Correlation of semi-quantitative findings of endolymphatic hydrops in MRI with the audiometric findings in patients with Meniere's disease

Purpose:To investigate the correlation between vestibular hydrops(VH),cochlearhydrops(CH),vestibular aqueduct non-visibility(VANV),and visually increased perilymphatic enhancement(VIPE)with the findings of pure-tone audiometry(PTA)in Meniere’s disease(MD)patients.Methods:In this cross-sectional study,53 ears belonging to 48 patients were divided into two groups and evaluated.In group“MD patients,”there were 24 ears of 19 patients diagnosed with the definite MD(14 patients with unilateral and 5 patients withbilateral involvements).The“control group”consisted of 29 non-symptomatic ears belonging to patients diagnosed with unilateral sudden sensory-neural hearing loss or unilateral schwannoma.All the patients underwent 2 sessions of temporal bone MRI using the same 3T system:an unenhanced axial T1,T2,and 3D-FLAIR MRI,an intravenous gadoliniumenhanced axial T1 fat-sat,and 4 h after the injection,an axial 3D-T2 cube and 3D-FLAIR session.VH,CH,VANV,and VIPE were assessed.Subsequently,the correlation between EH indices and PTA findings(in three frequency domains of low,middle,and high)were evaluated,and the predictive value of MRI was calculated.Results:VH was significantly correlated with the hearing threshold in the low,middle,and highfrequency domains.CH was also correlated with the hearing threshold in the low and middle domains.Contrarily,VIPE was not associated with hearing thresholds,and VANV was only correlated with the hearing threshold in low frequencies.Conclusion:The grade of VH,CH,and VANV were significantly correlated with the hearing thresholds in PTA.

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

Background:Evidence 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.Objective:This study aimed to investigate effects of different glucocorticoid administration methods on sudden hearing loss and Meniere’s disease.Methods:In 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 Kt,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.Result:WBC count,heart rate and blood pressure were higher in patients taking oral glucocorticoids,while body temperature,serum Kt 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.Conclusion:These findings suggest that systemic glucocorticoid administration can result in greater whole body responses than local administration,but with similar hearing treatment efficacy.

Identification of early-stage Meniere’s disease as a cause of unilateral tinnitus

Introduction:Meniere’s disease(MD)is characterized by episodic symptoms,including vertigo,hearing loss,and tinnitus.Objectives:in this study,cases of unilateral tinnitus were investigated for MD.Method:Twenty-two patients who presented with chronic unilateral tinnitus on puretone audiograms showing an average threshold better than 25 dB HL and thresholds in the lower frequencies worse than those in the higher frequencies by more than 10 dB were suspected to have early-stage MD and underwent electrocochleography(ECochG).Patients showing ECochG findings conclusive for MD were compared to a control group of patients presenting with chronic unilateral tinnitus on pure-tone audiograms showing an average threshold better than 25 dB and thresholds in the higher frequencies worse than those in the lower frequencies by more than 10 dB.Results:Eighteen of the 22 patients included in this study showed elevated summating potential amplitude to action potential amplitude ratios in ECochG(suggestive of endolymphatic hydrops due to MD)and were followed up for 2 months;14 of them experienced at least two attacks of vertigo or unsteadiness.In contrast,only one patient in the control group reported two or more attacks of vertigo or unsteadiness in the 2 month observation period.The incidence of this finding in the two groups was significantly different.Conclusion:Patients with early-stage MD can present with only unilateral tinnitus.Thus,the addition of“pure-tone audiograms showing lower-frequency thresholds worse than higher-frequency thresholds”to the probable MD category in the globally.

Study of cochlear hydrops analysis masking procedure in patients with Meniere＇s disease and otologically normal adults

Background The cochlear hydrops analysis masking procedure （CHAMP） is a new diagnostic technique for Meniere＇s disease （MD）. But its value has not been well proven. This study aimed to evaluate the diagnostic value of CHAMP for MD. Methods CHAMP test was taken in three populations using the Auditory Evoked Potential system delivered by Bio-logic Systems Corporation： （1） otologically normal subjects; （2） patients clinically diagnosed with definite MD; （3） patients clinically diagnosed with probable and possible MD. Results According to the comparison between the normal and definite MD group, if the abnormal criterion of CHAMP was defined as latency delay less than 0.3 ms, then the corresponding sensitivity was only 52%. However, if the abnormal criterion was defined as latency delay between 0.6 and 3.8 ms, then a sensitivity of 93% and a specificity of 100% can be achieved. The complex amplitude ratio showed a significant overlap between normal and definite MD group. If the abnormal criterion was defined as a complex amplitude ratio less than 0.95, the corresponding specificity was only 50%. However, if the abnormal cdtedon was defined as less than 0.80, the corresponding sensitivity was 60%, and the specificity was 97%. If the abnormal criterion of CHAMP was defined as latency delay less than 0.6 ms or the complex amplitude ratio less than 0.80, CHAMP result can be obtained in all subjects with good sensitivity and specificity. Conclusions CHAMP can differentiate patients with Meniere＇s disease from otologically normal subjects with high sensitivity and specificity. The recommended cdterion of abnormal CHAMP was a latency delay less than 0.6 ms or a complex amplitude ratio less than 0.80.

Characteristics of the Cochlear Symptoms and Functions in Meniere＇s Disease

Background：Meniere＇s disease is a unique,progressive disease of the inner ear.The complex manifestation presents diagnostic challenges.The cochlear symptoms often present before vertigo and tend to be ignored.This study aimed to analyze the characteristics of cochlear symptoms and functions associated with Meniere＇s disease to investigate the regularity of the development of this disorder.Methods：One-hundred fifteen patients who were diagnosed with definite unilateral Meniere＇s disease at the Hearing and Vestibular Clinic of the Department of Otorhinolaryngology of Beijing Tongren Hospital from August 2013 to November 2015 were recruited in this retrospective study.Initial symptoms,duration from initial symptoms to the diagnosis,hearing thresholds,audiogram patterns,and caloric test results were collected and analyzed for each patient.Data were analyzed using SPSS 13.0 statistical software by Spearman＇s correlation,Kruskal-Wallis H test,Chi-square test,and Fisher＇s exact test.Results：The average hearing threshold of these patients was 45.24 ± 18.40 dB HL.A majority of the patients （55.65%） were in Stage 3.The initial presentation of the disorder in 58 cases （50.43%） comprised only cochlear symptoms without vertigo.A weak,positive correlation was found between the degree of hearing loss and duration of the disease from initial symptoms to the diagnosis （rs =0.288,P =0.002）.Upward-sloping,inverted ＂V,＂ downward-sloping,and flat pattern were the main audiometric patterns observed with a distinctive distribution between stages （P 〈 0.001）.Based on the configurations of audiograms,the audiometric patterns had a weak correlation to the duration （rs =0.269,P =0.004）,and there was a tendency of duration to rising from upward-sloping,inverted ＂V＂,downward-sloping to flat pattern.（H =10.024,P =0.018）.Frequencies of tinnitus in 56 patients （64.4%） were at the lowest points of the audiograms,i.e.,the frequencies of the poorest hearing threshold.The patients at an advanced stage （Stage 3 [56] and Stage 4 [73]） exhibited a significantly higher abnormality of canal paresis than those at the earlier stages （Stage 1 [23] and Stage 2 [42]） （χ^2 =5.973,P=0.015）.Conclusions：Patients with definite Meniere＇s disease always have a moderate to severe sensorineural hearing loss before diagnosis.Cochlear symptoms are the most common initial presentation.With the progression of the duration,the hearing impairment becomes more severe and the distribution of the audiometric pattem is distinctive between stages.

Contralateral delayed endolymphatic hydrops: Clinical features and long term outcome

Background:Contralateral delayed endolymphatic hydrops(CDEH)is a clinical entity characterized by fluctuating low frequency hearing loss and/or vertigo,mimicking Meniere’s disease(MD),that manifests after the appearance of severe non-hydropic hearing loss(NHHL)at the other ear.Objectives:to describe the clinical features and the course of 57 patients affected by CDEH.Method:this is a retrospective study;57 patients affected by CDEH,out of 1065 patients seen in the same period and affected by MD,were subjected to otoscopy,PTA threshold evaluation,impedance testing,ABR,research of positioning nystagmus,vestibular function evaluated by means of bithermal caloric test under video-oculographic,and MRI with gadolinium.Results:the CDEH was definite in 24 cases(42%),probable in 2(4%)and possible in 31(54%).The mean PTA threshold at the hydropic ear was 41 dB.At the last follow-up,40 patients(70%)did not report vertigo or fluctuating hearing loss.Among the 17 patients who still reported symptomatology,11(64%)were affected by fluctuating hearing loss alone,4(23%)reported a subjective worsening of hearing loss and 2(12%)an acute vertigo crisis.Conclusions:contralateral delayed endolymphatic hydrops is a relatively rare form of Meniere disease that manifests more frequently as a definite form or with fluctuating low-frequency hearing loss.The prognosis at a long term follow-up is relatively good in terms of vertigo resolution.Contralateral delayed endolymphatic hydrops rarely determines a severe hearing loss in the better ear.

Cochlear implantation in patients with Meniere’s disease:A systematic review

Background:Meniere’s disease(MD)is an idiopathic disorder of the inner ear,which manifests as cochleo-vestibular dysfunction.Hearing loss will progress to a profound levelin a subset of patients with MD,and vestibular interventions can independently cause loss of hearing.The aim of this study was to systematically review the published literature describing the safety and efficacy of CI in patients with MD.Materials and methods:A systematic literature review was conducted in accordance PRISMA guidelines to identify articles that assessed at least one functional outcome in patients with MD who underwent CI.Demographic information,disease history,MD symptoms,outcomes measures,and complications related to CI were extracted from included studies.Results:In total,17 studies were included,and 182 patients with MD underwent CI.The weighted-mean age was 61.9 years(range 27-85).Study objective and methodology varied,and there was significant heterogeneity in CI outcome measures reported.In total,179(98.3%)of 182 patients reported objective improvements in at least one hearing metric after CI.A total of 69 patients(37.9%)reported vertigo or severe dizziness prior to CI,compared to 22 patients(15.4%)postoperatively.Two studies reported significant reductions in postoperative Tinnitus Handicap Inventory score(THI).Quality of life assessments varied between studies.Complications rates were low with only nine patients(4.9%)reporting a serious CI-related complication.Conclusions:This systematic review evaluated 17 studies describing the safety and efficacy of CI in patients with MD and encountered many challenges due to small sample sizes,and heterogeneity in study design and outcomes measured.Despite these limitations,this study of 182 patients is to the best of our knowledge the largest systematic review evaluating the safety and efficacy of CI in MD.The results of this study support the need for a standardized approach to evaluating outcomes of CI in patients with MD in future studies.