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European Annals of Otorhinolaryngology,... Apr 2015Endolymphatic pressure measurement is of interest both to researchers in the physiology and pathophysiology of hearing and ENT physicians dealing with Menière's disease... (Review)
Review
Endolymphatic pressure measurement is of interest both to researchers in the physiology and pathophysiology of hearing and ENT physicians dealing with Menière's disease or similar conditions. It is generally agreed that endolymphatic hydrops is associated with Menière's disease and is accompanied by increased hydrostatic pressure. Endolymphatic pressure, however, cannot be measured precisely without endangering hearing, making the association between hydrops and increased endolymphatic pressure difficult to demonstrate. Several integrated in vivo models have been developed since the 1960s, but only a few allow measurement of endolymphatic hydrostatic pressure. Models associating measurement of hydrostatic pressure and endolymphatic potential and assessment of cochlear function are of value to elucidate the pathophysiology of endolymphatic hydrops. The present article presents the main types of models and discusses their respective interest.
Topics: Ear, Inner; Endolymph; Endolymphatic Hydrops; Evidence-Based Medicine; Hearing Loss; Humans; Hydrostatic Pressure; Meniere Disease; Vestibular Function Tests
PubMed: 25467202
DOI: 10.1016/j.anorl.2014.05.004 -
European Annals of Otorhinolaryngology,... Jan 2020Endolymphatic hydrops features excess endolymph in the membranous labyrinth, and is a marker of Menière's disease. Between the early 1980s and late 2000s, MRI in... (Review)
Review
Endolymphatic hydrops features excess endolymph in the membranous labyrinth, and is a marker of Menière's disease. Between the early 1980s and late 2000s, MRI in Menière's disease aimed purely to rule out tumor or malformation as differential diagnoses for the pressure disorder. Progress in high-resolution MRI, however, now enables excess endolymph to be visualized in the membranous labyrinth, differentiating saccule and utricle in Menière's disease and in other clinical presentations such as cochleovestibular schwannoma. More recently, non-visibility of the saccule was demonstrated in a subgroup of Menière's disease patients, and utricle atelectasis in case of uni- or bilateral vestibular areflexia. Endolymph quantification remains highly controversial in terms of grading approach, but a simple semiology based on excess or deficient visualization of endolymph according to the compartment sheds light on the pathophysiological mechanisms of cochleovestibular disorder and may in future allow effective monitoring of medical and surgical treatment.
Topics: Endolymph; Humans; Magnetic Resonance Imaging; Meniere Disease; Saccule and Utricle
PubMed: 31831378
DOI: 10.1016/j.anorl.2019.11.001 -
F1000Research 2018Ménière's disease (MD) represents a heterogeneous group of relatively rare disorders with three core symptoms: episodic vertigo, tinnitus, and sensorineural hearing... (Review)
Review
Ménière's disease (MD) represents a heterogeneous group of relatively rare disorders with three core symptoms: episodic vertigo, tinnitus, and sensorineural hearing loss involving 125 to 2,000 Hz frequencies. The majority of cases are considered sporadic, although familial aggregation has been recognized in European and Korean populations, and the search for familial MD genes has been elusive until the last few years. Detailed phenotyping and cluster analyses have found several clinical predictors for different subgroups of patients, which may indicate different mechanisms, including genetic and immune factors. The genes associated with familial MD are , , , , , and . At least two mechanisms have been involved in MD: (a) a pro-inflammatory immune response mediated by interleukin-1 beta (IL-1β), tumor necrosis factor alpha (TNFα), and IL-6, and (b) a nuclear factor-kappa B (NF-κB)-mediated inflammation in the carriers of the single-nucleotide variant rs4947296. It is conceivable that microbial antigens trigger inflammation with release of pro-inflammatory cytokines at different sites within the cochlea, such as the endolymphatic sac, the stria vascularis, or the spiral ligament, leading to fluid imbalance with an accumulation of endolymph. Computational integration of clinical and "omics" data eventually should transform the management of MD from "one pill fits all" to precise patient stratification and a personalized approach. This article lays out a proposal for an algorithm for the genetic diagnosis of MD. This approach will facilitate the identification of new molecular targets for individualized treatment, including immunosuppressant and gene therapy, in the near future.
Topics: Genetic Testing; Genetic Therapy; Hearing Loss, Sensorineural; Humans; Immunosuppressive Agents; Meniere Disease; Molecular Targeted Therapy; Precision Medicine
PubMed: 30430003
DOI: 10.12688/f1000research.14417.1 -
Evolution of Endolymph Secretion and Endolymphatic Potential Generation in the Vertebrate Inner Ear.Brain, Behavior and Evolution 2018The ear of extant vertebrates reflects multiple independent evolutionary trajectories. Examples include the middle ear or the unique specializations of the mammalian... (Review)
Review
The ear of extant vertebrates reflects multiple independent evolutionary trajectories. Examples include the middle ear or the unique specializations of the mammalian cochlea. Another striking difference between vertebrate inner ears concerns the differences in the magnitude of the endolymphatic potential. This differs both between the vestibular and auditory part of the inner ear as well as between the auditory periphery in different vertebrates. Here we provide a comparison of the cellular and molecular mechanisms in different endorgans across vertebrates. We begin with the lateral line and vestibular systems, as they likely represent plesiomorphic conditions, then review the situation in different vertebrate auditory endorgans. All three systems harbor hair cells bathed in a high (K+) environment. Superficial lateral line neuromasts are bathed in an electrogenically maintained high (K+) microenvironment provided by the complex gelatinous cupula. This is associated with a positive endocupular potential. Whether this is a special or a universal feature of lateral line and possibly vestibular cupulae remains to be discovered. The vestibular system represents a closed system with an endolymph that is characterized by an enhanced (K+) relative to the perilymph. Yet only in land vertebrates does (K+) exceed (Na+). The endolymphatic potential ranges from +1 to +11 mV, albeit we note intriguing reports of substantially higher potentials of up to +70 mV in the cupula of ampullae of the semicircular canals. Similarly, in the auditory system, a high (K+) is observed. However, in contrast to the vestibular system, the positive endolymphatic potential varies more substantially between vertebrates, ranging from near zero mV to approximately +100 mV. The tissues generating endolymph in the inner ear show considerable differences in cell types and location. So-called dark cells and the possibly homologous ionocytes in fish appear to be the common elements, but there is always at least one additional cell type present. To inspire research in this field, we propose a classification for these cell types and discuss potential evolutionary relationships. Their molecular repertoire is largely unknown and provides further fertile ground for future investigation. Finally, we propose that the ultimate selective pressure for an increased endolymphatic potential, as observed in mammals and to a lesser extent in birds, is specifically to maintain the AC component of the hair-cell receptor potential at high frequencies. In summary, we identify intriguing questions for future directions of research into the molecular and cellular basis of the endolymph in the different compartments of the inner ear. The answers will provide important insights into evolutionary and developmental processes in a sensory organ essential to many species, including humans.
Topics: Animals; Ear, Inner; Electrophysiological Phenomena; Endolymph; Vertebrates
PubMed: 30415265
DOI: 10.1159/000494050 -
Neurochemistry International Jun 2020Glia use multiple mechanisms to mediate potassium fluxes that support neuronal function. In addition to changes in potassium levels within synapses, these ions are... (Review)
Review
Glia use multiple mechanisms to mediate potassium fluxes that support neuronal function. In addition to changes in potassium levels within synapses, these ions are dynamically dispersed through the interstitial parenchyma, perivascular spaces, leptomeninges, cerebrospinal fluid, choroid plexus, blood, vitreous, and endolymph. Neural circuits drive diversity in the glia that buffer potassium and this is reciprocal. Glia mediate buffering of potassium locally at glial-neuronal interfaces and via widespread networked connections. Control of potassium levels in the central nervous system is mediated by mechanisms operating at various loci with complexity that is difficult to model. However, major components of networked glial buffering are known. The role that potassium buffering plays in homeostasis of the CNS underlies some pathologic phenomena. An overview of potassium fluxes in the CNS is relevant for understanding consequences of pathogenic sequence variants in genes that encode potassium buffering proteins. Potassium flows in the CNS are described as follows: K1, the coordinated potassium fluxes within the astrocytic cradle around the synapse; K2, temporary storage of potassium within astrocytic processes in proposed microdomains; K3, potassium fluxes between oligodendrocytes and astrocytes; K4, potassium fluxes between astrocytes; K5, astrocytic potassium flux mediation of neurovasular coupling; K6, CSF delivery of potassium to perivascular spaces with dispersion to interstitial fluid between astrocytic endfeet; K7, astrocytic delivery of potassium to CSF and K8, choroid plexus (modified glia) regulation of potassium at the blood-CSF barrier. Components, mainly potassium channels, transporters, connexins and modulators, and the pathogenic sequence variants of their genes with the associated diseases are described.
Topics: Animals; Astrocytes; Central Nervous System; Homeostasis; Humans; Neuroglia; Oligodendroglia; Potassium
PubMed: 32194142
DOI: 10.1016/j.neuint.2020.104727 -
Cellular Physiology and Biochemistry :... 2011Pendrin (SLC26A4), a Cl(-)/anion exchanger encoded by the gene PDS, is highly expressed in the kidney, thyroid and inner ear epithelia and is essential for bicarbonate... (Review)
Review
Pendrin (SLC26A4), a Cl(-)/anion exchanger encoded by the gene PDS, is highly expressed in the kidney, thyroid and inner ear epithelia and is essential for bicarbonate secretion/chloride reabsorption, iodide accumulation and endolymph ion balance, respectively. The molecular mechanisms controlling pendrin activity in renal, thyroid and inner ear epithelia have been the subject of recent studies. The effects of ambient pH, the hormone aldosterone and the peptide uroguanylin (UGN; the "intestinal natriuretic hormone"), known modulators of electrolyte balance, on transcription of the pendrin gene, have been investigated. Luciferase reporter plasmids containing different length fragments of the human PDS (hPDS) promoter were transfected into renal HEK293, thyroid LA2, and inner ear VOT36 epithelial cells. Acidic pH decreased and alkaline pH increased hPDS promoter activity in transfected HEK293 and VOT36, but not in LA2 cells. Aldosterone reduced hPDS promoter activity in HEK293 but had no effect in LA2 and VOT36 cells. These pH and aldosterone-induced effects on the hPDS promoter occurred within 96-bp and 89-bp regions, respectively, which likely contain distinct response elements to these modulators. Injection of UGN into mice resulted in decreased pendrin mRNA and protein expression in the kidney. Exposure of transfected HEK293 to UGN decreased hPDS promoter activity. The findings provided evidence for the presence of a UGN response element within the 96-bp region overlapping with the pH response element on the hPDS promoter. Pendrin is also expressed in airway epithelium. The cytokins interleukin 4 (IL-4) and interleukin-13 (IL-13), known regulators of airway surface function, have been shown to increase hPDS promoter activity by a STAT6-dependent mechanism. In conclusion, systemic pH, the hormone aldosterone, and the peptide UGN influence renal tubular pendrin gene expression and, perhaps, pendrin-mediated Cl(-)/HCO(3)(-) exchange at the transcriptional level. Pendrin-driven anion transport in the endolymph and at the airway surface may be regulated transcriptionally by systemic pH and IL-3/IL-4, respectively. The distinct response elements and the corresponding transcription factors mediating the effect of these modulators on the PDS promoter remain to be identified and characterized.
Topics: Animals; Ear, Inner; Gene Expression Regulation; Humans; Ion Transport; Kidney; Membrane Transport Proteins; Promoter Regions, Genetic; Sulfate Transporters; Thyroid Gland
PubMed: 22116353
DOI: 10.1159/000335100 -
NeuroImage. Clinical 2021The origin of vestibular symptoms in patients with vestibular schwannoma (VS) is uncertain. We used intratympanic gadolinium-enhanced magnetic resonance imaging (MRI) to...
OBJECTIVE
The origin of vestibular symptoms in patients with vestibular schwannoma (VS) is uncertain. We used intratympanic gadolinium-enhanced magnetic resonance imaging (MRI) to confirm the labyrinthine lesions in patients with VS and to explore the features of endolymphatic hydrops (EH) in these patients.
METHODS
In total, 66 patients diagnosed with unilateral VS were enrolled in this study and underwent intratympanic gadolinium-enhanced MRI. The borders of the vestibule and endolymph were mapped on the axial MRI images, and the area and volume of vestibule and endolymph were automatically calculated using Osirix software, and the area and volume percentage of vestibular endolymph were obtained.
RESULTS
The area and volume percentages of vestibular endolymph on the affected side were significantly larger than those on the healthy side (both p < 0.001). Using Kendall's W test, we found that the area and volume percentages of vestibular endolymph on the affected side were consistent (p < 0.001), but the consistency was moderate (k = 0.574). The healthy side was also consistent (p < 0.001), and the degree of consistency was moderate (k = 0.444). We used 19.1% as the cut-off point to distinguish the presence or absence of vestibular EH; that is, the volume percentage of vestibular endolymph that was more than 19.1% were defined as the subgroup with hydrops, while the subgroup without hydrops included patients with a baseline level below 19.1%. No volume classification for vestibular EH was proposed. Based on this standard, 11/66 (16.7%) of the patients with VS in this study had vestibular EH.
CONCLUSIONS
The volume percentage of the vestibular endolymph was more accurate than the area percentage for assessing vestibular EH. Using 19.1% as the cut-off point to distinguish the presence or absence of vestibular EH, we found that 16.7% of patients with VS had varying degrees of vestibular EH. We believe that the vestibular symptoms in patients with VS may originate from the peripheral lesions.
Topics: Contrast Media; Endolymphatic Hydrops; Gadolinium; Humans; Magnetic Resonance Imaging; Neuroma, Acoustic; Vestibule, Labyrinth
PubMed: 33866299
DOI: 10.1016/j.nicl.2021.102656 -
Annals of the New York Academy of... Apr 2015Individuals working next to strong static magnetic fields occasionally report disorientation and vertigo. With the increasing strength of magnetic fields used for... (Review)
Review
Individuals working next to strong static magnetic fields occasionally report disorientation and vertigo. With the increasing strength of magnetic fields used for magnetic resonance imaging studies, these reports have become more common. It was recently learned that humans, mice, and zebrafish all demonstrate behaviors consistent with constant peripheral vestibular stimulation while inside a strong, static magnetic field. The proposed mechanism for this effect involves a Lorentz force resulting from the interaction of a strong static magnetic field with naturally occurring ionic currents flowing through the inner ear endolymph into vestibular hair cells. The resulting force within the endolymph is strong enough to displace the lateral semicircular canal cupula, inducing vertigo and the horizontal nystagmus seen in normal mice and in humans. This review explores the evidence for interactions of magnetic fields with the vestibular system.
Topics: Animals; Electromagnetic Fields; Humans; Magnetic Resonance Imaging; Vertigo; Vestibule, Labyrinth
PubMed: 25735662
DOI: 10.1111/nyas.12702