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Activities of carbonic anhydrase in the cochleae of guinea pigs with early experimental endolymphatic hydrops.

Normal auditory function depends on maintenance of the unique ion composition in the endolymph. Carbonic anhydrase in the inner ear has been suggested to play an important role in maintaining the ion concentration and regulating fluids of the inner ear. Cochlear dysfunction may indicate changes in the biochemical components and osmotic pressure of the inner ear fluids as well as inadequate generation of intracellular metabolic energy. Dysfunction of the inner ear was investigated in the early stages of endolymphatic hydrops. Normal adult albino guinea pigs were operated on to obliterate the endolymphatic ducts and sacs of the right ears to induce endolymphatic hydrops. The auditory function of experimental guinea pigs was assessed according to the difference between the preoperative and postoperative hearing thresholds of the auditory brainstem response (ABR). Vibratome sections of the hydropic cochlea (right) and control cochlea (left) were stained histochemically for the activities of carbonic anhydrase in this study. Decreased activity of this enzyme was not shown consistently in the stria vascularis and organ of Corti of the hydropic cochlea in the early stage of endolymphatic hydrops whereas auditory dysfunction in the hydropic ear was noted from the ABR threshold for the experimental animals with 2 months survival. The results of the present study suggest that further investigation concerning the role of carbonic anhydrase in the cochlear auditory function is necessary.

Animals↗

[Observation of cochlear microcirculation in experimental endolymphatic hydrops].

30 guinea pigs were made as a model of endolymphatic hydrops by obliteration of endolymphatic sac and duct. Cochlear microcirculation of the lateral wall in the third turn was observed and recorded. The diameters and velocities of the vessels were analysed in IBAS image system. The stria vascular vessels and spiral ligament vessels were analysed respectively. The data showed: 1. 1, 2 and 3 months after operation, the diameter of SLV averaely decreased 0.80 microns, 0.87 microns, 1.21 microns (P < 0.05) respectively and velocity decreased 100.8 microns/s, 141.0 microns/s, 136.0 microns/s (P < 0.05) respectively. 2. The diameter of SVV dilated 3 months after operation. With the elapse of time, SVV had a dilation tendency. These indicated that hydrops effect different changes in SLV and SVV. The overpressure of endolymphatic fluid may be the possible cause of changes in SVV and SLV.

Animals↗

Quantitative study of Scarpa's ganglion and vestibular sense organs in endolymphatic hydrops.

The density of vestibular hair cells and the number of neurons in Scarpa's ganglion were estimated for 11 temporal bones with endolymphatic hydrops. The ten subjects from which these bones were taken all exhibited decreased caloric response (when tested), fluctuating hearing loss, and episodic vertigo. The degeneration of vestibular sense organs was found within the "normal" range for all except one case, which showed total degeneration of the posterior crista ampullaris. Ganglion cell counts in all cases were low. In three of the ten subjects, counts fell below the lowest values seen in a sample of "normal" ears. These three subjects exhibited fluctuating hearing loss and episodic vertigo for more than six years prior to death. In cases of unilateral endolymphatic hydrops there was no significant difference between counts in the affected and unaffected ear. Thus, the apparent ganglion cell degeneration may be due to ear disease other than endolymphatic hydrops.

Aged↗

The relationship between vasopressin and endolymphatic hydrops in the guinea pig.

Clinical studies have shown that plasma vasopressin level is significantly elevated in patients with Meniere's disease. Other reports indicated that histamine induced a very quick and high elevation of vasopressin level and caused nystagmus in experimentally produced endolymphatic hydrops. We became interested in further investigating the details of this relationship by studying the effect of experimental endolymphatic hydrops and histamine upon plasma vasopressin level in the guinea pig. The results are as follows: 1) Histamine increased the plasma vasopressin level in normal guinea pigs. 2) There was no statistically significant difference in the plasma vasopressin level between the hydrops model and normal guinea pigs. 3) Histamine increased the plasma vasopressin level more in the hydrops model group than in normals. 4) Plasma vasopressin level was elevated in the vertiginous model caused by inner ear anesthesia. Our results support those of clinical investigators who reported that the plasma vasopressin level was elevated more in the Meniere's disease group than any other equilibrium disorder group. It is possible that vasopressin is in someway involved in the development of endolymphatic hydrops.

Animals↗

Vestibular evoked myogenic potentials and distortion-product otoacoustic emissions combined with glycerol testing in endolymphatic hydrops: their value in early diagnosis.

The present investigation was specifically designed to evaluate the ability of the glycerol test combined with pure tone audiometry, distortion-product otoacoustic emissions (DPOAEs), and vestibular evoked myogenic potentials (VEMPs) to diagnose endolymphatic hydrops early and to identify cases that may evolve toward Meniere's disease. This investigation consisted of 29 consecutive patients with mild dizziness or vertigo who received no treatment. Each patient underwent glycerol testing measured with conventional pure tone audiometry and with both DPOAEs and VEMPs. It is interesting to note that in 7 and 8 of the 29 cases, the VEMPs and DPOAEs, respectively, showed an improvement after glycerol administration that had not been shown on traditional audiometry. A further element worthy of consideration emerges from an analysis of the VEMP results compared to the DPOAE results that divided the patients into 4 groups. The first group had a postglycerol improvement with both methods, which would seem to suggest hydrops in both the anterior and posterior parts of the labyrinth. In the second and third groups, there was an improvement only either with VEMPs or DPOAEs, and this finding seems to indicate that only one endolymphatic compartment might be involved. In the last group, all patients had a positive glycerol test with positive DPOAEs on one side and with positive VEMPs on the other. Although endolymphatic hydrops can only be proven after death, a combination of VEMPs and DPOAEs with the glycerol test may permit early diagnosis of endolymphatic hydrops. These results clearly imply that these methods should be permanently included in the diagnostic protocol of patients with vestibular and audiological symptoms.

Adolescent↗

Effects of a nitric oxide synthase Type II inhibitor on compound action potential thresholds in experimental endolymphatic hydrops.

HYPOTHESIS: Nitric oxide (NO) is likely to be synthesized by nitric oxide synthase Type II (NOS II) action and may partake in the origin of changes of compound action potential (CAP) threshold observed in guinea pigs with induced endolymphatic hydrops. This study aimed to assess the action of a NOS II inhibitor on CAP thresholds in these experimental samples. BACKGROUND: In guinea pigs with experimental endolymphatic hydrops, there are lesions on the cochlea and progressive increase of CAP threshold. NOS II was found in the cochlea of this animal model, and it was inferred that NO can contribute by such alterations. METHODS: The animals were divided into two groups, in which eight received an intake of a NOS II inhibitor, aminoguanidine, and another eight served as a control group. During 16 weeks, CAP thresholds at 1,000, 2,000, 4,000 and 6,000 on electrocochleography were compared between the groups. RESULTS: The group that had an intake of aminoguanidine showed a lower increase on CAP thresholds at 2,000 (p < 0.05) and 6,000 Hz (p < 0.05) at the 12th postoperative week, and at 1,000 (p < 0.05), 2,000 (p < 0.001), 4,000 (p < 0.001), > and 6,000 Hz (p < 0.001) at the 16th week. CONCLUSION: We conclude that NOS II inhibitor reduced the elevation of CAP thresholds in experimentally induced endolymphatic hydrops.

Action Potentials↗

Antidiuretic hormone (ADH) and endolymphatic hydrops.

Plasma antidiuretic hormone (p-ADH) concentrations were determined with a radioimmunoassay, using a reversed-phase C18 silica column, in 300 patients with vertigo, dizziness and/or deafness; 119 of them had a diagnosis of Menière's disease. The p-ADH level was significantly elevated in patients with Meniere's disease and others with endolymphatic hydrops, e.g. cochlear Menière's disease or delayed hydrops. By contrast, the p-ADH level was not so high in cases without the endolymphatic hydrops. The increase in the p-ADH level was closely linked to vertigo attacks, the glycerol test results and an enhanced negative summating potential (-SP) in electrocochleogram (ECochG). These results lead to the assumption that disorders of ADH-dependent hormonal control in the inner ear may constitute the possible mechanism underlying vertiginous attacks and deafness in patients with endolymphatic hydrops.

Cochlea↗

Chronological changes of electrocochleogram in experimental endolymphatic hydrops. Special reference with AP output potential and hair cell cilia.

Chronological changes of the whole nerve action potential (AP), cochlear microphonics (CM) and summating potential (SP) in experimental endolymphatic hydrops in guinea pigs were studied during a period from 1 week to 13 months after the endolymphatic sac obliteration. Endolymphatic hydrops became extensive in month 3 and persisted thereafter. The threshold of AP increased with the lapse of time but good AP output potential was obtained, being maximum in month 3. The threshold of CM increased with the lapse of time. The CM output potential was the highest around week 3 when endolymphatic hydrops was slightly formed, and it decreased thereafter. SP responses at the frequencies of 8, 4, 1 and 0.5 kHz showed the reversed polarity of -SP in month 3 and changed towards potential 0 thereafter. In animals showing super-normal AP output potential, the cilia in the third row of outer hair cells were disarranged. This seems to be involved in recruitment.

Action Potentials↗

Verification of a new test of endolymphatic hydrops.

A new objective test of endolymphatic hydrops has been described previously. The test uses auditory brain-stem response (ABR) techniques to estimate the basilar membrane travelling wave velocity (TWV). The underlying hypothesis is that raised pressure in the scala media will lead to an increase in the stiffness of the basilar membrane and that this will give rise to a travelling wave velocity that is greater than normal. It was considered that verification of the technique could be obtained by carrying out the new test before and during a glycerol dehydration procedure. Patients who required this procedure for verification of the diagnosis or as a prognostic indicator for sacculotomy, underwent the following tests. Prior to dehydration an audiogram, the ABR TWV test and a blood sample (for plasma osmolality) were taken. The patient was then given the appropriate amount of glycerol mixed with lemon juice and laid on a bed in a test room. The audiogram and a blood sample were repeated every hour and the ABR TWV test was repeatedly carried out every twenty minutes between the other tests. Six of these procedures have been carried out. In five of them the 0.5 and 1 kHz average threshold improved by at least 10dB and in all those cases the ABR TWV test showed an abnormal pre-dehydration result which moved towards the normal value following dehydration. One patient gave ABR TWV results that were within normal limits before and during the procedure and gave an audiometric change of only 5dB.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Neurophysiologic assessment of endolymphatic hydrops.

An electrophysiological method for assessing endolymphatic hydrops of the cochlea in an animal model of Meniere's disease is described. Eighth nerve gross action potentials (AP) were evoked by tone bursts presented at discrete phases of a simultaneously delivered 50-Hz pure tone. Hydropic cochleas showed significantly less modulation of AP amplitude and latency than normal cochleas. This method may be useful in objectively evaluating Meniere's disease with electrocochleography.

Animals↗

Ménière's disease and delayed endolymphatic hydrops in children.

In order to study the characteristics of pediatric patients (< or = 15 years at onset) with endolymphatic hydrops disorders, we selected 5 patients with Ménière's disease (MD) and 3 patients with delayed endolymphatic hydrops (DEH) from 7418 patients who had visited the Neurotological Clinic of the Toyama Medical and Pharmaceutical University Hospital between 1979 and 1999. Clinical features were characterized as follows: (i) pediatric MD patients were rare, comprising approximately 1.5% of all the 337 MD patients in the sample; (ii) a fluctuating hearing loss, strong positive response in glycerol test, high SP/AP amplitude ratio in electrocochleography and moderate positive response in furosemide vestibulo-ocular reflex test were recorded; (iii) in 2 of the 5 pediatric MD patients, vertiginous attacks had persisted for a long period and they had undergone surgical procedures (sac operation and vestibular neurectomy); and (iv) the ipsilateral type of DEH was observed in 2/3 children, and contralateral DEH in 1/3.

Adolescent↗

Symptomatic versus asymptomatic endolymphatic hydrops: a histopathologic comparison.

One of the unanswered questions in Meniere's disease research is the pathophysiology by which the classic symptoms are produced. A histopathological study was undertaken to identify the pathological features of symptomatic endolymphatic hydrops and their relationship to these symptoms. Two groups of temporal bones were examined, compared, and described. The first group was from patients with symptoms of Meniere's disease (n = 29). Temporal bones in the second group were chosen for the presence of endolymphatic hydrops and the absence of otologic symptoms (n = 13). Significant differences were noted in the severity of hydrops, the frequency of membrane ruptures, the endolymphatic duct, and coexistent pathologic conditions. Ruptures were seen in 38% of temporal bones from symptomatic patients and in only 8% of temporal bones from patients with asymptomatic endolymphatic hydrops. Based on this study and a review of the literature, the authors believe that the symptoms and findings of Meniere's disease are explained best on the basis of both chemical and physical mechanisms being operational intermittently and together.

Cochlea↗

Idiopathic endolymphatic hydrops and the vestibular aqueduct.

The vestibular aqueduct (VA) and endolymphatic sac were examined topographically in 29 human ears with idiopathic endolymphatic hydrops. When compared with 34 control ears, there was found to be a slight but statistically significant reduction in the width (P less than 0.005) and length (P less than 0.05) of the vestibular aqueduct. A small hypoplastic endolymphatic sac was found in three of the ears with hydrops but was also present in one control ear. It would appear that a marked reduction in sac size is not a consistent feature in ears with endolymphatic hydrops.

Endolymphatic Sac↗

Different regulation of connexin26 and ZO-1 in cochleas of developing rats and of guinea pigs with endolymphatic hydrops.

Using confocal microscopy and morphometry, we analyzed the expression of connexin26 (Cx26) and ZO-1 in rat cochlea during the postnatal period to elucidate spatiotemporal changes in gap junctions and tight junctions during auditory development. We also studied changes in these junctions in experimental endolymphatic hydrops in the guinea pig. In the adult rat cochlear lateral wall, Cx26 was detected in fibrocytes in the spiral ligament and in the basal cell layer of the stria vascularis, whereas ZO-1 was detected in the apical surfaces of marginal cells and in the basal cell layer. During postnatal development, Cx26 expression increased mainly in the spiral ligament, whereas ZO-1 expression increased in the basal cell layer. The morphometry of Cx26 showed a sigmoid time course with a rapid increase on postnatal day (PND) 14, whereas that of ZO-1 showed a marked increase on PND 7. In experimental endolymphatic hydrops, the expression of Cx26 significantly decreased, whereas there were no obvious changes in the expression of ZO-1. These results indicate that gap junctions and tight junctions in the cochlea increase in a different spatiotemporal manner during the development of auditory function and that gap junctions and tight junctions in the cochlea are differentially regulated in experimental endolymphatic hydrops. (J Histochem Cytochem 49:573-586, 2001)

Animals↗

Degenerative changes in the organ of Corti and lateral cochlear wall in experimental endolymphatic hydrops and human Menière's disease.

The pathogenesis of sensorineural hearing loss in Menière's disease and experimental endolymphatic hydrops is not fully understood. At the light microscopic level, there is poor correlation between the histopathology and loss of sensitivity and speech discrimination. The results of electron microscopic investigation of histopathology and alterations in immunoreactivity in the organ of Corti and lateral cochlear wall in the hydropic guinea pig are presented. Loss of outer and inner hair cells and spiral ganglion cells, particularly in the apical turn was evident by light microscopy. By electron microscopy, further evidence of degeneration was detected in the cuticular plate of outer hair cells, neural endings of both inner and outer hair cells, myelinated dendritic fibers, spiral ganglion cells, and types I and II fibrocytes of the lateral cochlear wall. There was a marked decrease in immunoreactivity for a variety of enzymes, calcium binding proteins, structural proteins, and integral membrane proteins of gap junctions, particularly among type I and type II fibrocytes of the lateral cochlear wall. The evidence suggests that dysfunction and degeneration of hair cells, afferent neurons and fibrocytes of the lateral cochlear wall are involved in the pathogenesis of hearing loss in endolymphatic hydrops.

Animals↗

Diagnosis of endolymphatic hydrops by low-frequency masking.

Low-frequency masking is a new method for the diagnosis of endolymphatic hydrops. A short acoustic stimulus and a low-frequency masker tone are applied to the same ear in an adjustable phase relationship. We recorded phase-dependent masked thresholds from normal-hearing subjects, and patients with Ménière's disease and sensory hearing loss without vertigo. In normal hearing, there is a mean maximal difference in masking (modulation depth) of 28 dB between the phase delays of 0 degree and 270 degrees. In patients with sensory hearing loss without vertigo, modulation depth is reduced due to recruitment. In Ménière cases, the phase dependence may be totally absent and varies as the disease progresses. Therefore, repeated measurements of masking are required: patients and subjects with normal hearing were tested for a period of 1 year. Also, modulation depth is significantly reduced in the contralateral nonsymptomatic ears of Ménière patients. The results indicate that low-frequency masking is a quick, noninvasive and relevant method for the diagnosis of endolymphatic hydrops.

Acoustic Stimulation↗

Endolymphatic hydrops and III type allergic reaction.

IgG antibody and complement are both present in endolymphatic fluid, and it is possible that immune complex may form in endolymphatic fluid. However, there are still obscure points as to whether endolymphatic hydrops is due to immune complex, but it is quite possible that it is only one causative factor in endolymphatic hydrops.

Animals↗

The effect of glycerol on short-term experimental endolymphatic hydrops.

The aim of this paper was to evaluate the effect of one month of treatment with different dosages of glycerol on experimental endolymphatic hydrops produced by obliteration of endolymphatic sac and duct through an extradural approach. Forty-two guinea pigs were used. The animals, divided into six groups, received 0.5-2 gr/kg body weight of the drug per day for one month. The activity of glycerol was determined by statistical analysis of volumetric changes of scala media. Glycerol demonstrated the effect of reducing endolymphatic hydrops. The decrease in hydrops was influenced by the dosage suggesting a stria metabolic response.

Administration, Oral↗