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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.

Background: numerous researches on the pathological mechanism of Enlarged Vestibular Aqueduct (EVA) syndrome mainly focuses on the genetic characteristics of SLC26A4 gene and the function of its encoding protein, Pendrin. One of the limitations with these explanations is that it does not explain why cerebrospinal fluid pressure can affect clinical manifestations., Objectives: To establish a new approach to explain the clinical manifestations of EVA syndrome with biomechanical method., Material and Methods: 108 cases of EVA syndrome who received cochlear implantation were analyzed retrospectively. A cochlear model was built to reflect the differences of the structure in EVA syndrome with or without Mondini malformation. The CFD software was used to simulate and display the differences in mechanical pathogenic factors to which the model was subjected., Results: EVA syndrome patients with Mondini malformation suffer more mechanical damage from the cerebrospinal fluid pressure due to their structural reason and their symptoms appear earlier and progress faster., Conclusions: Biomechanics is an important aspect of pathological mechanism of EVA syndrome, and it provides a new angle for clinical decision-making.

Background: Inner ear hemorrhage is increasingly recognized as a cochlear lesion that can cause profound sudden sensorineural hearing loss (SSNHL). Objectives: To investigate changes of cochlear and vestibular function and to compare therapeutic recovery from profound SSNHL induced by different etiologies. Material and methods: Eighty patients with profound SSNHL (≥90 dB) were divided into an inner ear hemorrhage group and a non-inner ear hemorrhage group by MRI. Statistical analysis was performed to compare the therapeutic effects from vertigo and hearing loss and the outcomes of follow-up in the two groups. Results: There were significant differences between the two groups in terms of the overall 14-day therapeutic response rate (20 vs. 48%), the incidence of imbalance (26.7 vs. 6%), the incidence of semicircular canal dysfunction on the affected side (60 vs. 20%), the incidence of abnormal C-VEMP and O-VEMP on the affected side (63.3 vs. 38%; and 60 vs. 30%, respectively), the average hearing threshold (74.2 ± 10.7 vs. 53.6 ± 11.4 dB), and the word recognition score (65.5 ± 21.7 vs. 83.5 ± 24.5%) at a 12-month follow-up. Conclusions and significance: A higher percentage of patients with profound SSNHL induced by inner ear hemorrhage were associated with vertigo and had a poor prognosis.

Background: Diverticula in the internal auditory canal (IAC) have been reported in ears with otosclerosis., Objective: We evaluated hearing levels and vascular activity in ears with otosclerosis with and without IAC diverticula and clarify the significance of IAC diverticula., Materials and Methods: Sixty-one ears from 54 patients who underwent stapes surgery for otosclerosis [fenestral (48 ears) and retrofenestral (13 ears) groups] were included in the present study. Preoperative hearing levels on pure tone audiometry (PTA) and intraoperative measurements of blood flow were compared between the groups., Results: A total of 24 of 61 ears (39.3%) showed IAC diverticula, significantly higher than the frequency in ears without otosclerosis (3.7%). No significant differences in air- and bone-conduction thresholds on PTA were evident between ears with and without IAC diverticula in each group. Ears without IAC diverticula tended to show higher blood flow in the area anterior to the oval window than ears with IAC diverticula, but the difference was not significant., Conclusions: The incidence of the IAC diverticula in otosclerosis was significantly higher than in cases without otosclerosis. The existence of IAC diverticula was not evidently related to the severity of the disease from the perspective of hearing level and vascular activity.

Conclusions This study shows that the prevalence of auditory neuropathy spectrum disorder (ANSD) in the children with inner auditory canal (IAC) stenosis is much higher than those without IAC stenosis, regardless of whether they have other inner ear anomalies. In addition, the auditory characteristics of ANSD with IAC stenosis are significantly different from those of ANSD without any middle and inner ear malformations. Objectives To describe the auditory characteristics in children with IAC stenosis as well as to examine whether the narrow inner auditory canal is associated with ANSD. Method A total of 21 children, with inner auditory canal stenosis, participated in this study. A series of auditory tests were measured. Meanwhile, a comparative study was conducted on the auditory characteristics of ANSD, based on whether the children were associated with isolated IAC stenosis. Results Wave V in the ABR was not observed in all the patients, while cochlear microphonic (CM) response was detected in 81.1% ears with stenotic IAC. Sixteen of 19 (84.2%) ears with isolated IAC stenosis had CM response present on auditory brainstem responses (ABR) waveforms. There was no significant difference in ANSD characteristics between the children with and without isolated IAC stenosis.

Conclusion: The results support that chronic electromagnetic field exposure may cause damage by leading to neuronal degeneration of the auditory system., Objectives: Numerous researches have been done about the risks of exposure to the electromagnetic fields that occur during the use of these devices, especially the effects on hearing. The aim of this study is to evaluate the effects of the electromagnetic waves emitted by the mobile phones through the electrophysiological and histological methods., Methods: Twelve adult Wistar albino rats were included in the study. The rats were divided into two groups of six rats. The study group was exposed to the electromagnetic waves over a period of 30 days. The control group was not given any exposure to the electromagnetic fields. After the completion of the electromagnetic wave application, the auditory brainstem responses of both groups were recorded under anesthesia. The degeneration of cochlear nuclei was graded by two different histologists, both of whom were blinded to group information., Results: The histopathologic and immunohistochemical analysis showed neuronal degeneration signs, such as increased vacuolization in the cochlear nucleus, pyknotic cell appearance, and edema in the group exposed to the electromagnetic fields compared to the control group. The average latency of wave in the ABR was similar in both groups (p > 0.05).

Conclusion: An inner ear test battery may suggest bilateral involvement and aid in 'grading' of potential disease in a patient with actively symptomatic Meniere's disease (MD)., Objectives: This study applied an inner ear test battery in patients with bilateral MD to map the inner ear deficits in each ear., Methods: From 2009 to 2012, 100 (20%) of 498 MD patients were diagnosed with bilateral involvement, which was defined as established MD in one ear, and the opposite ear had inner ear symptoms combined with documented hearing loss. Each patient underwent audiometry, and ocular vestibular-evoked myogenic potential (oVEMP), cervical VEMP (cVEMP), and caloric tests., Results: Grading of inner ear deficits was based on the number of abnormal results in the inner ear test battery. Of 100 patients with bilateral MD, 54% had the same grade and 46% had different grades in their 2 ears. On the other hand, based on four-tone average, 79% had the same Meniere stage and 21% had different stages in their two ears. The difference between ears with asymmetric grades (46%) and ears with asymmetric stages (21%) accounts for 25% of bilateral MD patients, which may be caused by the saccular hydrops in the opposite ear.

Conclusions: Intraoperative computed tomography (iCT)-guided cochlear implantation is practical and effective for correct electrode placement in the cochlea of patients with congenital inner ear and/or complex middle ear malformation., Objectives: The operation in patients with inner ear and/or complex middle ear malformation including abnormal facial nerve course is difficult. This study evaluated the efficacy of cochlear implantation under the guidance of iCT to insure correct electrode placement., Methods: This was a prospective interventional case series. Ten patients with severe to profound sensorineural hearing loss due to ear malformations were enrolled, and iCT was used to confirm the right placement of electrodes., Results: Intraoperative CT was performed three times in one patient, twice in two, and once in the others. Interruption of the surgical process for each iCT until resumption of surgery was 9.64 ± 0.63 min. iCT revealed incorrectly positioned cochlear implants in two patients, which were immediately corrected. There were no reoperations due to misplacement of electrodes. iCT helped locate the cochlea in the middle ear of one patient with an abnormal facial nerve course. The overall intervention rate based on iCT findings was 30%., Level of Evidence: level 4.

This paper is a review of our most recent findings concerning the osteo-dynamics of the bony otic capsule and pathogenesis of otosclerosis. By exploring the spatial relationship between normal perilabyrinthine bone remodeling, the viability and spatial distribution of labyrinthine osteocytes, and the location of otosclerosis, a unique spatial pattern emerged. Bone remodeling is highly inhibited around the inner ear space. Most likely, inner ear anti-resorptive signals enter the bony otic capsule through the lacuno-canalicular porosity. The patency of this signaling pathway depends on the viability of individual osteocytes. In the young otic capsule the density of viable osteocytes is high and centripetally distributed. This arrangement may sustain a life-long osseus pathway for anti-resorptive signals even within a bone where a considerable loss of viable osteocytes must be expected, as demonstrated by a centripetal accumulation of dead osteocytes with age. The spatial distribution of dead osteocytes follows the same general pattern as otosclerosis. We suggest that clustering of dead osteocytes may impede the transmission of anti-resorptive signals locally, leaving such ghost regions susceptible to focal bone remodeling as in human otosclerosis. The preserved network of viable osteocytes around the depleted ghost regions may contain the process and distort the structure of bone remodeling into an abnormal otosclerotic pattern.

Conclusions: The blood-labyrinth barrier is impaired in association with the hydrops grade in Ménière's disease., Objectives: To investigate the relationship between endolymphatic hydrops and the clinical characteristics of patients with Ménière's disease revealed by 3 T magnetic resonance imaging (MRI)., Methods: A double dose of gadoteridol (Gd; 0.2 mmol/kg) was injected intravenously in 12 patients with Ménière's disease. We performed three-dimensional fluid attenuated inversion recovery MRI and three-dimensional real inversion recovery MRI 4 h later using a 3 T MRI unit. Ten patients had unilateral and two had bilateral Ménière's disease., Results: Fourteen ears with Ménière's disease showed intense Gd contrast on MRI compared with that in the 10 asymptomatic contralateral ears of patients with unilateral Ménière's disease (1.12 ± 0.36 vs 0.82 ± 0.15). The hydrops grade was correlated significantly with the contrast effect. The 14 ears with Ménière's disease had endolymphatic hydrops. Of the 10 contralateral ears of patients with unilateral Ménière's disease, 2 had endolymphatic hydrops in the cochlea and 6 had endolymphatic hydrops in the vestibule.

Conclusions: Our results show that among the patients with inner ear malformations, there were two patients with sensory cells of sacculus and inferior vestibular neurons and at least five patients with inferior vestibular neurons only, but no patients without sensory cells and vestibular neurons, as determined on the basis of vestibular evoked myogenic potentials (VEMPs)., Objective: The aim of this study was to investigate whether sensory cells of sacculus or inferior vestibular neurons or both are present in cochlear implant patients with inner ear malformations, particularly common cavity (CC) deformity, using VEMPs., Patients and Methods: Seven children with inner ear malformation who underwent cochlear implantation (CI) participated in this study. The patients had CC deformity (n=2), incomplete partition type I (n=2), incomplete partition type II (n=1), or a narrow internal auditory canal (IAC) (n=2). It was possible to record VEMPs before and after CI in three patients and not before but after CI in four patients. After surgery, VEMPs were recorded with the cochlear implant device switched both off and on., Results: Before the CI, two patients showed VEMPs and one patient showed no VEMPs, whereas in four patients no VEMPs could be recorded. All the patients showed VEMPs with the cochlear implant on.

Conclusion: Use of the Meniett pressure generator in patients with active Ménière's disease (MD) did not affect labyrinthine function., Objective: To investigate whether application of the Meniett pressure generator in patients with active MD significantly affects their labyrinthine function., Material and Methods: We measured hearing and labyrinth sensitivity in patients with established MD according to American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) criteria at five different time periods: at baseline; before ventilation tube placement; before Meniett treatment; immediately after Meniett treatment; and 1 month after cessation of Meniett treatment., Results: A total of 32 patients met the inclusion criteria and were included in the study. They were graded as having definite MD (AAO-HNS stages 2 and 3). No statistically significant changes in labyrinthine function were found during Meniett treatment.

Conclusion: If the high-frequency (HF) component of the R-R interval spectrum of the electrocardiogram (ECG) increases after the first stimulation during a bithermal caloric test, subsequent repeated stimulations may be likely to induce autonomic symptoms such as nausea., Objective: Nausea often occurs as a vestibulo-autonomic symptom during the caloric test. However, there have been only a few clinical studies of autonomic activity during this test. To determine whether certain factors can predict the development of nausea, we performed an autonomic function test by means of R-R interval spectral analysis of the ECG during caloric testing., Material and Methods: The study subjects were 53 healthy volunteers aged 22-39 years (41 males, 12 females; mean age 25.3 years) and a 35-year-old male patient with a labyrinthine defect. The caloric test was performed in 43 subjects using an air caloric apparatus with bithermal stimulation of alternate ears. After each stimulation, the subjects were asked about autonomic symptoms. Serial ECG records were obtained and R-R interval spectral analysis was performed., Results: Severe nausea was observed in 12 subjects (28%). In the group with severe nausea, the HF component of the R-R interval spectrum increased after the first caloric stimulation when autonomic symptoms had not yet become marked.

Objective: One of the clinical characteristics of benign paroxysmal positional vertigo (BPPV) is that the more quickly the head position changes, the more severe the vertigo. This suggests that the velocity of the head change is critical in determining the occurrence and severity of vertigo. The aim of this study was to examine factors determining the symptoms of BPPV using models of canalolithiasis and cupulolithiasis., Material and Methods: Canalolithiasis and cupulolithiasis models were prepared using the bullfrog posterior semicircular canal (PSC). The ampullary nerve discharges were compared between quick and slow positional changes to examine factors determining the symptoms of BPPV., Results: In the canalolithiasis model, the acceleration of the otoconia was greater for the quick positional change. This resulted in a greater discharge with a longer duration. With the slow positional change, the discharges were smaller and shorter. In the cupulolithiasis model, the discharges were sustained and their magnitude did not differ between the quick and slow positional changes. The canalolithiasis model influenced the magnitude of discharge of the PSC depending on the speed of the positional change., Conclusion: Canalolithiasis is the more likely mechanism of BPPV, which is characterized by various degrees of vertigo upon kinetic positional change.

It has taken many decades to arrive at today's concept of cupula mechanics in the stimulation of endolymphatic flows on the hair cells in the ampullae of the semicircular canal. While Steinhausen assumed free swing-door movement of the cupula in the 1930s, Hillman was the first to demonstrate firm cupula attachment to the ampulla wall as a physiological necessity in the 1970s. In contrast to the present clinical concepts of acute peripheral vestibular functional disorders (circulatory disturbances, viral or bacterial infection, altered electrolytes in the endolymph), this study examines the extent to which an impaired attachment mechanism can trigger peripheral vestibular disorders. For this purpose, we used a pigeon model (n = 8), in which mechanical detachment of the cupula from the ampulla wall was achieved by means of a targeted pressure increase in the ampulla of the lateral semicircular canal. In two additional animals the labyrinth was completely destroyed on one side in order to directly compare partial and complete vestibular disorders. In this way partial damage to the lateral semicircular canal ampulla presents a clinical picture whose symptoms are very similar to those of an idiopathic vestibular disorder in humans. Their intensity and course of compensation differ markedly from the symptoms of complete vestibular destruction. Subsequent histological examination revealed that the hair cells remained intact during the experimental detachment of the cupula. Our results thus show that only altered cupula mechanics seem to trigger the clinical picture of a peripheral vestibular disorder. This may result in completely new approaches to differential diagnosis and the therapy of vestibular neuronitis.

In order to assess the influence of canal paresis and compensation on the yaw-axis rotatory chair test parameters gain and time constant, a study was conducted in 435 patients diagnosed with unilateral peripheral vestibulopathy. Patients were grouped according to the amount of canal paresis found in the caloric test and by the result of a bedside examination of the vestibulo-ocular reflex (VOR). The time constant of the VOR diminishes as canal paresis increases and, when using a high-velocity sinusoidal test, there is a similar reduction in gain. Mean time constant was reduced when rotations were to the side of the lesion (ipsilesional) and to the normal (contralesional) and differences between both rotations were found for 21-80%, canal paresis. Ipsilesional gain was significantly less than contralesional gain, which had a normal result independent of the amount of canal paresis; differences between them were significant when canal paresis was > 41%. Spontaneous nystagmus significantly influenced the appearance of asymmetries in gain and time constant in the tests performed while, with high-velocity sinusoidal rotation, gain was found to be significantly different for ipsilesional and contralesional rotations in non-acute symptomatic patients.

Immersion in an illusory world is possible by means of virtual reality (VR), where environmental perception is modified by artificial sensorial stimulation. The application of VR for the assessment and rehabilitation of pathologies affecting the vestibular system, in terms of both diagnosis and care, could represent an interesting new line of research. Our perception of reality is in fact based on static and dynamic spatial information perceived by our senses. During head movements in a virtual environment the images on the display and the labyrinthine information relative to the head angular accelerations differ and therefore a visuo-vestibular conflict is present. It is known that mismatches between visual and labyrinthine information may modify the vestibulo-oculomotor reflex (VOR) gain. We studied the post-immersion modifications in 20 healthy subjects (mean age 25 years) exposed to a virtual environment for 20 min by wearing a head-mounted display. VOR gain and phase were measured by means of harmonic sinusoidal stimulation in the dark before, at the end of and 30 min after VR exposure. A VOR gain reduction was observed in all subjects at the end of VR exposure which disappeared after 30 min. Our data show that exposure to a virtual environment can induce a temporary modification of the VOR gain. This finding can be employed to enable an artificial, instrumental modification of the VOR gain and therefore opens up new perspectives in the assessment and rehabilitation of vestibular diseases.

3D analysis of eye movements during off vertical axis rotation (OVAR) was carried out in seven subjects with unilateral labyrinthine loss (ULL). The modulation component (MOC) of these patients was not different from that of normal subjects. However, the horizontal MOC was significantly smaller when the rotation was directed towards the diseased side as opposed to the healthy side. With rotation to the diseased side, most subjects exhibited a horizontal bias component (BIC) to the opposite side as compared with the diseased side in normal subjects. The vertical and torsional BICs were also influenced to some extent by lack of input from the unilateral labyrinth. These results indicate that a unilateral otolith organ plays a major role in the production of the horizontal BIC contralateral to the direction of rotation, which is strongly related to the velocity storage mechanism in the central nervous system. In addition, the unilateral otolith organ exerts some influence on the generation of the horizontal MOC and also the BIC of vertical and torsional eye movements.

Benign paroxysmal positional vertigo (BPPV) originating from the posterior semicircular canal (pSCC) is a common vestibular disorder that is easy to diagnose and usually easy to treat. The majority of patients with BPPV have no known inner ear disease; they have "primary" or "idiopathic" BPPV. However, a minority does have objective evidence of an inner ear disease on the same side as the BPPV and this group has "secondary" or "symptomatic" BPPV. Previous publications differ on the prevalence of secondary BPPV and about the types of inner ear diseases capable of causing it. In order to determine what proportion of patients have secondary as opposed to primary BPPV and which inner ear diseases are capable of causing secondary BPPV, we searched our database for the 10-year period from 1988 to 1997 and found a total of 2847 patients with BPPV. Of these, 81 (3%) had definite pSCC-BPPV secondary to an ipsilateral inner ear disease. Sixteen had Menière's disease, 24 had an acute unilateral peripheral vestibulopathy, 12 had a chronic unilateral peripheral vestibulopathy, 21 had chronic bilateral peripheral vestibulopathy and 8 had unilateral sensorineural hearing loss. It seems that any inner ear disease that detaches otoconia and yet does not totally destroy pSCC function can cause BPPV and that a case can be made for audiometry and caloric testing in all patients with BPPV.

The vestibulo-ocular reflex (VOR) allows clear vision during head movements by generating compensatory eye movements. Its response is reduced following damage to the vestibular endorgan, but recovers over time. The VOR is mediated by both direct and indirect anatomical pathways; most direct pathways include only two central synapses, both located in the brainstem. To investigate the possibility that a direct pathway is modified during the recovery of VOR gain, we measured the oculomotor response to single current pulses delivered to the vestibular labyrinth of two alert cats after plugging the contralateral horizontal canal. The response was also measured after motor learning induced by continuously worn lenses (optically induced motor learning) in two cats. The gain of the VOR was monitored concurrently. The eye movement evoked by a current pulse increased more than 100% during recovery from a plug. The electrically evoked eye movement did not change during optically induced motor learning either before the plug or after recovery. The gain of the VOR was modified in both situations. We conclude that direct VOR pathways are modified significantly during recovery after a plug, but not during optically induced learning. Our results suggest that significant modification of direct pathways may require a change in vestibular sensory input.

Electrophysiological changes were studied in the albino rat following instillation of Pseudomonas aeruginosa exotoxin A into the middle ear cavity through the tympanic membrane. Hearing threshold was measured by a burst-elicited, frequency-specific auditory brainstem response (ABR) technique prior to exposure, then 24 and 48 h, 5 days, 2 and 4 weeks after the toxin instillation. A single dose (1 microgram/20 microliters) of Pseudomonas aeruginosa exotoxin A raised the ABR threshold over the whole frequency range, by 5-25 dB, particularly in the high tones. All threshold shifts were of combined conductive and cochlear type, reversible, with deterioration starting at 24-48 h and recovery at 2-4 weeks. Effusion of serous fluid occurred at 24 or 48 h, resulting in conductive hearing loss. Latency/intensity curves revealed a cochlear component in addition to conductive hearing loss. Morphological examination by SEM showed slight and inconsistent derangement of OHCs. It is concluded that Pseudomonas aeruginosa exotoxin A causes middle ear inflammation, facilitating penetration to the inner ear and that this toxin also reversibly affects cochlear function.

We evaluated changes in the subjectively perceived gravitational vertical as an index of imbalance in the function of the right and left otolith organs. In addition to normal subjects (n = 25), we measured patients with a longstanding (mean 4.5 year +/- 3.2 SD; range 0.5-11.5 years) unilateral vestibular loss after surgery for acoustic neuroma (n = 32), patients with partial unilateral vestibular loss (n = 7) and patients with bilateral vestibular hyporeflexia (n = 8). Normal subjects could accurately align a vertical luminous bar to the gravitational vertical in an otherwise completely dark room (mean setting -0.14 degree +/- 1.11 SD). Patients with left-sided (complete; n = 13) or right-sided (complete; n = 19 and partial; n = 7) unilateral vestibular loss made mean angular settings at 2.55 degrees +/- 1.57 (SD) leftward and 2.22 degrees (+/-1.96 SD) rightward, respectively. These means differed highly significantly from the normal mean (p < 0.00001). In the time interval investigated (0.5-11.5 years) the magnitude of the tilt angle showed no correlation with the time elapsed since the operation. The mean setting by patients with clinically bilateral vestibular loss (-1.17 degrees +/- 1.96 SD; n = 8) did not significantly differ from the control group. The systematic tilts of the subjective vertical in patients with a unilateral vestibular impairment were correlated with their imbalance in canal-ocular reflexes, as reflected by drift during head-oscillation at 2 Hz (r2 = 0.44) and asymmetries in VOR-gain for head-steps (r2 = 0.48-0.67). These correlations were largely determined, by the signs of the asymmetries; correlation between the absolute values of the VOR gain asymmetries and subjective vertical angles proved to be virtually absent. We conclude that the setting of the subjective vertical is a very sensitive tool in detecting a left-right imbalance in otolith function, and that small but significant deviations towards the defective side may persist for many years (probably permanently) after unilateral lesions of the labyrinth or the vestibular nerve.

We investigated the effect of increased middle ear pressure on blood flow to the inner ear, middle ear, and facial nerve in guinea pigs using a nonradioactive microsphere technique. The elevation of middle ear pressure significantly reduced blood flow to the middle ear. Blood flow to the facial nerve also decreased due to the elevation of the middle ear pressure but the reduction was not significant. Blood flow to the inner ear did not change even after the middle ear pressure was increased. Findings are discussed in relationship to aural barotrauma, alternobaric vertigo and facial baroparesis.

In a retrospective study of 475 patients suffering from Meniere's disease, the hearing loss, both in the low and high frequencies, and the loss of vestibular function were studied over time. There were large variations of hearing loss from case to case but, overall, the low were more affected than the high frequencies, regardless of time elapsed since onset of disease. Hearing deteriorated over time and mean values of hearing loss correlated well with the elapsed time since onset of disease. There were also large variations of post-caloric labyrinthine preponderance (lp) from case to case but, overall, lp deteriorated with time. Mean values of lp, however, did not correlate that well with elapsed time since onset of disease as the hearing loss did. Nor was there any correlation over time between level of hearing loss and lp. It is concluded that in Meniere's disease the hearing, deteriorating over time, is mainly but not necessarily worse in the low frequencies; lp, indicating loss of vestibular function, does not correlate with level of hearing loss in most cases.

In the mammalian labyrinth, GABAA receptor subtypes are involved in the excitatory neurotransmission between the vestibular type II hair cells and the afferent neurons. Additional afferent ionophoric receptor channels, sensitive to further transmitter candidates, are discussed for both types I and II hair cells. GABA accelerates excitotoxic cell death in cortical neurons. This GABA-ergic neurotoxic action forms an appropriate pathophysiological model explaining peripheral labyrinthine disorders characterized by the spontaneous labyrinthine nystagmus and vertigo in man. A calculated GABAA receptor antagonism was envisaged in order to attenuate the pathological vestibular imbalance following one-sided GABA-accelerated vestibular neurotoxicity. Moderate allosteric blockers of the GABAA receptor channel and weak inverse agonists of the benzodiazepine binding site meet some requirements for potentially successful clinical application. The suppressing action of the suitable drugs picrotoxin and flumazenil on labyrinthine nystagmus and vertigo, tested in clinical trials, supports the hypothesis that GABAA receptors are involved in vestibular neurotransmission, even in humans. The test results promise the development of a successful vestibular receptorpharmacology in the near future.

Electrocochleographic recordings were made in 17 patients with Menièr's disease before and immediately after exposure to pressure applications in their middle ear. The variables of the response to clicks such as the SP/AP ratio and the width of the complex as well as the amplitudes of the SP in response to long tone bursts were evaluated. Improvement of electrocochleographic recordings, characterized by normalization of the waveforms and reduction of the SP, was found to be significant. The results indicate a mechanoelectric change in the hydroptic cochlea registered directly after exposure to middle ear pressure changes.

Evokation of caloric nystagmus in microgravity argues against thermal convection as a sufficient explanation for a caloric nystagmic response. By contrast, caloric nystagmus observed in weightlessness suggests a gravity independent mechanism. Here we demonstrate that direct caloric stimulation of isolated living vestibular hair cells from the guinea pig evoked mechanical responses of the sensory cells. Heating of the mechanoreceptor cells from 37 degrees C to 42 degrees C resulted in elongation cooling of the cells from 37 degrees C to 30 degrees C induced shortening of vestibular hair cells. The observed mechanism might contribute to the caloric evokation of a nystagmus both on earth and in orbit.

To evaluate biochemical changes of inner ear fluid following perilymphatic fistula (PLF), free amino acid (FAA) profiles of perilymph in experimental PLF were determined using high performance liquid chromatography (HPLC). Thirty-five guinea pigs were anesthetized and prepared as PLF models by perforating the round window membrane (RWM) of the left ear. Right ears served as controls. Samples (2 microliters) were aspirated from scala tympani through a RWM perforation. Animals were divided into two groups according to time of sampling following PLF induction: 2-week group (n = 17) and 4-week group (n = 18). Compound action potential (CAP) evoked by 1, 2, 4 and 8 kHz tone bursts were measured using a round window electrode from the left ear before PLF induction and from both ears before final sampling. RWM perforations were completely closed at the time of the final sampling in 8 of 17 animals from the 2-week group, and 15 of 18 animals from the 4-week group. In comparison with that in control ears, concentrations of FAA throughout all profiles was dramatically elevated in the PLF ears with a healed RWM perforation. Most PLF ears with persistent RWM perforation showed minimal differences between 2-week and 4-week groups. No remarkable CAP threshold changes were found at any frequencies tested following PLF induction in both the 2-week and 4-week groups. The unchanged 8 kHz threshold suggests that FAA concentration increases only at the basal end of the cochlea. FAAs accumulate within the basal end of scala tympani in ears with a healed RWM.(ABSTRACT TRUNCATED AT 250 WORDS)

In the present study, evoked otoacoustic emissions (EOAEs) were measured in 30 patients with tinnitus before and after intravenous lidocaine injection (1 mg/kg). For EOAE recordings, 1 kHz tone burst stimuli were used. Intravenous lidocaine injection resulted in suppression of tinnitus in 22 (73%) ears, and changes of EOAE amplitude (increase or decrease) in 18 (60%) ears. Of the 18 ears with EOAE amplitude changes, tinnitus disappeared or decreased in 17 (94%) ears. In 12 ears without changes of EOAE amplitude, tinnitus was suppressed in only 5 (42%). Changes in latency were not detected in any of the ears. These results indicate that there is a relationship between the effect of lidocaine in tinnitus suppression and changes in cochlear micromechanics caused by lidocaine.

The properties of the vestibulo-ocular reflex (VOR) were examined during sinusoidal passive head rotation in the dark at 1/6 Hz, in 9 normal subjects and 14 unilateral vestibular patients. Rotation speeds ranged from 90 to 180 degrees/s. The bias (offset of slow-phase velocity from zero) and gain in the VOR were estimated by using a polynomial (cubic) fit between head and slow-phase eye velocity, thereby allowing for possible non-linearities in the reflex. The gain in the VOR in this context refers to the linear components of the fit, and so predicts sensitivity only at low head velocities. The aim of the study was to verify previous theoretical predictions that VOR bias could vary with the rotation parameters, that this bias could be used to detect the side of a vestibular lesion even at low frequency rotation, and make non-linearities more obvious. Confirming these predictions, the VOR bias in a given test is never equal to any spontaneous nystagmus, even if present before rotation. The range of values for the gain in the VOR (as defined above) in normals and compensated unilateral vestibular patients overlap, so that they cannot be statistically separated into two response sets.(ABSTRACT TRUNCATED AT 250 WORDS)

The present material is based on a birth cohort of 2,512 children followed up from the antenatal period. Data on middle ear infections were collected until the children were 7 years. At the age of 7, audiometry was performed on 298 of them. For reasons other than middle ear infection, 32 children were excluded and the final material comprised 266 children. Only those children with clear differences in their history for otitis media were analysed. Although the pure tone averages (0.5-3 kHz) showed no major changes, different mean air-conduction (AC) thresholds were associated with different histories of otitis media. The 35 healthy children (with no otitis media episodes until 2 years of age) showed the best mean AC thresholds. The 51 children with recurrent acute otitis media (RAOM; > or = 4 episodes of acute otitis media) showed worse thresholds at the high frequencies and those 13 who had had secretory otitis media (SOM) at all frequencies. The children with a history of otitis media, either RAOM or SOM, more often had hearing thresholds exceeding 20 dB. Our results might indicate inner ear involvement in these long-term follow-up hearing thresholds.

One hundred thirty-seven patients with chronic disabling paroxysmal positional vertigo were treated surgically with singular neurectomy (SN) during the years 1972-1994. One hundred forty-six SN were performed on these patients. One hundred twenty-eight patients underwent unilateral SN while 9 patients underwent bilateral sequential SN. The patients ranged in age from 25 to 86 years with a mean of 57 years and a female to male ratio of 92: 45. One hundred thirty-eight patients (94%) experienced complete relief of vertigo following SN. Three patients (2%) experienced partial relief of positional vertigo following SN and 5 patients failed to have any improvement of symptoms following SN (4%). A partial sensorineural occurred following SN in 4 patients (3%) in this series. The results indicate that SN is a safe and effective surgical treatment for chronic disabling BPV caused by the posterior semicircular canal.

The various constituents of the tubules of the human adult intraosseous endolymphatic sac were stained with lectins and Millon reaction to reveal some of the carbohydrate and protein-containing substances in the endolymph. The amount of each substance was determined by digitizing sequential temporal bone sections. Differing amounts of the various identified substances were found in sacs from patients with Meniere's disease, patients with labyrinthine fibrosis, and controls.

Pneumococcal otitis media is frequent in young children and could lead to labyrinthitis post-implantation. To assess the risk, and methods of minimizing it by a graft to the round window around the electrode entry point, we have used a cat animal model of pneumococcal otitis media. Twenty-one kittens were used in the study. Thirty-two cochleas were implanted when the kittens were 2 months of age. Fourteen cochleas were implanted without using a graft (12 were available for study); 9 had a fascial graft, and 9 a Gelfoam graft (7 were available for study). The implanted kittens had their bullae inoculated with Streptococcus pneumoniae 2 months after implantation and were sacrificed 1 week later. There were also 9 unimplanted control ears which were inoculated when the animals were 4 months of age. Labyrinthitis occurred in 44% of unimplanted control, 50% of implanted ungrafted, and 6% of implanted grafted (fascia and Gelfoam) cochleas. There was no statistically significant difference between the unimplanted control and the implanted cochleas (p < 0.05). There was, however, a difference between the implanted-ungrafted and implanted grafted cochleas, but not between the use of fascia and Gelfoam to graft the round window entry point. As a result, the data indicates that cochlear implantation does not increase the risk of labyrinthitis following pneumococcal otitis media, but it is desirable to use fascia as a graft to the round window around the electrode entry point.

Three dimensional Fourier transformation constructive interference in steady state (3DFT-CISS) magnetic resonance imaging (MRI) allows a detailed visualization of the membranous labyrinth of the inner ear. In this study the endolymphatic duct and sac is investigated in patients with Menière's disease using 3DFT-CISS MRI. In addition, the distance between the vertical part of the posterior semicircular canal and the fossa posterior is quantified.

Blood viscosity and plasma viscosity were measured in 51 patients with sudden deafness (SD) and 70 controls with normal hearing. Blood viscosity and plasma viscosity in patients with SD at their first medical examination were significantly higher than in the control group. The difference in viscosimetry results between the two groups was greater at higher shear rates. The data obtained in viscosimetry and pure-tone audiometry were analyzed after dividing the patients into a high viscosity group and a normal viscosity group. The correlation between average hearing level in pure-tone audiogram and blood viscosity or plasma viscosity was positive. The values of the O2-transport capacity of the blood demonstrated a negative correlation with average hearing level in patients with SD before treatment. During the course of treatment, blood viscosity and plasma viscosity decreased with the improvement of hearing impairment. When the distribution of average hearing level was 40 to 79 dB, a few of the patients with "recovery" or "good improvement" and most of patients with "fair improvement" or "no change" belonged to the low viscosity group. And, most of the patients with flat type hearing impairment and a few patients with high tone type hearing impairment belonged to the high viscosity group. These results suggest that many patients with SD have increased blood viscosity and plasma viscosity, and that this increase may play a significant role in the etiology of SD. There are also some differences in etiologic factors concerning type of hearing impairment and prognosis. In conclusion, the present study points to the importance of measuring blood viscosity and plasma viscosity in patients with SD, since blood and/or plasma viscosity may be involved in its etiology and prognosis.

Dysfunction of the mechanical properties of the basilar membrane is a potential cause of presbycusis. In cases of minimal sensorineural or strial degeneration it is believed to play a major role. The membrane has been shown to be partly composed of fibronectin. Fibronectin immunoreactivity is diminished in aged rats. Mesothelial cell line the perilymphatic surface of the membrane and are reduced in number in the aged rat cochlea. Fibronectin immunoreactivity was examined in human temporal bone sections (6 months to 92 years old). Hematoxylin and eosin stained section (17 to 97 years) were immunoreactivity was demonstrable in the human cochlea, but was not reduced, even in the eldest cases examined The number of mesothelial cells was reduced, however, and was related to the age of the individual, but not to the clinical diagnosis or audiogram shape. These two factors do not, therefore, appear to give rise to hearing losses associated with presbycusis.

Nine cats with the cochlear aqueduct surgically blocked were anaesthetised, put in a pressure chamber, and stepwise exposed to hypobaric pressure, each of the two steps lasting 5 min. The chamber pressure was reduced to -5 and -7 kPa. Concluding the experiment the cats were observed for 5 min after restoration of atmospheric pressure. The perilymph, tympanic cavity, cerebrospinal fluid, central venous, arterial and surrounding chamber pressures were continuously recorded. Reduced chamber pressure caused relative overpressure within the tympanic cavity, thereby inducing pressure gradients between the chamber, the tympanic cavity, the labyrinth and the cerebrospinal fluid and vascular pressures respectively. Our results demonstrate that hypobaric effects on the labyrinth are mediated via pressure changes in the tympanic cavity and not by systemic, vascular or cerebrospinal fluid influence. Substantial perilymph overpressure lasted throughout the hypobaric conditions. The magnitude and the duration of this overpressure were influenced by the patency of the Eustachian tube whereas the rate of the pressure change in the chamber was of little importance. As atmospheric pressure was restored in the chamber the overpressure in the labyrinth was reversed to underpressure--relative to pre-experimental conditions.

Most attempts at developing a model of autoimmune inner ear disease have focused on the immunization of healthy animals with cochlear tissue. We have chosen an alternate route of studying this entity utilizing the MRL-lpr/lpr (Lupus) mouse, an animal known to spontaneously develop multisystemic, organ nonspecific autoimmune disease. We report on the auditory pathology found in animals at early stages of this systemic disease. At the onset of clinical signs of illness (cachexia, weight loss, lethargy) animals were sacrificed and their cochleas and kidney prepared for morphologic analysis. Significant pathology was seen in the MRL/lpr animals involving the basal and middle turns of the cochlea which could not be correlated with the presence or degree of glomerulonephritis. Findings included outer and inner haircell degeneration, strial edema and degeneration, and an acellular infiltrate in the tunnel of Corti. Cochlear pathology was not found in control animals. Thus, at early stages of systemic disease, MRL/lpr mice manifest significant cochlear pathology not seen in control animals. The implications of these results with regard to the pathogenesis of these lesions as well as their clinical relevance are discussed.

Mercury deposition and its relationship to the inner ear function in methylmercury-poisoned rats were examined. Rats were poisoned with methylmercury chloride. After the appearance of signs of poisoning, the cerebellar and inner ear tissues were examined histochemically using an autoradiographic procedure. Mercury deposits were typically found in the cerebellum and appeared in parts of the vestibular nerves, cochlear nerves, spiral ganglion and stria vascularis. In the vestibule, a slight mercury deposition was observed in the acoustic maculae and in the cochlea; only one instance of mercury deposit in the organ of Corti was detected. The effect of mercury on the inner ear function was also assessed on the basis of immunohistochemical localization of substance P which is believed to be a neurotransmitter in the inner ear. However, no pathological changes were detected. These findings suggest that methylmercury deposition has little effect on the inner ear function.

Collapse of the membranous labyrinth of the pars superior is a morphological change observed in 50% of animals with experimentally produced perilymph fistula, although the extent and degree of the collapse may vary greatly. The moderately collapsed membranous labyrinth may drift with CSF and/or perilymph pressure changes and this may stimulate sensory cells of the utricle and/or semicircular canals if the sensory cells are intact and the collapsed wall is in contact with the otolithic membrane and/or cupula. This condition is termed "floating" labyrinth. Caloric irregularity is often observed in electronystagmograms recorded from animals with experimental perilymph fistula. This is also observed in patients with perilymph fistula. Partial destruction of the vestibular organs using argon laser was performed in a patient with perilymph fistula who was incapacitated because of persistent positional vertigo after closure of the oval window fistula. Irradiation of the argon laser beam was directed to the macula utriculi, utriculoampullary nerve and singular nerve. The hearing of the patient was maintained, and vertigo disappeared after laser labyrinthectomy.

In a previous study (1) it was found that in healthy subjects Ocular (Counter) Rotation is mainly due to otolith stimulation and only to a minor extent induced by slanted visual structures. Stimulation of the neck by tilting the trunk laterally upwards did not result in a systematic rotation of the eyes. In the present study it was found that subjects with bilateral loss of vestibular function showed a higher visually induced ocular rotation. Tilting the head (cervical stimulation) or the whole body (somatosensory stimulation) also led to a considerable OCR, demonstrating substitution of other sensory modalities for the loss of vestibular function. Estimates of the subjective horizontal were noisy, demonstrating the lack of an adequate gravitational reference signal.

An increase in the ratio of the summating potential to the action potential components of the electrocochleogram is known to be a feature of endolymphatic hydrops. We investigated the value of the SP/AP ratio in response to condensation and rarefaction click stimuli delivered separately. In patients with electrophysiological evidence of endolymphatic hydrops there was found to be a significantly greater SP/AP ratio to condensation clicks than rarefaction clicks. This finding supports the hypothesis that the increased SP/AP ratio in hydrops is due to mechanical asymmetry of the basilar membrane.

A technique for rapid, balanced hot/cold stimulation of the vestibular receptor is presented. During continuous aural irrigation the temperature of the irrigation fluid is switched between hot and cold values at times computed according to a mathematical model of heat conduction in the labyrinth area. As a result, the induced temperature difference across the lateral semicircular canal describes an approximately sinusoidal time course, reaching peak values of equal magnitude but opposite sign. Application of the test to 32 clinical subjects demonstrated that the heat conduction model and the analysis used in timing the sequence of thermal pulses was accurate. We expect that, with further refinements, the new technique will prove superior to conventional caloric test methods in the detection and measurement of subtle as well as gross abnormalities of the vestibular system.

It was demonstrated in experiments on normal subjects that moderate ambient pressure changes, creating overpressure in the middle ear, may induce a vestibular reaction. In other experiments on subjects suffering from acute attacks of Meniere's disease, relief of symptoms was achieved by means of ambient pressure changes of the same magnitude.

A midline loudness balance test was made in 10 normal ears with blocked ear canals and in 104 conductively deaf ears with loss not exceeding 45 dB. The test was made by giving the tones simultaneously in the ears at 10, 60 and 80 dB sensation levels for 500 and 2 000 Hz. In the normal group the midline balance method indicated no reduction from the threshold differences at any of the three levels whereas significant reductions occurred in all groups of conduction deafness. The occurrence of true partial recruitment in conduction deafness demonstrated by ABLB (Fowler's) test was attributed to altered middle ear mechanics, loud tones stimulating the inner ear relatively more effectively than tones near the threshold level. The midline balance method is more closely dependent upon bone conducted sounds and on the altered relative movements of the labyrinthine windows.

Inner ear function was assessed by a frequency-specific (+/-100 Hz) auditory brainstem response (ABR) technique after a single instillation of a suspension of purified E. coli lipopolysaccharide in sterile water into the round window (RW) niche in rats. Instillation of endotoxin caused a transient concentration and tonotopically dependent dysfunction of the inner ear. The largest threshold impairment occurred in the high-frequency region anatomically located close to the RW. At 31.5 kHz the threshold impairment persisted throughout the study. Morphologic damage to the inner ear was not detected at the light microscopic level when using serial sections from decalcified specimens. Our study supports the clinical hypothesis that remnants of non-viable bacteria such as endotoxins, when trapped in the middle ear, can promote middle ear effusion and dysfunction of the inner ear.

A report is presented of experiments on guinea pigs and pigeons concerning behavioural and electrophysiological responses of the labyrinth to sound. In the guinea pigs the cochlea was destroyed on both sides by CO2 laser radiation and fenestration of the lateral semicircular canal was done. In the pigeons the cochlea was removed before fenestration. On account of the results obtained, fenestration of the lateral canal in patients with bilateral total cochlear loss can be considered as an alternative to monopolar cochlear implants.