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The Laryngoscope Apr 1981A perilymph fistula is an abnormal communication between the fluids surrounding the membranous labyrinth and the middle ear space. Because of the potential hazards of... (Review)
Review
A perilymph fistula is an abnormal communication between the fluids surrounding the membranous labyrinth and the middle ear space. Because of the potential hazards of meningitis, permanent hearing loss, and occasionally incapacitating vestibular symptoms, early recognition and prompt repair of the perilymph leak is important. One hundred thirty three cases of perilymph fistulas are presented, stressing the clinical characteristics, evaluation, and management of patients with this otologic entity. The historical and contemporary literature on this subject is reviewed. A pathophysiological basis for perilymph fistula formation is presented, based upon certain anatomic, physiologic and mechanical principles involving the temporal bone and surrounding structures. For the purpose of publication, the Materials and Methods and Evaluation sections of the paper have been omitted. These are available from the author upon request in mimeographed form. The interested reader may wish to refer to items 80 and 133 in the Bibliography, previous publications by the author in which the bulk of the data used in the omitted sections can be found.
Topics: Cerebrospinal Fluid; Child; Female; Fistula; Humans; Infant; Labyrinth Diseases; Labyrinthine Fluids; Male; Middle Aged; Perilymph; Stapes Surgery
PubMed: 7012493
DOI: 10.1288/00005537-198104000-00007 -
Archives of Otolaryngology--head & Neck... Jul 1992
Review
Topics: Animals; Fistula; Humans; Labyrinth Diseases; Perilymph
PubMed: 1627287
DOI: 10.1001/archotol.1992.01880070023004 -
Archives of Otolaryngology--head & Neck... Jul 1992A perilymph fistula may be defined as an abnormal opening between the inner ear and the external surface of the labyrinth capsule, which allows the escape of perilymph... (Review)
Review
A perilymph fistula may be defined as an abnormal opening between the inner ear and the external surface of the labyrinth capsule, which allows the escape of perilymph and causes specific disorders of hearing, balance, or both. Its diagnosis remains somewhat controversial. This communication presents a defined set of clinical diagnostic criteria, the historic context by which those criteria have been developed, the histopathologic and clinical studies by which they have been challenged or substantiated, and the deficiencies that remain in our understanding of the pathogenic mechanisms that allow for abnormal cochlear or vestibular function.
Topics: Fistula; Humans; Labyrinth Diseases; Perilymph
PubMed: 1627286
DOI: 10.1001/archotol.1992.01880070017003 -
Proteomics Mar 2021The vast majority of sensorineural hearing loss is caused by impairment of the inner ear cells. Proteomic analysis of perilymph may therefore improve our understanding...
The vast majority of sensorineural hearing loss is caused by impairment of the inner ear cells. Proteomic analysis of perilymph may therefore improve our understanding of inner ear diseases and hearing loss. However, the investigation of the human perilymph proteome was limited due to technical difficulties in perilymph sampling. The guinea pig (Cavia porcellus) is frequently used as an experimental model in preclinical hearing research. In this study, we analyzed samples of perilymph collected from 12 guinea pigs to overcome limited experimental information regarding its proteome. We identified a total of 1413 proteins, establishing a greatly expanded proteome of the previously inferred guinea pig perilymph. This provides a comprehensive proteomic resource for the research community, which will facilitate future molecular-phenotypic studies using the guinea pig as an experimental model of relevance to human inner ear biology.
Topics: Animals; Guinea Pigs; Perilymph; Proteome; Proteomics
PubMed: 33459488
DOI: 10.1002/pmic.202000138 -
Ear, Nose, & Throat Journal Jan 1989PLF is an enigmatic otologic disease that may be congenital or acquired through surgery, trauma, neoplasm, or infection. The exact incidence of PLF is unknown, primarily... (Review)
Review
PLF is an enigmatic otologic disease that may be congenital or acquired through surgery, trauma, neoplasm, or infection. The exact incidence of PLF is unknown, primarily because it is difficult to diagnose. A careful, thorough history is important in recognizing PLF. If a PLF exists, repair can be effected simply and quickly and may be very beneficial to the patient. There is a small but real risk of total hearing loss with operation to repair any PLF. Currently, the most useful diagnostic tests for PLF involve the vestibular system, and further studies of vestibular responses to PLF hold promise in improving our diagnostic ability.
Topics: Fistula; Humans; Labyrinth Diseases; Labyrinthine Fluids; Perilymph
PubMed: 2656229
DOI: No ID Found -
Hearing Research Apr 2022Sensorineural hearing losses (SNHLs) are a significant public health issue, and the hearing loss field is desperately in need of effective therapy. Pathophysiological...
INTRODUCTION
Sensorineural hearing losses (SNHLs) are a significant public health issue, and the hearing loss field is desperately in need of effective therapy. Pathophysiological mechanisms are not yet clearly understood in the absence of validated methods to assess the inner ear content. Proteomic and metabolomic analysis of perilymph is opening new research perspectives for SNHLs. We aimed to demonstrate the feasibility of an innovative mass spectrometry (MS) strategy using porous silicon chips (PSCs) to investigate the low molecular weight (LMW) protein and metabolite content of human perilymph. Our second objective was to stratify perilymph samples according to their MS profiles and compare these results with clinical data.
MATERIAL AND METHODS
Perilymph samples obtained during cochlear implant surgery from patients with SNHLs were retrieved from a validated biobank. To focus on LMW entities, we used a PSC enrichment protocol before MALDI-ToF MS analysis. PSCs were used as a LMW molecular preanalytical stabilizer and amplifier. Patients' clinical data and SNHL characteristics were retrieved retrospectively from medical charts.
RESULTS
We successfully acquired and compared 59 exploitable MS profiles out of 71 perilymph samples. There was a good correlation between duplicates. Comparing both ears from the same patient, we found good reproducibility even when there was a one-year interval between samplings. We identified three distinct groups when comparing the samples' metabolomic profiles and four homogeneous groups comparing their LMW proteome profiles. Clinical data analysis suggested that some groups shared clinical or preanalytical characteristics.
CONCLUSION
This proof-of-concept study confirms that LMW proteome and metabolome content of perilymph can be analyzed with PSCs. Based on protein profiles, we managed to stratify perilymp samples according to their molecular composition. These results must be confirmed with a larger population, and sampling methods require improvement, but this approach seems promising. In the future, this approach may pave the way for companion test strategies to precisely diagnose and define potential molecular targets for audioprotective therapies.
Topics: Hearing Loss, Sensorineural; Humans; Perilymph; Porosity; Proteome; Proteomics; Reproducibility of Results; Retrospective Studies; Silicon; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization
PubMed: 35152040
DOI: 10.1016/j.heares.2022.108457 -
Acta Oto-laryngologica Aug 2021Otological diseases including Meniere's disease (MD) involve endolymphatic hydrops (EH), which can be visualized by magnetic resonance imaging (MRI) with gadolinium...
BACKGROUND
Otological diseases including Meniere's disease (MD) involve endolymphatic hydrops (EH), which can be visualized by magnetic resonance imaging (MRI) with gadolinium contrast agents, but the temporal changes of contrast in the inner ear have not been evaluated.
OBJECTIVES
We investigated the permeability of the blood-perilymph barrier (BPB) in ears with EH to evaluate the severity of the inner ear disturbances.
MATERIALS AND METHODS
The study included 32 ears from 16 patients with EH or related diseases who underwent MRI. The permeability of the BPB was assessed by the signal-intensity ratio (SIR) at four-time points: before and at 10 min, 4 h, and 24 h after administration of gadolinium for assessing EH.
RESULTS
Cochlear EH was found in 25 of the 32 ears, and vestibular EH in 11. The rate of EH was significantly higher in symptomatic ears; however, the existence of EH was not related to SIR values. Nevertheless, SIR values in the basal turn were significantly higher 4 and 24 h after injection of gadolinium in patients aged ≥50 years.
CONCLUSION AND SIGNIFICANCE
Higher SIR values observed in older patients with EH indicate severe disturbances of the BPB in the cochlea, which may account for intractable inner ear disturbances in older patients.
Topics: Adult; Aged; Audiometry, Pure-Tone; Capillary Permeability; Cochlea; Contrast Media; Ear, Inner; Endolymphatic Hydrops; Female; Gadolinium; Humans; Magnetic Resonance Imaging; Male; Meniere Disease; Middle Aged; Perilymph
PubMed: 34346271
DOI: 10.1080/00016489.2021.1957500 -
Archives of Oto-rhino-laryngology 1980Freeze fracture replicas of the guinea pig inner ear were studied under the electron microscope to define the blood-perilymph barrier morphologically. This barrier is...
Freeze fracture replicas of the guinea pig inner ear were studied under the electron microscope to define the blood-perilymph barrier morphologically. This barrier is represented basically by the continuous endothelium of the inner ear capillaries. The endothelial cells contain only a few micropinocytotic vesicles in contrast to those of the stria vascularis vessels. The cochlear plexus also exhibits some special differences. Tight junctions of the continuous mesothelial type connect the endothelial cells of the inner ear capillaries. Cell membranes of these cells possess a lower particle density than those of the stria vascularis vessels. In general, the blood-perilymph barrier is morphologically similar to the blood-brain barrier.
Topics: Animals; Capillaries; Cochlear Aqueduct; Ear, Inner; Endothelium; Female; Guinea Pigs; Labyrinthine Fluids; Male; Microcirculation; Microscopy, Electron; Perilymph
PubMed: 7469927
DOI: 10.1007/BF00455891 -
Journal of Neurology Oct 2018
Topics: Adult; Aged; Endolymph; Female; Humans; Male; MicroRNAs; Middle Aged; Perilymph
PubMed: 29666983
DOI: 10.1007/s00415-018-8862-3 -
Journal of Proteome Research May 2017The knowledge about the etiology and pathophysiology of sensorineural hearing loss (SNHL) is still very limited. This study aims at the improvement of understanding...
The knowledge about the etiology and pathophysiology of sensorineural hearing loss (SNHL) is still very limited. This study aims at the improvement of understanding different types of SNHL by proteome analysis of human perilymph. Sampling of perilymph was established during inner ear surgeries (cochlear implantation, vestibular schwannoma surgeries), and safety of the sampling method was determined by checking hearing threshold with pure-tone audiometry postoperatively. An in-depth shot-gun proteomics approach was performed to identify cochlear proteins and the individual proteome in perilymph of patients. This method enables the identification and quantification of protein composition of perilymph. The proteome of 41 collected perilymph samples with volumes of 1-12 μL was analyzed by data-dependent acquisition, resulting in overall 878 detected protein groups. At least 203 protein groups were solely identified in perilymph, not in reference samples (serum, cerebrospinal fluid), displaying a specific protein pattern for perilymph. Samples were grouped by patient's age and surgery type, leading to the identification of some proteins specific to particular subgroups. Proteins with different abundances between different sample groups were subjected to classification by gene ontology annotations. The identified proteins might serve as biomarkers to develop tools for noninvasive inner ear diagnostics and to elucidate molecular profiles of SNHL.
Topics: Adult; Aged; Aged, 80 and over; Child; Child, Preschool; Cochlea; Gene Ontology; Hearing Loss, Sensorineural; Humans; Infant; Middle Aged; Perilymph; Proteins; Proteome; Proteomics; Sampling Studies
PubMed: 28282143
DOI: 10.1021/acs.jproteome.6b00986