Prevalence of Endolymphatic Hydrops in Cochlear Implant Candidates with Idiopathic Profound Sensorineural Hearing Loss.

OBJECTIVE: To determine the prevalence of endolymphatic hydrops (EH) in cochlear implant (CI) candidates with idiopathic profound sensorineural hearing loss (SNHL) and its influence on the preservation of audiovestibular function after cochlear implantation. STUDY DESIGN: Prospective case series. SETTING: Tertiary referral center. PATIENTS: CI candidates with idiopathic progressive SNHL, but without classic EH-associated symptoms. INTERVENTIONS: Delayed intravenous gadolinium-enhanced inner ear fluid-attenuated inversion recovery magnetic resonance imaging as well as pure-tone audiograms, video head impulse tests, and vestibular evoked myogenic potentials before and 4 weeks after cochlear implantation. MAIN OUTCOME MEASURES: Prevalence of EH before cochlear implantation, audiovestibular function before and after surgery in hydropic and nonhydropic ears. RESULTS: Thirty-two ears in 16 CI candidates were included. Nine ears (28%) with EH were detected. Although preoperative hearing thresholds, utricular function, and semicircular canal function were not different between the two groups, saccular function was reduced in hydropic ears. Ten subjects received a unilateral CI. Of these, 3 (30%) showed EH on the implanted side. There was no difference regarding postoperative hearing loss between the two groups, but the results point toward a higher vulnerability of hydropic ears with respect to loss of otolith function after cochlear implantation. CONCLUSIONS: This is the first study showing that EH can be assumed in about one third of CI candidates with idiopathic profound SNHL, but no classic EH-associated symptoms. Preliminary results suggest that EH has no influence on the preservation of cochlear function but could be a risk factor for loss of otolith function after cochlear implantation.

Changes in endolymphatic hydrops after vestibular neurectomy observed in magnetic resonance imaging - A pilot study.

OBJECTIVES: The aim was to evaluate endolymphatic hydrops in patients with Ménière's disease before and after vestibular neurectomy to verify if the endolymphatic space dilatation, observed in magnetic resonance imaging, regressed within several months after surgery. METHODS: Magnetic resonance imaging was performed after intravenous gadolinium injection in four patients with unilateral definite Ménière's disease before and eight months after vestibular neurectomy. Clinical symptoms, audiovestibular tests, and endolymphatic hydrops in magnetic resonance imaging were evaluated. RESULTS: Endolymphatic hydrops was visualized in preoperative magnetic resonance imaging in three out of four analyzed patients. In the remaining one, an asymmetrical contrast enhancement in the affected ear was found. After the vestibular neurectomy, all four patients presented a complete resolution of vertigo episodes and improved functional level. Significant postoperative hearing deterioration was found in two patients. In the follow-up magnetic resonance imaging, no reduction of the endolymphatic hydrops was visualized. A reduction of asymmetrical contrast enhancement in one patient was found. CONCLUSIONS: Magnetic resonance imaging of the inner ear is a helpful diagnostic tool for Menière's disease. Vestibular neurectomy is an effective treatment for intractable vertigo; however, there is no endolymphatic hydrops regression evidence within several months after the surgery. Therefore, further studies with a long follow-up period and repeated magnetic resonance imaging are needed to assess the vestibular neurectomy's impact on endolymphatic hydrops. Nevertheless, magnetic resonance imaging supports the clinical diagnosis of Ménière's disease and may help understand its pathophysiology.

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.

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.

[A review of endolymphatic sac-related surgeries and their possible mechanisms].

Endolymphatic sac-related surgeries are a series of effective procedures to treat patients with Ménière's disease. However, there is controversy since they were proposed in 1927. Although most studies have shown the effectiveness of endolymphatic decompression and endolymphatic shunting, the mechanisms of them are still unclear. Some studies suggested that these procedures work because the endolymphatic hydrops relieves after surgery. However, the new one proposed recently, endolymphatic duct blockage, seems to contradict to the relief theory. Therefore, this article reviewed the mechanisms of endolymphatic hydrops and the development of a series of sac surgeries to better understand the possible mechanisms of sac surgeries.

Change in endolymphatic hydrops 2 years after endolymphatic sac surgery evaluated by MRI.

OBJECTIVE: This study was performed to determine whether endolymphatic sac surgery improves vestibular and cochlear endolymphatic hydrops 2 years after sac surgery and to elucidate the relationship between the degree of improvement of endolymphatic hydrops and the changes in vertigo symptoms, the hearing level, and the summating potential/action potential ratio (-SP/AP ratio) by electrocochleography (ECochG) in patients with Ménière's disease (MD). METHODS: Twenty-one patients with unilateral MD who underwent sac surgery were included in this study. All patients underwent gadolinium-enhanced magnetic resonance imaging (Gd-MRI) before and 2 years after sac surgery. We evaluated the difference in vestibular and cochlear endolymphatic hydrops between before and after surgery in both ears and compared these findings with the frequency of vertigo attacks, hearing level, and ECochG findings. RESULTS: In affected ears, the presence of vestibular endolymphatic hydrops and the frequency of vertigo attacks significantly decreased after surgery. However, affected ears showed no significant improvement in the presence of cochlear endolymphatic hydrops or the -SP/AP ratio by ECochG; there was also no significant improvement or deterioration in the hearing level. CONCLUSION: The present findings suggest that sac surgery reduces vestibular endolymphatic hydrops and prevents aggravation of cochlear endolymphatic hydrops, and these changes lead to a reduction of vertigo attacks and suppress the progression of hearing impairment associated with vertigo attacks.

Endolymphatic sac drainage for the treatment of Ménière's disease.

OBJECTIVE: To describe the results obtained with endolymphatic sac drainage in patients with Ménière's disease. METHOD: A retrospective case review study was conducted of 95 Ménière's disease patients who underwent endolymphatic sac drainage in a tertiary care referral centre, after failing a long course of medical management. The main outcome measures were vertigo control and hearing preservation. RESULTS: In patients with unilateral disease, vertigo control was obtained in 94.3 per cent of patients. A significant improvement in cochlear function was seen in 14 per cent of patients, and hearing was preserved or improved in 88 per cent. For the bilateral group, vertigo control was obtained in 85.7 per cent of patients and cochlear function improved in 28 per cent. Hearing preservation was attained in 71 per cent of these patients. CONCLUSION: Endolymphatic sac drainage is a good surgical option for patients with incapacitating endolymphatic hydrops, providing a high percentage of vertigo control and hearing preservation.

Identification of operculum and surgical results in endolymphatic sac drainage surgery.

To increase the accuracy of identification of the endolymphatic sac during endolymphatic sac drainage surgery, we focused on the intra-operative identification of the operculum as a structural marker of the endolymphatic sac and the vestibular aqueduct at the rugose portion. Then, we examined the relationship between identification of the operculum and surgical outcomes. We used 100 cases with intractable Meniere's disease who received endolymphatic sac drainage with local steroid administration, superior to traditional endolymphatic sac drainage surgery and non-surgical medical treatment. According to operation records, we divided these 100 cases into those with intra-operative identification of the operculum (n=72) and those without (n=28) and compared the surgical outcomes. The ratio of intra-operative identification of operculum (OPC(+)) was 72.0%. Better hearing results were observed significantly more often in patients with OPC(+), although there were no significant differences in vertigo results between OPC(+) and OPC(-). We concluded that the endolymphatic sac might be exposed adequately at rugose portion and opened for the drug delivery, resulting in better surgical results.

Changes in endolymphatic hydrops visualized by magnetic resonance imaging after sac surgery.

The purpose of the study was to observe changes in endolymphatic hydrops by using intratympanic injection of gadolinium and magnetic resonance imaging (MRI) before and after endolymphatic sac surgery in patients with unilateral Meniere's disease. Thirteen patients with unilateral Meniere's disease undergoing endolymphatic sac surgery were retrospectively and prospectively analyzed. Three-dimensional fluid-attenuated inversion recovery or three-dimensional real inversion recovery MRI was performed 24 h after an intratympanic injection of gadolinium to grade the presence of endolymphatic hydrops. Among the 13 patients with hydrops confirmed by preoperative MRI, vestibular hydrops had no significant change in all patients; cochlear hydrops became negative in 2 patients, and remained unchanged in the other 11 patients after surgery. Definite vertigo attacks were substantially controlled in one patient and completely controlled in 12 patients during a follow-up period of 8-34 months after surgery. The hearing levels were improved in 3 patients, remained unchanged in 7 patients, and decreased in 3 patients. In conclusion, endolymphatic sac surgery does not always alleviate endolymphatic hydrops in patients with Meniere's disease. Relief from vertigo cannot always be attributed to the remission of hydrops. A change in hearing levels cannot be explained by hydrops status alone.

High Resolution MRI Shows Presence of Endolymphatic Hydrops in Patients Still Symptomatic After Endolymphatic Shunt Surgery.

OBJECTIVE: Endolymphatic hydrops has been well described in patients with Ménière's syndrome; however, causation has not been established. Decompression of the endolymphatic sac has been proposed as a means to relieve hydrops and improve vertigo symptoms, but the efficacy of the surgery is debated. Until recently, there have been few objective measures of efficacy other than patients' subjective symptoms. Recent archival human temporal bone studies have shown that patients continue to have hydrops after shunt surgery. We propose using high-resolution magnetic resonance imaging (MRI) to determine the efficacy of endolymphatic shunt surgery (ELS) in patients who continue to experience vertigo. PATIENTS: Four patients presented with continued vertigo after ELS. INTERVENTIONS: Magnetic resonance imaging sequences included "cisternographic" three-dimensional T2, and delayed intravenous-enhanced three-dimensional fluid-attenuation inversion recovery (DIVE-3D-FLAIR) sequences, performed with 2350 ms (bright perilymph) and 2050 ms (bright endolymph) inversion times. The bright endolymph images were subtracted from bright perilymph images to create a composite image with bright perilymph, dark endolymph, and intermediate bone signals. MAIN OUTCOME MEASURES: MRI finding of endolymphatic hydrops. RESULTS: In all five affected ears in four patients who continued to experience severe vertigo, hydrops was found on high resolution MRI on the operated ear. The appearance on MRI was no different than in patients with endolymphatic hydrops (EH) who have not had surgery. CONCLUSIONS: The present study demonstrates the persistence of endolymphatic hydrops in patients who have failed ELS. Future studies evaluating for the presence or absence of endolymphatic hydrops in patients who claim to obtain relief from ELS.

Effects of endolymphatic sac decompression surgery on endolymphatic hydrops.

CONCLUSIONS: The present findings suggest that complete control of vertigo after endolymphatic sac decompression surgery (ESDS) does not always depend on improved vestibular function or reduced endolymphatic hydrops. Vertigo control is, however, associated with hearing stability. OBJECTIVE: Among surgical treatments for intractable Meniere's disease, ESDS is performed to preserve and improve inner ear function. We examined the correlation between changes in vertigo frequency and neuro-otologic function to understand the condition of the inner ear in patients whose vertigo was completely controlled after undergoing ESDS. METHODS: This was a retrospective cross-tabulation study. Between 1997 and 2001, we treated 52 patients with intractable vertigo using ESDS and followed the patients regularly for 2 years. Postoperatively we evaluated and recorded changes in vertigo attack frequency, maximum slow phase eye velocity, worst hearing level, and glycerol test results according to modified American Academy of Otolaryngology-Head and Neck Surgery 1995 criteria. RESULTS: We found no correlation between vertigo control and vestibular function. There was also no correlation between vertigo control and negative conversion of the glycerol test. There was a significant correlation between vertigo control and hearing control .

Changes in endolymphatic hydrops after sac surgery examined by Gd-enhanced MRI.

CONCLUSION: Endolymphatic hydrops could be a reversible inner ear pathological condition. After sac surgery, hydrops was reduced and symptoms went into remission in some cases, although vertigo suppression was not always a result of the reduced hydrops. OBJECTIVE: To examine the changes in endolymphatic hydrops detected by gadolinium (Gd) contrast-enhanced magnetic resonance imaging (MRI) before and 6 months after endolymphatic sac surgery in patients with unilateral Ménière's disease. METHODS: Fluid-attenuated inversion recovery MRI was obtained 4 h after intravenous administration or 24 h after intratympanic administration of Gd contrast medium. An enlarged negative stain corresponding to the cochlear duct and endolymphatic space of the vestibule was assessed as hydrops. RESULTS: Of seven patients with hydrops confirmed by MRI before surgery, both cochlear and vestibular hydrops became negative in two, cochlear hydrops became negative in one, both hydrops were present, but reduced, in one, and there was no change in three patients. The number of vertigo spells was reduced in all cases at 6-12 months after surgery. As for the three cases of negative hydrops, vertigo was completely suppressed. In two cases in which hearing level improved, hydrops became negative after surgery.

Otolithic membrane damage in patients with endolymphatic hydrops and drop attacks.

OBJECTIVES: 1. Evaluate the otolithic membrane in patients with endolymphatic hydrops (EH) and vestibular drop attacks (VDA) undergoing ablative labyrinthectomy. 2. Correlate intraoperative findings to archival temporal bone specimens of patients with EH. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. SPECIMEN SOURCE: 1. Patients undergoing labyrinthectomy for incapacitating Ménière's disease (MD), delayed EH, VDA, or acoustic neuroma (AN) between 2004 and 2011. 2. Archival temporal bone specimens of patients with MD. INTERVENTIONS: Ablative labyrinthectomy. MAIN OUTCOME MEASURES: Examination of the utricular otolithic membrane. RESULTS: The otolithic membrane of the utricle was evaluated intraoperatively in 28 patients undergoing labyrinthectomy. Seven (25%) had a history of VDA, 6 (21%) had delayed EH, 9 (32%) had MD, and 6 (21%) had AN. All patients with VDA showed evidence of a disrupted utricular otolithic membrane, whereas only 50% and 56% of patients with delayed EH and MD, respectively, demonstrated otolithic membrane disruption (p = 0.051). None of the patients with AN showed otolithic membrane disruption (p = 0.004). The mean thickness of the otolithic membrane in 5 archival temporal bone MD specimens was 11.45 micrometers versus 38 micrometers in normal specimens (p = 0.001). CONCLUSION: The otolithic membrane is consistently damaged in patients with VDA. In addition, there is a significantly higher incidence of otolithic membrane injury in patients with MD and delayed EH compared with patients without hydrops, suggesting that the underlying pathophysiology in VDA results from injury to the otolithic membrane of the saccule and utricle, resulting in free-floating otoliths and atrophy.

Diplopia due to skew deviation following neurotologic procedures.

OBJECTIVE: To describe and characterize diplopia resulting from skew deviation after cerebellopontine angle (CPA) surgery and labyrinthectomy. PATIENTS AND INTERVENTIONS: Retrospective case series of 4 patients who developed vertical diplopia from skew deviation after resection of tumors in the CPA or labyrinthectomy MAIN OUTCOME MEASURE: Complete neuro-opthalmologic examination including opticokinetic testing, confrontational visual field assessment, color plate, pupillary reflex, slit lamp examination, and head tilt test. RESULTS: Four patients with residual hearing preoperatively developed skew deviation immediately after surgical intervention, including translabyrinthine (n = 1) and retrosigmoid (n = 2) approaches to the CPA and labyrinthectomy (n = 1). Neuro-ophthalmologic examination demonstrated intact extraocular movements, and 2- to 14-mm prism diopter hypertropia on both primary gaze and head tilt testing. In all cases, skew deviation resolved spontaneously with normalization of the neuro-ophthalmologic examination within 10 weeks. CONCLUSION: Patients undergoing CPA surgery or labyrinthectomy can develop postoperative diplopia due to skew deviation as a consequence of acute vestibular deafferentation. Patients with significant hearing preoperatively, a probable marker for residual vestibular function, may be especially at risk for developing skew deviation postoperatively.

Endolymphatic sac tumor with overexpression of V2 receptor mRNA and inner ear hydrops.

CONCLUSION: We reported previously that hyperactivation of vasopressin type-2 receptor (V2R)-mediated signaling in the endolymphatic sac could affect endolymphatic fluid metabolism, resulting in the pathogenesis of endolymphatic hydrops. Taken together with the present endolymphatic sac tumor (ELST) study, it is suggested that disorder of V2R signaling in the endolymphatic sac for any reason could be involved in the pathogenesis of endolymphatic hydrops. Although it is due to tumor genesis in ELST, it is idiopathic in nature in Meniere's disease. OBJECTIVE: We encountered two cases of ELST showing Meniere's disease-like symptoms. Both cases were suspected of having endolymphatic hydrops using neuro-otological examinations. To clarify the histopathological diagnosis of ELST and the molecular pathogenesis of endolymphatic hydrops, we performed histopathological and molecular biological examinations of the endolymphatic sac. METHODS: ELSTs in two rare cases were removed completely through the transmastoidal approach. V2R mRNA expression was examined using real-time PCR. RESULTS: The first case was diagnosed as inflammatory granulation adjacent to the endolymphatic sac, i.e. pseudo-ELST, and the second case was diagnosed as papillary adenoma of ELST. V2R mRNA expression was up-regulated in the endolymphatic sac of both cases as seen in Meniere's disease compared with controls.

Histopathology after endolymphatic sac surgery for Ménière's syndrome.

BACKGROUND: The putative goal of sac surgery in Ménière's syndrome is to promote the flow of endolymph from the labyrinth to the endolymphatic sac and thereby relieving hydrops. There are scant published histopathologic data whether sac surgery actually accomplishes this goal. OBJECTIVE: To determine whether sac surgery relieves hydrops by examining the histopathologic changes in temporal bones obtained from individuals who had undergone sac surgery during life for Ménière's syndrome. METHODS: Temporal bones were examined from 15 patients who had sac surgery. Data on the presence and severity of hydrops, histology of the sac, and whether the procedure relieved vertigo were collected. RESULTS: The surgery failed to expose the sac in 5 cases; 4 of the 5 had relief from vertigo. The sac was exposed, but the shunt failed to reach the lumen of the sac in 8 cases; 4 of the 8 had relief from vertigo. The shunt was successfully placed within the lumen of the sac in 2 cases; both cases failed to experience relief from vertigo. Endolymphatic hydrops was present in all 15 cases. CONCLUSION: Endolymphatic sac surgery does not relieve hydrops in patients with Ménière's syndrome. Yet, sac surgery relieves vertigo in some patients, but the mechanism of such symptomatic relief remains unknown.

Magnetic resonance imaging of guinea pig cochlea after vasopressin-induced or surgically induced endolymphatic hydrops.

OBJECTIVE: To investigate the ability to detect the in vivo cochlear changes associated with vasopressin-induced and surgically induced endolymphatic hydrops using MRI at 3 tesla (T). STUDY DESIGN: Prospective, animal model. SETTING: Animal laboratory. SUBJECTS AND METHODS: In group 1, five guinea pigs underwent post-gadolinium temporal bone MRI before and after seven and 14 days of chronic systemic administration of vasopressin by osmotic pump. In group 2, five guinea pigs underwent temporal bone MRI eight weeks after unilateral surgical ablation of the endolymphatic sac. Three-tesla high-resolution T1-weighted sequences were acquired pre- and postcontrast administration. Region of interest signal intensities of the perilymph and endolymph were analyzed manually. Quantitative evaluation of hydrops was measured histologically. RESULTS: Gadolinium preferentially concentrated in the perilymph, allowing for distinction of cochlear compartments on 3.0-T MRI. The T1-weighted contrast MRI of vasopressin-induced hydropic cochlea showed significant increases in signal intensity of the endolymph and perilymph. Surgically induced unilateral hydropic cochlea also showed increased signal intensity, compared with the control cochlea of the same animal, but less of an increase than the vasopressin group. The histological degree of hydrops induced in the vasopressin group was comparable to previous studies. CONCLUSIONS: In vivo postcontrast MRI of the inner ear demonstrated cochlear changes associated with chronic systemic administration of vasopressin and surgical ablation of the endolymphatic sac. Understanding the MRI appearance of endolymphatic hydrops induced by various methods contributes to the future use of MRI as a possible tool in the diagnosis and treatment of Ménière's disease.

Time course of vestibular function changes of experimental endolymphatic hydrops in guinea pigs.

OBJECTIVE: To investigate the relationship between endolymphatic hydrops and vestibular dysfunction. METHODS: 20 pigmented guinea pigs were used: 15 for a hydrops group and 5 for a sham group. Endolymphatic hydrops was produced by electrocauterization of the endolymphatic sac on the left ears. In the horizontal vestibuloocular reflex (HVOR) study, HVOR responses were recorded before and 1, 2, and 4 weeks after surgery in 9 animals of the hydrops group and 5 animals of the sham group. HVOR gain under sinusoidal rotation with a maximal head velocity of 45 degrees /s and frequencies of 0.05, 0.1, 0.2, 0.4 and 0.8 Hz was analyzed. In the nystagmus study, spontaneous nystagmus was recorded in all animals of the hydrops and the sham groups for 1 h in the dark and the maximum slow-phase velocity was measured before and 1, 2, 4 weeks after surgery. Morphological changes in the inner ear were measured light microscopically. RESULTS: In the hydrops group, the HVOR gains at all stimulation frequencies seemed to decrease 1 week after surgery and recover 2 or 4 weeks after surgery; however, there were no statistical differences among HVOR gains in any periods after surgery. The incidence of spontaneous nystagmus gradually increased after surgery and the direction and onset showed large variation. The duration of nystagmus was approximately 10 min. The degree of endolymphatic hydrops showed large variation. In the sham group, HVOR gains at all stimulation frequencies showed no statistically different change in any period after surgery. In the sham group, no animal showed spontaneous nystagmus. CONCLUSION: Experimentally induced endolymphatic hydrops seems to contribute to vestibular dysfunction to some extent. We speculated that when endolymphatic hydrops is progressing, vestibular dysfunction might occur.