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Background: Ménière's disease (MD) is a chronic inner ear disorder with a multifactorial etiology. Decreased visualization of the endolymphatic duct (ED) and sac (ES) is thought to be associated with MD, although controversy exists about whether this finding is specific toMD. Recent literature has revealed that two distinct ES pathologies, developmental hypoplasia and epithelial degeneration, can be distinguished in MD using the angular trajectory of the vestibular aqueduct (ATVA) or ED-ES system as a radiographic surrogate marker. It has been suggested that these two subtypes are associated with distinct phenotypical features. However, the clinical differences between the ATVA subtypes require further validation. Research objective: The objective of this study is to investigate whether (1) non-visualization of the ED-ES system is a discriminative radiological feature for MD in a cohort of vertigo-associated pathologies (VAPs) and whether (2) different angular trajectories of the ED-ES systemin MD are associated with distinguishable clinical features. Setting: The study was conducted in the Vertigo Referral Center (Haga Teaching Hospital, The Hague, the Netherlands). Methods: We retrospectively assessed 301 patients (187 definite MD and 114 other VAPs) that underwent 4h-delayed 3D FLAIR MRI. We evaluated (1) the visibility of the ED-ES system between MD and other VAP patients and (2) measured the angular trajectory of the ED-ES system. MD patients were stratified based on the angular measurements into αexit ≤ 120° (MD-120), αexit 120°-140° (MD-intermediate), or αexit ≥ 140° (MD-140). Correlations between ATVA subgroups and clinical parameters were evaluated. Results: Non-visualization of the ED-ES system was more common in definite MD patients compared with other VAPs (P < 0.001). Among definite MD patients, the MD-140 subtype demonstrated a longer history of vertigo (P = 0.006), a higher prevalence of bilateral clinical disease (P = 0.005), and a trend toward a male preponderance (p = 0.053). No significant differences were found between ATVA subgroups regarding the presence or severity of auditory symptoms, or the frequency of vertigo attacks. Conclusion: Non-visualization of the ED-ES systemis significantly associated with MD. Among MD patients with a visible ED-ES system, we demonstrated that the MD-140 subtype is associated with a longer disease duration, a higher prevalence of bilateral MD, and a trend toward a male preponderance. [ABSTRACT FROM AUTHOR]

BackgroundMénière's disease (MD) is a chronic inner ear disorder with a multifactorial etiology. Decreased visualization of the endolymphatic duct (ED) and sac (ES) is thought to be associated with MD, although controversy exists about whether this finding is specific to MD. Recent literature has revealed that two distinct ES pathologies, developmental hypoplasia and epithelial degeneration, can be distinguished in MD using the angular trajectory of the vestibular aqueduct (ATVA) or ED-ES system as a radiographic surrogate marker. It has been suggested that these two subtypes are associated with distinct phenotypical features. However, the clinical differences between the ATVA subtypes require further validation.Research objectiveThe objective of this study is to investigate whether (1) non-visualization of the ED-ES system is a discriminative radiological feature for MD in a cohort of vertigo-associated pathologies (VAPs) and whether (2) different angular trajectories of the ED-ES system in MD are associated with distinguishable clinical features.SettingThe study was conducted in the Vertigo Referral Center (Haga Teaching Hospital, The Hague, the Netherlands).MethodsWe retrospectively assessed 301 patients (187 definite MD and 114 other VAPs) that underwent 4h-delayed 3D FLAIR MRI. We evaluated (1) the visibility of the ED-ES system between MD and other VAP patients and (2) measured the angular trajectory of the ED-ES system. MD patients were stratified based on the angular measurements into αexit ≤ 120° (MD-120), αexit 120°-140° (MD-intermediate), or αexit ≥ 140° (MD-140). Correlations between ATVA subgroups and clinical parameters were evaluated.ResultsNon-visualization of the ED-ES system was more common in definite MD patients compared with other VAPs (P < 0.001). Among definite MD patients, the MD-140 subtype demonstrated a longer history of vertigo (P = 0.006), a higher prevalence of bilateral clinical disease (P = 0.005), and a trend toward a male preponderance (p = 0.053). No significant differences were found between ATVA subgroups regarding the presence or severity of auditory symptoms, or the frequency of vertigo attacks.ConclusionNon-visualization of the ED-ES system is significantly associated with MD. Among MD patients with a visible ED-ES system, we demonstrated that the MD-140 subtype is associated with a longer disease duration, a higher prevalence of bilateral MD, and a trend toward a male preponderance.

Purpose: To describe an anatomical variant that should be consider in patients with hearing loss. Methods: An 8-year-old girl underwent to temporal bone computed tomography for the evaluation of bilateral conductive hearing loss and further assessment of possible enlarged vestibular aqueduct or high jugular bulb on brain magnetic resonance imaging (MRI). Results: CT of temporal bone showed a cystic cavity with bony sclerotic margins extending from the right jugular foramen to the vestibular aqueduct. Bony dehiscence of the jugular foramen with the right carotid canal was also noted. On brain MRI, there was no evidence of enlargement of the endolymphatic duct and sac on T2 thin-section gradient echo sequence. Time of flight MR angiography did not show arterial flow in the cavity. Contrast enhanced MR venography confirmed the presence of a high right jugular bulb with a diverticulum extending into the vestibular aqueduct due to jugular bulb-vestibular aqueduct dehiscence. Conclusion: Knowledge of high jugular bulb-vestibular aqueduct dehiscence is important in the assessment of patients with otologic symptoms such as vertigo, tinnitus and hearing loss. [ABSTRACT FROM AUTHOR]

Objective: We aimed to evaluate the diagnostic performance of some anatomical variables with regard to endolymphatic sac (ES) and duct (ED), measured by non-contrast three-dimensional sampling perfection with application-optimized contrasts using different flip angle evolutions (3D-SPACE) magnetic resonance imaging (MRI), in differentiating vestibular migraine (VM) from unilateral Ménière's disease (MD). Methods: In this study, 81 patients with VM, 97 patients with unilateral MD, and 50 control subjects were enrolled. The MRI-visualized parameters, such as the distance between the vertical part of the posterior semicircular canal and the posterior fossa (MRI-PP distance) and visibility of vestibular aqueduct (MRI-VA), were measured bilaterally. The diagnostic value of the MRI-PP distance and MRI-VA visibility for differentiating VM from unilateral MD was examined. Results: (1) Compared with the VM patients, patients with unilateral MD exhibited shorter MRI-PP distance and poorer MRI-VA visibility. No differences in the MRI-PP distance and MRI-VA visibility were detected between patients with VM and control subjects. (2) No significant interaural difference in the MRI-PP distance and MRI-VA visibility was observed in patients with VM and those with unilateral MD, respectively. (3) Area under the curve (AUC) showed a low diagnostic value for the MRI-PP distance and MRI-VA visibility, respectively, in differentiating between the VM and unilateral MD. Conclusions: Based on non-enhanced MRI-visualized measurement, anatomical variables with regard to the endolymphatic drainage system differed significantly between the patients with VM and those with unilateral MD. Further investigations are needed to improve the diagnostic value of these indices in differentiating VM from unilateral MD. [ABSTRACT FROM AUTHOR]

ObjectiveWe aimed to evaluate the diagnostic performance of some anatomical variables with regard to endolymphatic sac (ES) and duct (ED), measured by non-contrast three-dimensional sampling perfection with application-optimized contrasts using different flip angle evolutions (3D-SPACE) magnetic resonance imaging (MRI), in differentiating vestibular migraine (VM) from unilateral Ménière's disease (MD).MethodsIn this study, 81 patients with VM, 97 patients with unilateral MD, and 50 control subjects were enrolled. The MRI-visualized parameters, such as the distance between the vertical part of the posterior semicircular canal and the posterior fossa (MRI-PP distance) and visibility of vestibular aqueduct (MRI-VA), were measured bilaterally. The diagnostic value of the MRI-PP distance and MRI-VA visibility for differentiating VM from unilateral MD was examined.Results(1) Compared with the VM patients, patients with unilateral MD exhibited shorter MRI-PP distance and poorer MRI-VA visibility. No differences in the MRI-PP distance and MRI-VA visibility were detected between patients with VM and control subjects. (2) No significant interaural difference in the MRI-PP distance and MRI-VA visibility was observed in patients with VM and those with unilateral MD, respectively. (3) Area under the curve (AUC) showed a low diagnostic value for the MRI-PP distance and MRI-VA visibility, respectively, in differentiating between the VM and unilateral MD.ConclusionsBased on non-enhanced MRI-visualized measurement, anatomical variables with regard to the endolymphatic drainage system differed significantly between the patients with VM and those with unilateral MD. Further investigations are needed to improve the diagnostic value of these indices in differentiating VM from unilateral MD.

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. [ABSTRACT FROM AUTHOR]

High signal intensity in the endolymphatic duct (ED) is occasionally observed on magnetic resonance imaging (MRI) in ears that have otological disorders. The signal intensity (SI) in the ED on post-contrast MRI was investigated in subjects with various otological disorders, and the meaning of high SI in the ED was evaluated. 392 patients with otological disorders and 21 controls without otological symptoms underwent 3 T MRI. The SIs of the ED and the cerebellum were measured, the SI ratio (SIR) was calculated, and ears with SIR ≥4 were identified. A high SIR was identified in the ED of 3.7% of ears affected by definite Meniere's disease (dMD), 100% of ears affected by large vestibular aqueduct syndrome (LVAS), and 7.1% of ears with no otological symptoms. On the whole, a significant relationship was found between the existence of vestibular or cochlear EH and the SIR in the ED. The MRI finding of high SI in the ED may indicate the mechanism of inner ear disturbances in ears with otological disorders, especially in those with LVAS, and it may suggest an underlying disorder in some ears in which otological symptoms are not apparent. [ABSTRACT FROM AUTHOR]

Meniere's disease remains enigmatic, and has no treatment with sufficient evidence. The characteristic histopathological finding is endolymphatic hydrops, suggesting either an overproduction or decreased reabsorption of endolymph in the human inner ear. This study presents the first analysis of the vascular plexus around the human endolymphatic duct using micro computed tomography and coherent synchrotron radiation with phase contrast imaging. Using a software program, data were processed by volume-rendering with scalar opacity mapping to create transparent three-dimensional reconstructions. A rich vascular plexus was discovered around the endolymphatic duct that drained into collecting channels, linked to the vestibular venous outflow system. This network is believed to make up the principal route for endolymph outflow, and its associated malfunction may result in endolymphatic hydrops and Meniere's disease. [ABSTRACT FROM AUTHOR]

Background: Recently, 3-Tesla magnetic resonance imaging (MRI) after an intravenous gadolinium injection has been used to describe the endolymphatic space (ELS). Objectives: This study described the histopathological differences between idiopathic sudden sensorineural hearing loss (ISSNHL) and fluctuating sensorineural hearing loss (FSNHL) by examining the ELS. Additionally, the relationship between the affected cochlear and vestibular ELS/total fluid space (TFS) volume ratio and the duration from the onset to MRI in patients with FSNHL were evaluated. Material and methods: This study included 205 individuals without vertigo: 47 controls, 94 with ISSNHL, and 64 with FSNHL. The TFS and ELS volumes were measured and the ELS/TFS volume ratios (%) were evaluated. Results: The cochlear and vestibular ELS/TFS volume ratios of the affected ear in patients with FSNHL were significantly higher than that in those with ISSNHL. There was no correlation between the duration from FSNHL onset to the MRI scan in the affected cochlea and vestibule. Conclusion and significance: There were differences in the form of hearing fluctuation and the extended ELS volume between ISSNHL and FSNHL. ISSNHL cases with severe ELS extension were likely to change to FSNHL. [ABSTRACT FROM AUTHOR]

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The aqeductus vestibuli (aqueduct) is believed to connect to the saccule in embryos and adults. However, in embryos, the saccule and utricle are known to communicate widely to provide a common endolymph space "atrium". Using sagittal histological sections from five embryos (crown-rump length or CRL, 14–21 mm), nine early fetuses (CRL 24–35 mm) and 12 midterm and near-term fetuses (CRL 82–272 mm), we revisited the development and growth of the human ear aqueduct. The atrium took on a thick tube-like appearance as an antero-inferior continuation of the aqueduct, but soon divided into multiple gulfs. Most of the gulfs corresponded to the ampullae of semicircular ducts, while one gulf at the antero-medio-inferior corner corresponded to the future saccule. Notably, in eight of the 14 embryos and early fetuses, the aqueduct ended at the utricle near the primitive ampulla of the anterior (superior) or posterior semicircular duct. Conversely, an embryo of CRL 21 mm was the smallest specimen in which the aqueduct joined the gulf-like saccule. At midterm and near-term, the growing perilymph space separated the aqueduct from the utricle and appeared to push the aqueduct toward the saccule. A topographical change occurred between the embryonic superiorly located utricle and the inferiorly-located saccule to create the antero-posterior arrangement in adults. Consequently, the vestibular end of the aqueduct was most likely to migrate anteriorly from the utricle to the saccule at 6–8 weeks possibly due to differential growth of the endothelium. Previous reconstructions of the embryonic aqueduct might be biased by the adult morphology. [ABSTRACT FROM AUTHOR]

Abstract The development of the inner ear complex cytoarchitecture and functional geometry requires the exquisite coordination of a variety of cellular processes in a temporal manner. At early stages of inner ear development several rounds of cell proliferation in the otocyst promote the growth of the structure. The apoptotic program is initiated in exceeding cells to adjust cell type numbers. Apoptotic cells are cleared by phagocytic cells that recognize the phosphatidylserine residues exposed in the cell membrane thanks to the energy supplied by autophagy. Specific molecular programs determine hair and supporting cell fate, these populations are responsible for the functions of the adult sensory organ: detection of sound, position and acceleration. The neurons that transmit auditory and balance information to the brain are also born at the otocyst by neurogenesis facilitated by autophagy. Cellular senescence participates in tissue repair, cancer and aging, situations in which cells enter a permanent cell cycle arrest and acquire a highly secretory phenotype that modulates their microenvironment. More recently, senescence has also been proposed to take place during vertebrate development in a limited number of transitory structures and organs; among the later, the endolymphatic duct in the inner ear. Here, we review these cellular processes during the early development of the inner ear, focusing on how the most recently described cellular senescence participates and cooperates with proliferation, apoptosis and autophagy to achieve otic morphogenesis and differentiation. Highlights • The development of the inner ear requires the coordination in time and space of strictly regulated cellular processes. • Cellular senescence emerges as a complementary process to apoptosis and autophagy to sculpt the developing inner ear. • Understanding the mechanisms of otic cell specification and survival should help the development of regenerative strategies. [ABSTRACT FROM AUTHOR]

The lower stations of the auditory system display a complex anatomy. The inner ear labyrinth is composed of several interconnecting membranous structures encased in cavities of the temporal bone, and the cerebellopontine angle contains fragile structures such as meningeal folds, the choroid plexus (CP), and highly variable vascular formations. For this reason, most histological studies of the auditory system have either focused on the inner ear or the CNS by physically detaching the temporal bone from the brainstem. However, several studies of neuroimmune interactions have pinpointed the importance of structures such as meninges and CP; in the auditory system, an immune function has also been suggested for inner ear structures such as the endolymphatic duct (ED) and sac. All these structures are thin, fragile, and have complex 3D shapes. In order to study the immune cell populations located on these structures and their relevance to the inner ear and auditory brainstem in health and disease, we obtained a clarified-decalcified preparation of the rat hindbrain still attached to the intact temporal bone. This preparation may be immunolabeled using a clearing protocol (based on iDISCO+) to show location and functional state of immune cells. The observed macrophage distribution suggests the presence of CP-mediated communication pathways between the inner ear and the cochlear nuclei. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

In order to investigate the genetic causes of hearing loss in a Chinese proband with nonsyndromic hearing loss and enlarged vestibular aqueduct (EVA), we conducted clinical and genetic evaluations in a deaf proband and her parents with normal hearing. 20 exons and flanking splice sites of the SLC26A4 gene were screened for pathogenic mutations by PCR amplification and bidirectional sequencing. As a control, a group of 400 healthy newborns from the same ethnic background were subjected to SLC26A4 gene screening using the same method. The proband harbored two mutations in the SLC26A4 gene in the form of compound heterozygosity. She was found to be heterozygous for a novel mutation c.574delC (p.Leu192Ter) in exon 5 and for the known mutation c.919-2A>G(c.IVS7-2A>G). Her mother was a heterozygous carrier of the c.919-2A>G mutation, and her father was a heterozygous carrier of the c.574delC and therefore co-segregated with the genetic disease. The c.574delC mutation was absent in 400 healthy newborns. The frameshift mutation causes the leucine (Leu) at amino acid position 192 to become a termination codon, leading to termination of protein sequence coding. This study demonstrates that the novel frameshift mutation c.574delC (p.Leu192Ter) in compound heterozygosity with c.919- 2A>G in the SLC26A4 gene is the main cause of deafness in a family. Our study will expand the spectrum of known SLC26A4 mutations in the Chinese population, providing more information on genetic counseling, and diagnosis in hearing loss with EVA. [ABSTRACT FROM AUTHOR]

Abstract The isolated anatomical position and blood-labyrinth barrier hampers systemic drug delivery to the mammalian inner ear. Intratympanic placement of drugs and permeation via the round- and oval window are established methods for local pharmaceutical treatment. Mechanisms of drug uptake and pathways for distribution within the inner ear are hard to predict. The complex microanatomy with fluid-filled spaces separated by tight- and leaky barriers compose various compartments that connect via active and passive transport mechanisms. Here we provide a review on the inner ear architecture at light- and electron microscopy level, relevant for drug delivery. Focus is laid on the human inner ear architecture. Some new data add information on the human inner ear fluid spaces generated with high resolution microcomputed tomography at 15 μm resolution. Perilymphatic spaces are connected with the central modiolus by active transport mechanisms of mesothelial cells that provide access to spiral ganglion neurons. Reports on leaky barriers between scala tympani and the so-called cortilymph compartment likely open the best path for hair cell targeting. The complex barrier system of tight junction proteins such as occludins, claudins and tricellulin isolates the endolymphatic space for most drugs. Comparison of relevant differences of barriers, target cells and cell types involved in drug spread between main animal models and humans shall provide some translational aspects for inner ear drug applications. Highlights • Inner ear fluid compartments are separated by barriers with varying leakiness. • Oval and round window are permeable for several chemical compounds. • Endolymph compartment is tightly sealed and vulnerable to sensory epithelium damage. • Hair cells containing Cortilymph is permeable to perilymph via the basilar membrane. • Active transport mechanisms by mesothelial cells provide a pathway to neurons. [ABSTRACT FROM AUTHOR]

Considerable diversity has been documented in most sensory systems of elasmobranchs (sharks, rays, and skates); however, relatively little is known about morphological variation in the auditory system of these fishes. Using magnetic resonance imaging (MRI), the inner ear structures of 26 elasmobranchs were assessed in situ. The inner ear end organs (saccule, lagena, utricle, and macula neglecta), semi-circular canals (horizontal, anterior, and posterior), and endolymphatic duct were compared using phylogenetically-informed, multivariate analyses. Inner ear variation can be characterised by three primary axes that are influenced by diet and habitat, where piscivorous elasmobranchs have larger inner ears compared to non-piscivorous species, and reef-associated species have larger inner ears than oceanic species. Importantly, this variation may reflect differences in auditory specialisation that could be tied to the functional requirements and environmental soundscapes of different species., (© 2023. The Author(s).)

Plastic bronchitis is a poorly understood and uncommon diagnosis, arising from multiple etiologies. Traditional treatment consists of steroids and vasodilators, with thoracic duct embolization emerging as a new procedural therapy. Herein, abnormal lymphatic vessels were noted on lymphangiography in an adult patient with debilitating plastic bronchitis, but anterograde lymphatic access was not feasible due to the patient's morbid obesity and non-visualization of retroperitoneal lymphatics. After trans-venous thoracic duct access could not be established, direct transcervical thoracic duct access was performed. A thoracic duct stent-graft was placed, excluding the abnormal bronchial lymphatics and maintaining physiologic anterograde flow through the central lymphatics. At three-month follow-up, the patient's condition had resolved. [ABSTRACT FROM AUTHOR]

Objectives: A case-controlled imaging study demonstrated that saccular hydrops was specific to Meniere's disease (MD), but only present in a subset of patients. Here, we compared patients with definite MD, vertigo and sensorineural hearing loss (SNHL) to elucidate the relationship between saccular hydrops and extent of SNHL.Methods: In this prospective study, we performed 3D-FLAIR sequences between 4.5 and 5.5 h after contrast media injection in patients with MD (n=20), SNHL (n=20), vertigo (n=20) and 30 healthy subjects. Two radiologists independently graded saccular hydrops. ROC analysis was performed to determine the hearing loss threshold to differentiate patients with saccular hydrops.Results: Saccular hydrops was found in 11 of 20 MD patients, 10 of 20 SNHL patients and in none of the vertigo patients and healthy subjects. In SNHL patients, 45 dB was the threshold above which there was a significant association with saccular hydrops, with sensitivity of 100 % and specificity of 90 %. In MD patients, 40 dB was the threshold above which there was a significant association with saccular hydrops, with sensitivity of 100 % and specificity of 44 %.Conclusions: Our results indicate saccular hydrops as a feature of worse than moderate SNHL rather than MD itself.Key Points: • MRI helps clinicians to assess patients with isolated low-tone sensorineural hearing loss. • Saccular hydrops correlates with sensorineural hearing loss at levels above 40 dB. • Vertigo patients without sensorineural hearing loss do not have saccular hydrops. • Saccular hydrops is described in patients without clinical diagnosis of Meniere's disease. [ABSTRACT FROM AUTHOR]

The article focuses on the study on the electrocochleography of Ménière's disease (MD) patients who received Endolymphatic sac decompression (ESD) surgery. Topics discussed include mastoidectomy performed to remove bony wall between sigmoid sinus and posterior semicircular canal, indication of endolymphatic pressure relief, and treatment of endolymphatic hydrops.

Diseases that cause hearing loss and/or vertigo in humans such as Meniere's disease are often studied using animal models. The volume of endolymph within the inner ear varies with these diseases. Here, we used a mouse model of increased endolymph volume, endolymphatic hydrops, to develop a computer-aided objective approach to measure endolymph volume from images collected in vivo using optical coherence tomography. The displacement of Reissner's membrane from its normal position was measured in cochlear cross sections. We validated our computer-aided measurements with manual measurements and with trained observer labels. This approach allows for computer-aided detection of endolymphatic hydrops in mice, with test performance showing sensitivity of 91% and specificity of 87% using a running average of five measurements. These findings indicate that this approach is accurate and reliable for classifying endolymphatic hydrops and quantifying endolymph volume. [ABSTRACT FROM AUTHOR]

Objective: The purpose of the study was to investigate the difference in the responses of endolymphatic hydrostatic pressure to isoproterenol, β-adrenergic receptor agonist, between pars superior and pars inferior.Methods: The hydrostatic pressure of endolymph and perilymph and endolymphatic potential in the ampulla and the cochlea during the intravenous administration of isoproterenol were recorded using a servo-null system in guinea pigs.Results: The hydrostatic pressure of endolymph and perilymph in the ampulla and cochlea was similar in magnitude. Isoproterenol significantly increased hydrostatic pressure of ampullar and cochlear endolymph and perilymph with no change in the ampullar endolymphatic potential and endocochlear potential, respectively. The isoproterenol-induced maximum change of endolymphatic hydrostatic pressure in ampulla was significantly (p<0.01) smaller than that in the cochlea. In ears with an obstructed endolymphatic sac, the action of isoproterenol on endolymphatic hydrostatic pressure in the ampulla disappeared like that in the cochlea.Conclusion: Isoproterenol elevates endolymphatic hydrostatic pressure in different manner between the vestibule and the cochlea. [ABSTRACT FROM AUTHOR]

Enlarged vestibular aqueduct (EVA) syndrome is a common congenital inner ear malformation characterized by a vestibular aqueduct with a diameter larger than 1.5 mm, mixed or sensorineural hearing loss that ranges from mild to profound, and vestibular disorders that may be present with a range from mild imbalance to episodic objective vertigo. In our study, we present the case of a patient with unilateral enlarged vestibular aqueduct and bilateral endolymphatic hydrops (EH). EH was confirmed through anamnestic history and audiological exams; EVA was diagnosed using high-resolution CT scans and MRI images. Therapy included intratympanic infusion of corticosteroids with a significant hearing improvement, more evident in the ear contralateral to EVA. Although most probably unrelated, EVA and EH may present with similar symptoms and therefore the diagnostic workup should always include the proper steps to perform a correct diagnosis. Association between progression of hearing loss and head trauma in patients with a diagnosis of EVA syndrome is still uncertain; however, these individuals should be advised to avoid activities that increase intracranial pressure to prevent further hearing deterioration. Intratympanic treatment with steroids is a safe and well-tolerated procedure that has demonstrated its efficacy in hearing, tinnitus, and vertigo control in EH. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: To measure the volume of the endolymph drainage system in temporal bone specimens with Ménière disease, as compared with specimens with endolymphatic hydrops without vestibular symptoms and with nondiseased specimens STUDY DESIGN: Comparative human temporal bone analysis.Methods: We generated three-dimensional models of the vestibular aqueduct, endolymphatic sinus and duct, and intratemporal portion of the endolymphatic sac and calculated the volume of those structures. We also measured the internal and external aperture of the vestibular aqueduct, as well as the opening (if present) of the utriculoendolymphatic (Bast's) valve and compared the measurements in our three study groups.Results: The volume of the vestibular aqueduct and of the endolymphatic sinus, duct, and intratemporal endolymphatic sac was significantly lower in the Ménière disease group than in the endolymphatic hydrops group (P <.05). The external aperture of the vestibular aqueduct was also smaller in the Ménière disease group. Bast's valve was open only in some specimens in the Ménière disease group.Conclusions: In temporal bones with Ménière disease, the volume of the vestibular aqueduct, endolymphatic duct, and intratemporal endolymphatic sac was lower, and the external aperture of the vestibular aqueduct was smaller as compared with bones from donors who had endolymphatic hydrops without vestibular symptoms and with nondiseased bones. The open status of the Bast's valve in the Ménière disease group could be secondary to higher retrograde endolymph pressures caused by smaller drainage systems. These anatomic findings could correlate with the reason that some patients with hydrops develop clinical symptoms, whereas others do not.Level Of Evidence: N/A Laryngoscope, 127:E170-E175, 2017. [ABSTRACT FROM AUTHOR]

The aim of this study was to observe morphological changes of the cultured otocysts isolated from various stages of the chick embryo. Isolated otocysts were dissected from embryonic day, E2.5-4.5 of incubation (HH stage 16-26) according to stages of developing inner ear. Morphology of the chick otocyst exhibited an ovoid shape. The width and height of the otocyst were 0.2 mm and 0.3 mm, respectively. Elongation of a tube-like structure, the endolymphatic duct, was found at the dorsal aspect of the otocyst. The cultured otocyst is lined by the otic epithelium and surrounding periotic mesenchymal cells started to migrate outwards the lateral aspect of such epithelium. Notably, the acoustic-vestibular ganglion (AVG) was observed at the ventrolateral aspect of the otocyst. Appearance of AVG in vitro can be applied for studying chemical-induced ototoxicity and sensorineural hearing loss. It was concluded that the organcultured otocyst of the chick embryo could be used as a model to study sensory organ development of avian inner ear. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Competing Interests: Conflicts of interest: MEN reports stock ownership in 3M, Amgen, Cardinal Health, Johnson & Johnson, Medtronic, and Pfizer. No conflicts of interests to declare for the remaining author.

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. [ABSTRACT FROM AUTHOR]

Objective: To observe any changes in stria vascularis and cochlear hair cells in patients with syphilis.Materials and Methods: We examined 13 human temporal bone samples from 8 patients with syphilis (our syphilis group), as well as 12 histopathologically normal samples from 9 age-matched patients without syphilis (our control group). We compared, between the two groups, the mean area of the stria vascularis (measured with conventional light microscopy connected to a personal computer) and the mean percentage of cochlear hair cell loss (obtained from cytocochleograms).Results: In our syphilis group, only 1 (7.7%) of the 13 samples had precipitate in the endolymphatic or perilymphatic spaces; 8 (61.5%) of the samples revealed the presence of endolymphatic hydrops (4 cochlear, 4 saccular). The mean area of the stria vascularis did not significantly differ, in any turn of the cochlea, between the 2 groups (P>0.1). However, we did find significant differences between the 2 groups in the mean percentage of outer hair cells in the apical turn (P<0.026) and in the mean percentage of inner hair cells in the basal (P=0.001), middle (P=0.004), and apical (P=0.018) turns. In 7 samples in our syphilis group, we observed either complete loss of the organ of Corti or a flattened organ of Corti without any cells in addition to the absence of both outer and inner hair cells.Conclusion: In this study, syphilis led either to complete loss of the organ of Corti or to significant loss of cochlear hair cells, in addition to cochleosaccular hydrops. But the area of the stria vascularis did not change. [ABSTRACT FROM AUTHOR]

Ménière’s attack has been shown to temporarily alter the vestibuloocular reflex (VOR). A patient with unilateral Ménière’s disease was serially evaluated with the video Head Impulse Test during single, untreated episodes of acute vertigo. Spontaneous nystagmus activity was concurrently recorded in order to establish the three typical phases of Ménière’s attack (irritative, paralytic, and recovery) and correlate them with VOR performance. The onset of attack was associated with a quick change in VOR gain on the side of the affected ear. While a rapidly progressive reduction of the VOR was evident at the paralytic nystagmus phase, in the recovery phase the VOR gain returned to normal and the direction of the previous nystagmus reversed. The membrane rupture potassium intoxication theory provides a good foundation with which to explain these dynamic VOR changes and the observed triphasic direction behavior of the spontaneous nystagmus. We additionally postulated that endolymphatic fluid displacement could have a synergic effect during the earliest phase of attack. [ABSTRACT FROM AUTHOR]

Purpose: During vestibular schwannoma surgery there is a risk of endolymphatic duct and sac injury, which may cause a loss or a deterioration of hearing. The goal of the study was to evaluate the empirical utility of presurgical planning using CT with the bone window for the hearing preservation surgery. Methods: The study was performed on 14 human temporal bones. CT scans with the bone window were obtained in the standard position. Safe drilling line was evaluated and after that drilling distances were analysed: the lateral drilling distance, total length of internal acoustic meatus, drilled length of internal acoustic meatus. After this, a surgical exposure was performed, using size of a drill tip as measuring scale. The dura was excised and endolymphatic duct was injected with a latex. Revision of the internal acoustic meatus was performed with the use of a microscope. Results: Mean results of safe drilling coefficients were: lateral drilling distance: 10 ± 2 mm, total length of internal acoustic meatus: 9 ± 2 mm, drilled length of internal acoustic meatus: 7 ± 2 mm. In all cases, no endolymphatic duct injury was observed. Conclusions: Preoperative radiological planning using the safe drilling coefficients is of value for the hearing preservation surgery in vestibular schwannoma. The size of the drilling tip may be used as an intraoperative measuring scale during this procedure. However, CT with a bone window is a necessary tool for the purposes of this procedure. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

There are a variety of techniques available to investigate endolymph dynamics, primarily seeking to understand the cause of endolymphatic hydrops. Here we have taken the novel approach of injecting, via a glass micropipette, fluorescein isothiocyanate–dextran (FITC-dex) and artificial endolymph into scala media of anaesthetized guinea pigs, with subsequent imaging of the inner ear using Light Sheet Fluorescence Microscopy (LSFM) as a means to obtain highly resolved 3D visualization of fluid movements. Our results demonstrate endolymph movement into the utricle, semicircular canals and endolymphatic duct and sac when more than 2.5 μl of fluid had been injected into scala media, with no apparent movement of fluid into the perilymphatic compartments. There was no movement of endolymph into these compartments when less than 2.5 μl was injected. The remarkable uptake of the FITC-dex into the endolymphatic duct, including an absorption into the periductal channels surrounding the endolymphatic duct, highlights the functional role this structure plays in endolymph volume regulation. [ABSTRACT FROM AUTHOR]

Objectives: Melanin pigmentation is present in the human inner ear. In this study, we quantify the melanin pigmentation in the vestibular system and examine racial differences of vestibular melanin pigmentation using human cadaveric temporal bone specimens.Study Design: Basic research.Setting: Laboratory.Subjects and Methods: Light microscopy was used to examine specimens from 40 left temporal bones from the Johns Hopkins Human Temporal Bone Collection. Color images of (1) ampulla of the horizontal canal, (2) utricular wall, (3) endolymphatic duct, and (4) posterior ampullary nerve as it enters the posterior canal were acquired with a digital camera attached to the microscope and image acquisition software. Acquired images of each anatomic area of interest were processed offline through ImageJ. Melanin content was then compared according to ethnicity, age, sex, and location.Results: Fifteen African American and 25 Caucasian specimens were analyzed. Mean age was 68.8 years. African American specimens had a significantly greater amount of pigment at all 4 sampled locations as compared with Caucasian specimens (P < .01). Between sexes, there was a statistically significant difference (P < .05) at the posterior ampullary nerve, with males having more than females. Melanin content was not associated with age.Conclusions: There is greater melanin pigmentation within the vestibular system of African Americans than in Caucasians, similar to what has been described in the cochlea. Racial differences in vestibular physiologic function have been observed in the literature and may be explained by differences in melanin pigmentation. [ABSTRACT FROM AUTHOR]

The utriculo-endolymphatic valve (UEV) has an uncertain function, but its opening and closure have been predicted to maintain a constant endolymphatic pressure within the semicircular canals (SCCs) and the utricle of the inner ear. Here, the study׳s aim was to examine the role of the UEV in regulating the capabilities of the 3 SCCs in sensing angular acceleration by using the finite element method. The results of the developed model showed endolymphatic flow and cupula displacement patterns in good agreement with previous experiments. Moreover, the open valve was predicted to permit endolymph exchange between the 2 parts of the membranous labyrinth during head rotation and, in comparison to the closed valve, to result in a reinforced endolymph flow in the utricle and an enhanced or weakened cupula deflection. Further, the model predicted an increase in the size of the orifice would result in greater endolymph exchange and thereby to a greater impact on cupula deflection. The model findings suggest the UEV plays a crucial role in the preservation of inner ear sensory function. [ABSTRACT FROM AUTHOR]

Volume homeostasis of the cochlear endolymph depends on radial and longitudinal endolymph movements (LEMs). LEMs measured in vivo have been exclusively recognized under physiologically challenging conditions, such as experimentally induced alterations of perilymph osmolarity or endolymph volume. The regulatory mechanisms that adjust LEMs to the physiological requirements of endolymph volume homeostasis remain unknown. Here, we describe the formation of an aquaporin (AQP)-based 'water shunt' during the postnatal development of the mouse cochlea and its regulation by different triggers. The final complementary expression pattern of AQP5 (apical membrane) and AQP4 (basolateral membrane) in outer sulcus cells (OSCs) of the cochlear apex is acquired at the onset of hearing function (postnatal day (p)8-p12). In vitro, hyperosmolar perfusion of the perilymphatic fluid spaces or the administration of the muscarinic agonist pilocarpine in cochlear explants (p14) induced the translocation of AQP5 channel proteins into the apical membranes of OSCs. AQP5 membrane translocation was blocked by the muscarinic antagonist atropine. The muscarinic M3 acetylcholine (ACh) receptor (M3R) was identified in murine OSCs via mRNA expression, immunolabeling, and in vitro binding studies using an M3R-specific fluorescent ligand. Finally, the water shunt elements AQP4, AQP5, and M3R were also demonstrated in OSCs of the human cochlea. The regulation of the AQP4/AQP5 water shunt in OSCs of the cochlear apex provides a molecular basis for regulated endolymphatic volume homeostasis. Moreover, its dysregulation or disruption may have pathophysiologic implications for clinical conditions related to endolymphatic hydrops, such as Ménière's disease. [ABSTRACT FROM AUTHOR]

In the last 4 years the authors observed a trend that correcting vitamin D deficiency in newly diagnosed cases of Meniere's disease decreased the necessity of the ablative therapy with intratympanic gentamicin. According to their hypothesis, vitamin D supplementation may indeed have a beneficial effect in Meniere's disease if the symptoms are caused by a local postviral autoimmune reaction. Vitamin D has a strong immunomodulatory role, one of which is the regulation of the expression of pro-inflammatory mediators. The authors suggest further epidemiological studies to decide if there is a connection between vitamin D deficiency and Meniere's disease. [ABSTRACT FROM AUTHOR]