Endolymphatic Duct and Sac Decompression: A New Life for an Old Technique.

BACKGROUND: Ménière's disease is an inner ear disorder causing recurrent vertigo, hearing loss, and tinnitus. Diagnosis is based on the variability of the symptoms over time and absence of radiological abnormalities. Medical therapy is effective only in a small percentage of patients. Surgical strategies remain controversial. In this article, we revisit a surgical technique neglected over the years: endolymphatic sac surgery. METHODS: Fifty-four patients affected by Ménière's disease underwent endolymphatic duct and sac decompression. According to the American Academy of Otolaryngology-Head and Neck Surgery criteria, vertigo control was evaluated with follow-up at 6 months, 1 year, and 2 years from the intervention. Hearing results were evaluated before the surgery and at 2 years of follow-up using the pure tone average. The results were compared with similar techniques of endolymphatic sac surgery described in the literature. RESULTS: According to the American Academy of Otolaryngology-Head and Neck Surgery criteria classification, 2 years after surgery, 87% patients achieved complete control of vertigo (class A). The hearing remained stable in 93.5% of patients. The results appear compatible with other publications data regarding endolymphatic sac surgeries. CONCLUSION: The duct and endolymphatic sac decompression allows the control of vertigo and preserves hearing from the pathological effects of Ménière's disease. The revised technique allows the functional restoration of endolymphatic homeostasis.

Endolymphatic Duct Blockage as a Surgical Treatment Option for Ménière's Disease.

Endolymphatic duct blockage is a relatively new treatment option for Ménière's disease, aiming to reduce vertigo attacks while sparing hearing and equilibrium. After a regular mastoidectomy, the posterior semicircular canal is identified, and Donaldson's line is determined. This is a line through the horizontal semicircular canal, crossing the posterior semicircular canal. The endolymphatic sac is usually found at this site under the posterior semicircular canal. The bone of the endolymphatic sac and the dura are thinned until the sac is skeletonized, after which the endolymphatic duct is identified. The duct is then blocked with a titanium clip. Using a computerized tomography (CT) scan, the position is confirmed. Follow-up visits take place 1 week, 6 weeks and 1 year after surgery. To this day, only one prospective trial assessing this method has been conducted, comparing this new method to endolymphatic sac decompression. Results of the duct blockage are promising, with 96.5% of the patients free of vertigo after 2 years. However, further research is required.

A comparison of endolymphatic duct blockage, endolymphatic sac drainage and endolymphatic sac decompression surgery in reversing endolymphatic hydrops in Meniere's disease.

BACKGROUND: To explore the differences between endolymphatic duct blockage, endolymphatic sac drainage and endolymphatic sac decompression surgery in the reversal of endolymphatic hydrops (EH) in patients with intractable Meniere's disease (MD). METHODS: A total of 27 MD patients receiving endolymphatic duct blockage surgery (n = 10), endolymphatic sac drainage surgery (n = 9) and endolymphatic sac decompression surgery (n = 8) underwent gadolinium-enhanced inner ear magnetic resonance imaging (MRI) scans prior to, 2 weeks after and at > 12 months following surgery. RESULTS: In the group with endolymphatic duct blockage, the second MRI revealed no changes in EH, whereas the third MRI revealed a reversal of vestibular EH in 3 patients and a downgrading of cochlear hydrops in 2 of these 3 patients, who presented with an improvement in their hearing and complete control of vertigo. In the group with endolymphatic sac drainage, the second MRI showed a reversal of EH in 4 patients, and no changes in EH in the remaining 5 patients, whereas the third MRI showed that those 4 patients who presented with a reversal of EH at the second MRI stage remained unchanged except a recurrence of vestibular hydrops in 1 patient. All 4 patients exhibited a complete control of vertigo, but hearing improved in 1, worsened in 1 and remained unchanged in 2. In the group with endolymphatic sac decompression, both the second and third MRI examination revealed no reversal of EH. CONCLUSIONS: The present study has shown that both endolymphatic duct blockage surgery and endolymphatic sac drainage surgery have the potential to reduce EH in certain MD patients, but none of the patients receiving endolymphatic sac decompression surgery showed reversal of their EH.

Endolymphatic duct blockage for refractory Ménière's disease: assessment of intraoperative CSF leak on short-term surgical outcomes.

BACKGROUND: Endolymphatic sac decompression has shown limited success for the treatment of Ménière's disease (MD). We have published a novel technique with very promising results for the treatment of refractory MD: the Endolymphatic Duct Blockage (EDB) with two titanium clips. One of the challenges of this technique was an increased incidence of intraoperative Cerebrospinal Fluid (CSF) Leak. OBJECTIVE: To assess the effect of intraoperative CSF Leak on short-term surgical outcomes. METHODS: Retrospective comparative study in a tertiary care center (61 patients). MD patients who underwent EDB were included. Intraoperative CSF Leaks were documented. Surgical outcomes assessed were the presence of postoperative Benign Paroxysmal Positional Vertigo (BPPV), aural fullness, tinnitus, vertigo spells and pure tone average (PTA), speech discrimination scores (SDS) and bone conduction thresholds (BCT). Data were collected for these visits: preoperative, 1 week, 1 and 6 months postoperatively. RESULTS: Outcomes for the CSF Leak group (CSF +) (n = 22) were compared to remaining patient (CSF-) (n = 39). There was no significant difference in the occurrence of postoperative BPPV, aural fullness, tinnitus and vertigo spells. There was no significant difference in PTA, BCT or SDS between our groups at any visit. CONCLUSIONS: Intraoperative CSF Leak may cause a temporary non-significant worsening of hearing in the first-month postoperatively without any difference at 6 months.

Endolymphatic Duct Blockage for Refractory Ménière's Disease: Assessment of Endolymphatic Sac Biopsy on Short-Term Surgical Outcomes.

OBJECTIVE: Endolymphatic sac decompression (ESD) for the treatment of Ménière's disease (MD) has had limited success and variable results in the literature. We have devised a novel technique that involves blocking the endolymphatic duct with surgical clips. In a separate effort to study the endolymphatic sac (ELS), we have sectioned the lateral part of the main body of the ELS as biopsies from a subset of patients. We aimed to evaluate the effect of the lateral part sectioning of the ELS on short-term surgical outcomes. MATERIALS AND METHODS: This was a single-physician, retrospective study in a tertiary medical center. MD patients underwent endolymphatic duct blockage (EDB) surgery with or without ELS biopsy. The assessed surgical outcomes were the occurrence of benign paroxysmal positional vertigo (BPPV), intraoperative CSF leaks, aural fullness, tinnitus, vertigo spells, and pure tone audiometry. Data were collected at the following visits: preoperatively 1st week, 1st month, and 6th months. RESULTS: A total of 63 patients were included. The outcomes of the biopsy group (EDB+B) (n=27) were compared to those of the EDB group (n=36) at each visit. There was no significant difference in the occurrence of postoperative BPPV, CSF leaks, aural fullness, tinnitus, or vertigo spells. The SDS, the PTA, and bone conduction were not significantly different at any visit, and PTA variations were similar in both groups. CONCLUSION: Our results suggest that aggressive decompression of the ELS by sectioning the sac does not benefit patients in the early postoperative period. The short-term success of EDB surgery is attributable to the effective obstruction of the endolymphatic duct regardless of pressure in the ELS.

Endolymphatic duct blockage: quality of life assessment of a novel surgical technique for Ménière disease.

The aim of this study is to evaluate the quality of life (QOL) of patients treated by endolymphatic duct blockage (EDB) for Ménière's disease with a dedicated questionnaire. This is a retrospective cross-sectional study which included 54 patients diagnosed with severe, refractory Ménière's disease according to the AAO-HNS criteria and treated with EDB between 2010 and 2013. Answers to the first 38 questions have assigned scores from 0 to 4 (0 corresponding to the poorest QOL). A preoperative score called S1 was calculated as follows: S1 = sum of preoperative question scores/maximum possible preoperative score ×100. The same formula was used to calculate the postoperative score S2. The change in QOL score, S3, was then calculated (S3 = S2-S1). All answers were analyzed anonymously. Statistical analysis was done using Student t test and Chi square test. A response rate of 89 % was obtained with the Ménière's disease outcome questionnaire. The preoperative (S1) score was 21.4 (±12.6) and the postoperative score (S2) was 64.6 (±21.6) with a change in QOL (S3) of 43.3 (p < 0.001). Postoperatively, 89.9 % reported no Ménière's attacks (p < 0.001). Seventy-nine percent (15/19) of the questions showed a significant improvement after surgery. These results show that EDB is associated with a significant improvement of the QOL of patients suffering from severe Ménière's disease.

Evaluation of the usefulness of three-dimensional optical coherence tomography in a guinea pig model of endolymphatic hydrops induced by surgical obliteration of the endolymphatic duct.

Optical coherence tomography (OCT) has advanced significantly over the past two decades and is currently used extensively to monitor the internal structures of organs, particularly in ophthalmology and dermatology. We used ethylenediamine tetra-acetic acid (EDTA) to decalcify the bony walls of the cochlea and investigated the inner structures by deep penetration of light into the cochlear tissue using OCT on a guinea pig model of endolymphatic hydrops (EH), induced by surgical obliteration of the endolymphatic duct. The structural and functional changes associated with EH were identified using OCT and auditory brainstem response tests, respectively. We also evaluated structural alterations in the cochlea using three-dimensional reconstruction of the OCT images, which clearly showed physical changes in the cochlear structures. Furthermore, we found significant anatomical variations in the EH model and conducted graphical analysis by strial atrophy for comparison. The physical changes included damage to and flattening of the organ of Corti­evidence of Reissner's membrane distention­and thinning of the lateral wall. These results indicate that observation of EDTA-decalcified cochlea using OCT is significant in examination of gradual changes in the cochlear structures that are otherwise not depicted by hematoxylin and eosin staining.

Endolymphatic duct blockage: a randomized controlled trial of a novel surgical technique for Ménière's disease treatment.

OBJECTIVES: To compare the effectiveness of the endolymphatic duct blockage (EDB) and the endolymphatic sac decompression (ESD) to control Ménière's disease symptoms and to evaluate their effect on hearing level. STUDY DESIGN: Prospective nonblinded randomized study. SETTING: Tertiary medical center. SUBJECTS AND METHODS: Fifty-seven patients affected by a refractory Ménière's disease were included out of which 22 underwent an ESD and 35 underwent an EDB. Five periods of follow-up were considered: 0 to 1 week, 1 week to 6 months, 6 to 12 months, 12 to 18 months, and 18 to 24 months. Mean outcome measurements consisted of vertigo control, tinnitus, aural fullness, instability, and hearing level. Hearing level was evaluated using pure-tone average (PTA) and speech discrimination score (SDS). RESULTS: There was no significant difference between the 2 groups in the number of vertigo spells per months preoperatively (P = .153). Twenty-four months postoperatively, 96.5% of the EDB group had achieved a complete control of vertigo spells against 37.5% of the ESD group with a statistically significant difference (P = .002). There was a better control of tinnitus and aural fullness with EDB (P = .021 and P = .014, respectively). There was no statistically significant difference in hearing level preoperatively (P = .976) and 24 months postoperatively (P = .287) between the 2 groups. Hearing level was preserved in each group with no significant difference between the preoperative and the postoperative levels (P > .05). CONCLUSION: EDB is more effective than the traditional ESD in controlling the symptoms of Ménière's disease. It is a novel surgical technique with promising results for a complete treatment of Ménière's disease. There are no significant complications or adverse effect.

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.

Surgical induction of endolymphatic hydrops by obliteration of the endolymphatic duct.

Surgical induction of endolymphatic hydrops (ELH) in the guinea pig by obliteration and obstruction of the endolymphatic duct is a well-accepted animal model of the condition and an important correlate for human Meniere's disease. In 1965, Robert Kimura and Harold Schuknecht first described an intradural approach for obstruction of the endolymphatic duct (Kimura 1965). Although effective, this technique, which requires penetration of the brain's protective covering, incurred an undesirable level of morbidity and mortality in the animal subjects. Consequently, Andrews and Bohmer developed an extradural approach, which predictably produces fewer of the complications associated with central nervous system (CNS) penetration.(Andrews and Bohmer 1989) The extradural approach described here first requires a midline incision in the region of the occiput to expose the underlying muscular layer. We operate only on the right side. After appropriate retraction of the overlying tissue, a horizontal incision is made into the musculature of the right occiput to expose the right temporo-occipital suture line. The bone immediately inferio-lateral the suture line (Fig 1) is then drilled with an otologic drill until the sigmoid sinus becomes visible. Medial retraction of the sigmoid sinus reveals the operculum of the endolymphatic duct, which houses the endolymphatic sac. Drilling medial to the operculum into the area of the endolymphatic sac reveals the endolymphatic duct, which is then packed with bone wax to produce obstruction and ultimately ELH. In the following weeks, the animal will demonstrate the progressive, fluctuating hearing loss and histologic evidence of ELH.

Bilateral Ménière's disease.

PURPOSE OF REVIEW: Bilateral Ménière's disease treatment can be extremely challenging and, like its unilateral variant, remains highly controversial. There are few articles in the literature that have focused specifically on the treatment of bilateral Ménière's disease, despite significant numbers of unilateral cases evolving toward bilaterality - a process which complicates management and is not simple to foresee. This study reviews the most up-to-date concepts in its diagnosis and treatment, with a special focus on recent advances. RECENT FINDINGS: Recent advances in electrocochleography, vestibular-evoked myogenic potential, and intratympanic gadolinium-based contrast agent MRI may have the potential to diagnose bilateral endolymphatic hydrops, and predict unilateral to bilateral Ménière's disease progression. Although more prospective studies are necessary, recent studies show that intermittent pressure therapy (Meniett device), intratympanic steroids, and endolymphatic sac surgery appear to be efficacious and well tolerated nonablative management options. Moreover, triple semicircular canal occlusion surgery has recently been used successfully in Ménière's disease. SUMMARY: Bilateral Ménière's disease can be challenging to diagnose via clinical suspicion and pure-tone audiometry alone. Therefore, adjunctive diagnostic tools including electrocochleography, vestibular-evoked myogenic potential, and intratympanic gadolinium-based contrast agent MRI are of even greater value, and may predict progression to bilaterality - allowing better optimization of treatment. Ablative treatments are relatively contraindicated due to the risks of bilateral vestibular and cochlear hypofunction. Nonablative treatments such as the Meniett device, intratympanic steroids, and endolymphatic sac surgery appear to be efficacious, and useful alternatives after conservative measures fail. Systemic aminoglycoside therapy is reserved for only the most severe and intractable cases.

[Surgery for vertigo]. / Chirurgie des vertiges.

Spontaneous recovery or central compensation makes surgical procedures rare in patients with vertigo. Surgery for vertigo proposed after pharmacological or physical therapy fails to eliminate Ménière's disease and some very rare cases of paroxystic positional vertigo. The main target in treating Ménière's disease is to promote vestibular compensation, which is possible only with a nonprogressive and stable deficit leading to readjustment of vestibular reflexes. Surgical procedures can be classified as nondestructive (endolymphatic sac decompression, vestibular nerve decompression, patching of perilymphatic fistulas), selectively destructive (middle fossa or retrosigmoid vestibular neurotomy, lateral semi-circular plugging) and destructive (labyrinthectomy). Surgical indications essentially concern incapacitating vertigo and depend mainly on hearing status. In Ménière's disease, vestibular neurotomy can be regarded as the gold standard considering its good results on vertiginous episodes; however, scoring with functional and quality-of-life scales bring out residual deficiency in some cases.

Morphology and function of Bast's valve: additional insight in its functioning using 3D-reconstruction.

The utriculo-endolymphatic valve was discovered by Bast in 1928. The function of Bast's valve is still unclear. By means of orthogonal-plane fluorescence optical sectioning (OPFOS) microscopy 3D-reconstructions of the valve and its surrounding region are depicted. The shape of the duct at the utricular side is that of a flattened funnel. In the direction of the endolymphatic duct and sac this funnel runs into a very narrow duct. The valve itself has a rigid 'arch-like' configuration. The opposing thin, one cell-layer thick, utricular membrane is highly compliant. We propose that opening and closure of the valve occurs through movement of the flexible base/utricular membrane away from and toward the relatively rigid valve lip.

Evaluation and management of endolymphatic sac and duct tumors.

The endolymphatic sac tumor is a slow-growing, locally aggressive neoplasm that originates from the epithelium of the endolymphatic sac and duct. Disease progression can lead to profound sensorineural hearing loss, posterior fossa invasion, brainstem compression, drop metastasis, and eventual death. Early diagnosis and surgical attention are the primary objectives in the management of patients who have endolymphatic sac tumor. This article describes the latest rationale and techniques for hearing preservation surgery and a review of the latest developments surrounding this disease entity.

Unusual cases of delayed endolymphatic hydrops.

The objective of this report is to emphasize the clinical features of delayed endolymphatic hydrops in comparison with Ménière's disease. Four cases were selected from a series of 16 examined between the years 1993 and 2005, due to their unusual characteristics. A short clinical history and significant tests are presented for each of the four cases. The cases of delayed endolymphatic hydrops discussed in this article suggest that hydrops is the most important underlying pathology that causes the hearing loss and the vestibular symptoms both in the better ear and in the ear with severe hearing loss. They also suggest that this condition probably occurs in patients with congenitally sensitive ears that make them prone to the development of the late hydrops.

Endolymphatic sac tumor located around semicircular canals.

We report a case of endolymphatic sac tumor (ELST). A 48-year-old female had recurrent and slowly progressive hearing loss, accompanied with dizziness like Meniere's disease. A tumor was located around the semicircular canals, and was detected on CT and MRI. The patient underwent total removal of the tumor using a transmastoid approach. Histopathological examinations agreed with features of an ELST. The tumor was highly suspected to have originated from the rugose portion of the endolymphatic sac or the endolymphatic duct, based on surgical and imaging studies. Structure of the membranous labyrinth was preserved regardless of the existence of the tumor around semicircular canals with bone destruction. ELSTs seem to have an osteolytic or osteophilic nature, by examining patterns of tumor infiltration.

An interstitial network of podoplanin-expressing cells in the human endolymphatic duct.

The human endolymphatic duct (ED) with encompassing interstitial connective tissue (CT) is believed to be important for endolymph resorption and fluid pressure regulation of the inner ear. The periductal CT cells are interconnected via numerous cellular extensions, but do not form vessel structures. Here we report that the periductal CT is populated by two distinct cell phenotypes; one expressing podoplanin, a protein otherwise found on lymph endothelia and on epithelia involved in fluid fluxes, and a second expressing a fibroblast marker. A majority of the interstitial cells expressed podoplanin but not the lymphatic endothelial cell markers hyaluronan receptor (LYVE-1) or vascular endothelial growth factor receptor-3 (VEGFR-3). The fibroblast marker positive cells were found close to the ED epithelium. In the mid- and distal parts of the ED, these cells were enriched under folded epithelia. Furthermore, subepithelial CT cells were found to express activated platelet derived growth factor (PDGF)-beta receptors. Cultured CT cells from human inner ear periductal and perisaccular explant tissues were identified as fibroblasts. These cells compacted a three-dimensional collagen lattice by a process that could be promoted by PDGF-BB, a factor involved in interstitial fluid pressure regulation. Our results are compatible with the notion that the periductal CT cells are involved in the regulation of inner ear fluid pressure. By active compaction of the periductal CT and by the formation of villous structures, the CT cells could modulate fluid fluxes over the ED epithelium as well as the longitudinal flow of endolymph in the ED.

[Dissection of endolymphatic duct in Meniere's disease]. / Rassechenie éndolimfaticheskogo protoka pri bolezni Men'era.

The authors believe that endolymphatic sac (ES) surgery produces the highest and most persistent effect in comparison with all other surgical interventions in Meniere's disease (MD). Conventional views on the mechanisms underlying a beneficial effect of the ES surgery are revised and a new surgical technique is proposed. A positive effect of the operation, according to the authors, depends not on ES decompression but on the ES break taking place in separation of the dura mater from the bone skull in the outlet area of the aqueduct of the vestibule (AV). When the AV is separated from the sac, a rise in endolymphatic pressure is relieved by the endolymph pushing through the AV as it is not connected with the ES. 10 year follow-up has shown that this technique excludes subsequent endolymphatic hydrops.

Three new surgeries for treatment of intractable Meniere's disease.

OBJECTIVE: This study aimed to describe the rationales for and preliminary results of three new types of surgery for the treatment of intractable Meniere's disease, all involving insertion of a capillary tube into the endolymphatic duct. This study also aimed to compare the contrasting surgical strategies of endolymphatic sac enhancement versus sac supplantation. STUDY DESIGN AND SETTING: The study design was a retrospective review of 129 surgeries conducted by the author at Chang Gung Memorial Hospital since 1993: 51 cases of Huang/Gibson inner ear shunt implantation, 52 cases of intraductal capillary tube implantation (ICTI), and 26 cases of ICTIin combination with endolymphatic sac ballooning surgery (ESBS). PATIENTS: This study is limited to patients with classic Meniere's disease whose vertiginous symptoms were disabling and refractory to dietetic and medical treatment. MAIN OUTCOME MEASURES: Comparison of preoperative and postoperative conditions (e.g., vertigo control, hearing, disability) using American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) 1985 criteria and chi-square statistical method was measured. RESULTS: After 1 year of follow-up, use of the Huang/Gibson shunt resulted in a 94.1% vertigo control rate (complete or substantial) and fairly good hearing results, ICTI by itself resulted in an 88.5% rate of vertigo control and relatively unremarkable hearing results, and the ICTI in combination with ESBS (ICTI/ESBS) achieved a vertigo control rate of 96.1% in addition to good hearing results. CONCLUSIONS: The 1-year follow-up results for Huang/Gibson shunt implantation and sac-preserving ICTI/ESBS have approximately duplicated the excellent performance of the Arenberg implant after the same follow-up period, perhaps attributable in part to enhancement of endolymph flow through the endolymphatic duct.

Long-term hearing results following vestibular surgery in Meniere's disease.

OBJECTIVES: The purpose of this study is to evaluate the long-term hearing changes following vestibular surgery in patients with Meniere's disease. STUDY DESIGN: This is a retrospective analysis of patients operated on in a tertiary referral center setting. METHODS: Preoperative, postoperative, and 3- to 9-year postoperative audiograms were analyzed in two patient groups. Twenty-one patients underwent posterior fossa vestibular neurectomy (VN) and five, mastoid endolymphatic sac decompression and shunt (ELS). All frequencies, four-frequency pure-tone averages (PTAs), spondee thresholds, and speech recognition scores were compared for operated ear against nonoperated ear of VN subjects. The results were subjected to a covariance analysis. VN and ELS patients whose hearing deteriorated from "serviceable" (PTA < or =70 dB hearing level) and speech recognition > or =30%) to nonserviceable status were compared using nonparametric statistics. RESULTS: Progressive hearing loss beyond the rate of change of the normal contralateral ear was evident in all patients. Serviceable hearing dropped from 81% to 43% of patients an average of 4 years following VN. CONCLUSIONS: VN patients have significant hearing deterioration over time in the operated ear. This finding suggests that continued postoperative medical management is necessary for patients undergoing VN.