Asymmetry and tuning shift of the cervical vestibular evoked myogenic potential indicate saccular dysfunction in idiopathic normal pressure hydrocephalus.

OBJECTIVE: The purpose of this study was to investigate the effects of excessive cerebrospinal fluid (CSF) retention on the peripheral vestibular function and the inner ear fluid in patients with idiopathic normal pressure hydrocephalus (iNPH). METHODS: In 25 patients with iNPH (14 females, age 65-88 years), cervical vestibular evoked myogenic potential (cVEMP) was measured before the spinal tap test. The asymmetry ratios (ARs) and tuning properties in 500 Hz and 1,000 Hz short-tone burst stimuli of cVEMP were evaluated. Furthermore, cVEMP was measured in an age-matched control group of 12 non-iNPH patients. RESULTS: Seven (28%) iNPH patients exhibited a cVEMP asymmetry (AR > 33%). cVEMP tuning was significantly shifted to a higher frequency in the iNPH group than in the age-matched control group. CONCLUSIONS: One-fourth of patients with iNPH had obvious saccular dysfunction. A high rate of a shift in cVEMP tuning in the iNPH group indicated that excessive CSF accumulation propagated to the endolymph and perilymph. SIGNIFICANCE: Saccular dysfunction might be one of the possible causes of imbalance in iNPH, and the shift in cVEMP tuning may be a determining factor in the diagnosis and treatment strategy.

Emissões otoacústicas evocadas na doença de Ménière / Otoacoustic emissions evoked in Ménière's disease

RESUMO Objetivo Verificar as respostas das emissões otoacústicas (EOA) evocadas por estímulo transiente e produto de distorção em indivíduos com doença de Ménière. Métodos Estudo transversal com casuística composta por 60 indivíduos de 19 a 75 anos de idade, distribuídos em dois grupos: grupo estudo, com 32 indivíduos com diagnóstico médico de doença de Ménière, sem outros riscos, e grupo controle formado por 28 indivíduos com perda coclear, sem doença de Ménière, pareado por idade e gênero ao grupo estudo. Critério de elegibilidade: curva tipo A, sem perda condutiva ou mista ou suspeita de alteração retrococlear. A avaliação audiológica foi composta por anamnese, inspeção do meato acústico externo, audiometria tonal limiar, logoaudiometria, medidas de imitância acústica e emissões otoacústicas evocadas por estímulo transiente e produto de distorção. Resultados Os indivíduos com Ménière apresentaram maior ocorrência de perda unilateral, zumbido pitch grave, vertigem e plenitude auricular em relação ao controle. Nesses indivíduos, houve maior incompatibilidade entre os resultados das EOA e da audiometria tonal: nas perdas unilaterais, observaram-se alterações nas EOA nas orelhas com limiares auditivos normais do lado contralateral, caracterizando disfunções cocleares. Nas orelhas com perda coclear, houve presença de EOAT (por estímulo transiente) e ausência de EOAPD (produto de distorção), contrapondo-se ao grupo controle, que apresentou ausência de EOAT e de EOAPD, como o esperado em perdas cocleares de outras etiologias. Conclusão A pesquisa das emissões na doença de Ménière identificou disfunção coclear na orelha contralateral nos casos unilaterais e presença de EOAT com ausência de EOAPD nas orelhas com perda auditiva, diferenciando-se das perdas cocleares de outras etiologias.

ABSTRACT Purpose To verify the responses of Evoked Otoacoustic Emissions by transient stimulus and distortion product in individuals with Ménière's Disease. Methods Cross-sectional study with a sample composed of 60 individuals, aged 19 to 75 years, divided into two groups: study group, with 32 individuals with a medical diagnosis of Ménière's disease, without other risks and a control group formed by 28 individuals with cochlear loss without Meniere's disease, age and sex matched to the study group. Eligibility criteria: type A curve, without conductive or mixed loss or suspected retrocochlear alteration. The audiological evaluation consisted of anamnesis, inspection of the external acoustic meatus, pure tone audiometry, logoaudiometry, measures of acoustic immittance and transient evoked otoacoustic emissions and distortion product. Results Individuals with Ménière's disease had a higher occurrence of unilateral hearing loss, low pitch tinnitus, vertigo and ear fullness in relation to the control. In these individuals, there was greater incompatibility between the results of OAE and pure tone audiometry: in unilateral hearing loss, alterations in OAE were observed in ears with normal hearing thresholds on the contralateral side, characterizing cochlear dysfunctions. In the ears with cochlear loss, there was the presence of TEOAE and absence of DPOAE, in contrast to the control group, which showed the absence of TEOAE and DPOAE, as expected in cochlear losses of other etiologies. Conclusion The investigation of emissions in Ménière's disease identified cochlear dysfunction in the contralateral ear in unilateral cases and the presence of TOAE with absence of DPOAE in ears with hearing loss, differentiating from cochlear losses of other etiologies.

Quantification of endolymphatic hydrops and its correlation with Meniere's disease clinical features.

OBJECTIVE: We aimed to quantitatively evaluate the degree of endolymphatic hydrops and its correlation with the clinical features of Meniere's disease. METHODS: We retrospectively collected data from patients with Meniere's disease who underwent gadolinium-enhanced magnetic resonance imaging (MRI) at our department from January 2018 to December 2019. Mimics software was used to perform three-dimensional modelling of the labyrinth, and volume information was obtained to calculate the endolymphatic hydrops index (EHI). A correlation analysis was conducted with data from pure tone audiometry, electrocochleography (EchoG), vestibular myogenic-evoked potential (VEMP) testing, caloric testing and video head impulse testing (vHIT). A two-dimensional method was also employed to calculate the hydrops ratio (HR) of cochlea and vestibule. The test-retest reliability of EHI/HR from different operators was evaluated. RESULTS: A total of 23 affected ears were examined, and the EHI was significantly correlated with Meniere's disease stage, low-frequency hearing threshold, EchoG summating potential/action potential ratio (-SP/AP) and VEMP binaural asymmetry ratio, but no significant correlation was observed between EHI and the caloric test or vHIT. The Intraclass correlation coefficient (ICC) of EHI data calculated by two otologists was 0.946 (p < .001). And the ICC of cochlea and vestibule HR were 0.844 and 0.832 (p < .001). CONCLUSION: Mimics software can be used to quantitatively evaluate the degree of endolymphatic hydrops and have shown higher test-retest reliability than traditional two-dimensional evaluation method. Endolymphatic hydrops correlates with clinical data, such as Meniere's disease stage, low-frequency hearing threshold, EchoG and VEMP asymmetry ratio.

Evaluation of the blood-perilymph barrier in ears with endolymphatic hydrops.

BACKGROUND: Otological diseases including Meniere's disease (MD) involve endolymphatic hydrops (EH), which can be visualized by magnetic resonance imaging (MRI) with gadolinium contrast agents, but the temporal changes of contrast in the inner ear have not been evaluated. OBJECTIVES: We investigated the permeability of the blood-perilymph barrier (BPB) in ears with EH to evaluate the severity of the inner ear disturbances. MATERIALS AND METHODS: The study included 32 ears from 16 patients with EH or related diseases who underwent MRI. The permeability of the BPB was assessed by the signal-intensity ratio (SIR) at four-time points: before and at 10 min, 4 h, and 24 h after administration of gadolinium for assessing EH. RESULTS: Cochlear EH was found in 25 of the 32 ears, and vestibular EH in 11. The rate of EH was significantly higher in symptomatic ears; however, the existence of EH was not related to SIR values. Nevertheless, SIR values in the basal turn were significantly higher 4 and 24 h after injection of gadolinium in patients aged ≥50 years. CONCLUSION AND SIGNIFICANCE: Higher SIR values observed in older patients with EH indicate severe disturbances of the BPB in the cochlea, which may account for intractable inner ear disturbances in older patients.

Altered mapping of sound frequency to cochlear place in ears with endolymphatic hydrops provide insight into the pitch anomaly of diplacusis.

A fundamental property of mammalian hearing is the conversion of sound pressure into a frequency-specific place of maximum vibration along the cochlear length, thereby creating a tonotopic map. The tonotopic map makes possible systematic frequency tuning across auditory-nerve fibers, which enables the brain to use pitch to separate sounds from different environmental sources and process the speech and music that connects us to people and the world. Sometimes a tone has a different pitch in the left and right ears, a perceptual anomaly known as diplacusis. Diplacusis has been attributed to a change in the cochlear frequency-place map, but the hypothesized abnormal cochlear map has never been demonstrated. Here we assess cochlear frequency-place maps in guinea-pig ears with experimentally-induced endolymphatic hydrops, a hallmark of Ménière's disease. Our findings are consistent with the hypothesis that diplacusis is due to an altered cochlear map. Map changes can lead to altered pitch, but the size of the pitch change is also affected by neural synchrony. Our data show that the cochlear frequency-place map is not fixed but can be altered by endolymphatic hydrops. Map changes should be considered in assessing hearing pathologies and treatments.

Intracranial Distribution of Intravenously Administered Gadolinium-based Contrast Agent over a Period of 24 Hours: Evaluation with 3D-real IR Imaging and MR Fingerprinting.

PURPOSE: To evaluate the feasibility for the detection of slight contrast effects after intravenous administration of single dose gadolinium-based contrast agent (IV-SD-GBCA), the time course of the GBCA distribution up to 24 h was examined in various fluid spaces and brain parenchyma using 3D-real IR imaging and MR fingerprinting (MRF). METHODS: Twenty-four patients with a suspicion of endolymphatic hydrops were scanned at pre-administration and at 10 min, 4 and 24 h post-IV-SD-GBCA. 3D-real IR images and MRF at the level of the internal auditory canal were obtained. The signal intensity on the 3D-real IR image of the cerebrospinal fluid (CSF) in the cerebellopontine angle cistern (CPA), Sylvian fissure (Syl), lateral ventricle (LV), and cochlear perilymph (CPL) was measured. The T1 and T2 values of cerebellar gray (GM) and white matter (WM) were measured using MRF. Each averaged value at the various time points was compared using an analysis of variance. RESULTS: The signal intensity on the 3D-real IR image in each CSF region peaked at 4 h, and was decreased significantly by 24 h (P< 0.05). All patients had a maximum signal intensity at 4 h in the CPA, and Syl. The mean CPL signal intensity peaked at 4 h and decreased significantly by 24 h (P < 0.05). All patients but two had a maximum signal intensity at 4 h. Regarding the T1 value in the cerebellar WM and GM, the T1 value at 10 min post-IV-GBCA was significantly decreased compared to the pre-contrast scan, but no significant difference was observed at the other time points. There was no significant change in T2 in the gray or white matter at any of the time points. CONCLUSION: Time course of GBCA after IV-SD-GBCA could be evaluated by 3D-real IR imaging in CSF spaces and in the brain by MRF.

Assessment of the Progression of Vertical Semicircular Canal Dysfunction and Increased Vestibular Endolymphatic Hydrops in Patients With Early-Stage Ménière Disease.

Importance: Vertical semicircular canals and endolymphatic hydrops play important roles in the pathophysiological mechanisms of Ménière disease. However, their characteristics and associations with disease progression during medical treatment have not been determined. Objective: To examine the function of both the horizontal and vertical semicircular canals in patients with Ménière disease and to evaluate the change in endolymphatic hydrops volume during medical treatment, including treatment with diuretic therapy, over a 2-year period. Design, Setting, and Participants: This prospective longitudinal observational cohort study included 55 patients with definite unilateral Ménière disease and was performed in a tertiary care hospital in Japan. Participants were enrolled between April 1, 2017, and January 31, 2018, and those with vestibular migraine were excluded. All participants received education regarding diet and lifestyle modifications and treatment with betahistine mesylate (36 mg daily) and/or an osmotic diuretic (42-63 mg daily). Patients were followed up for vertigo and hearing evaluations at least once per month for more than 12 months and were instructed to record episodes of vertigo in a self-check diary. Audiometry was performed monthly, video head impulse testing and caloric testing were performed every 4 months, and magnetic resonance imaging was conducted annually. Data were analyzed from May 15, 2017, to January 31, 2020. Main Outcomes and Measures: Neurootological testing to evaluate vestibuloocular reflex gain over time, magnetic resonance imaging to evaluate the change in endolymphatic hydrops volume over time, and monthly vertigo and hearing evaluations for more than 12 months. Results: Among 55 participants with definite Ménière disease, 32 patients (58.2%) were female, and the mean (SD) age was 59.0 (15.1) years. The median disease duration was 2 years (interquartile range, 0-4 years), with 43 patients (78.2%) having an early stage (ie, disease duration ≤4 years) of Ménière disease. Over the 2-year study period, the vestibuloocular reflex gain decreased from 0.76 to 0.56 in the superior semicircular canals, for a difference of 0.20 (95% CI, 0.14-0.26) and from 0.68 to 0.50 in the posterior semicircular canals, for a difference of 0.18 (95% CI, 0.14-0.22). The maximum slow-phase velocity and vestibuloocular reflex gain in the horizontal semicircular canals were maintained. The volume ratio of vestibular endolymphatic hydrops increased from 19.7% to 23.3%, for a difference of 3.6% (95% CI, 1.4%-5.8%). The frequency of vertiginous episodes decreased, and the hearing level over the study period worsened from 40.9 dB to 44.5 dB, for a difference of 3.5 dB (95% CI, 0.7-6.4 dB). Conclusions and Relevance: In this study, during a 2-year period of medical treatment among patients with Ménière disease, vestibuloocular reflex gain decreased in the vertical semicircular canals but was maintained in the horizontal semicircular canals; the endolymphatic hydrops volume ratio increased, and the frequency of vertiginous episodes decreased. These findings describe the pathological progression of chronic Ménière disease and expand the understanding of its pathophysiological characteristics during the early stage of disease.

Comparison of furosemide-loading cervical vestibular-evoked myogenic potentials with magnetic resonance imaging for the evaluation of endolymphatic hydrops.

BACKGROUND: Furosemide-loading cervical vestibular-evoked myogenic potential (FVEMP), in which vestibular function is improved via diuretics-induced dehydration, can be used to estimate the presence of endolymphatic hydrops, one characteristic of Menière's disease. Inner ear magnetic resonance imaging (MRI) can also reveal endolymphatic hydrops.Aims/Objective: This study aimed to compare and confirm the usefulness of these two examination methods for the diagnosis of Menière's disease. METHODS: Twenty patients with definite unilateral Menière's disease were included. All subjects underwent both, FVEMP and inner ear MRI examinations. The results were then compared statistically between the affected and contralateral ears and among the methods. RESULTS: FVEMP and inner ear MRI of the cochlea, saccules, and utricles yielded positive results indicative of endolymphatic hydrops in 55.0%, 60.0%, 45.0%, and 45.0% of cases, respectively. The results of FVEMP were more consistent with those of the cochlea (κ = 0.8) than with those of the saccules or utricles by inner ear MRI (κ = 0.6). CONCLUSIONS: FVEMP appears to be a good and minimally invasive option for evaluating endolymphatic hydrops. However, the combination of FVEMP and inner ear MRI may yield even more accurate evaluations of endolymphatic hydrops.

Live imaging and functional changes of the inner ear in an animal model of Meniere's disease.

The symptoms of Meniere's disease (MD) are generally considered to be related to endolymphatic hydrops (EH). There are many recent reports supporting the possibility that vasopressin (VP) is closely linked to the formation of EH in Meniere's disease. Based on this, we developed a clinically relevant animal model of Meniere's disease in which a VP type 2 receptor agonist was administered after electrocauterization of the endolymphatic sac. We report live imaging of the internal structure, and functional changes of the inner ear after electrocauterization of the endolymphatic sac and administration of a VP type 2 receptor agonist. In this model, the development of EH was visualized in vivo using optical coherence tomography, there was no rupture of Reissner's membrane, and low-tone hearing loss and vertiginous attacks were observed. This study suggested that acute attacks are caused by the abrupt development of EH. This is the first report of live imaging of the development of EH induced by the administration of a VP type 2 receptor agonist.

A Revised Surgical Approach to Induce Endolymphatic Hydrops in the Guinea Pig.

Endolymphatic hydrops is an enlargement of scala media that is most often associated with Meniere's disease, though the pathophysiologic mechanism(s) remain unclear. In order to adequately study the attributes of endolymphatic hydrops, such as the origins of low-frequency hearing loss, a reliable model is needed. The guinea pig is a good model because it hears in the low-frequency regions that are putatively affected by endolymphatic hydrops. Previous research has demonstrated that endolymphatic hydrops can be induced surgically via intradural or extradural approaches that involve drilling on the endolymphatic duct and sac. However, whether it was possible to create an endolymphatic hydrops model using an extradural approach that avoided dangerous drilling on the endolymphatic duct and sac was unknown. The objective of this study was to demonstrate a revised extradural approach to induce experimental endolymphatic hydrops at 30 days post-operatively by obliterating the endolymphatic sac and injuring the endolymphatic duct with a fine pick. The sample size consisted of seven guinea pigs. Functional measurements of hearing were made and temporal bones were subsequently harvested for histologic analysis. The approach had a success rate of 86% in achieving endolymphatic hydrops. The risk of cerebrospinal fluid leak was minimal. No perioperative deaths or injuries to the posterior semicircular canal occurred in the sample. The presented method demonstrates a safe and reliable way to induce endolymphatic hydrops at a relatively quick time point of 30 days. The clinical implications are that the presented method provides a reliable model to further explore the origins of low-frequency hearing loss that can be associated endolymphatic hydrops.

Contralesional subjective visual horizontal predicts endolymphatic hydrops.

Background: The subjective visual horizontal (SVH) is a test of utricular function that assesses conjugate ocular torsion which is a component of the ocular tilt reaction (OTR). In unilateral destructive peripheral vestibular lesions, the OTR and so the SVH tilt is usually ipsiversive.Aims/objective: Our study aimed to profile the causes of a contraversive SVH tilt in patients with a confirmed unilateral peripheral vestibular deficit.Materials and methods: The clinical records, nystagmus and vestibular investigation characteristics of 52 patients with a unilateral canal paresis (CP) on caloric of ≥30%, a contraversive SVH tilt of ≥4 degrees and at least one pure tone audiometry were retrospectively analysed.Results: The most common diagnosis of patients (n = 39) with a contraversive SVH and ipsilesional CP was endolymphatic hydrops: 35 (67.3%) had Meniere's disease (MD) and 4 (7.7%) had delayed endolymphatic hydrops (DEH). The remaining 13 (25%) of cases had other peripheral aetiologies or an unknown diagnosis. 16 (30.8%) patients had ictal spontaneous nystagmus at the time of SVH or caloric testing.Conclusions and significance: A contraversive SVH with unilateral CP suggests endolymphatic hydrops.

Different findings of morphological changes and functional decline in the vestibule and the semicircular canal in ipsilateral delayed endolymphatic hydrops.

OBJECTIVE: To elucidate the pathophysiological process by analyzing the correlation between morphological and functional changes in patients with delayed endolymphatic hydrops (DEH). METHODS: Twenty-nine patients diagnosed with DEH were enrolled in this retrospective study. All patients were assessed using the caloric test, cervical and ocular vestibular evoked myogenic potentials, and gadolinium-enhanced magnetic resonance imaging (MRI) of the inner ear. RESULTS: According to the MRI, the hydrops localization was categorized as hydrops in the vestibule (saccule and utricle) (14%), hydrops in the vestibule and cochlea (72%), and hydrops in the vestibule, cochlea, and lateral semicircular canal (LSCC) (14%). Vestibular hydrops could definitely be observed as function declined; however, a dysfunction of both the saccule and utricle was not always present when vestibular hydrops was detected with MRI. In the LSCC, a decline in functional tests was not necessarily accompanied by morphological abnormalities. However, dysfunction could definitely be detected when LSCC hydrops was observed with MRI. CONCLUSIONS: Hydrops can be found mainly in the vestibule as shown by MRI. In the vestibule, abnormalities are commonly morphologic rather than functional, whereas in the LSCC a functional deterioration can be detected more frequently than morphological changes. SIGNIFICANCE: Our findings can provide a new perspective on the functional and morphological characteristics of patients with DEH.

Effects of endolymphatic hydrops on acoustic energy absorbance.

Background: The presence of endolymphatic hydrops (EH) may cause hearing loss and affect the transmission of acoustic energy to the inner ear.Objectives: Acoustic energy absorbance on wideband acoustic immittance (WAI) was evaluated, focusing especially on EH in the vestibule.Material and methods: A total of 32 ears from 16 patients who underwent 3-T magnetic resonance imaging (MRI) to evaluate the presence of EH were examined, retrospectively. The degree of EH in the vestibule was classified into three grades (no, mild, and significant), and pure tone audiometry (PTA) and WAI were measured before and after a glycerol drip.Results: Ears with significant EH showed significantly higher hearing levels and air-bone gaps (ABG), and higher absorbance values on WAI at low frequencies (560-600 Hz) than ears with mild or no EH. Changes in absorbance values were observed in some ears without threshold change on PTA.Conclusions and Significance: This study showed significantly higher absorbance values of acoustic energy with significant vestibular EH at low frequencies. Considering ABGs observed in ears with significant EH, the presence of EH in the vestibule might cause an obstacle to the transmission of acoustic energy to the inner ear.

Results in caloric test, video head impulse test and inner ear MRI in patients with Ménière's disease.

OBJECTIVE: Our aim was to elucidate relationships between results from the caloric test (c-test), video Head Impulse Test (vHIT) and inner ear gadolinium-enhanced MRI (ieMRI) in patients with endolymphatic hydrops (EH), especially patients with Ménière's disease (MD). METHODS: We managed 1789 successive patients at the Vertigo/Dizziness Center in Nara Medical University from May 2014 to December 2018. After providing informed consent for vertigo/dizziness examinations, 281 patients were hospitalized to check their inner ear function for proper diagnosis and treatment. Then 76 participants underwent the c-test, vHIT and ieMRI. Among these 76 cases, 20 were diagnosed with MD (20/76; 26.3%) and 56 were non-MD (56/76; 73.7%) according to the 2015 diagnostic guideline of the International Classification of Vestibular Disorders. The MD group included 15 unilateral and 5 bilateral cases. The non-MD group included 22 benign paroxysmal positional vertigo, 10 vestibular neuritis, 8 sudden deafness with vertigo, 6 orthostatic dysregulation, 4 vestibular neuropathy and 6 others. Results in these examinations in the side of an active lesioned inner ear were representative in each peripheral case. RESULTS: Twenty-nine of the 76 patients (38.1%) showed discrepant results between the c-test (outside of normal range) and vHIT (within normal range). Twenty-two of 76 patients (28.9%) had a positive EH sign on ieMRI. The c-test/vHIT discrepancy percentage in MD (14/20; 70.0%) was significantly higher than that in non-MD (15/56; 26.8%) (p=0.00179). The positive EH sign in ieMRI percentage in MD (15/20; 75.0%) was significantly higher than that in non-MD (7/56; 12.5%) (p=0.0015). There was a significant positive relationship between the c-test/vHIT discrepancy and the positive EH sign (p=0.00058) in all 76 cases combined. However, there was no significant relationship between c-test/vHIT discrepancy and positive EH sign (p=0.13) in the 20 MD cases. Considering the 15 unilateral and 5 bilateral MD cases, the c-test/vHIT discrepancy was observed in 14 of the 25 affected ears. Positive signs of vestibular EH herniation into the cupula in the lateral semicircular canal was seen in 14 of the 25 MD ears. There was significant relationship between the c-test/vHIT discrepancy and EH herniation (p=0.0012) in MD ears. CONCLUSION: The present results suggest that patients with MD could have inner ear EH significantly more often than those with non-MD. In cases with MD, a positive EH sign on ieMRI did not always indicate a c-test/vHIT discrepancy; both findings may occur due to herniation of vestibular EH adjacent to the lateral semicircular canal.

A possible mechanism of the formation of endolymphatic hydrops and its associated inner ear disorders.

The pathology of Meniere's disease (MD) is well established to be endolymphatic hydrops. However, the mechanism underlying deafness and vertigo of MD or idiopathic endolymphatic hydrops is still unknown. In order to evaluate the pathogenesis of deafness and vertigo in MD, it seems to be rational to investigate the interrelationship between hydrops and inner ear disorders using animals with experimentally-induced endolymphatic hydrops. In spite of intense efforts by many researchers, the mechanism of vertiginous attack has been unexplained, because animals with experimental hydrops usually did not show vertiginous attack. Recently, there are two reports to succeed to evoke vertiginous attack in animals with experimental hydrops. In the present paper were first surveyed past proposals about underlying mechanism of the development of hydrops and inner ear disorders associated with hydrops, and were discussed the pathogenetic mechanism of vertiginous attack in hydrops. In conclusion, abrupt development of hydrops was thought to play a pivotal role in the onset of vertiginous seizure.

Evolution of Meniere's Disease from MD 1.0, via MD 1.5, to MD 2.0.

Background: Elder Meniere's disease (MD) patients ultimately lose their vestibular function. Objective: This study utilized an inner ear test battery to investigate evolution of MD. Methods: Total 278 elder MD patients aged >65 years were divided into three groups. Ninety-four patients with bilateral MD (188 ears) were assigned to Group A. The remaining 184 patients with unilateral MD were further divided into two groups. Group B consisted of 20 affected ears with normal vestibular function on the opposite ears, while Group C indicated 184 unaffected ears. All patients underwent an inner ear test battery. Results: Inner ear deficits in Group B declined from the cochlea via the saccule, utricle to semicircular canals. In contrast, Groups A and C did not significantly differ in the abnormality rates of cervical vestibular-evoked myogenic potential (cVEMP), ocular VEMP and caloric tests, indicating that Group C (unaffected ears) may partly share the same mechanism like Group A (affected ears), namely aging and hydropic effects. Conclusion and significance: Evolution of MD may progress from unilateral MD (MD 1.0), via unilateral MD coupled with asymptomatic hydrops on opposite ear (MD 1.5), toward bilateral MD (MD 2.0), where the number 1.0-2.0 means the number of clinically affected ears.

Magnetic resonance imaging of the endolymphatic space in patients with acute low-tone sensorineural hearing loss.

OBJECTIVE: The aim of this study was to measure the volume of the endolymphatic space (ELS) and to investigate prognosis in patients with acute low-tone sensorineural hearing loss (ALHL). METHODS: A total of 61 ALHL patients participated; 47 were definite while 14 were probable ALHL cases. The definite ALHL patients were classified into three groups: A, "Cure"; B, "No cure"; and C, "Recurrence." Also, nine patients for whom diagnosis changed from ALHL to cochlear Meniere's disease (cMD) without vertigo (ALHL-cMD group). Images of the inner ear fluid space, positive perilymph, and positive endolymph were acquired using a 3T magnetic resonance scanner. Three-dimensional (3D) images were semi-automatically reconstructed using anatomical and tissue information to fuse the 3D images of the inner ear fluid space with the 3D ELS images. RESULTS: Patients in the no cure group showed a significantly higher ELS/total fluid space (TFS) volume ratio in the affected cochlear region than the patients of the other groups. Additionally, the affected vestibular ELS/TFS volume ratio in the cure group was significantly lower than that in the recurrence group. There were significantly higher cochlear and vestibular ELS/TFS ratios in ALHL-cMD patients than in control subjects. CONCLUSIONS: These results indicate that the cochlear ELS/TFS volume ratio should be considered when investigating the extent of recovery, while the extended ELS in the vestibule should be considered when investigating cases of recurrence. Thus, our study suggests that the severe extended ELS appeared likely to change to cMD and that the prognostic determination of ALHL is possible.

Clinical Features of Ears With Otosclerosis and Endolymphatic Hydrops.

OBJECTIVE: Endolymphatic hydrops (EH) has been reported in ears with otosclerosis. The objective of this study was to investigate the clinical features of ears with otosclerosis and EH on magnetic resonance imaging (MRI) and identify predictors for the presence of EH. STUDY DESIGN: Retrospective study. SETTING: University hospital. MATERIALS AND METHODS: Forty-six ears from 37 patients with otosclerosis were included in the present study. INTERVENTIONS: The subjects were divided into three groups, those with no, mild, or significant EH, based on 3-T MRI with intravenous injection of gadolinium. Hearing levels and the extent of otosclerotic lesions graded based on the computed tomography (CT) findings were compared among the groups. Moreover, to examine the vascular activity of the disease, intraoperative measurements of blood flow were also evaluated. MAIN OUTCOME MEASURES: Imaging, hearing levels, and blood flow values. RESULTS: The overall rate of EH was 58.7% (27 of 46 ears); cochlear EH (52.2%) was more frequent than vestibular EH (26.1%). Average thresholds in ears with significant EH were significantly higher at several frequencies, both on air and bone conduction, than those with no or mild EH. Significant EH was more frequently observed in ears with advanced stages on CT than in those without advanced stages. The values of blood flow in the area anterior to the oval window were higher in some ears with EH than in ears without EH. CONCLUSION: EH was frequently present in ears with otosclerosis, especially those with severe hearing loss or advanced disease on CT.

The Biomechanics of Lesion Formation in Endolymphatic Hydrops: Single and Double Hit Mechanisms.

BACKGROUND: The vestibular membranes of the cochlea and saccule are subject to two simultaneous constraints as they deform in endolymphatic hydrops. Boundary tethers impose a bulge-type constraint during pressure-induced transverse membrane displacement, while inherent elasticity imposes a stretch-type constraint during stress-induced longitudinal membrane distention. OBJECTIVE: The aim of this study is to reconcile the effect of these dual constraints on membrane deformation. It is hypothesized that it is the interaction of these constraints that determines whether a stable membrane configuration can be achieved or progression to endolymphatic hydrops will occur. METHODS: Reissner's membrane was modeled as a flat elastic ribbon that was bound along its lateral edges and subject to trans-mural pressure. The bulge and stretch constraints on membrane deformation were formulated mathematically. A graphic solution of the constraint functions was used to examine the nature of the interaction and determine how pressure and elasticity influence the hydropic process. RESULTS: The graphic analysis shows how bulge and stretch phenomena interact to achieve an equilibrium point that satisfies both physical requirements. Nominal values of pressure and elasticity are projected to result in a stable membrane equilibrium in the precritical zone with the modest isolated increases in either parameter alone compatible with stability. However, a sufficiently large increase in either pressure or elasticity alone can constitute a single hit mechanism to exceed the critical point and destabilize the membrane. Moreover, simultaneous modest increases in both pressure and elasticity, neither of which would be sufficient in its own right, can be additive and constitute a double hit mechanism to destabilize the membranes as well. Finally, extreme values of pressure and elasticity that fail to intersect imply that no solution is feasible and that the affected membranes will fail immediately. CONCLUSIONS: Sufficiently large increases in either endolymphatic pressure or membrane elasticity alone can destabilize the membranes and constitute single hit mechanisms for inducing hydrops. Combined moderate increases in both trans-mural pressure and membrane elasticity can also destabilize the membranes and constitute a double hit mechanism for hydrops induction.

Clinical Features and Endolymphatic Hydrops in Patients With MRI Evidence of Hydrops.

OBJECTIVES:: The purpose of this study was to investigate the correlation between grades of endolymphatic hydrops (ELH) and clinical characteristics and determine the detailed clinical characteristics of Ménière's disease (MD) patients with evidence of hydrops based on magnetic resonance imaging (MRI). METHODS:: One hundred ninety-eight MD patients (396 ears) with MRI evidence of hydrops were included. ELH grades were evaluated using the Nakashima grading standard. Correlations between the extent of ELH and clinical features were evaluated. Detailed clinical characteristics were analyzed to assess the clinical diagnostic criteria. RESULTS:: Of 198 patients, ELH was observed in 100% of cases on the clinically affected side and 8.6% of cases on the asymptomatic side. In addition, 98.5% of ELH was classified as moderate or significant grade. Low-frequency hearing loss was significantly correlated with the extent of both vestibular and cochlear hydrops, whereas the vertigo attack frequency showed no significant correlation with ELH grades. The disease duration of MD with bilateral ELH was longer than that with unilateral ELH. The clinical characteristics were variant and did not completely fit the proposed diagnostic criteria. CONCLUSIONS:: MRI findings have relevance to the clinical severity, to a certain extent, but not vestibular symptoms. The proposed diagnostic criteria based on clinical characteristics may be partially effective; analysis of the detailed clinical characteristics of MD was meaningful. Diagnosis of MD based on both MRI and clinical symptoms could facilitate an early diagnosis.