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PubMed1995

Perilymph fistula pathophysiology.

A great deal of the controversy associated with perilymph fistula is due to the lack of a sensitive, specific way of detecting and thus diagnosing one. The existing subjective, clinical observations need to be replaced with an objective, scientific methodology having higher specificity and sensitivity for the detection of perilymph fistula. Three detection methods presently under development are presented here. One uses a miniendoscope to observe the middle ear. This procedure is less traumatic than inspection with tympanotomy. Thus it is less likely to create artifacts that can be mistaken for a fistula leakage. The "yes-no" observation decision is to be replaced with a graded quantitative measure. The second method concentrates on detected leakage from inner to middle ear. beta 2-Transferrin, a unique endogenous substance found in perilymph and cerebrospinal fluid, can be detected with one- or two-dimensional gel electrophoresis and immunoblotting. The third method is a system identification fistula test. It measures a change in a physiologic response that is caused by the presence of the fistula. Classic biophysical models of the vestibular end organs can be used to predict the dynamics of responses to systems identification stimuli.

Biomarkers↗

PubMed2000

Hydroxyl radical formation in the perilymph of asphyxic guinea pig.

To elucidate the role of hydroxyl radical (.OH) species in the generation mechanism of the cochlear pathology induced by transient asphyxia and subsequent re-ventilation, the concentrations of 2,3-hydroxybenzoic acid (DHBA) and 2,5-DHBA, major products arising from the attack of .OH upon salicylate, were measured in the perilymph of the guinea pig by the high performance liquid chromatography-electrochemical/UV method. The mean value of 2, 3-DHBA concentration in the perilymph significantly increased from the pre-asphyxic level (6.4 microM) to 7.6 microM and 8.8 microM during asphyxia of 3 min duration and at 5 min after the onset of re-ventilation, respectively. The 2,5-DHBA concentration was 7.9 microM before asphyxia, and also significantly increased to 11.5 microM and 16.2 microM during and after asphyxia, respectively. These results strongly indicated that .OH was generated in the perilymph of the asphyxic and re-ventilated guinea pig cochlea, and the significance of this increased .OH in generating anoxia and re-perfusion injury is discussed with respect to iron and oxygen-derived free radicals.

Animals↗

PubMed2002

Reduction in the endocochlear potential caused by Cs(+) in the perilymph can be explained by the five-compartment model of the stria vascularis.

In an earlier publication (Takeuchi et al., Biophys. J. 79 (2000) 2572-2582), we proposed that K(+) channels in intermediate cells within the stria vascularis may play an essential role in the generation of the endocochlear potential (EP), and we presented an extended version of the five-compartment model of the stria vascularis. In search of further evidence supporting the five-compartment model, we studied the effects of Cs(+) added to the perilymph on guinea pig EP. Cs(+) is known as a competitive K(+) channel blocker. Both the scala tympani and the scala vestibuli of four cochlear turns were perfused at a flow rate of 10 microl/min, and the EP was recorded from the second cochlear turn. Cs(+) at 30 mM caused a biphasic change in the EP; the EP increased transiently from a control level of 89.6 mV to 94.8 mV within 10 min, and then decreased to a steady level of 24.5 mV within the next 40 min. We propose that the initial transient increase in the EP results from Cs(+)-mediated blockade of K(+) conductance in the basolateral membrane of hair cells, and that the subsequent EP decrease is due to effects of Cs(+) on the stria vascularis. We believe that Cs(+) in the perilymph is able to access the stria vascularis by being taken up by fibrocytes in the spiral ligament and then being transported to intermediate cells because it is known that Cs(+) is taken up via Na(+),K(+)-ATPase and that gap junctions connect fibrocytes in the spiral ligament to basal cells and basal cells to intermediate cells. To clarify the effect of intracellular Cs(+) on the electrophysiological properties of intermediate cells, these cells were dissociated from guinea pigs and studied by the whole-cell patch-clamp method. Intracellular Cs(+) depolarized intermediate cells in a dose-dependent manner. In addition, efflux of Cs(+) from the intermediate cell was much less than the efflux of K(+). Thus, Cs(+) may accumulate in the intermediate cell, which depolarizes the cell, which in turn decreases the EP. We conclude that the five-compartment model of the stria vascularis can explain the EP decrease caused by Cs(+) in the perilymph.

Animals↗

PubMed1991

Perilymph fistula--a diagnostic dilemma.

A retrospective series is presented of 51 cases operated on for suspected perilymph fistula. In 26 ears a fistula was identified at surgery. A positive fistula test was found to strongly indicate a perilymph fistula but was more often negative than positive in surgically demonstrated fistula ears. Other vestibular tests were found to be of little value in the pre-operative diagnosis. Ears with a surgically demonstrated fistula and sensorineural hearing loss had either flat or downward-sloping audiograms. Difficulties in diagnosing a perilymph fistula at tympanotomy are discussed. At follow-up, vestibular symptoms were found to be eliminated or improved in 96 per cent of cases with surgically demonstrated fistulae and in 68 per cent of cases in which no fistula was detected at tympanotomy but hearing improved significantly in only one ear (4 per cent) of the former group and in five ears (20 per cent) of the latter group.

Adolescent↗

PubMed1991

Bilateral perilymph fistula in an adult.

Sudden hearing loss is a symptom caused by a variety of diseases, among them also a perilymph fistula. The occurrence of bilateral perilymph fistula is rare, and was reported only associated with head trauma. A case of a bilateral perilymph fistula, without head trauma in an adult construction worker is reported. To the best of our knowledge this is the first bilateral case caused by physical effort to be reported in the literature.

Audiometry↗

PubMed1985

Perilymph fistula.

Perilymph fistula occurs when endolymph and perilymph mix, or when perilymph leaks into the middle ear space. Sensorineural hearing loss and/or vertigo may result. This paper reviews the pertinent anatomy and physiology of the inner ear, clinical presentations, diagnosis, treatment, and prognosis as a guide for the clinician.

Adult↗

PubMed1983

Effect of sound stimulation at several levels on concentrations of primary amines, including neurotransmitter candidates, in perilymph of the guinea pig inner ear.

Perilymph, which bathes the sensory cells of the cochlea, was collected from guinea pigs exposed to noise and analyzed via two cation-exchange HPLC procedures with fluorescence detection, resolving 51 and 81 primary-amine compounds, respectively, at a sensitivity limit of 0.1 pmol relative to leucine. During a first period, each animal was either exposed to noise at 80, 90, or 115 decibels sound-pressure level or maintained in silence (controls), and during a second period, the same animal was maintained in silence. Perilymph was collected during both periods, and perilymphatic components were compared, within animals and across animals, for several levels of sound stimulation. A gamma-aminobutyric acid-like component was elevated in the first period in proportion to stimulus intensity by the various methods of comparison, suggesting an auditory-neurotransmitter role for this component. Aspartic acid was elevated in the second period, 2-3.5 h after onset of sound stimulation, compatible with the release of aspartic acid from central auditory synapses. In addition, a methionine-enkephalin-like component, distinct from leucine-enkephalin, was detected in perilymph from control animals and was elevated in response to noise at 115 decibels. Regression coefficients, determined for the relation between sound intensity and first-period concentrations or the difference between first and second-period concentrations, indicated zero linear regression at p = 0.05 for glutamic acid, aspartic acid, glycine, taurine, and 39 other perilymphatic components, consistent with the hypothesis that these compounds are unlikely to be peripheral auditory neurotransmitters.

Acoustic Stimulation↗

PubMed1984

Correlation of amikacin concentrations in perilymph and plasma of continuously infused guinea pigs.

A commercially available radioimmunoassay kit was modified to enable us to measure, in triplicate, the amikacin concentration in 1 microliter of perilymph fluid. Amikacin levels in plasma and perilymph were measured in guinea pigs after continuous intravenous infusion at four different dosing rates. After a 4-h infusion, a good linear correlation was found between the amikacin concentration in plasma and the dosing rate. Likewise, a significant linear relationship was found between concentrations of amikacin in perilymph and plasma (y = 0.21x + 2.56; r = 0.67; n = 45) after 6 h of infusion. These results suggest nonsaturation kinetics at the concentrations used.

Amikacin↗

PubMed1990

Histopathologic study of the perilymph-suctioned labyrinth.

A small amount of perilymph was suctioned from the scala tympani through the round window membrane of guinea pigs. The animals either were killed immediately after the suctioning or were kept alive for 1 to 3 months. For morphologic study, the conventional celloidin embedding method was employed, and the temporal bones were serially sectioned. Various kinds and degrees of changes in the membranous labyrinth were observed: hydrops, collapse, and rupture. Loss of outer hair cells in the upper turns of the cochlea was noted in the 1- to 3-month recovery specimens. These findings are identical to those found in animals in which artificial perilymph was injected into the subarachnoid space in order to produce experimental perilymphatic fistula. After comparing morphologic changes of the membranous labyrinth following use of each method, we concluded that perilymph suctioning from the round window can be used as an animal model of perilymphatic fistula.

Animals↗

PubMed1992

Chronic perilymph fistula in the guinea pig with implications in the human.

To improve understanding of the pathophysiology of perilymph fistulas, a predictable animal model of a chronic fistula was developed. Our findings suggest that guinea pig fistulas do not remain patent for prolonged periods. By extrapolating these findings to humans, we postulate that the symptoms and signs of perilymph fistula are possibly due not to one prolonged constant fistula, but rather a series of "blowouts" from an inherent congenital or posttraumatic weak spot in either the round or oval window. We feel that a diagnosis of perilymph fistula must be considered in any patient presenting with a Meniere's-like symptom set concomitant with a congenital inner ear deformity or a history of inner ear trauma.

Acoustic Stimulation↗

PubMed1992

Endolymphatic hydrops induced by immune response of the endolymphatic sac: relation to perilymph antibody levels.

This study investigated the effect of a secondary immune response in the endolymphatic sac on the development of endolymphatic hydrops in guinea pigs. Following secondary keyhole limpet hemocyanin (KLH) challenge directly to the sac in systemically presensitized animals, hydrops rapidly developed within 1 week, then gradually reduced by 4 weeks. After 5 weeks, hydrops gradually recurred and developed again. Mean perilymph anti-KLH antibody levels rose from day 1 to 4 weeks and then decreased. The elevation of perilymph anti-KLH antibody levels on day 2 was due to an increased vascular permeability and there was a local regeneration by the challenge of the sac after day 7. The development of hydrops correlated well with mean perilymph anti-KLH antibody levels in the period from day 1 to day 7. After 2 weeks, there was no correlation between them. These results suggest that an immune response of the sac may induce an excess secretion of endolymph in the initial stage and may further lead to irreversible dysfunction of the sac at a later stage.

Animals↗

PubMed1995

Disturbance of regulation of sodium by cis-diamminedichloroplatinum in perilymph of the guinea pig cochlea.

We studied the acute effects of cis-diamminedichloroplatinum (CDDP) on the cochlear partition and inner ear fluid in the guinea pig. At 48 hours after the administration of a single intramuscular injection of CDDP, 12.5 mg/kg of body weight, the endocochlear resting potential (EP) was significantly decreased to 32.1 +/- 1.8 mV in the treated animals, versus 80.6 +/- 1.0 mV in the control animals. There was a significant rise in potassium (K+), sodium (Na+), and chlorine (Cl-) in the endolymph of the animals treated with CDDP as compared with the control animals. Only Na+ was found to increase significantly in the perilymph, reaching more than twice the level of the control animals; both K+ and Cl- remained within the normal range. Serum electrolytes also remained within the normal range. Evaluation of modified ionic permeabilities across the endolymph-perilymph barrier showed an apparent increase in Na+ permeability and a normal range of K+ and Cl- permeabilities. Histopathologic examination of the cochlea showed a moderate collapse of the endolymphatic space, with atrophy of the stria vascularis and destruction of the outer hair cells. The findings suggest that the acute changes produced in the cochlea by administration of CDDP were attributable to a breakdown in the regulation of Na+ metabolism in the perilymph.

Animals↗

PubMed1991

Perilymph fistulas: the House Ear Clinic experience.

The diagnosis, evaluation, and surgical treatment of perilymph fistulas has recently been the focus of a great deal of attention in otology. Authors have focused on perilymph fistulas as the cause of hearing loss and vertigo in many diverse situations. Additionally, surgical repair has been suggested when there is little objective support for intervention. To address some of the problems inherent in the diagnosis and treatment of perilymph fistulas, records of patients operated on at the House Ear Clinic during the past 12 years were reviewed retrospectively. Eighty-six patients were surgically explored for fistulas during this period. Thirty-five (40.7%) fistulas were found, and 51 ears were patched whether fistulas were found or not. Of the 80 patients who were seen for follow-up, 35 (43.8%) were subjectively better, and 45 (56.2%) were the same. Although the number of fistulas found and the number of patients improved were similar, the composition of the two groups was different. On the basis of audiometric results, improvement in hearing occurred in only 18.7% of the patients. None of the demographic factors or diagnostic tests were predictive of either the presence of a fistula or the therapeutic outcome. Further work is required to facilitate the preoperative diagnosis of fistulas and to design appropriate surgical intervention.

Adolescent↗

PubMed1976

Symposium: Congenital anomalies of the middle ear. IV. Management of profuse perilymph leak.

Profuse perilymph leak during an otological operation can be controlled by inserting an epidural teflon cannula into the lumbar subarachnoid space and draining away the excess spinal fluid. After about 100 cc is removed the perilymph leak stops, and the oval window can be sealed with a living seal, such as vein, and the operation completed. The catheter is left in the subarachnoid space for about four days, with a bottle on the distal end positioned to remove no more than 150 cc of spinal fluid per day. Results with two patients in which this maneuver was used to control profuse perilymph leak are reported.

Catheterization↗

PubMed1981

Steep gradients of amino acids between cochlear endolymph and perilymph.

Levels of 19 free amino acids in cochlear endolymph and perilymph of scala vestibuli of the guinea pig were determined using reverse phase high performance liquid chromatography (HPLC) of the o-phthaldialdehyde-ethanethiol derivatives with fluorescence detection. Aspartate and glutamate were found to be significantly higher in endolymph than in perilymph, confirming results from another study using a different analytical method. The remaining 17 amino acids were significantly lower in the endolymph, in many cases by an order of magnitude. The data are compared with results on utricular endolymph and vestibular perilymph obtained by HPLC in another study from our laboratory. Possible implications and interpretations of the results are discussed.

Amino Acids↗

PubMed1986

Two emerging perilymph fistula "syndromes" in children.

This paper proposes two relatively common "syndromes" seen in children with perilymph fistulas using illustrative case reports. One, a 2 1/2-year-old child with bilaterally symmetric progressive sensorineural hearing loss, was found to have bilateral oval and round window fistulas. The other was a child who presented originally with an unexplained unilateral sensorineural loss. It was only when hearing deteriorated in the opposite ear several years later that a perilymph fistula was suspected and confirmed by tympanotomy. The clinical presentations and other details of these cases diverge widely, but may represent two emerging syndromes of perilymph fistulas in children: 1. a congenital or hereditary predisposition, and the other, 2. sudden hearing loss in the "good" ear of children with an unexplained sensorineural hearing loss in the opposite ear.

Audiometry↗

PubMedDate not supplied

The effect of intracisternally injected ouabain on perilymph electrolyte concentrations.

A means for altering the ionic composition of perilymph utilizing the cochlear aqueduct and cerebrospinal fluid in chinchillas is introduced. A decrease in sodium and an increase in potassium concentration in perilymph was observed after ouabain was injected into cerebrospinal fluid. The time course of changes in the perilymph paralleled the CSF change but with a time lag which had not reached equilibrium within 60 minutes.

Animals↗

PubMedDate not supplied

Perilymph production and cochlear blood flow.

In rodents at least, the main sources of the perilymph fluid are (1) influx of CSF through the cochlear aqueduct, and (2) blood flow dependent local production within the cochlea. Experimental data are presented that give a ratio of 22:78 percent for those sources. The perilymph production thus derives mainly from the cochlear blood flow. It is concluded that measurements of the perilymph production can be used as indirect measurements of the inner ear blood flow under various experimental conditions. Two experimental examples are referred to.

Acidosis, Respiratory↗