Cochlear Basal Turn Research Articles

The characteristics of three-dimensional real inversion recovery MRI sequence in delayed Meniere’s disease

Background Delayed Meniere’s disease (DMD) is characterized by episodic vertigo occurring after a significant delay following longstanding sensorineural hearing loss. However, its pathogenesis and characteristics remain unclear. Objectives To explore the characteristics of endolymphatic hydrops (EH) in DMD and investigate its pathology using MRI. Materials and methods 17 patients diagnosed with DMD were included in this study. A 3D-real-IR MRI sequence was utilized to visualize and grade EH. The signal intensity of the basal cochlear turn was compared to that of the cerebellar white matter to calculate the CC ratio. A paired t-test was employed to assess the differences in the CC ratio between the affected and unaffected sides in patients with unilateral DMD. Results EH was present in 100% of cases on the affected side. Unilateral EH was seen in 82.4%, and bilateral EH in 23.5%. Vestibular EH without cochlear involvement occurred in 29.4%, and cochlear EH without vestibular involvement in 5.9%. The CC ratio was significantly higher on the affected side (p < 0.01). Conclusions and significance A significant grade of vestibular EH was observed in DMD, with bilateral EH being relatively common. The increased contrast on the affected side may reflect the underlying pathology in DMD patients.

Cochlear Implantation: Predicting Round Window Niche Visibility Using One Measurement in High-Resolution Temporal Bone Computed Tomography.

To evaluate the accuracy of a single measurement in temporal bone computed tomography in predicting the round window niche (RWN) visibility during cochlear implantation. A prospective study was conducted on 148 patients (165 ears) who had a cochlear implant (CI) from January 2010 to December 2018 at a tertiary CI center. The measurement was done for the angle of the basal turn of the cochlea (ABTC), which we defined as the angle formed by the cochlear basal turn and the cranium mid-sagittal plane, by 2 readers blindly from the axial images of computed tomography. The RWN visibility was classified according to the observation during surgery (through posterior tympanotomy) into full visibility, partial visibility, and invisibility. The measured angle was then correlated to the intra-operative visibility of the RWN. The average ABTC was 57.48° ± 4.05° (range: 45.0-68.0), and the RWN was found to be fully visible in 85%, partially visible in 11%, and invisible in 4% of the studied ears. The receiver operating characteristic analysis revealed a significant discriminating ability in predicting RWN visibility (P <.001) at a threshold ABTC angle of 58.5°. The mean ABTC was 56.71° ± 3.74°, 61.00°, and 63.86° ± 2.67° for fully visible, partially visible, and invisible RWN, respectively. A statistical significant difference was found (P = .0002) when comparing the ABTC in patients with partially visible/invisible RWN (61.80° ± 2.87°) with the fully visible RWN (56.71° ± 3.74°). Round window niche visibility could be predicted by measuring the ABTC in relation to the cranium's mid-sagittal plane in CT preoperatively. An ABTC bigger than 58.5° could be an indication of poorly visible RWN.

Age-related alterations in efferent medial olivocochlear-outer hair cell and primary auditory ribbon synapses in CBA/J mice.

Hearing decline stands as the most prevalent single sensory deficit associated with the aging process. Giving compelling evidence suggesting a protective effect associated with the efferent auditory system, the goal of our study was to characterize the age-related changes in the number of efferent medial olivocochlear (MOC) synapses regulating outer hair cell (OHC) activity compared with the number of afferent inner hair cell ribbon synapses in CBA/J mice over their lifespan. Organs of Corti of 3-month-old CBA/J mice were compared with mice aged between 10 and 20 months, grouped at 2-month intervals. For each animal, one ear was used to characterize the synapses between the efferent MOC fibers and the outer hair cells (OHCs), while the contralateral ear was used to analyze the ribbon synapses between inner hair cells (IHCs) and type I afferent nerve fibers of spiral ganglion neurons (SGNs). Each cochlea was separated in apical, middle, and basal turns, respectively. The first significant age-related decline in afferent IHC-SGN ribbon synapses was observed in the basal cochlear turn at 14 months, the middle turn at 16 months, and the apical turn at 18 months of age. In contrast, efferent MOC-OHC synapses in CBA/J mice exhibited a less pronounced loss due to aging which only became significant in the basal and middle turns of the cochlea by 20 months of age. This study illustrates an age-related reduction on efferent MOC innervation of OHCs in CBA/J mice starting at 20 months of age. Our findings indicate that the morphological decline of efferent MOC-OHC synapses due to aging occurs notably later than the decline observed in afferent IHC-SGN ribbon synapses.

Insertion trauma of a novel inner ear catheter for intracochlear drug delivery.

Even with recent research advances, effective delivery of a compound to its target cells inside the inner ear remains a challenging endeavor due to anatomical and physiological barriers. Direct intracochlear drug administration with an inner ear catheter (IEC) aims to overcome this obstacle and strives to provide a safe and efficient way for inner ear pharmacotherapy. The goal of this study was to histologically and audiologically evaluate the traumatic properties of a novel IEC for intracochlear drug delivery in a large animal model. Seven inner ears of piglets that had undergone intracochlear fluorescein isothiocyanate dextran application via an IEC (n = 4) or round window membrane (RWM) puncture with a needle (n = 3) followed by sequential apical perilymph sampling were histologically analyzed. Additionally, obtained objective auditory compound action potential and cochlear microphonic measurements were compared. Cochlear cryosections were stained using hematoxylin and eosin, and preservation of inner ear structures was investigated. Moreover, one cochlea was methylmethacrylate-embedded and analyzed with the IEC in situ. Histological evaluation revealed an atraumatic insertion and subsequent compound application in a majority of IEC-inserted inner ears. Click cochlear compound action potential (CAP) shifts in the IEC groups reached a maximum of 5 dB (1.25 ± 2.5 dB) post administration and prior to perilymph sampling. In comparison, application by RWM puncture generated a maximum click CAP hearing threshold shift of 50 dB (23.3 ± 23.1 dB) coinciding with coagulated blood in the basal cochlear turn in one specimen of the latter group. Furthermore, in situ histology showed an atraumatic insertion of the IEC demonstrating preserved intracochlear structures. The IEC appears to be a promising and efficient way for inner ear drug delivery. The similarities between the porcine and human inner ear enhance the clinical translation of our findings and increase confidence regarding the safe applicability of the IEC in human subjects.

Evaluation of cochlear and auditory brainstem functions in COVID-19 patients; a case control study

BackgroundMany viral infections can cause hearing loss due to affection of cochlear hair cells or neurogenic pathway. Although, the damage secondary to viral infections is mainly cochlear affection; auditory brainstem can be affected as well. It was predicted that SARS-COV-2 infection can similarly affect the auditory system. This study aimed to detect affection in auditory system and if present investigate the possible site of lesion (up to the level of the brain stem) in relation to COVID-19 infection.MethodsThis is a case control study, where the study group constituted of thirty adults, diagnosed with COVID-19 at least 2 weeks prior to testing and up to 6 months, without previous auditory complaints pre-COVID-19 or other risk factors that could affect the auditory pathway. Fifteen adult participants that were age and gender matched to the study group with no previous history of covid-19 infection constituted the control group. Audiological evaluations done to all participants were pure-tone and speech audiometry, tympanometry, transient-evoked otoacoustic emission with and without contralateral suppression and auditory brainstem response measurements.ResultsThe study group showed significantly worse pure tone thresholds at high frequencies 4 and 8 kHz (p < 0.01), significantly worse transient-evoked otoacoustic emission signal to noise ratio at 2800 Hz and 4000 Hz (p < 0.05) and significantly lower total suppression index (p<0.05). On the other hand, there was no significant difference between both groups in auditory brainstem response wave latencies (p > 0.05).ConclusionCOVID-19 had subtle effect on cochlear basal turn, and it is shown that the auditory efferent system may also be affected, while the auditory nerve and afferent brainstem pathways seems to be spared. Moreover, the absence of the symptoms of auditory dysfunction postcovid-19 does not guarantee normal auditory functions.

The impact of the size and angle of the cochlear basal turn on translocation of a pre-curved mid-scala cochlear implant electrode

Scalar translocation is a severe form of intra-cochlear trauma during cochlear implant (CI) electrode insertion. This study explored the hypothesis that the dimensions of the cochlear basal turn and orientation of its inferior segment relative to surgically relevant anatomical structures influence the scalar translocation rates of a pre-curved CI electrode. In a cohort of 40 patients implanted with the Advanced Bionics Mid-Scala electrode array, the scalar translocation group (40%) had a significantly smaller mean distance A of the cochlear basal turn (p < 0.001) and wider horizontal angle between the inferior segment of the cochlear basal turn and the mastoid facial nerve (p = 0.040). A logistic regression model incorporating distance A (p = 0.003) and horizontal facial nerve angle (p = 0.017) explained 44.0–59.9% of the variance in scalar translocation and correctly classified 82.5% of cases. Every 1mm decrease in distance A was associated with a 99.2% increase in odds of translocation [95% confidence interval 80.3%, 100%], whilst every 1-degree increase in the horizontal facial nerve angle was associated with an 18.1% increase in odds of translocation [95% CI 3.0%, 35.5%]. The study findings provide an evidence-based argument for the development of a navigation system for optimal angulation of electrode insertion during CI surgery to reduce intra-cochlear trauma.

The association between high jugular bulb and mastoid pneumatization in adults.

The purpose of this study was to analyze the relationship between the degree of high jugular bulb (HJB) and mastoid pneumatization using high-resolution computed tomography (HRCT). Between April 2019 and June 2022, HRCT of the temporal bone was retrospectively analyzed in 1,025 patients. By excluding the other coexistent pathologies, 113 patients with HJBs were recruited for the study. The degree of the HJBs were defined as follows: Grade I, JB situated between inferior annulus of tympanic membrane and cochlear basal turn (CBT). Grade II, JB situated between CBT and lateral semicircular canal (LSC). Grade III, JB situated above LSC. The volume of mastoid pneumatization was based on HRCT images using a 3D reconstruction. There were 32 male and 81 female subjects (mean age, 41.2 ± 14.0 years; age range, 18-80 years). The male group included 16 Grade I, 28 Grade II and 6 Group III HJB subjects. The female group included 38 Grade I, 62 Grade II and 31 Group III HJB cases. In the different groups of HJB, the mastoid cell volume differences were also not statistically significant (p = 0.165). In the classification, Grade II was most common (90/181, 49.7%). This study found no correlation between mastoid air cell volume and HJB, suggesting that HJB may not affect the mastoid air cell development and disease occurrence. These data must be considered exploratory, requiring more extensive cross-sectional studies.

Blood-labyrinth barrier breakdown in Meniere's disease.

We compared the signal intensity ratio (SIR) of the cochlear basal turn between Meniere's disease and healthy controls to investigate potential damage of the blood-labyrinth barrier in Meniere's disease. Thirty patients diagnosed with unilateral definite Meniere's disease and 24 healthy controls were enrolled. 3D-FLAIR scan was conducted to assess the grades of endolymphatic hydrops in Meniere's patients while measuring the SIR of cochlear basal turns in both groups. The differences of bilateral SIR between Meniere's disease and healthy control were compared, and the correlation between the SIR on affected ear in Meniere's disease and the grades of cochlear and vestibular hydrops were analyzed. SIR of affected ear in Meniere's disease exhibited significant increase compared to that of unaffected ear. No significant difference was observed in SIR between the two ears in the healthy control. Furthermore, the SIR of unaffected side in Meniere's disease was higher than that of both ears in healthy controls. The SIR in affected ear of Meniere's disease exhibited positive correlation with hydrops in both cochlea and vestibula. The permeability of blood-labyrinth barrier is increased in Meniere's disease, in combination with the typical criteria of Meniere's disease it may be a good biological marker. Destruction of blood-labyrinth barrier may be one of the causes of endolymphatic hydrops in Meniere's disease.

Applications of visualizing cochlear basal turn in cochlear implantation.

To report a reliable method in obtaining optimal cochlear basal turn and cross-section (c/s) of internal auditory canal (IAC) supporting Cochlear implantation (CI) procedure. Computer tomography (CT) and magnetic resonance image (MRI) scans of potential CI candidates from 2018 to 2022 from the tertiary center were considered for analysis. Slicer software was used in three-dimensional (3D) segmentation of inner ear and for capturing the cochlear basal turn. A total of 1932 head scans were made available for the analysis and out of which 1866 scans had normal anatomy (NA) inner ear. Incomplete partition (IP) type-I was identified in 19 ears, IP type-II in 27 ears, IP type-III in 6 ears, cochlear hypoplasia (CH) type-I in 6 ears, CH type-II in 1 ear, CH type-III in 3 ears, and CH type-IV is 3 ears, and enlarged vestibular aqueduct syndrome in 1 ear. 3D segmented inner ear helped in successfully obtaining the cochlear basal turn and the c/s of IAC in all anatomical types. Time taken to capture the cochlear basal turn with the help of 3D segmented inner ear was <1 min. Within the NA category, five cases showed scalar ossification, and its extent was identified in the cochlear basal turn. The identification and the extent of ossification in the scala tympani, shape of the basal turn, and the cochlear size measurement in cochlear basal turn has high clinical relevance as this helps in surgical planning and in choosing appropriate electrode length. Level of evidence: Level 2 to the best of our understanding.

Influence of CX3CR1 Deletion on Cochlear Hair Cell Survival and Macrophage Expression in Chronic Suppurative Otitis Media.

Our objective was to determine whether the receptor CX3CR1 is necessary for the recruitment of macrophages to the cochlea in chronic suppurative otitis media (CSOM) and if its deletion can prevent hair cell loss in CSOM. CSOM is a neglected disease that afflicts 330 million people worldwide and is the most common cause of permanent hearing loss among children in the developing world. It is characterized by a chronically discharging infected middle ear. We have previously demonstrated that CSOM causes macrophage associated sensory hearing loss. The receptor CX3CR1 is expressed on macrophages, which have been shown to be increased at the time point of outer hair cell (OHC) loss in CSOM. In this report, we examine the influence of CX3CR1 deletion (CX3CR1-/-) in a validated model of Pseudomonas aeruginosa (PA) CSOM. The data show no difference in OHC loss between the CX3CR1-/- CSOM group and CX3CR1+/+ CSOM group (p = 0.28). We observed partial OHC loss in the cochlear basal turn, no OHC loss in the middle and apical turns in both CX3CR1-/- and CX3CR1+/+ CSOM mice at 14 days after bacterial inoculation. No inner hair cell (IHC) loss was found in all cochlear turns in all groups. We also counted F4/80 labeled macrophages in the spiral ganglion, spiral ligament, stria vascularis and spiral limbus of the basal, middle, and apical turn in cryosections. We did not find a significant difference in the total number of cochlear macrophages between CX3CR1-/- mice and CX3CR1+/+ mice (p = 0.97). The data did not support a role for CX3CR1 macrophage associated HC loss in CSOM.

A prestin-targeting peptide-guided drug delivery system rearranging concentration gradient in the inner ear: An improved strategy against hearing loss

Hearing loss is mainly due to outer hair cell (OHC) damage in three cochlear turns. Local administration via the round window membrane (RWM) has considerable otological clinical potential in bypassing the blood–labyrinth barrier. However, insufficient drug distribution in the apical and middle cochlear turns results in unsatisfactory efficacy. We functionalized poly (lactic-co-glycolic acid) nanoparticles (PLGA NPs) with targeting peptide A665, which specifically bound to prestin, a protein uniquely expressed in OHCs. The modification facilitated the cellular uptake and RWM permeability of NPs. Notably, the guide of A665 towards OHCs enabled more NPs perfusion in the apical and middle cochlear turns without decreasing accumulation in the basal cochlear turn. Subsequently, curcumin (CUR), an appealing anti-ototoxic drug, was encapsulated in NPs. In aminoglycoside-treated guinea pigs with the worst hearing level, CUR/A665-PLGA NPs, with superior performance to CUR/PLGA NPs, almost completely preserved the OHCs in three cochlear turns. The lack of increased low-frequencies hearing thresholds further confirmed that the delivery system with prestin affinity mediated cochlear distribution rearrangement. Good inner ear biocompatibility and little or no embryonic zebrafish toxicity were observed throughout the treatment. Overall, A665-PLGA NPs act as desirable tools with sufficient inner ear delivery for improved efficacy against severe hearing loss.

Anatomical Features of Children With Mondini Dysplasia: Influence on Cochlear Implantation Performance.

To analyze the long-term auditory performance after cochlear implantation (CI) and identify anatomical features of Mondini dysplasia associated with post-CI outcomes. Retrospective study. Tertiary care academic center. We enrolled 49 ears with Mondini dysplasia who underwent CI with more than 7 years of follow-up and age at CI- and sex-matched control group with radiologically normal inner ears. The development of auditory skills after CI was evaluated using word recognition scores (WRSs). The anatomical features were measured based on temporal bone computed tomography and magnetic resonance imaging, involving the width of the bony cochlear nerve canal (BCNC), cochlear basal turn, enlarged vestibular aqueduct, cochlear height, and diameter of the cochlear nerve (CN). CI in ears with Mondini dysplasia showed comparable benefits and improvement of auditory performance to controls during the 7 years of follow-up. In Mondini dysplasia, four (8.2%) ears showed narrow BCNC (<1.4 mm) with poorer WRS (58 ± 17%) than those with normal-sized BCNC, which had WRS (79 ± 10%) comparable to that of the control group (77 ± 14%). In Mondini dysplasia, the maximum ( r = 0.513, p < 0.001) and minimum ( r = 0.328, p = 0.021) CN diameters had positive correlations with post-CI WRS. The maximum CN diameter ( β = 48.347, p < 0.001) and BCNC width ( β = 12.411, p = 0.041) were significant factors that influence the post-CI WRS in multiple regression analysis. Preoperative anatomical evaluation, especially BCNC status and CN integrity, may serve as predictive markers for post-CI performance.

Temporal pitch processing in an animal model of normal and electrical hearing

Cochlear-implant users typically exhibit only limited sensitivity to temporal fine structure (TFS). We are developing a cat animal model in which to explore alternative modes of electrical stimulation that might improve TFS sensitivity. Normal-hearing cats were trained to detect changes in rates of acoustical pulse trains. The pulses were non-resolved harmonic complexes that were band-limited to stimulate the basal cochlear turn, which is the location that is accessible to feline cochlear implants. The rate sensitivity of that temporal pitch was like that of human listeners but shifted upward in rate by ∼1 octave. Scalp recordings from sedated cats showed a frequency following response (FFR) across all tested rates, up to 600 pulses per second. Changes in pulse rates elicited an Acoustic Change Complex (ACC) that showed a rate sensitivity parallel to that of perception. That suggests that the ACC can be used as a surrogate for psychophysical performance. Cats that were implanted chronically with conventional intra-scalar cochlear implants exhibited electrically-evoked FFR and ACC that showed rate sensitivity largely similar to that in normal-hearing cats. These results validate the cat animal model for ongoing studies of transmission of TFS with electrical cochlear stimulation. [Work supported by the NIDCD and the Wellcome Trust]

Uneventful Cochlear Implantation through Round Window Approach in a Child with Persistent Stapedial Artery: A Case Report

Persistent stapedial artery (PSA) is a rare congenital vascular anomaly, with both clinical and surgical implications. When encountered during middle ear surgery, it represents a challenge to the otologic surgeon, since damage to this artery could lead to massive intraoperative bleeding and poor surgical exposure. Here we report the case of a 3-year-old boy with prelingual and profound bilateral sensorineural hearing loss who was referred to our Otolaryngology-Head and Neck Surgery Department for cochlear implant evaluation. Pre-operative computed tomography (CT) examination of the temporal bone confirmed the diagnosis of PSA. Further evaluation showed no contraindication for surgery and the patient underwent cochlear implantation using the classic transmastoid, facial recess approach. During surgery the vessel was coursing far enough from the round window niche, making it possible to achieve safe and complete electrode insertion into the scala tympani of the cochlear basal turn. This case demonstrates that cochlear implantation is safe and feasible in patients with PSA. Thorough analysis of the preoperative temporal bone CT scan might help with surgical planning by identifying this anomaly prior to surgery.

Robotized Cochlear Implantation under Fluoroscopy: A Preliminary Series.

It is known that visual feedback by fluoroscopy can detect electrode array (EA) misrouting within the cochlea while robotized EA-insertion (rob-EAI) permits atraumatic cochlear implantation. We report here our unique experience of both fluoroscopy feedback and rob-EAI in cochlear implant surgery. We retrospectively analyzed a cohort of consecutive patients implanted from November 2021−October 2022 using rob-EAI, with the RobOtol®, to determine the quality of EA-insertion and the additional time required. Twenty-three patients (10 females, 61+/−19 yo) were tentatively implanted using robot assistance, with a rob-EAI speed < 1 mm/s. Only three cases required a successful revised insertion by hand. Under fluoroscopy (n = 11), it was possible to achieve a remote rob-EAI (n = 8), as the surgeon was outside the operative room, behind an anti-radiation screen. No scala translocation occurred. The additional operative time due to robot use was 18+/−7 min with about 4 min more for remote rob-EAI. Basal cochlear turn fibrosis precluded rob-EAI. In conclusion, Rob-EAI can be performed in almost all cases with a low risk of scala translocation, except in the case of partial cochlear obstruction such as fibrosis. Fluoroscopy also permits remote rob-EAI.

A novel three-step process for the identification of inner ear malformation types.

We hypothesize that visualizing inner-ear systematically in both cochlear view (oblique coronal plane) and in mid-modiolar section (axial plane) and following three sequential steps simplifies, identification of inner-ear malformation types. Pre-operative computer-tomography (CT) scans of temporal bones of 112 ears with various inner ear malformation (IEM) types were taken for analysis. Images were analyzed using DICOM viewers, 3D slicer, and OTOPLAN®. The inner-ear was captured in the oblique-coronal plane for the measurement of length and width of cochlear basal turn which is also called as A-, and B-values respectively (Step 1). In the same plane, the angular-turns of lateral-wall (LW) of cochlear basal turn were measured (Step 2). As Step 3, the mid-modiolar section of inner ear was captured in the axial plane by following the A-value and perpendicular to cochlear view. From the mid-modiolar section, the outer-contour of inner ear was captured manually by following contrasting gray area between fluid filled and bony promontory and was compared to known resembling objects to identify IEM types (Step 3). Following reference values have emerged from our analysis: A-, and B-values (Step 1) on average are >8 mm and >5.5 mm respectively, in normal cochleae (NA), enlarged vestibular aqueduct syndrome (EVAS), incomplete partition (IP) type-I and -II, whereas it is <8 mm and <5.5 mm respectively, in IP type-III and cochlear hypoplasia (CH). Angular-turn of LW is consistently observed in cochlear basal turn (Step 2), is 540° in NA and EVAS, 450° in IP-II, and 360° in IP types I & III. In subjects with CH type, angular-turn of LW is either 360° or 450° or 540°. In true mid-modiolar section, outer-contour of inner-ear (Step-3), other than in CH and cystic inner-ear malformations, resembles recognizable shapes of known objects. Absence of EVA is an additional characteristic that confirms diagnosis of CH when the A-, B-values, and angular-turn of LW can be similar to other anatomical types. Drawing a straight line along posterior edge of internal auditory canal (IAC) in axial view can differentiate a true common cavity (CC) from cochlear aplasia-vestibular cavity (VC). Three-step process proposed in this study captures inner-ear in cochlear view as well in mid-modiolar sections visualizing key features of inner-ear in identification of IEM types. Level 1.

17O-labeled water distribution in the human inner ear: Insights into lymphatic dynamics and vestibular function.

We evaluated the inner ear distribution of 17O-labeled saline administered to the human tympanic cavity. Magnetic resonance imaging was performed after intratympanic administration in five healthy volunteers and one patient with cochlear endolymphatic hydrops. In all volunteers, 17O-labeled water permeated the cochlear basal turn and vestibule at 30 min and disappeared gradually within 2-4 h. All participants experienced positional vertigo lasting a few hours to a few days. Visualization of 17O-labeled water distribution in the endolymphatic space of the posterior ampulla showed indistinct separation of endolymph and perilymph in the cochlea and most of the vestibule in all participants. Intralabyrinthine distribution of 17O-labeled water differed from that in previous reports of intratympanically administered gadolinium-based contrast agent. 17O-labeled water in the endolymphatic space may cause heavier endolymph and positional vertigo. These results of this study may add new insights for investigating the distribution and the effects of molecules in the inner ear after the intratympanic administration in living humans.

Typical and Atypical Symptoms of Petrous Apex Cholesterol Granuloma: Association with Radiological Findings.

Objective: Petrous apex cholesterol granuloma (PACG) is a lesion that can give rise to different symptoms, and correlations with etiopathology are ambiguous. The aim of this study is to analyze the association between PACG symptoms and radiological findings at presentation, in order to establish a reproduceable pre-operative radiological evaluation and guide the surgical indication. Methods: PACG patients were collected in two tertiary care hospitals. All cases underwent CT/MRI to evaluate the cyst localization and erosion of surrounding structures. Typical and atypical symptoms were then analyzed and compared to radiologic findings established in accordance with the literature. Results: Twenty-nine patients were recruited; the most common symptoms were headache (69%), diplopia (20.7%) and fainting (24.1%), an atypical clinical manifestation related to jugular tubercle involvement. Significant associations between symptoms and radiologic findings were noted in terms of headache and temporal lobe compression (p = 0.04), fainting and jugular tubercle erosion (p < 0.001), vestibular symptoms and internal auditory canal erosion (p = 0.02), facial paresthesia and Meckel’s cave compression (p = 0.03), diplopia and Dorello canal involvement (p = 0.001), and tinnitus and cochlear basal turn erosion (p < 0.001). All patients were treated via an endoscopic–endonasal approach, in which extension was tailored to each case. At a median follow-up of 46 months, 93.1% of patients experienced resolution of symptoms. Conclusions: This clinico-radiological series demonstrates associations between symptoms and anatomical subsites involved with PACG. Hence, it may guide the surgeon at the time of surgical decision, since it asserts that typical and atypical symptoms are actually related to PACG.

Frequency Mapping of a Precurved Electrode Array in the Internal Auditory Canal for a Pediatric Cochlear Implant Recipient.

Review the effectiveness of an alternative mapping procedure of a precurved electrode array in the internal auditory canal (IAC). A 7-year-old bilateral cochlear implant (CI) recipient of precurved arrays transferred to the study site and demonstrated no speech recognition with the left CI. Imaging revealed bilateral incomplete partition type III malformations. For the left CI, four contacts were observed in the basal cochlear turn and 18 contacts were coiled in the IAC. The family decided against revision surgery. Pitch ranking was completed to map the contacts in the IAC that were perceptually discrete and tonotopically organized. For the left CI, PB-K word recognition improved from no recognition to 32% after 1 month and to 52% after 3 months. In the bilateral CI condition, performance improved from 56 to 72% after 1 month and 80% after 3 months. A precurved array in the IAC creates difficult management decisions. Direct stimulation of the auditory nerve resulted in better monaural and bilateral speech recognition, likely due to better spectral representation of the speech signal. Individualizing the map using imaging and behavioral findings may improve performance for malpositioned arrays when revision surgery is not pursued.

Efficient Delivery of Adeno-Associated Virus into Inner Ear In Vivo Through Trans-Stapes Route in Adult Guinea Pig.

Adeno-associated virus (AAV) are potent vectors to achieve treatment against hearing loss resulting from genetic defects. However, the effects of delivery routes and the corresponding transduction efficiencies for clinical applications remain elusive. In this study, we screened AAV vectors of three serotypes (AAV 8 and 9 and Anc80L65) into the inner ears of adult normal guinea pigs through trans-stapes (oval window) and trans-round window delivery routes in vivo. Trans-stapes route is akin to stape surgeries in humans. Then, auditory brainstem response (ABR) measurements were conducted to evaluate postoperative hearing, and inner ear tissues were harvested for transduction efficiency analysis. Results showed that AAV8 targeted partial inner hair cells (IHCs) in cochlear basal turn; AAV9 targeted IHCs in cochlear basal and second turn, also a part of vestibular hair cells (VHCs). In contrast, Anc80L65 contributed to green fluorescent proteins (GFP) signals of 80 - 95% IHCs and 67 - 91% outer hair cells (OHCs), as well as 69% VHCs through the trans-round window route, with 15-20 decibel (dB) ABR threshold shifts. And, through the trans-stapes (oval window) route, there were 71 - 90% IHCs and 42 - 81% OHCs, along with 64% VHCs demonstrating GFP positive, and the ABR threshold shifts were within 10 dB. This study revealed AAV could be efficiently delivered into mammalian inner ear cells in vivo through the trans-stapes (oval window) route with postoperative hearing preservation, and both delivery routes showed promise of virus-based clinical translation of hearing impairment treatment.