Delayed Endolymphatic Hydrops Research Articles

Improvement of vertigo symptoms and acoustic power absorbance in cases with endolymphatic hydrops.

The pathophysiology and symptoms underlying Meniere's disease (MD) manifest as endolymphatic hydrops (EH), potentially impacting acoustic power absorbance in vestibular EH. The longitudinal effects of middle ear pressure therapy (MEPT) and conservative therapies for EH by magnetic resonance imaging (MRI) and on acoustic power absorbance on wideband acoustic immittance (WAI) were evaluated, and their changes were compared with clinical symptoms. Eleven patients with definite MD or delayed endolymphatic hydrops (DEH), resistant to conservative therapies and who continued MEPT for 1 year, were included. Vertigo scores, hearing levels, acoustic power absorbance on WAI, and degrees of EH on 3-T MRI were evaluated and compared before and after the treatments. One year after the start of MEPT, all cases showed symptomatic improvement in vertigo score; however, the degrees of EH showed no improvements except in one case. In the affected ears with EH, their absorbances on WAI improved, particularly at 1580-1905 or 2400-2953 Hz (p < .05). Alleviation of vestibular symptoms with the therapy of MD was not necessarily associated with improved EH. Vestibular symptoms could be related to the change in the impedance of inner ear pressure, which was proven by the normalization of acoustic power absorbance. Assessments of acoustic power absorbance may provide useful information for physiological conditions and causative factors of vertigo in ears with EH. 4.

Long-term effects of middle ear pressure therapy with the EFET01 device in patients with Ménière’s disease and delayed endolymphatic hydrops in Japan

Background Long-term efficacy of middle ear pressure therapy (MEPT) with the EFET01 device in patients in Japan with definitive Ménière’s disease (MD) and delayed endolymphatic hydrops (DEH) was evaluated. Objective To examine the effects of reducing vertigo attacks and improving hearing of MD and DEH patients by using MEPT with the EFET01 device for two years after treatment. Material and Methods A retrospective study was conducted of 32 MD patients and 2 DEH patients treated by MEPT with the EFET01 device from December 2018 to April 2021. According to Japan Society for Equilibrium Research (JSER) guidelines, patients were investigated for the frequency of vertigo attacks and change in hearing levels during a period of 6 months before to 18–24 months after start of treatment. Results The frequency of vertigo attacks significantly decreased in both MD and DEH patients, and hearing level has remained stable in the majority of our patients after treatment. Conclusion Our study clarified that MEPT with the EFET01 device was effective in controlling vertigo symptoms of MD and DEH. It should be considered a safe option for patients failing medical treatment. Significance The efficacy of MEPT with the EFET01 was shown over a 2-year follow-up period.

The value of 3D quantitative scoring of endolymphatic hydrops in the diagnosis and differential diagnosis of Ménière's disease.

We aimed to explore the application of three-dimensional (3D) quantitative scoring of endolymphatic hydrops (EH) based on gadolinium-contrast MRI in both the diagnosis and differential diagnosis of Ménière's disease (MD). Local threshold method based on signal intensities was used to quantitatively measure volumes, and the degree of EH was scored by 3D quantitative scoring. The receiver-operating characteristic (ROC) curve was used to compare the diagnostic efficacy of this method against conventional evaluation methods. The scores of MD, sudden sensorineural hearing loss (SSNHL), delayed endolymphatic hydrops (DEH), and vestibular migraine (VM) group were compared and correlated with clinical examination results, including pure tone audiometry (PTA), electrocochleogram, and caloric test. The sensitivity and specificity of 3D quantitative scoring were 94.1% and 92.2%, while conventional evaluation methods were 84.3% and 88.2%, respectively. Cochlear score (CR), vestibular score (VR), semicircular canal score (SR), and total scores (TR) in MD group were significantly higher than those in SSNHL group. The rate of diagnosis of MD was significantly higher in the MD group (92.2%) than VM group (27.3%). In MD, PTA was significantly correlated with CR and SR, the ratio of summating potential to action potential (-SP/AP) of electrocochleogram was significantly correlated with CR, VR, and SR, also canal paresis (CP) value of caloric test was significantly correlated with SR. 3D quantitative scoring demonstrated better diagnostic efficacy than conventional evaluation methods for Ménière's disease, and it may be an effective clinical tool to distinguish MD from SSNHL and VM. The clinical practicality of inner ear-enhanced MRI was further confirmed. IV.

Estimated number and prevalence of patients with delayed endolymphatic hydrops in Japan: a nationwide survey

Background Delayed endolymphatic hydrops (DEH) is a rare disease, and the actual number of patients in Japan remains unknown. Objective To investigate the number and prevalence of patients with DEH in Japan. Methods In total, 781 departments of otolaryngology in Japan were selected for survey by stratified random sampling according to the total number of hospital beds. We sent questionnaires to the target departments and collected data regarding the number of patients with DEH who visited those departments in 2019. Results The overall response rate was 68.0% (531 departments). The estimate number of patients with DEH in Japan was 962, and the prevalence was calculated to be 0.8 per 100,000 population. Conclusion Patients with DEH were extremely rare in Japan. Significance This may be the first nationwide epidemiological study on the number and prevalence of patients with DEH in Japan or in the world.

Effects of endolymphatic space volume on discrepancy of results between caloric test and video head impulse test

ObjectiveThe caloric test (C-test) and video head impulse test (vHIT) are known to occasionally show contradictory results in patients with Meniere's disease (MD). The reasons underlying this discrepancy between the two tests are currently unclear. We aimed to reveal the mechanisms responsible for this discrepancy by performing volumetric evaluation of the endolymphatic space (ELS) by using endoluminal contrast-enhanced inner ear MRI (ieMRI). MethodsWe enrolled 136 patients (174 ears) who visited the vertigo/dizziness center of our university and underwent the C-test and vHIT between February 2018 and February 2020. Inner ear MRI was also performed to determine the presence of endolymphatic hydrops (EH). The percentage of patients diagnosed with each vestibular disease was as follows: MD, 23.0%; benign paroxysmal positional vertigo (BPPV), 17.8%; bilateral vestibular disorder (BVD), 9.2%; sudden deafness with vertigo (SD), 8.0%; peripheral dizziness (PD), 7.5%; unilateral vestibular disorder (UVD), 6.9%; vestibular neuritis (VN), 6.3%; delayed endolymphatic hydrops (DEH), 3.4%; central dizziness (CD), 2.9%; Hunt syndrome (Hunt), 1.2%; and other disorders (OD), 13.8%. ResultsAmong the ears in the present study, 46.0% (80/174) showed a discrepancy in the results of the C-test and vHIT, and the disease-related distribution of patients showing this discrepancy was as follows; MD, 38.8% (27/80; p = 0.0019); BVD, 13.8% (11/80); UVD, 12.5% (10/80); SD, 7.5% (6/80); BPPV, 6.3% (5/80); PD, 6.3% (5/80); VN, 3.8% (3/80); DEH, 3.8% (3/80); CD, 2.5% (2/80); Hunt, 0.0% (0/80); and OD, 10.0% (8/80). In all cases, the discrepancy presented as a positive C-test result and negative vHIT result. The ELS ratio was measured for the whole inner ear, cochlea, vestibule, and semicircular canal, and the relationships between the rates and the presence of discrepancy was examined. Inner ear ELS ratio was 17.9% ± 10.8% in patients with the discrepancy and 15.2% ± 8.8% in those without the discrepancy (p = 0.036). Cochlear ELS ratio was 14.9% ± 11.3% in patients with the discrepancy and 11.9% ± 10.3% in those without the discrepancy (p = 0.0012). Vestibular ELS ratio was 22.3% ± 16.2% in patients with the discrepancy and 17.2% ± 12.7% in those without the discrepancy (p = 0.032). Semicircular canal ELS ratio was 18.0% ± 11.0% in patients with the discrepancy and 16.5% ± 9.6% in those without the discrepancy (p = 0.442). ConclusionThe volume of the ELS may affect the discrepancy of results between the C-test and vHIT.

A 14-year nationwide epidemiological analysis of delayed endolymphatic hydrops in Japan

Background Delayed endolymphatic hydrops (DEH) is an inner ear disease that causes recurrent vertigo in the ipsilateral ear or fluctuating hearing in the contralateral ear due to endolymphatic hydrops secondary to preceding deafness. There are few reports of large, multicentre studies investigating the clinical-epidemiological characteristics of DEH. Objective This study aimed to clarify the characteristics of DEH in Japan. Methods Clinical data on 662 patients with DEH were analysed by nationwide, multicentre surveys conducted by the Peripheral Vestibular Disorders Research Group of Japan. Results The proportion of ipsilateral DEH (IDEH) was slightly higher than that of contralateral DEH (CDEH) at 55.4%. The time delay between onset of precedent deafness and onset of DEH was significantly longer for CDEH than for IDEH. The most common cause of precedent deafness was a disease of unknown cause with onset in early childhood (33.1%). Epidemiological characteristics were not significantly different between CDEH with and without vertigo. Conclusion DEH appearing to be caused by viral labyrinthitis has a high rate of onset within 40 years of precedent deafness. Clinical and epidemiological characteristics of IDEH, CDEH with vertigo, and CDEH without vertigo were very similar. Significance The clinical-epidemiological characteristics of DEH in Japan were clarified.

12-month effect of middle ear pressure therapy with the EFET01 device for intractable definite Meniere’s disease and delayed endolymphatic hydrops after certification by the public health insurance system in Japan

Background Middle ear pressure therapy (MEPT) is effective for intractable vertigo in patients with definite Meniere’s disease (MD) and treatment-refractory delayed endolymphatic hydrops (DEH). Four-month MEPT with the EFET01®, an MEPT device developed in Japan and covered by national health insurance since September 2018, has shown efficacy. However, efficacy and safety after 12 months of treatment, which is appropriate for determining the therapeutic effect of MEPT devices, is unclear. Objectives Examine the therapeutic effect of 12-month MEPT using the ETET01®. Material and Methods Patients underwent MEPT using the EFET01® from September 2018 to July 2021. Thirty-three patients followed for >12 months were enrolled in this retrospective study. Clinical data were evaluated in the first and second 6-month treatment periods. Data from the second 6-month period were compared with data from an MEPT study using a different device. Results MEPT with the EFET01® significantly improved vertigo in the first period, with further improvement in the second period. The efficacy and safety were comparable to MEPT with other devices. Conclusions MEPT with the EFET01® is effective for intractable vertigo in patients with definite MD and DEH, and 12-month follow-up is recommended. Significance The efficacy of 12-month MEPT with the EFET01® was demonstrated.

Efficiency of a novel middle ear pressure device for intractable definite Meniere’s disease and delayed endolymphatic hydrops after certification by the public health insurance system in Japan

Background Middle ear pressure therapy (MEPT) is effective in treating intractable vertigo in patients with definite Meniere’s disease (MD) and delayed endolymphatic hydrops (DEH) refractory to conservative treatment. A novel middle ear pressure device, the EFET01®, which requires no transtympanic ventilation tubes, was developed in Japan, approved by the Japanese Ministry of Health, Labour and Welfare, and has been used under Japanese national health insurance since September 2018. Objectives To examine short-term therapeutic effect of MEPT using the ETET01® compared with previous clinical trial results. Methods Patients selected according to Japan Society for Equilibrium Research (JSER) guidelines underwent MEPT using the EFET01 from September 2018 to July 2021, and 44 patients were enrolled in this retrospective study. Clinical data analysed at 4 months after the start of MEPT were compared with those of the previous clinical trial for the EFET01. Results MEPT using the EFET01 showed the same therapeutic efficacy as that of the previous clinical trial, i.e. improvement in the intensity and frequency of vertigo with no effect on hearing, even under JSER guidelines for proper use of MEPT. Conclusion MEPT using the EFET01 provided an effective treatment option for intractable vertigo in patients with definite MD and DEH.

Anatomical variation of inner ear may be a predisposing factor for unilateral M\xe9ni\xe8re\u2019s disease rather than for ipsilateral delayed endolymphatic hydrops

ObjectiveRadiological anatomical variations, measured by magnetic resonance imaging (MRI), were evaluated in patients with ipsilateral delayed endolymphatic hydrops (DEH) and unilateral Ménière’s disease (MD). The role of anatomical variations in different subtypes of hydropic ear disease was investigated.MethodsTwenty-eight patients with ipsilateral DEH, 76 patients with unilateral MD, and 59 control subjects were enrolled. The radiological indices included the distance between the vertical part of the posterior semicircular canal and the posterior fossa (MRI-PP distance) and the visibility of vestibular aqueduct (MRI-VA). These variations among patients with DEH, MD, and control subjects were compared. The correlation between radiological anatomical variations and clinical features or audio-vestibular findings was also examined.Results(1) MRI-PP distance in the affected side of unilateral MD was shorter than that in ipsilateral DEH (Z = − 2.481, p = 0.013) and control subjects (Z = − 2.983, p = 0.003), while the difference of MRI-PP distance between the affected side of ipsilateral DEH and control subjects was not statistically significant (Z = − 0.859, p = 0.391). (2) There was no significant interaural difference of MRI-PP distance in patients with unilateral MD (Z = − 0.041, p = 0.968) and ipsilateral DEH (t = − 0.107, p = 0.915) respectively. (3) No significant interaural difference of MRI-VA visibility was observed in patients with unilateral MD (χ2 = 0.742, p = 0.389) and ipsilateral DEH (χ2 = 0.327, p = 0.567) respectively. (4) No correlation was found between these anatomical variables and clinical features or audio-vestibular findings in patients with unilateral MD and ipsilateral DEH respectively (p > 0.05).ConclusionsAnatomical variations of inner ear may be a predisposing factor in the pathogenesis of unilateral MD rather than ipsilateral DEH.Key Points• Patients with ipsilateral delayed endolymphatic hydrops showed normal distance between the vertical part of the posterior semicircular canal and the posterior fossa.• Compared to patients with ipsilateral delayed endolymphatic hydrops and control subjects, patients with unilateral Ménière’s disease exhibited shorter distance between the vertical part of the posterior semicircular canal and the posterior fossa.• Anatomical variations of inner ear may be a predisposing factor in the pathogenesis of unilateral Ménière’s disease rather than ipsilateral delayed endolymphatic hydrops.

Clinical features of vestibular syncope associated with tumarkin attacks in delayed endolymphatic hydrops

Objective: To analyze the clinical characteristics of vestibular syncope (VS) associated with drop attacks (DA) in delayed endolymphatic hydrops (DEH). Methods: DEH cases with complete data were retrospectively analyzed, including three DEH cases with DA and VS (VS group), and six DEH cases without DA or VS (control group). The clinical profile, the results of neurotological examinations [such as pure tone audiometry, electrocochleography (EcochG), caloric test, vestibular evoked myogenic potentials (VEMP), and video head impulse test (vHIT)] and treatment outcomes were analyzed. Results: (1) In the VS group, there were three cases of ipsilateral DEH; in the control group, there were six cases of ipsilateral type. One case in each group had a history of migraine. (2) The prevalence of abnormal results in caloric test, vHIT, cervical VEMP, and ocular VEMP in the VS group was 3/3, 1/3, 2/2, and 2/2, respectively, and in the control group was 3/6, 0/3, 1/6, and 4/6, respectively. Two cases in each group underwent EcochG, and no identifiable waveform was elicited on the affected side, and-SP/AP ratio of unaffected side was less than 0.4. (3) Patients in both groups were initially treated with conservative medication. Two cases in the VS group subsequently received intratympanic injections of dexamethasone. No DA or VS occurred during a follow-up period lasting over one year. All patients achieved good control of vertigo during the follow-up period. Conclusions: VS may occur in the patients with DEH. The differential diagnosis of syncope in patients with otogenic vertiginous disease can help improve clinical diagnosis and treatment.

Long-term effect of transtympanic intermittent pressure therapy using a tympanic membrane massage device for intractable meniere’s disease and delayed endolymphatic hydrops

Background A 12-month follow-up study showed that middle ear pressure treatment with a transtympanic membrane massage (TMM) device had a similar effect to a Meniett device. Objectives The effects of pressure treatment with a TMM device were retrospectively compared to the effects of treatment with a Meniett device in patients with Meniere’s disease (MD) and delayed endolymphatic hydrops (DEH) who were followed for a minimum of 24 months. Materials and Methods Twenty-seven patients were treated with the TMM device and 14 patients were treated with a Meniett device. The insertion of a transtympanic ventilation tube was necessary for the Meniett device but not for the TMM device. Results In patients treated with the TMM and Meniett devices, the frequency of vertigo significantly improved at 19–24 months after treatment. The distribution of vertigo at 19–24 months after treatment did not differ between the patients treated with the two types of devices. Pressure treatment for 8 months or more was suitable to achieve remission. Conclusions and Significance Middle ear pressure treatment for 8 months or more with a TMM or Meniett device was equally effective and provided minimally invasive treatment options for intractable MD and DEH.

Effects of transtympanic intermittent pressure therapy using a new tympanic membrane massage device for intractable Meniere’s disease and delayed endolymphatic hydrops: a prospective study

Background The retrospective study showed that the effect of the middle ear pressure treatment by the tramstympanic membrane massage (TMM) device was similar to that of the Meniett device. Objectives The new TMM device named EFET device was prospectively evaluated in patients with Meniere’s disease (MD) and delayed endolymphatic hydrops (DEH) and we compared the effects to the Meniett device. Materials and methods 23 ears of 19 patients were treated with an EFET device, and 17 ears of 15 patients were treated with the Meniett device. All patients suffering from intractable MD and DEH were treated for 4 months. The insertion of a transtympanic ventilation tube was necessary for the Meniett device, but not the EFET device. Results In patients treated by the EFET and Meniett devices, the frequency of vertigo significantly improved after treatment. The distribution of vertigo outcomes at 4 months after treatment did not differ between patients treated with the both devices. Conclusions and significance Middle ear pressure treatment by the EFET device is effective and provides minimally invasive options for intractable MD and DEH like the Meniett device.

Evaluation of Patients with Delayed Endolymphatic Hydrops by Videonystagmography, Vestibular-Evoked Myogenic Potentials, and Electrocochleography

Introduction: The categorization of delayed endolymphatic hydrops (DEH) based on the ear which produces vertigo may sometimes cause misdiagnosis. Objectives: The aim of this study was investigating the vestibular-evoked myogenic potentials (VEMPs), electrocochleography (ECoG), and videonystagmography (VNG) in cases with DEH to determine the ear that originates symptoms. Methods: In this cross-sectional study, 34 patients – 20 males and 14 females – with profound unilateral sensorineural deafness and vertigo attacks were recruited and evaluated by the ECoG, VNG, and VEMPs tests. Results: The average age was around 43; the summating potential/action potential was abnormal in 29.4% of patients in their normal auditive ear. In 32.4, 17.6, and 50% of cases with a deaf ear, absent, normal, and abnormal VEMPs results were sequentially observed, respectively. In normal-hearing ears, absent, normal, and abnormal VEMPs were observed in 23.5, 50, and 26.5%, respectively. In the normal-hearing ear, the distribution of abnormal VEMPs was 26.5%, and in the deaf ear, this parameter was abnormal in 50% of the opposite ear (p value = 0.00021). In the VNG test, among patients with a normal-hearing ear, results in 27 and 7 patients were sequentially normal and hypofunction. Conclusion and Significance: The probability of a hypofunction VNG test in a normal-hearing ear might be greater when the VEMPs results of the contralateral deaf ear are normal. In patients with a normal-hearing ear, the distribution of abnormal VEMPs in the contralateral deaf ear is greater, although the intact side may also manifest abnormality in VEMPs tests. The initial evaluation should begin in a deaf ear as well as for the normal-hearing ear ere utilizing ablation surgery.

The transcriptome characteristics of vestibular organs from delayed endolymphatic hydrops patients (Meniere's disease).

To identify genes that are related to delayed endolymphatic hydrops (DEH) in patients by RNA-Seq analysis. Observational study. Eye & ENT Hospital, Fudan University (Shanghai, China). We collected the entire vestibular system from four patients with DEH who underwent labyrinthectomy. Three control samples were collected from patients with acoustic neuroma or facial neuroma treated via the translabyrinthine approach. High-throughput RNA-Seq analysis was performed to investigate gene expression in the pathological vestibular system. Our bioinformatic analysis identified 17 genes that were upregulated and eight genes that were downregulated in patients with DEH compared with the controls. The altered gene expression profile suggested that DEH is closely related to neuropathy and autoimmune disease. In addition, many of the differentially regulated genes were involved in cell adhesion, suggesting a role of cell adhesion in DEH. Immunofluorescence analysis confirmed the expression of PMP2 and CLDN19 in the cytoplasm of hair cells and scattered expression of MPZ at cell junctions. The protein expression levels were higher in specimens from patients with Ménière's disease and DEH compared with controls. The protein expression profile of vestibular organs in patients with endolymphatic hydrops exhibited a degree of similarity to that of Ménière's disease. Endolymphatic hydrops is characterised by autoimmune abnormalities. DEH and Ménière's disease are likely to be different manifestations of the same disease, with disparate clinical symptoms. RNA-Seq is a useful analytical tool to characterise the vestibular pathology based on its transcriptome.

Long-term efficacy of triple semicircular canal plugging in the treatment of patients with ipsilateral delayed endolymphatic hydrops

This study aims to explore the long-term efficacy of triple semicircular canal plugging (TSCP) in the treatment of intractable ipsilateral delayed endolymphatic hydrops (DEH), so as to provide an alternative therapy for this disease. Forty-eight patients diagnosed with ipsilateral DEH referred to vertigo clinic of our hospital between Dec. 2010 and Dec. 2017, were included in this study for retrospective analysis. All patients were followed up for 2 years. Vertigo control and auditory functions were measured and analyzed. Pure tone audiometry, caloric test, and vestibular evoked myogenic potential (VEMP) were performed in two-year follow-up. Forty-five patients who accepted intratympanic gentamicin (26.7 mg/mL) twice given one week apart were selected as a control group. The total control rate of vertigo in TSCP group was 97.9% (47/48) in the two-year follow-up, with complete control rate of 83.3% (40/48) and substantial control rate of 14.6% (7/48). The rate of hearing loss was 22.9% (11/48). The total control rate of vertigo in intratympanic gentamicin group was 80.0% (36/45), with complete control rate of 57.8% (26/45) and substantial control rate of 22.2% (10/45), and the rate of hearing loss was 20.0% (9/45). The vertigo control rate of TSCP was significantly higher than that of intratympanic gentamicin (χ2 = 6.01, p < 0.05). There was no significant difference of hearing loss rate between two groups. (χ2 = 0.12, p > 0.05). TSCP, which can reduce vertiginous symptoms in patients with intractable ipsilateral DEH, represents an effective therapy for this disorder.

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.

Dissociation of Caloric and Video Head Impulse Tests in Patients With Delayed Endolymphatic Hydrops.

Delayed endolymphatic hydrops (DEH) represents a rare clinical entity characterized by intermittent vertigo attacks mimicking those of Ménière's disease (MD) in a patient with a prior sensorineural hearing loss. Some vestibular tests have been employed in patients with DEH. These tests provide useful diagnostic information and facilitate clinical decision-making. Here, we retrospectively studied the features of video head impulse test (vHIT) and examined its relationship with caloric test used in DEH patients. Included in this study were 17 patients with ipsilateral DEH and 2 with contralateral DEH. Among them, 73.7% (14/19) showed abnormal caloric test response (76.5% in ipsilateral DEH and 50% in contralateral DEH). Meanwhile, only 15.8% (3/19) of patients yielded abnormal horizontal vHIT results (11.8% in ipsilateral DEH and 50% in contralateral DEH). Abnormal caloric response in the presence of a preserved vHIT was common in DEH patients, especially those with ipsilateral DEH. This dissociation might be a distinctive pattern of vestibular deficit in DEH.

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

ObjectiveTo elucidate the pathophysiological process by analyzing the correlation between morphological and functional changes in patients with delayed endolymphatic hydrops (DEH). MethodsTwenty-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. ResultsAccording 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. ConclusionsHydrops 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. SignificanceOur findings can provide a new perspective on the functional and morphological characteristics of patients with DEH.

Bilateral delayed endolymphatic hydrops evaluated by bilateral intratympanic injection of gadodiamide with 3T-MRI.

The purpose of this study was to assess the diagnostic performance of 3T MRI after intratympanic injection of gadodiamide for delayed endolymphatic hydrops (DEH), and assess the relationship between endolymphatic hydrops (ELH) and vestibular function in patients diagnosed with DEH and confirmed by 3T MRI. Nineteen patients clinically diagnosed with DEH (11 ipsilateral DEH, 8 contralateral DEH) participated in this study. Diluted gadodiamide was administered to the bilateral tympanic cavity by injection through the tympanic membrane. At 24 hours post-injection, the ELH was evaluated by MRI. Patient vestibular functions were evaluated by caloric testing and cVEMP. ELH was observed in all patients (19/19: positive rate 100%). The distribution patterns of ELH varied between the cochlear or vestibular region. Vestibular ELH was observed in the affected ear in all ipsilateral DEH patients. In the contralateral DEH patients, however, there were individual differences in the distribution patterns of ELH. Six patients (1 ipsilateral DEH, 5 contralateral DEH) had bilateral ELH. No obvious relationships were observed between ELH and vestibular function. ELH distribution was complicated, particularly in the contralateral DEH cases. It was difficult to identify the existence of ELH by vestibular functional testing alone; therefore, 3T MRI is thought to be useful for identifying the affected ear. A significant number of cases had “bilateral” DEH, particularly among the contralateral DEH cases, indicating that we should pay careful attention to this pathology when treating DEH.

Delayed endolymphatic hydrops. Special emphasis on nystagmus associated with episodes and contribution of chemical labyrinthectomy

ObjectivesThe main objective was to describe spontaneous nystagmus characteristics during an episode of delayed endolymphatic hydrops (DEH), including an initial vertical upbeating nystagmus in one patient. The secondary objective was to highlight the contribution of chemical labyrinthectomy. MethodsEpisodic vertigo after a prolonged period of time of sensorineural hearing loss (profound or total) in one ear characterized ipsilateral DEH and was associated with the development of hearing loss in the opposite ear in contralateral DEH. ResultsTen patients met the criteria for DEH: 7 ipsilateral and 3 contralateral. Three (all ipsilateral DEH) were examined during a vertigo episode. Two patients had a typical horizontal-torsional nystagmus beating contralaterally to the hearing loss. One patient showed atypic initial vertical upbeating nystagmus with a slight torsional component, which secondarily became horizontal-torsional beating contralaterally to the hearing loss. Four patients had disabling vertigo with unilateral total deafness (ipsilateral DEH), successfully treated by 1-3 transtympanic gentamycin (Gentalline®) injections. ConclusionNystagmus direction during vertigo episodes varies, and may initially present as vertical upbeating nystagmus, which, to our knowledge, has not been previously reported in DEH or Menière's disease. This nystagmus might reflect an inhibition of the superior semicircular canal (on the hearing-impaired side), suggesting incipient hydrops in this canal. Chemical labyrinthectomy is a simple and effective procedure in unilateral DEH, especially as the patient often suffers from total deafness.