑神经损伤的病因、发病机制和临床特点,及引起多脑神经损伤疾病的临床特点。方法报道1例Ramsay-Hunt’s综合征及引起多脑神经损伤的临床资料,结合相关文献进行复习。结果通过治疗,患者面瘫有明显好转,水疱干涸,结痂。喉镜检查双侧软腭活动对称,双侧声带活动正常。结论多脑神经的损伤使Ramsay-Hunt’s综合征患者的临床表现复杂多样,为临床医师的诊断带来了较大的难度。     

Volumetric measurements of the inner ear in patients with Meniere’s disease using three-dimensional magnetic resonance imaging

Conclusion: This study described the lateralities of axial length of inner ear (ALIE), of the volume of inner ear (VIE) and age-related differences of the volume of inner ear components in patients with chronic rhinosinusitis (CRS). Age-related differences were found in ALIE and the positive correlation in ALIE and the volume of the cochlea (VC) of the affected ear in patients with Meniere’s disease (MD). Objective: To identify side or sex-related differences in the ALIE, the length of the spiral canal of cochlea (LSCC), and the volume of components of the inner ear in MD and CRS.

Methods: Thirty-two with unilateral MD and 14 with CRS were included. Images were acquired with a 3.0-tesla unit using SPACE sequences. The ALIE was measured and the VIE, VC, the volume of the vestibule (VV), and of the semi-circular canals (VSC) were also measured.

Results: In CRS, ALIE of the right ear in males was significantly longer than in females. Patients younger than 60 years old with CRS had a significantly larger VIE, VC, and VSC than older than 60. In MD, the ALIE in older than 60 was longer than below 60.     

Vestibular Drop Attacks and Meniere’s Disease as Results of Otolithic Membrane Damage—A Numerical Model

Meniere’s disease (MD) is a condition of the inner ear with symptoms affecting both vestibular and hearing functions. Some patients with MD experience vestibular drop attacks (VDAs), which are violent falls caused by spurious vestibular signals from the utricle and/or saccule. Recent surgical work has shown that patients who experience VDAs also show disrupted utricular otolithic membranes. The objective of this study is to determine if otolithic membrane damage alone is sufficient to induce spurious vestibular signals, thus potentially eliciting VDAs and the vestibular dysfunction seen in patients with MD. We use a previously developed numerical model to describe the nonlinear dynamics of an array of active, elastically coupled hair cells. We then reduce the coupling strength of a selected region of the membrane to model the effects of tissue damage. As we reduce the coupling strength, we observe large and abrupt spikes in hair bundle position. As bundle displacements from the equilibrium position have been shown to lead to depolarization of the hair-cell soma and hence trigger neural activity, this spontaneous activity could elicit false detection of a vestibular signal. The results of this numerical model suggest that otolithic membrane damage alone may be sufficient to induce VDAs and the vestibular dysfunction seen in patients with MD. Future experimental work is needed to confirm these results in vitro.

     

A case against spiral ligament atrophy as a cause of Ménière's disease

The etiology of Ménière's disease and endolymphatic hydrops has been very difficult to determine. In order to develop rational therapies for patients with Ménière's disease the etiology needs to be established. In the current study an examination was made of the spiral ligament in temporal bones from individuals with Ménière's and endolymphatic hydrops and normal control temporal bones. No evidence of atrophy in either the length or width or the fibrocyte cell density was seen in the temporal bones with Ménière's disease. The role of the spiral ligament in the development of endolymphatic hydrops is still unclear.     

Kimura’s Disease — report of a case masquerading as a parotid tumor

Kimura’s Disease is a part of an ill- defined spectrum of vascular lesions of the subcutis. It is a benign chronic inflammatory condition producing subcutaneous tumour like nodules chiefly in the head and neck region with predilection for periauricular areas. It is characterised histologically by dense fibrosis, lymphoid infiltration, vascular proliferation and eosinophilia. The condition has a preponderance in the for eastern countries. It is extremely uncommon in Indian subcontinent. There has been no reported case from the Indian subcontinent in the English Literature. We report such a rare case in which the condition presents as parotid masses. We also present its CT, MRI & histopathology features and a brief review of literature.     

Radiological findings of the cochlear aqueduct in patients with Meniere’s disease using high-resolution CT and high-resolution MRI

The objective of the present study was to evaluate the cochlear aqueduct (CA) in Meniere’s disease (MD) and to disclose radiological differences of CA between MD and non-MD patients by means of high-resolution computed tomography (HRCT) and high-resolution magnetic resonance imaging (HRMRI). Radiological data of 86 ears of MD patients which were separated into 52 ears of diseased side group (MD-D group) and 34 ears of contralateral non-affected side group of unilateral MD (MD-ND group), 27 ears of patients with sensorineural hearing loss (SNHL group) and 56 ears of patients with somatoform dizziness and normal hearing (control group) were analyzed retrospectively. The bony type of CA, the bony length of CA, and the bony width of CA medial orifice was measured in HRCT. The visibility of CA in HRMRI was scored. Fluid length in CA and fluid width in medial orifice were measured in HRMRI. Data were compared between MD-D, MD-ND, SNHL, and control group. There were no significant differences in the bony type of CA, bony length of CA, bony width of CA medial orifice, and fluid width of CA medial orifice between MD-D, MD-ND, SNHL and control group (p > 0.05). However, CA fluid length of MD-D (5.13 ± 1.88 mm) and of MD-ND group (5.44 ± 1.81 mm) was significantly shorter than fluid length of SNHL (6.90 ± 1.55 mm) (p < 0.001, p = 0.001) and of control group (7.43 ± 1.24 mm) (p < 0.001, p < 0.001). The ratio between CA fluid length and CA bony length was the smallest in MD-D group (0.403; p = 0.009). CA bony dimensions of affected ears of MD are normal, but CA fluid length is decreased.     

Magnetic resonance-based volumetric measurement of the endolymphatic space in patients with Meniere’s disease and other endolymphatic hydrops-related diseases

ObjectiveTo employ magnetic resonance imaging (MRI) to measure the volume of the inner ear endolymphatic space (ELS) in patients with acute low-tone sensorineural hearing loss (ALHL), sudden deafness (SD), cochlear Meniere’s disease (cMD), and unilateral MD (uMD) compared with control subjects (CS) with chronic rhinosinusitis.MethodsForty-one patients with ALHL, 82 with SD, 48 with cMD, 72 with uMD, and 47 CS participated in the study. With the exception of all uMD patients, none of the subjects had vertigo. Images of the inner ear fluid space, positive perilymph signal, and positive endolymph signal were acquired using a 3-T MRI scanner. Three-dimensional images were reconstructed semi-automatically by using anatomical and tissue information to fuse the inner ear fluid space images and the ELS images.ResultsThe cochlear ELS/total fluid space (TFS) volume ratio was 10.2 ± 6.7% (mean ± standard deviation) in the CS group, 12.1 ± 5.7% in ALHL patients, 15.2 ± 8.7% in SD patients, 18.1 ± 8.2% in cMD patients, and 21.9 ± 16.4% in uMD patients. The vestibular ELS/TFS volume ratio was 17.7 ± 10.2% in the CS group, 18.9 ± 8.3% in ALHL patients, 19.9 ± 11.3% in SD patients, 22.5 ± 13.7% in cMD patients, and 35.7 ± 24.1% in uMD patients. The cochlear ELS/TFS volume ratio in patients with uMD was similar to that in the cMD group and significantly higher than that in the CS, ALHL, and SD groups (CS = ALHL < SD < cMD = uMD: p < 0.05 for CS vs. SD and p < 0.01 for CS vs. cMD). The vestibular ELS/TFS volume ratio in patients with uMD was significantly higher than that in the CS and all other patient groups (CS = ALHL = SD = cMD < uMD: p < 0.01 for uMD vs. all other groups).ConclusionThe cochlear ELS volume of patients with MD and other endolymphatic hydrops-related diseases differed from that of CS. Our results suggest that ALHL may not be caused by endolymphatic hydrops. We confirmed the presence of extended ELS in patients with SD.     

Bell’s palsy workup:Does audiometry add value?

Objective:To investigate if routine audiometry in Bell’s palsy patients has prognostic value.Methods:Retrospective case review was conducted on all Bell’s palsy patients(n紏191)seen at the tertiary otolaryngology specialist outpatient clinic from 2015 to 2017.Correlation of ipsilesional audiometric thresholds with patients’time-to-recovery and initial clinical severity(measured by House-Brackmann(HB)scoring)were used for the prognostic outcome measure.Audiometry results were analyzed using three contiguous frequency pure-tone average(1kHz,2kHz,4kHz).Statistical analysis was done via Stata(v13.1),significance tests were 2-sided at 5%significance level.Results:There was no significant difference between audiometric thresholds between the ipsilesional ear and the contralateral ear(p=0.87).Time-to-recovery was significantly longer for patients with severe initial presentation as compared to mild and moderate severity(p<0.01).There was no correlation found between the audiometry results and HB score at presentation(p=0.39).There was no correlation found between ipsilesional audiometric thresholds and time-to-recovery(p=0.58).Conclusion:Our study suggests that routine audiometry has limited prognostic value in Bell’s palsy patients.     

Effects of posterior tympanotomy with steroids at round window on hearing recovery after revision surgery for intractable Meniere’s disease

Conclusions: At the second postoperative year, there were no significant differences between results for vertigo and hearing after endolymphatic sac drainage with steroid instillation surgery (EDSS) and EDSS with posterior tympanotomy with steroids at the round window (EDRW). In particular, as regards hearing recovery to the preoperative level, the periods after EDRW were shorter than those after the second EDSS. Objectives: Patients sometimes faces recurrent problems years after EDSS due to endolymphatic sac closure and/or disease progression. In the present study, we examined the effects of EDRW on vertigo and hearing after revision surgery for intractable relapsed Meniere’s disease. Methods: Sixteen patients with Meniere’s disease had revision surgery due to intractable recurrence of disease, and were followed up regularly at least for 2 years. As revision surgery, EDSS was performed repeated in eight cases and EDRW was performed in the other eight. There were no significant differences between the patients’ backgrounds in the two groups. Results: Periods of hearing recovery to the preoperative level were 11.5 ± 4.4 months after the first EDSS, although it took 16.4 ± 2.6 months longer after revision surgery with the second EDSS (p = 0.038 < 0.05: first EDSS vs second EDSS) and was 10.0 ± 3.3 months shorter after revision surgery with EDRW (p = 0.010 < 0.05: second EDSS vs EDRW).