Ampulla Of Semicircular Canal Research Articles

A mouse model of autoimmune inner ear disease without endolymphatic hydrops

Autoimmune inner ear disease (AIED) is an organ-specific disease characterized by irreversible, prolonged, and progressive hearing and equilibrium dysfunctions. The primary symptoms of AIED include asymmetric sensorineural hearing loss accompanied by vertigo, aural fullness, and tinnitus. AIED is divided into primary and secondary types. Research has been conducted using animal models of rheumatoid arthritis (RA), a cause of secondary AIED. However, current models are insufficient to accurately analyze vestibular function, and the mechanism underlying the onset of AIED has not yet been fully elucidated. Elucidation of the mechanism of AIED onset is urgently needed to develop effective treatments. In the present study, we analyzed the pathogenesis of vertigo in autoimmune diseases using a mouse model of type II collagen-induced RA. Auditory brain stem response analysis demonstrated that the RA mouse models exhibited hearing loss, which is the primary symptom of AIED. In addition, our vestibulo-oculomotor reflex analysis, which is an excellent vestibular function test, accurately captured vertigo symptoms in the RA mouse models. Moreover, our results revealed that the cause of hearing loss and vestibular dysfunction was not endolymphatic hydrops, but rather structural destruction of the organ of Corti and the lateral semicircular canal ampulla due to an autoimmune reaction against type II collagen. Overall, we were able to establish a mouse model of AIED without endolymphatic hydrops. Our findings will help elucidate the mechanisms of hearing loss and vertigo associated with AIED and facilitate the development of new therapeutic methods.

Optimizing vestibular implant electrode positioning using fluoroscopy and intraoperative CT imaging

Purpose Vestibular implant electrode positioning close to the afferent nerve fibers is considered to be key for effective and selective electrical stimulation. However, accurate positioning of vestibular implant electrodes inside the semicircular canal ampullae is challenging due to the inability to visualize the target during the surgical procedure. This study investigates the accuracy of a new surgical protocol with real-time fluoroscopy and intraoperative CT imaging, which facilitates electrode positioning during vestibular implant surgery.Methods Single-center case-controlled cohort study with a historic control group at a tertiary referral center. Patients were implanted with a vestibulocochlear implant, using a combination of intraoperative fluoroscopy and cone beam CT imaging. The control group consisted of five patients who were previously implanted with the former implant prototype, without the use of intraoperative imaging. Electrode positioning was analyzed postoperatively with a high-resolution CT scan using 3D slicer software. The result was defined as accurate if the electrode position was within 1.5 mm of the center of the ampulla.ResultsWith the new imaging protocol, all electrodes could be positioned within a 1.5 mm range of the center of the ampulla. The accuracy was significantly higher in the study group with intraoperative imaging (21/21 electrodes) compared to the control group without intraoperative imaging (10/15 electrodes), (p = 0.008).Conclusion The combined use of intraoperative fluoroscopy and CT imaging during vestibular implantation can improve the accuracy of electrode positioning. This might lead to better vestibular implant performance.

Three-dimensionally visualized ossification and mineralization process of the otic capsule in a postnatal developmental mouse.

We aimed to elucidate the ossification process of the otic capsule in postnatal C57BL/6 mice and depict the three-dimensional (3D) process of otoconial mineralization in vivo. The otic capsules of C57BL/6 mice were stained with alizarin red and imaged/compared using micro-computed tomography on postnatal day (P) between P0 and P8, P10, P15, and P30 and 3-4 months old (P3-4Mo). We reconstructed 3D images of the otic capsule and otoconia and measured the bone mineral density using x-ray absorptiometry on each age. The 3D reconstructed otic capsule images revealed two ossification centers of the otic capsule at P0. One was observed around the ampulla of the superior semicircular canal and utricle, and the other was observed around the ampulla of the posterior semicircular canal. The cross-sectional views demonstrated that modiolar ossification developed from the base to the apex from P4 to P8. The inter-scalar septum ossified bidirectionally from the modiolus and bony otic capsule from P8 to P15. The mineralized otoconia were first detected in the utricle at P3 and saccular otoconia at P6. The density of the utricle and saccular otoconia showed different growth trends. To the best of our knowledge, this is the first study to demonstrate the 3D appearance of the otic capsule and otoconia in different developmental stages of mice. We also revealed that modiolar and inter-scalar septal calcification is the final event in the cochlea and that it can be susceptible to pathological conditions (cochlear congenital malformations and hereditary vestibular diseases). The unique features of the ossification process and duration may explain these pathological conditions observed in humans. 3.

Symptom: Vertigo After Labyrinthectomy

Symptom: Vertigo After Labyrinthectomy

The clinical significance of direction-fixed mono-positional apogeotropic horizontal nystagmus.

SUMMARYObjectiveA mono-positional persistent, direction-fixed apogeotropic nystagmus (MPosApoNy) is very challenging for the neuro-otologist. MPosApoNy can be found in patients suffering from a partially compensated acute unilateral vestibulopathy; with a normal caloric test, one can speculate the presence of “trapped” otolithic debris located close to the ampulla of the horizontal semicircular canal.MethodsAmong 957 patients suffering from vertigo and dizziness, we selected 53 cases of MPosApoNy.ResultsIn 28 patients, caloric test showed a canal paresis on the same side of the MPosApoNy. In the remaining 25 cases, MPosApoNy was the only clinical finding. We hypothesised the presence of horizonal canal lithiasis and patients were treated with a Gufoni manoeuvre, followed by a forced prolonged position.ConclusionsPerforming bedside examination in a patient suffering from vertigo, the presence of MPosApoNy may be due to: a) facilitation of a subclinical nystagmus due to the mechanism of apogeotropic reinforcement; b) horizontal canal lithiasis with ‘trapped’ otoliths close to the ampulla. The disappearance of MPosApoNy following a repositioning manoeuvre or conversion in a typical form of canalolithiasis may represent the best method to confirm this hypothesis.

Fitting the determined impedance in the guinea pig inner ear to Randles circuit using square error minimization in the range of 100 Hz to 50 kHz

Objective. Several lumped and distributed parameter models of the inner ear have been proposed to improve vestibular implant stimulation. The models should account for all significant physical phenomena that influence the current propagation, such as the electrical double layer (EDL) and medium polarization. The electrical properties of the medium are reflected in the electrical impedance; therefore, the study aimed to measure the impedance in the guinea pig inner ear and construct its equivalent circuit. Approach. The electrical impedance was measured from 100 Hz to 50 kHz between a pair of platinum electrodes immersed in 0.9% NaCl saline solution using sinusoidal voltage signals. The Randles circuit was fitted to the measured impedance in the saline solution in order to estimate the EDL parameters () of the electrode interface in saline. Then, the electrical impedance was measured between all combinations of the electrodes located in the semicircular canal ampullae and the vestibular nerve in the guinea pig in vitro. The extended Randles circuit considering the medium polarization () together with EDL parameters () obtained from the saline solution was fitted to the measured impedance of the guinea pig inner ear. The Warburg element was assumed negligible and was not considered in the guinea pig model. Main results. For the set-up used, the obtained EDL parameters were: The average values of intra-, extracellular resistances, and membrane capacitance were respectively. Significance. The obtained values of the model parameters can serve as a good estimation of the EDL for modelling work. The EDL, together with medium polarization, plays a significant role in the electrical impedance of the guinea pig inner ear, therefore, they should be considered in electrical conductivity models to increase the credibility of the simulations.

Magnetic Resonance 3D Measurement of the Endolymphatic Space in 100 Control Human Subjects

Three Dimensional-magnetic resonance imaging of the inner ear endolymphatic space was performed in 100 control human subjects. Ear endolymphatic space was visualized using contrast-enhanced magnetic resonance imaging with intravenous gadolinium injection. The images were acquired on a 3-tesla magnetic resonance scanner using a 32-channel array head coil for all patients. The ear endolymphatic space and total fluid space volumes of the inner ear, cochlea, and vestibule were measured, and the ear endolymphatic space in the cochlea and vestibule was classified into four categories. The cochlea categories were: c-1, no ear endolymphatic space; c-2, ear endolymphatic space visualized only until the upper turn; c-3, ear endolymphatic space visualized from the upper turn to the second turn; and c-4, ear endolymphatic space visualized up to the basal turn. The vestibule categories were: v-1, no ear endolymphatic space; v-2, ear endolymphatic space visualized only in the utriculus; v-3, ear endolymphatic space visualized in the utriculus and sacculus separately; and v-4, ear endolymphatic space visualized in the utriculus and sacculus together. The mean TFS volumes of the inner ear, cochlea, and vestibule were 282.1 ± 33.2 µL, 112.9 ± 15.9 µL, and 69.1 ± 9.9 µL, respectively, and that of the ampulla of the posterior semicircular canal was 7.8 ± 1.7 µL. The mean ear endolymphatic space/total fluid space volume ratio in the cochlea was 10.3 ± 6.7% and that in the vestibule was 17.3 ± 12.2%. This ratio in the cochlea was between the results of categories c-1 and c-2 and that in the vestibule between those of categories v-2 and v-3. Two subjects had ear endolymphatic space extending from the vestibule to the posterior non-ampullated crus of the lateral semicircular canal. These findings can be applied as standard reference values for further research.

Dilatation of the Endolymphatic Space in the Ampulla of the Posterior Semicircular Canal: A New Clinical Finding Detected on Magnetic Resonance Imaging.

To investigate the clinical features of ears with dilatation of the endolymphatic space in the ampulla of the posterior semicircular canal on magnetic resonance imaging. Retrospective study. A university hospital. This study included 1,842 ears from 934 patients who underwent 3-T magnetic resonance imaging with gadolinium to investigate the presence of endolymphatic hydrops. Age, sex distribution, hearing thresholds on pure-tone audiometry, and vestibular symptoms were compared between cases of unilateral and bilateral dilatation of the endolymphatic space in the ampulla of the posterior semicircular canal. Forty-eight ears (17 men and 14 women; mean age 49.9 yrs) showed dilatation of the endolymphatic space in the ampulla of the posterior semicircular canal. Age and the rate of chronic sensorineural hearing loss were significantly higher in the unilateral group (14 ears) than in the bilateral group (34 ears). The average hearing thresholds and rates of vestibular symptoms reported did not differ between unilateral and bilateral cases, but some patients showed positional nystagmus. Dilatation of the endolymphatic space in the ampulla was observed selectively in the posterior semicircular canal, though its pathogenesis was not clear. Such dilatation is not usually accompanied by vestibular endolymphatic hydrops, and it may be a cause of vertigo and dizziness.

Bone Conduction Stimulation Applied Directly to the Otic Capsule: Intraoperative Assessment in Humans.

Aim was to investigate the innovative method of direct acoustic bone conduction (BC) stimulation applied directly to the otic capsule and measured intraoperatively by promontory displacement in living humans. The objective was to find the best stimulation site that provides the greatest transmission of vibratory energy in a living human and compare it with the results previously obtained in cadavers. The measurements were performed in 4 adult patients referred to our department for vestibular schwannoma removal via translabyrinthine approach. The measurements were performed in the operated site. The cadaver data were obtained in our previous study and here they are reanalyzed for comparison. Promontory displacement was measured using a commercial scanning laser Doppler vibrometer. The laser Doppler vibrometer points located on the promontory were used to analyze the promontory displacement amplitude. Cochlear stimulation was induced with BC stimulation through an implant positioned at three sites. The first site was on the skull surface at the squamous part of the temporal bone (BC No. 1), the second at the bone forming the ampulla of the lateral semicircular canal (BC No. 2), and the third between the superior and lateral semicircular canals (BC No. 3). BC No. 2 and BC No. 3 were located directly on the otic capsule. Four frequencies in total were tested (500, 1000, 2000, and 4000 Hz), one at a time. In patients, the detailed analysis of promontory displacement amplitudes revealed the BC No. 1 magnitude to be the smallest and significantly different from BC No. 2 and No. 3 at all measured frequencies. Transmission of vibratory energy at BC No. 2 and BC No. 3 was the most effective and similar with a small and insignificant difference at 500, 1000, and 4000 Hz, and a significant difference at 2000 Hz. The results observed in cadavers were similar to those in living humans. However, a few differences were observed when comparing patients and cadavers. Small and insignificant differences were found for BC No. 1. Almost the same results were obtained for BC No. 2 and BC No. 3 in cadavers as in living humans, with only BC No. 3 measurements results at 500 Hz at the limit of statistical significance, with no other significant differences observed. The results of this study indicate that the promontory vibration amplitude increases when the BC stimulation location approaches the cochlea. BC No. 1 stimulation located on the squama caused overall smaller displacement than both BC No. 2 and No. 3 screwed to the ampulla of the lateral semicircular canal and to the midpoint between the semicircular canals, respectively. In our opinion, the results of BC stimulation applied directly to the otic capsule present a potential new stimulation site that could be introduced in the field of BC hearing rehabilitation.

Detailed Analysis of the Space between the Semicircular Canals for the Purpose of Direct Bone Conduction Stimulation of the Inner Ear

Introduction: Until recently, all locations for bone conduction (BC) stimulator described in the literature were situated outside of the real otic capsule. In recent studies 2 new sites for the BC titanium implant were proposed to directly stimulate the cochlea from the closest possible distance, which was the bone forming the ampulla of the lateral semicircular canal (SC) and the bone between the superior and lateral SC. They proved to be the most efficient in terms of transmission of vibratory energy into the inner ear and could be introduced in the field of BC hearing rehabilitation. To the best of our knowledge the anatomy of the space between SC has not been studied so far. However, screwing the BC implant into the proposed new locations directly at the otic capsule and drilling the bone near the SC cast doubt on the safety of this procedure. In this study we aimed to present a detailed analysis of the anatomy of the otic capsule, especially as regards the space between the SC that seems to be safer. Methods: Sixteen fresh frozen cadaveric temporal bones scanned with micro-computed tomography and analyzed using the multiplanar reconstruction option. The anatomy of the space between the SC was analyzed in detail for the purpose of direct BC stimulation of the inner ear. Results: At least 3 mm of bony tissue is available above the bony space between the crura of the superior SC above the lateral SC, where the new location for the titanium BC implant is proposed. As regards the limitations of the length of screw the BC implant to be screwed, the smallest distance is at least 4 mm of bone thickness. Conclusions: The bone between the crura of the superior SC is the best placement to screw the BC implant directly to the otic capsule. The implant direction should be parallel to the plane of the lateral SC. This location, the direction, and the limitation of the screw length of the BC implant to a maximum of 7 mm present the lowest potential risk of damage to the inner ear.

Effectiveness of Bone Conduction Stimulation Applied Directly to the Otic Capsule Measured at Promontory: Assessment in Cadavers

Introduction: The aims of this study included: (a) to develop a method of direct acoustic bone conduction (BC) stimulation applied directly to the otic capsule, (b) to investigate the effect of different stimulation sites on the promontory displacement amplitude, and (c) to find the best stimulation site (among 2 located directly on the otic capsule and 1 standard site approved for clinical use) that provides the greatest transmission of vibratory energy. Methods: Measurements were performed on 9 cadaveric whole human heads. A commercial scanning laser Doppler vibrometer was used. The promontory displacement was recorded in response to BC stimulation delivered by an implant at 3 sites: BC1 on the squamous part of the temporal bone, BC2 on the ampulla of the lateral semicircular canal, and BC3 between the semicircular canals. The displacement of the promontory was analyzed in detail. Results: The results show that BC1 caused an overall smaller promontory displacement than both sites BC2 and 3. BC3 stimulation is more efficient than that at BC2. Conclusions: BC is an effective method of acoustic stimulus delivery into the inner ear, with the effectiveness increasing when approaching closer to the cochlea. Placing the implant directly on the labyrinth and thus applying vibrations directly to the otic capsule is possible and very effective as proved in this study. The results are encouraging and represent the potential of new stimulation sites that could be introduced in the field of BC hearing rehabilitation as the possible future locations for implantable BC hearing devices.

Transmastoid labyrinthectomy

Transmastoid labyrinthectomy is a highly effective procedure for controlling vertigo attacks in patients with refractory Meniere's disease and ipsilateral nonserviceable hearing. Performed under general anesthesia, the procedure involves a cortical mastoidectomy with subsequent opening and removal of the vestibular neuroepithelium of the 3 semicircular canal ampullae, as well as opening and ablation of the utricle and saccule of the vestibule. Transmastoid labyrinthectomy typically requires several days of hospitalization to permit acute central compensation, and a period of vestibular physical therapy is typically necessary for patients to return to normal activity levels by 2 months. Transmastoid labyrinthectomy is a safe procedure with low rates of complications. Hearing rehabilitation can be performed via cochlear implantation, when indicated. Overall, transmastoid labyrinthectomy provides high rates of relief from vertigo and remains the gold standard for surgical treatment of Meniere's disease. Transmastoid labyrinthectomy is a highly effective procedure for controlling vertigo attacks in patients with refractory Meniere's disease and ipsilateral nonserviceable hearing. Performed under general anesthesia, the procedure involves a cortical mastoidectomy with subsequent opening and removal of the vestibular neuroepithelium of the 3 semicircular canal ampullae, as well as opening and ablation of the utricle and saccule of the vestibule. Transmastoid labyrinthectomy typically requires several days of hospitalization to permit acute central compensation, and a period of vestibular physical therapy is typically necessary for patients to return to normal activity levels by 2 months. Transmastoid labyrinthectomy is a safe procedure with low rates of complications. Hearing rehabilitation can be performed via cochlear implantation, when indicated. Overall, transmastoid labyrinthectomy provides high rates of relief from vertigo and remains the gold standard for surgical treatment of Meniere's disease.

Experimental Investigation of Electric Signal Transmission Through Vestibular Organ Tissues

Experimental data on electric signal transmission through tissues of the vestibular organ of a guinea pig are presented. Stimulating electrodes are implanted in three ampullae of semicircular canals to which an alternating voltage of 170 mV is applied. The receiving electrode is implanted in the termination of vestibular nerves. The impedance of the vestibular organ tissues has been measured depending on the stimulating signal frequency. It is shown that the amplitude phase characteristics of the resulting signal at the termination of the vestibular nerves depend on the position of the stimulating electrodes and on the stimulating signal frequency. The presence of conductive disturbance currents and their influence on the resulting signal waveforms are established. A method for eliminating the influence of the conductive disturbance currents by means of application of additional electric pulses with a certain phase shift to the stimulating electrodes is suggested.

Round Window Membrane Motion Induced by Bone Conduction Stimulation at Different Excitation Sites: Methodology of Measurement and Data Analysis in Cadaver Study.

The aim of this study was to investigate the following: (1) the vibration pattern of the round window (RW) membrane in human cadavers during air (AC) and bone conduction (BC) stimulation at different excitation sites; (2) the effect of the stimulation on the fluid volume displacement (VD) at the RW and compare the VD between BC and AC stimulation procedures; (3) the effectiveness of cochlear stimulation by the bone implant at different excitation sites. The RW membrane vibrations were measured by using a commercial scanning laser Doppler vibrometer. The RW vibration amplitude was recorded at 69 measurement points evenly distributed in the measurement field covering the entire surface of the RW membrane and a part of the surrounding bony surface. RW vibration was induced first with AC and then with BC stimulation through an implant positioned at two sites. The first site was on the skull surface at the squamous part of the temporal bone (implant no. 1), a place typical for bone-anchored hearing aids. The second site was close to the cochlea at the bone forming the ampulla of the lateral semicircular canal (implant no. 2). The displacement amplitude (dP) of the point P on the promontory was determined and used to calculate the relative displacement (drRW) of points on the RW membrane, drRW = dRW - dP. VD parameter was used to analyze the effectiveness of cochlear stimulation by the bone implant screwed at different excitation sites. RW membrane displacement amplitude of the central part of the RW was similar for AC and BC implant no. 1 stimulation, and for BC implant no. 2 much larger for frequency range >1 kHz. BC implant no. 2 causes a larger displacement amplitude of peripheral parts of the RW and the promontory than AC and BC implant no. 1, and BC implant no. 1 causes larger than AC stimulation. The effect of BC stimulation exceeds that of AC with identical intensity, and that the closer BC stimulation to the otic capsule, the more effective this stimulation is. A significant decrease in the value of VD at the RW is observed for frequencies >2 kHz for both AC and BC stimulation with BC at both locations of the titanium implant placement. For frequencies >1 kHz, BC implant no. 2 leads to a significantly larger VD at the RW compared to BC implant no. 1. Thus, the closer to the otic capsule the BC stimulation is located, the more effective it is. Experimental conditions allow for an effective acoustic stimulation of the inner ear by an implant screwed to the osseous otic capsule. The mechanical effect of BC stimulation with a titanium implant placed in the bone of the ampulla of the lateral semicircular canal significantly exceeds the effect of an identical stimulation with an implant placed in the temporal squama at a conventional site for an implant anchored in the bone. The developed research method requires the implementation on a larger number of temporal bones in order to obtain data concerning interindividual variability of the observed mechanical phenomena.

Morphology of the Petrosal in Late Miocene Baleen Whales of Northwestern Ciscaucasia

Morphological features of the membranous labyrinth in extinct and extant baleen whales and their ancestors are discussed. The tomography of the membranous labyrinth of two species of Late Miocene baleen whales from Adygea has shown that Kurdalagonus, Zygiocetus, and Cetotherium have a comparable number of cochlear turns with representatives of herpetocetins, i.e., about three turns. Representatives of the genus Kurdalagonus differ from Zygiocetus in the structure of semicircular canals, the longer crus commune (much shorter in Zygiocetus), and in the size of the ampulla of the lateral semicircular canal. In addition, representatives of the genus Kurdalagonus differ from Zygiocetus and Cetotherium in the structure of the round window, which is much wider and has a more rounded shape.

Postoperative MR imaging features after superior semicircular canal plugging in Minor syndrome.

To report the postoperative magnetic resonance imaging (MRI) features after superior semicircular canal plugging in patients with Minor syndrome. The MRI examinations with 3D T2-weighted SPACE sequence of 12 patients with superior semicircular canal dehiscence syndrome (SCDS) were retrospectively assessed. Two radiologists independently evaluated the presence of a filling defect of the superior semicircular canal above the superior ampulla and the common crus using an oblique plane parallel to the superior semicircular canal (Pöschl's plane). Postoperative MRI showed a filling defect above the ampulla of the superior semicircular canal and the common crus in 8/12 patients (67%). Three patients (3/12; 25%) had a filling defect involving also the superior ampulla that caused postoperative labyrinthitis with labyrinthine enhancement on MRI in 2 patients. One patient (1/12; 8%) had incomplete plugging of superior semicircular canal with abnormal functional tests and remaining symptoms. Postoperative MRI shows a normal plugging aspect of the superior semicircular canal in 67% of patients. MRI can reveal complications that may have therapeutic implications.

Incomplete and delayed Sox2 deletion defines residual ear neurosensory development and maintenance.

The role of Sox2 in neurosensory development is not yet fully understood. Using mice with conditional Islet1-cre mediated deletion of Sox2, we explored the function of Sox2 in neurosensory development in a model with limited cell type diversification, the inner ear. In Sox2 conditional mutants, neurons initially appear to form normally, whereas late- differentiating neurons of the cochlear apex never form. Variable numbers of hair cells differentiate in the utricle, saccule, and cochlear base but sensory epithelium formation is completely absent in the apex and all three cristae of the semicircular canal ampullae. Hair cells differentiate only in sensory epithelia known or proposed to have a lineage relationship of neurons and hair cells. All initially formed neurons lacking hair cell targets die by apoptosis days after they project toward non-existing epithelia. Therefore, late neuronal development depends directly on Sox2 for differentiation and on the survival of hair cells, possibly derived from common neurosensory precursors.

The image variations in mastoid segment of facial nerve and sinus tympani in congenital aural atresia by HRCT and 3D VR CT

ObjectiveTo find the variations of middle ear structures including the spatial pattern of mastoid segment of facial nerve and the shapes of the sinus tympani in patients with congenital aural atresia (CAA) by using the high-resolution (HR) CT and 3D volume rendered (VR) CT images. MethodsHRCT was performed in 25 patients with congenital aural atresia including six bilateral atresia patients (n=25, 21 males, 4 females, mean age 13.8 years, range 6–19). Along the long axis of the posterior semicircular canal ampulla, the oblique axial multiplanar reconstruction (MPR) was set to view the depiction of the round window and the mastoid segment of facial nerve. Volumetric rending technique was used to demonstrate the morphologic features. HRCT and 3D VR findings in atresia ears were compared with those in 19 normal ears of the unilateral ears of atresia patients. ResultsOn the basic plane, the horizontal line distances between the mastoid segment of the facial nerve and the round window (h-RF) in atresia ears significantly decreased compared to the control ears (P<0.05). There was a significant negative correlation between the sinus tympani area (a-ST) and the distance between the horizontal lines of FN and RW midpoint (h-RF) (P<0.05). The mean area of sinus tympani in atresia group is larger (P<0.05). The shapes of the sinus tympani were classified into three categories: the cup-shaped, the pear-shaped and the boot-shaped. Area measurement indicated that the boot-shaped sinus tympani was a special variation with a large area, which only appears in CAA group. There were a significant difference between the area of the boot-shaped group and the other two groups (P<0.05). The morphologic differences of ST and other middle ear structures can also be observed visually in 3D VR CT images. ConclusionHRCT and 3D VR CT could help a better understanding of different kinds of variations in mastoid segment of facial nerve and sinus tympani in CAA ears. And it may further help surgeons to make the correct decision for hearing rehabilitation.

Assessment of the Effects of Superior Canal Dehiscence Location and Size on Intracochlear Sound Pressures

Superior canal dehiscence (SCD) is a defect in the bony covering of the superior semicircular canal. Patients with SCD present with a wide range of symptoms, including hearing loss, yet it is unknown whether hearing is affected by parameters such as the location of the SCD. Our previous human cadaveric temporal bone study, utilizing intracochlear pressure measurements, generally showed that an increase in dehiscence size caused a low-frequency monotonic decrease in the cochlear drive across the partition, consistent with increased hearing loss. This previous study was limited to SCD sizes including and smaller than 2 mm long and 0.7 mm wide. However, the effects of larger SCDs (>2 mm long) were not studied, although larger SCDs are seen in many patients. Therefore, to answer the effect of parameters that have not been studied, this present study assessed the effect of SCD location and the effect of large-sized SCDs (>2 mm long) on intracochlear pressures. We used simultaneous measurements of sound pressures in the scala vestibuli and scala tympani at the base of the cochlea to determine the sound pressure difference across the cochlear partition - a measure of the cochlear drive in a temporal bone preparation - allowing for assessment of hearing loss. We measured the cochlear drive before and after SCDs were made at different locations (e.g. closer to the ampulla of the superior semicircular canal or closer to the common crus) and for different dehiscence sizes (including larger than 2 mm long and 0.7 mm wide). Our measurements suggest the following: (1) different SCD locations result in similar cochlear drive and (2) larger SCDs produce larger decreases in cochlear drive at low frequencies. However, the effect of SCD size seems to saturate as the size increases above 2-3 mm long and 0.7 mm wide. Although the monotonic effect was generally consistent across ears, the quantitative amount of change in cochlear drive due to dehiscence size varied across ears. Additionally, the size of the dehiscence above which the effect on hearing saturated varied across ears. These findings show that the location of the SCD does not generally influence the amount of hearing loss and that SCD size can help explain some of the variability of hearing loss in patients. i 2014 S. Karger AG, Basel

Prosthetic Implantation of the Human Vestibular System

A functional vestibular prosthesis can be implanted in human such that electrical stimulation of each semicircular canal produces canal-specific eye movements while preserving vestibular and auditory function. A number of vestibular disorders could be treated with prosthetic stimulation of the vestibular end organs. We have previously demonstrated in rhesus monkeys that a vestibular neurostimulator, based on the Nucleus Freedom cochlear implant, can produce canal-specific electrically evoked eye movements while preserving auditory and vestibular function. An investigational device exemption has been obtained from the FDA to study the feasibility of treating uncontrolled Ménière's disease with the device. The UW/Nucleus vestibular implant was implanted in the perilymphatic space adjacent to the three semicircular canal ampullae of a human subject with uncontrolled Ménière's disease. Preoperative and postoperative vestibular and auditory function was assessed. Electrically evoked eye movements were measured at 2 time points postoperatively. Implantation of all semicircular canals was technically feasible. Horizontal canal and auditory function were largely, but not totally, lost. Electrode stimulation in 2 of 3 canals resulted in canal-appropriate eye movements. Over time, stimulation thresholds increased. Prosthetic implantation of the semicircular canals in humans is technically feasible. Electrical stimulation resulted in canal-specific eye movements, although thresholds increased over time. Preservation of native auditory and vestibular function, previously observed in animals, was not demonstrated in a single subject with advanced Ménière's disease.