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Lin Chuang Er Bi Yan Hou Tou Jing Wai... Sep 2020Perilymphatic fistula(PLF) is defined as an abnormal communication between the fluid(perilymph) -filled space of the inner ear and the air-filled space of the... (Review)
Review
Perilymphatic fistula(PLF) is defined as an abnormal communication between the fluid(perilymph) -filled space of the inner ear and the air-filled space of the middle ear and mastoid, or cranial spaces. At present the diagnostic criteria for perilymphatic fistula is the fistula hole confirmed by the microscope and endoscope between the middle ear and inner ear, the hole is located in the round or oval window, fractured bony labyrinth, microfissures, anomalous footplate, and can occur after head trauma or barotrauma, chronic inflammation, or in otic capsule dehiscence. Recently, the cochlin-tomoprotein(CTP) detected from the middle ear. CTP, the shortest isoform of cochlin encoded by the COCH gene, has been proven to be a perilymph-specific protein which is not expressed in blood, cerebrospinal fluid and saliva but is highly expressed in lymphatic fluid of the inner ear and is used as a diagnostic biochemical marker for perilymph fistula. The CTP test based on ELISA was performed on the lavage fluid of patients with suspected perilymph fistula to obtain comparatively accurate test results. This paper reviewed the diagnostic value of CTP in perilymphatic fistula.
Topics: Extracellular Matrix Proteins; Fistula; Humans; Perilymph; Saliva; Skull; Vestibular Diseases
PubMed: 33040516
DOI: 10.13201/j.issn.2096-7993.2020.09.021 -
Frontiers in Cell and Developmental... 2022The high complexity of the cellular architecture of the human inner ear and the inaccessibility for tissue biopsy hampers cellular and molecular analysis of inner ear...
The high complexity of the cellular architecture of the human inner ear and the inaccessibility for tissue biopsy hampers cellular and molecular analysis of inner ear disease. Sampling and analysis of perilymph may present an opportunity for improved diagnostics and understanding of human inner ear pathology. Analysis of the perilymph proteome from patients undergoing cochlear implantation was carried out revealing a multitude of proteins and patterns of protein composition that may enable characterisation of patients into subgroups. Based on existing data and databases, single proteins that are not present in the blood circulation were related to cells within the cochlea to allow prediction of which cells contribute to the individual perilymph proteome of the patients. Based on the results, we propose a human atlas of the cochlea. Finally, druggable targets within the perilymph proteome were identified. Understanding and modulating the human perilymph proteome will enable novel avenues to improve diagnosis and treatment of inner ear diseases.
PubMed: 35573665
DOI: 10.3389/fcell.2022.847157 -
Frontiers in Neurology 2020A perilymphatic fistula (PLF) is an abnormal communication between the perilymph-filled inner ear and the middle ear cavity, mastoid, or intracranial cavity. A PLF most... (Review)
Review
A perilymphatic fistula (PLF) is an abnormal communication between the perilymph-filled inner ear and the middle ear cavity, mastoid, or intracranial cavity. A PLF most commonly forms when the integrity of the oval or round window is compromised, and it may be trauma-induced or may occur with no known cause (idiopathic). Controversy regarding the diagnosis of idiopathic PLF has persisted for decades, and the presenting symptoms may be vague. However, potential exists for this condition to be one of the few etiologies of dizziness, tinnitus, and hearing loss that can be treated surgically. The aim of this review is to provide an update on classification, diagnosis, and treatment of PLF. Particular attention will be paid to idiopathic PLF and conditions that may have a similar presentation, with subsequent information on how best to distinguish them. Novel diagnostic criteria for PLF and management strategy for PLF and PLF-like symptoms is presented.
PubMed: 33041986
DOI: 10.3389/fneur.2020.01046 -
Hearing Research Mar 2019The blood-perilymph barrier serves a critical role by separating the components of blood from inner ear fluids, limiting traffic of cells, proteins and other solutes...
The blood-perilymph barrier serves a critical role by separating the components of blood from inner ear fluids, limiting traffic of cells, proteins and other solutes into the labyrinth, and allowing gas (O-CO) exchange. Inflammation produces changes in the blood-perilymph barrier resulting in increased vascular permeability. It is commonly thought that compromise of the blood-inner ear barrier would lead to hearing impairment through loss of the endocochlear potential (EP). In fact, the effect of increasing cochlear vascular permeability on hearing function and EP is poorly understood. We used a novel method to measure the integrity of the blood-perilymph barrier and demonstrated the effects of barrier compromise on ABR threshold and EP. We also investigated the contribution of CX3CR1 cochlear macrophages and CCR2 inflammatory monocytes to barrier function after systemic exposure to lipopolysaccharide (LPS). We found that systemic LPS induced a profound change in vascular permeability, which correlated with minimal change in ABR threshold and EP. Macrophage depletion using CX3CR1-DTR mice did not alter the baseline permeability of cochlear vessels and resulted in preservation of barrier function in LPS-treated animals. We conclude that cochlear macrophages are not required to maintain the barrier in normal mice and activated macrophages are a critical factor in breakdown of the barrier after LPS. CCR2 null mice demonstrated that LPS induction of barrier leakiness occurs in the absence of CCR2 expression. Thus, enhanced aminoglycoside ototoxicity after LPS can be linked to the expression of CCR2 in inflammatory monocytes, and not to preservation of the blood-perilymph barrier in CCR2 knockout mice.
Topics: Animals; CX3C Chemokine Receptor 1; Capillary Permeability; Cochlea; Diphtheria Toxin; Heparin-binding EGF-like Growth Factor; Lipopolysaccharides; Macrophage Activation; Macrophages; Mice; Mice, Knockout; Mice, Transgenic; Monocytes; Ototoxicity; Perilymph; Receptors, CCR2
PubMed: 30710792
DOI: 10.1016/j.heares.2019.01.006 -
Frontiers in Molecular Neuroscience 2017The inner ear is a very complex sensory organ whose development and function depend on finely balanced interactions among diverse cell types. The many different kinds of... (Review)
Review
The inner ear is a very complex sensory organ whose development and function depend on finely balanced interactions among diverse cell types. The many different kinds of inner ear supporting cells play the essential roles of providing physical and physiological support to sensory hair cells and of maintaining cochlear homeostasis. Appropriately enough, the gene most commonly mutated among subjects with hereditary hearing impairment (HI), , encodes the connexin-26 (Cx26) gap-junction channel protein that underlies both intercellular communication among supporting cells and homeostasis of the cochlear fluids, endolymph and perilymph. lies at the DFNB1 locus on 13q12. The specific kind of HI associated with this locus is caused by recessively-inherited mutations that inactivate the two alleles of the gene, either in homozygous or compound heterozygous states. We describe the many diverse classes of genetic alterations that result in DFNB1 HI, such as large deletions that either destroy the gene or remove a regulatory element essential for expression, point mutations that interfere with promoter function or splicing, and small insertions or deletions and nucleotide substitutions that target the coding sequence. We focus on how these alterations disrupt and Cx26 functions and on their different effects on cochlear development and physiology. We finally discuss the diversity of clinical features of DFNB1 HI as regards severity, age of onset, inner ear malformations and vestibular dysfunction, highlighting the areas where future research should be concentrated.
PubMed: 29311818
DOI: 10.3389/fnmol.2017.00428 -
AJNR. American Journal of Neuroradiology Mar 2020Ménière disease is characterized by endolymphatic hydrops, whereas perilymphatic enhancement on MR imaging has been suggested to be of additional value in diagnosing...
BACKGROUND AND PURPOSE
Ménière disease is characterized by endolymphatic hydrops, whereas perilymphatic enhancement on MR imaging has been suggested to be of additional value in diagnosing Ménière disease. This study evaluates the presence of endolymphatic hydrops and perilymphatic enhancement in patients with Ménière disease and with other vertigo-associated inner ear pathology.
MATERIALS AND METHODS
A 3D-FLAIR sequence 4 hours after intravenous gadolinium injection was performed to visualize the endolymph and perilymph in 220 patients suspected of having Ménière disease. Patients' ears were retrospectively categorized as having Ménière disease (probable or definite) or other vertigo-associated inner ear pathology not attributable to Ménière disease. Endolymphatic hydrops was evaluated using a visual classification system, and perilymphatic enhancement was scored both visually and quantitatively.
RESULTS
Endolymphatic hydrops was present in 137 (91.9%) of the definite Ménière disease ears and in 9 (7.0%) of the ears with other vertigo-associated inner ear pathology ( < .001). The combination of endolymphatic hydrops and visually increased perilymphatic enhancement was present in 122 (81.9%) definite Ménière disease ears compared with 4 (3.1%) ears with other vertigo-associated inner ear pathology ( < .001). This combination increases the positive predictive value from 0.94 for endolymphatic hydrops and 0.91 for perilymphatic enhancement to 0.97. The addition of measured perilymphatic enhancement leads to a moderate decrease in sensitivity from 0.92 for endolymphatic hydrops to 0.86.
CONCLUSIONS
The combination of perilymphatic enhancement and endolymphatic hydrops in patients suspected of having Ménière disease increases the positive predictive value in the diagnosis of definite Ménière disease.
Topics: Adult; Aged; Aged, 80 and over; Endolymphatic Hydrops; Female; Gadolinium DTPA; Humans; Magnetic Resonance Imaging; Male; Meniere Disease; Middle Aged; Perilymph; Predictive Value of Tests; Retrospective Studies
PubMed: 32029469
DOI: 10.3174/ajnr.A6410 -
Indian Journal of Otolaryngology and... Aug 2022This study has aimed to determine the anatomical site of labyrinthine fistula in patients of chronic suppurative otitis media at our centre. Labyrinthine fistulae (LF)...
This study has aimed to determine the anatomical site of labyrinthine fistula in patients of chronic suppurative otitis media at our centre. Labyrinthine fistulae (LF) are caused by abnormal communications between the inner ear and surrounding structures resulting in perilymph leakage and hearing loss. Labyrinthine fistula represents as erosive loss of the enchondral bone overlying the semicircular canals without loss of perilymph. The manifestations of fistula like vertigo, hearing loss vary in severity and complexity, commonly ranging from very mild to incapacitating. Cholesteatoma induced fistula most commonly involves lateral semicircular canal probably because of its close proximity to the middle ear, but can involve other semicircular canals and rarely cochlea. This is a retrospective analysis of 36 patients of chronic suppurative otitis media with history of vertigo undergoing tympanomastoid surgery in whom there was an evidence of labyrinthine fistula on HRCT scan of temporal bone. The incidence of patients with labyrinthine fistula presenting with vertigo, nystagmus, sensorineural hearing loss, history of vertigo were analysed. The anatomical location of the fistula was supported by Radiological evidence. Patients underwent either canal wall down mastoidectomy or cortical mastoidectomy. The anatomical site and length of the labyrinthine fistula were analysed. Amongst the 36 patients of chronic suppurative otitis media with labyrinthine fistula 22 (61.1%) patients had atticoantral disease, 4 (11.1%) patients had chronic otitis media with extensive granulation, 2 (5.5%) patients had Tubotympanic disease with polyps, 4 (11.1%) patients had Tuberculous otitis media, 1 (2.77%) patient had Tubotympanic disease with extensive tympanosclerosis eroding the dome of lateral semicircular canal, 1 (2.77%) patient had extensive cholesteatoma with cerebellar abscess, 1 (2.77%) patient had fistula in the promontory following trauma, 1 (2.77%) patient had extensive tympanosclerosis with erosion of promontory. It was noticed that, in 14 (38.88%) patients the fistula was at the centre, in 17 (47.22%) patients the fistula is towards the ampullary end of horizontal semicircular canal and in 5 (13.88%) patients the fistula was towards the non ampullary end of lateral semicircular canal. The maximum length of fistula noticed was 6 mm and the minimum length of the fistula noticed was 2 mm. Labyrinthine fistula are most commonly noticed in the ampullary end of the lateral semicircular canal. The average length of the fistula was found to be 4 mm. Careful elevation of the cholesteatoma matrix over the endosteal membrane and immediate placement of temporal fascia over the exposed fistula is important to avoid injury to the inner ear. Maximum number of fistula were seen in the atticoantral type of Chronic suppurative otitis media. Prior knowledge of anatomical location of the fistulous tract in HRCT temporal bone is important to address the fistula.
PubMed: 36032823
DOI: 10.1007/s12070-020-01857-2 -
Frontiers in Bioengineering and... 2023Cisplatin (CIS) is widely used to treat various cancers but can cause ototoxicity and sensory hair cell loss in the inner ear. Copper induces an excessive production of...
Cisplatin (CIS) is widely used to treat various cancers but can cause ototoxicity and sensory hair cell loss in the inner ear. Copper induces an excessive production of reactive oxygen species (ROS) in hair cells, leading to the development of various antioxidants. This study aimed to evaluate the potential antioxidant properties of curcumin (CUR) in the inner ear organ of corti-1 cells (OC1) and animal models (zebrafish and guinea pigs). Graphene oxide quantum dots (GOQDs) enabled CUR to penetrate the round window membrane (RWM) and maintain the concentration in the perilymph after inner ear administration. The results showed that CUR/GOQDs had favorable biocompatibility and strongly affected ROS generation induced by CIS in OC1 cells. DCFHDA Green staining demonstrated that CUR/GOQDs successfully reversed the decrease in mitochondrial membrane potential induced by CIS and rescued cells from early cuproptosis, which was confirmed by FDX1 staining. Additionally, the experiment found that CUR decreased the expression of cuproptosis proteins (FDX1, LIAS, and LIPT1) and increased the expression of the Bcl-2 protein. The results demonstrate that CUR/GOQDs is a promising therapeutic agent that can prevent CIS-induced ototoxicity by blocking the cuproptosis signal pathway.
PubMed: 37152642
DOI: 10.3389/fbioe.2023.1183197 -
Lin Chuang Er Bi Yan Hou Tou Jing Wai... Nov 2022To investigate the relationship between imaging characteristics and intraoperative perilymph gusher in patients with Mondini inner ear malformation in cochlear...
To investigate the relationship between imaging characteristics and intraoperative perilymph gusher in patients with Mondini inner ear malformation in cochlear implantation, in order to provide basis and clinical guidance for predicting of intraoperative perilymph gusher before cochlear implantation. According to Sennaroglu's classification method, children with severe sensorineural hearing loss screened from January 2020 to December 2021 were divided into Mondini group, simple enlarged vestibular aqueduct group and normal inner ear group according to inclusion criteria strictly. The images of temporal bone HRCT and inner ear MRI were post-processed, some relative indicators were measured, including cochlear height and width of vestibular aqueduct, etc., and the gusher situation during cochlear implantation was recorded. The mean value of each indicator among the three groups were compared respectively, and the differences of each indicator between the gusher group and the non-gusher group were analyzed. There were statistically significant differences in cochlear height, length of cochlear bottom turn, width of cochlear aperture, vestibular length and vestibular width among the Mondini group(24 cases), simple EVA group(15 cases) and normal inner ear group(28 cases). The incidence of gusher of Mondini group in cochlear implantation was 30.77%(8/26). The outer diameter of the VA([3.10±0.74]mm) and the middle width of the VA([1.90±0.68] mm) in the gusher group were wider than those in the non-gusher group, and the difference was statistically significant. The incidence of intraoperative gusher in patients with EVA was 20.00%(3/15), and there was statistically significant difference in the length of endolymph sac between gusher group and non-gusher group(<0.05). The causes of intraoperative perilymph gusher in patients with Mondini inner ear malformation are complex. The enlarged vestibular aqueduct may be one of the anatomical basis. Whether it can be used to guide the preoperative assessment of the risk of intraoperative perilymph gusher need to be further confirmed by a large sample of clinical research from multiple centers in the future.
Topics: Child; Humans; Perilymph; Hearing Loss, Sensorineural; Vestibular Aqueduct; Cochlear Implantation; Vestibule, Labyrinth; Retrospective Studies
PubMed: 36347576
DOI: 10.13201/j.issn.2096-7993.2022.11.006 -
British Medical Journal (Clinical... Jul 1986
Topics: Ear, Inner; Fistula; Humans; Labyrinthine Fluids; Perilymph
PubMed: 3089460
DOI: 10.1136/bmj.293.6541.220