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Competing Interests: The authors disclose no conflicts of interest.

Purpose: Radiographic review of pathologies that associate with third window syndrome., Methods: Case series and literature review., Results: Eight unique third window conditions are described and illustrated, including superior, lateral, and posterior semicircular canal dehiscence; carotid-cochlear, facial-cochlear, and internal auditory canal-cochlear dehiscence, labyrinthine erosion from endolymphatic sac tumor, and enlarged vestibular aqueduct., Conclusion: The present study highlights the characteristic imaging features and symptoms to differentiate third window pathologies for expedient diagnosis and management planning., Competing Interests: Declaration of competing interest The authors report no relevant conflict of interest in submitting this article for publication., (Copyright © 2023. Published by Elsevier Inc.)

Purpose: To evaluate perioperative findings and audiological and vestibular outcomes in patients operated for cholesteatoma with labyrinthine fistulas. Also to assess radiological fistula size., Materials and Methods: Patients who underwent surgery for a labyrinthine fistula caused by a cholesteatoma between 2015 and 2020 in a tertiary referral center were retrospectively investigated. Fistula size was determined on preoperative CT scan. Bone and air conduction pure tone average thresholds were obtained pre- and postoperatively. Clinical outcomes, such as vertigo and otorrea were also evaluated. Main purpose was to determine whether there is a correlation between fistula size and postoperative hearing. Furthermore, perioperative findings and vestibular outcomes are evaluated., Results: 21 patients (22 cases) with a labyrinthine fistula were included. There was no significant change after surgery in bone conduction pure tone average (preoperatively 27.6 dB ± 26.7; postoperatively 30.3 dB ± 34.3; p = 0.628) or air conduction pure tone average (preoperatively 58.7 dB ± 24.3; postoperatively 60.2 dB ± 28.3; p = 0.816). Fistula size was not correlated to postoperative hearing outcome. There were two patients with membranous labyrinth invasion: one patient was deaf preoperatively, the other acquired total sensorineural hearing loss after surgery., Conclusions: Sensorineural hearing loss after cholesteatoma surgery with labyrinthine fistula is rare. Fistula size and postoperative hearing loss are not correlated, however, membranous labyrinthine invasion seems to be related to poor postoperative hearing outcomes. Therefore, additional preoperative radiological work up, by MRI scan, in selected cases is advocated to guide the surgeon to optimize preoperative counselling., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Objective: To determine the incidence and volume of pneumolabyrinth using early temporal bone computed tomography (TBCT) findings and evaluate the correlation between pneumolabyrinth and various clinical variables., Study Design: Retrospective case review., Setting: Tertiary academic center., Patients: Fifty-three consecutive adult cochlear implantation (CI) recipients (53 ears) with normal inner ears who underwent high-resolution TBCT within 3 days after CI were included., Intervention: Axial TBCT images were used for detection and measurement of pneumolabyrinth., Main Outcome Measures: Incidence and volume of pneumolabyrinth were evaluated. In addition, the following clinical variables were analyzed to evaluate the correlation with pneumolabyrinth volume: demographics, implant type, surgical approach, postoperative dizziness, and preservation of functional residual hearing., Results: Pneumolabyrinth was present in all patients, and the average volume was 8.496 mm3. Pneumolabyrinth was confined to the cochlea (pneumocochlea) in all patients except for one who also had pneumovestibule. The pneumolabyrinth volume in patients with postoperative dizziness was significantly larger than that in patients without postoperative dizziness (10.435 mm3 vs. 8.016 mm3; p = 0.029). Multivariate analysis showed that a larger volume of pneumolabyrinth was significantly associated with postoperative dizziness (odds ratio, 1.420; 95% confidence interval, 1.114-1.808; p = 0.005)., Conclusion: A certain volume of pneumolabyrinth was always present in the early stage after CI, and the volume of pneumolabyrinth measured using early TBCT findings was significantly associated with the occurrence of postoperative dizziness., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2021, Otology & Neurotology, Inc.)

Autoimmune and autoinflammatory inner ear diseases (AIED/AID) are characterized by the symptom of sensorineural hearing loss (SNHL). To date, standardized diagnostic tools for AIED/AID are lacking, and clinically differentiating AIED/AID from chronic otitis media (COM) with SNHL is challenging. This retrospective study aimed to construct a magnetic resonance imaging (MRI)-based decision tree using classification and regression tree (CART) analysis to distinguish AIED/AID from COM. In total, 67 patients were enrolled between January 2004 and October 2019, comprising AIED/AID (n = 18), COM (n = 24), and control groups (n = 25). All patients underwent 3 T temporal bone MRI, including post-contrast T1-weighted images (postT1WI) and post-contrast FLAIR images (postFLAIR). Two radiologists evaluated the presence of otomastoid effusion and inner ear contrast-enhancement on MRI. A CART decision tree model was constructed using MRI features to differentiate AIED/AID from COM and control groups, and diagnostic performance was analyzed. High-intensity bilateral effusion (61.1%) and inner ear enhancement (postFLAIR, 93.8%; postT1WI, 61.1%) were the most common findings in the AIED/AID group. We constructed two CART decision tree models; the first used effusion amount as the first partitioning node and postT1WI-inner ear enhancement as the second node, whereas the second comprised two partitioning nodes with the degree of postFLAIR-enhancement of the inner ear. The first and second models enabled distinction of AIED/AID from COM with high specificity (100% and 94.3%, respectively). The amount of effusion and the degree of inner ear enhancement on MRI may facilitate the distinction between AIED/AID and COM with SNHL using decision tree models, thereby contributing to early diagnosis and intervention., (© 2021. The Author(s).)

Middle ear cholesteatoma is a destructive lesion that can potentially erode the bone. Cholesteatoma is a clinical diagnosis in the vast majority of cases. Patients must be systematically investigated for the presence of signs suggestive of complications, the most common of which is labyrinthine fistula. However, the clinical features of labyrinthine fistula are inconstant and the fistula sign may sometimes be negative. CT scan of the petrous temporal bone is performed systematically to specify the site and extension of the cholesteatoma, and to assess the extent of osteolysis that can result in exposure of the membranous labyrinth. Surgical treatment has three main objectives: complete resection of the cholesteatoma, which is the only way to avoid residual cholesteatoma, prevention of recurrence by an adapted, preferably one-step, technique, and restoration of good quality hearing. Hydrodissection of the cholesteatoma matrix in the presence of labyrinthine fistula is a simple technique that can achieve the three main general objectives of cholesteatoma surgery., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Background: The presence of endolymphatic hydrops (ELH) in patients with Meniere's disease (MD) is considered a pathological hallmark., Aims/objectives: We aimed to conduct a quantitative volumetric measurement of inner ear ELH in patients with unilateral MD (uMD). The values of uMD with and without herniation into the posterior semi-circular canal (h-PSC) and the lateral semi-circular canal (h-LSC) were compared using 3 D magnetic resonance imaging., Material and Methods: This study included 130 individuals (47 controls and 83 patients with uMD). We measured the total fluid space (TFS) and endolymphatic space (ELS) volumes. We also evaluated the ELS/TFS volume ratios (%)., Results: The ELS/TFS volume ratios in the inner ear, cochlea, and vestibule were significantly different between the affected and contralateral sides in patients with h-PSC. Moreover, the ELS/TFS volume ratios of the inner ear, vestibule, and semi-circular canals in the affected ear were significantly higher in patients with h-PSC than in those without h-PSC. The vestibular ELS/TFS volume ratio in the affected ear was significantly higher in patients with h-LSC than in those without h-LSC., Conclusions and Significance: H-LSC is present in extended vestibular ELH. However, this is a result of ELH progression in the inner ear.

Competing Interests: The authors disclose no conflicts of interest.

Background: Salivary gland choristoma (SGCh) is a rare benign tumor reported in several unusual sites, such as the gastrointestinal tract, the optic nerve, and the internal auditory canal, but never reported in the inner ear., Case Presentation: An 8-year-old girl with a history of left profound congenital hearing loss presented to us with ipsilateral progressive severe facial nerve palsy (House-Brackmann Grade VI). The left tympanic membrane was swollen with a pulsatile tumor. Radiological investigations revealed a multilocular tumor in the inner ear extending into the middle ear and internal auditory canal (IAC). We performed a partial resection of the tumor by transmastoid approach to preserve the anatomical structure of the facial nerve. The tumor was pathologically diagnosed as SGCh. Two years after surgery, her facial function recovered to House-Brackmann Grade II and the residual tumor did not show regrowth on MRI., Conclusions: Although the natural course of this rare tumor is unknown, a partial resection is an acceptable treatment procedure when functional recovery of the facial nerve is anticipated.

Objectives/hypothesis: Disturbed perilymph behind a labyrinth fistula can lead to hearing deterioration; thus, delicate manipulation is required during surgery for cholesteatomatous fistulae with matrix extension to the perilymphatic space (EPS). However, it remains challenging to identify the EPS preoperatively. This study aimed to evaluate the diagnostic value of computed tomography (CT) for preoperative prediction of the EPS of cholesteatomatous fistulae., Study Design: Retrospective study., Methods: We included serial high-resolution CT images showing a cholesteatomatous bone defect in the lateral semicircular canal (LSC) requiring mastoidectomy. CT and intraoperative findings were analyzed retrospectively. Using axial CT planes, we evaluated the length and angle between the margins of bone defects. Receiver operating characteristic (ROC) curves were constructed to determine the cutoff points., Results: We extracted data from 30 bone defects, of which six (20.0%) showed EPS intraoperatively. Bone defects with EPS (n = 6) had significantly greater length and angle values than those without EPS (n = 24) (P < .001 for both, Wilcoxon rank sum test). For length and angle, the area under the curve was 0.944 (95% confidence interval [CI]: 0.858-1.000) and 0.951 (95% CI: 0.875-1.000), respectively, according to the ROC analysis, and the optimal cutoff values were 3.65 mm and 71.6°, respectively, with 100% sensitivity and 91.67% specificity for both., Conclusions: Results demonstrated that a length >3.65 mm and an angle >71.6° for LSC bone defects on axial CT images are reliable diagnostic markers of EPS. Preoperative high-resolution CT analysis can provide surgeons with a more conscientious preparation for handling deeper labyrinth fistulae., Level of Evidence: 4 Laryngoscope, 131:E1301-E1307, 2021., (© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).)

Objective: Identify and define specific preoperative and postoperative characteristics of intracanalicular meningiomas (ICMs) in order to improve their diagnosis and management, and to differentiate them from intrameatal vestibular schwannomas (IMVSs)., Methods: Preoperative symptomatology, magnetic resonance imaging (MRI), and postoperative outcomes of 28 ICMs were analyzed. The results were compared to the literature and IMVSs treated by our group., Results: Anacusis and progressive hearing loss were more frequent in the present population than the cases reviewed (P = .0064 and P = .0001, respectively). Hearing loss affected more than 90% of the patients, with anacusis in 32.1% of the cases. Facial palsy affected 17.9% of the patients. In comparison to IMVSs, preoperative anacusis was more associated to meningiomas (P = .0037), and the facial nerve was more compromised in ICMs than IMVSs, both preoperatively (P = .0011) and at follow-up (P < .0001). According to a re-evaluation of preoperative MRIs and comparison with IMVSs, linear tumor borders, and linear morphology along the internal auditory canal wall, but not the presence of a dural tail, were significantly more present in ICMs (P = .0035, P = .0004, P = .1963, respectively). These characteristics could have led to a correct preoperative diagnosis in 61% of our cases., Conclusion: Contrariwise to IMVSs, the frequent preoperative anacusis and facial palsy demonstrate the more aggressive nature of ICMs, which also carry a higher risk of postoperative facial palsy and difficulty to preserve hearing. An attentive evaluation of imaging should ease diagnosis, and asymptomatic or stable ICMs should be enrolled in a wait-and-scan protocol., Level of Evidence: 4 Laryngoscope, 131:E413-E419, 2021., (© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).)

Background: Perilymph gusher (PLG) is a rare complication of otologic surgery attributed to a communication between the cochlea and the internal auditory canal (IAC). Subtle patency between the cochlear basal turn and IAC has recently been identified on computed tomography (CT) as a risk factor, specifically when the defect is > 0.75 mm., Objectives: Investigate the prevalence of radiographic cochlear basal turn patency., Materials and Methods: Patients with CT of the temporal bones and inner ears interpreted as "normal" were included. An otologist and a radiologist independently reviewed CTs to measure radiographic dehiscence in an oblique plane along the interface of the cochlea and IAC. Known PLGs were excluded., Results: Two hundred and ten ears were included (88 conductive or mixed hearing loss, 62 sensorineural hearing loss, 41 audiometrically normal ears). 71 ears (33.8%) were radiographically patent. Mean defect width was 0.41 mm (0.15-0.7 mm). Defect width was not associated with type of hearing loss, age, or gender. No defects were wider than 0.75 mm., Conclusions: Radiographic patency of the cochlear basal turn may be present in patients with hearing loss and normal hearing, but patency > 0.75 mm (i.e. risk for PLG) is rare.

Pneumolabyrinth (PL) is the presence of air within the vestibule, semicircular canals, or cochlea. It represents an abnormal connection between the inner ear and middle ear spaces. PL most commonly occurs after blunt head trauma, followed by penetrating injuries. Temporal fractures may or may not accompany. Prognosis of hearing loss is poor, while prognosis of vestibular symptoms is good. Herein we present a 45-year-old female with unilateral pneumolabyrinth, who presented with significant dizziness and unilateral total hearing loss after a car accident.

Background: High signal intensity in the endolymphatic duct (ED) is occasionally observed on magnetic resonance imaging (MRI) in ears that have otological disorders., Objective: The signal intensity (SI) in the ED on post-contrast MRI was investigated in subjects with various otological disorders, and the meaning of high SI in the ED was evaluated., Material and Methods: 392 patients with otological disorders and 21 controls without otological symptoms underwent 3 T MRI. The SIs of the ED and the cerebellum were measured, the SI ratio (SIR) was calculated, and ears with SIR ≥4 were identified., Results: A high SIR was identified in the ED of 3.7% of ears affected by definite Meniere's disease (dMD), 100% of ears affected by large vestibular aqueduct syndrome (LVAS), and 7.1% of ears with no otological symptoms. On the whole, a significant relationship was found between the existence of vestibular or cochlear EH and the SIR in the ED., Conclusion: The MRI finding of high SI in the ED may indicate the mechanism of inner ear disturbances in ears with otological disorders, especially in those with LVAS, and it may suggest an underlying disorder in some ears in which otological symptoms are not apparent.

Background: Perilymphatic fistula (PLF) is a breach in a labyrinthine window. The opening might decrease the radiological density of the window. Aims/objectives: To evaluate the radiological density of the labyrinthine windows by virtual endoscopy on CT scan. Materials and methods: This prospective study included 47 adult patients with PLF and 98 control patients. Diagnosis of PLF was based on a composite radio clinical score and/or intra operative visualization of the fistula and/or resolution of the symptoms after surgery. On routine CT-scan, labyrinthine windows were examined by virtual endoscopy. The reconstruction threshold was gradually increased until a virtual opening appeared (opening threshold [OT]) and compared to the contralateral window (OT difference). Results: The OT difference was higher in patients than in controls (60.2 ± 10.36 (SEM), n  = 47 versus 28.0 ± 2.29 Hounsfield units (HUs), n  = 98, p  < .01 unpaired t -test). A ROC analysis showed that at an OT difference of 31.5 UH had a sensitivity of 75% and a specificity of 75% for the PLF diagnosis. Conclusions: CT-scan virtual endoscopy and threshold variation provided high specificity and sensitivity in the PLF diagnosis. Significance: This post processing of radiological data appears to enhance the diagnostic value of CT scan.

Background: We evaluated the usefulness of CT and MRI for the diagnosis of perilymphatic fistula (PLF) of the round (RW) and/or oval (OW) windows, with surgery as gold standard., Methods: We retrospectively enrolled 17 patients who presented a surgically confirmed PLF of the round (RW) or oval (OW) windows. All patients were imaged by CT + MRI (T2W SSFP without contrast) prior to surgery (= gold standard). Two radiologists, analyzed the RW and OW on the side of the clinical symptoms and sensitivity (Se) + Specificity (Sp) were calculated., Results: Round window fistula was the most frequent (71%). The best sign of PLF on imaging was a fluid filling of the window niches, which had good Se (83-100% for RW, 66-83% for OW) and Sp (60% for RW, 91-100% for OW). Disorientation of the footplate and pneumolabyrinth were also only observed in 50% of OW PLF., Conclusion: The combination of CT and MRI is a reliable tool for a fast and accurate diagnosis of round and oval window perilymphatic fistula, with good sensitivity (> 80%). The most common sign of PLF on imaging is the presence of a fluid-filling in the RW (especially if > 2/3 of the RW niche) or in the OW niches on both CT and MRI. A disorientation of the footplate or the presence of a pneumolabyrinth are clearly in favor of an oval window perilymphatic fistula.

Objective: This study aims to investigate the application value of magnetic resonance (MR) hydrography of the inner ear in cochlear implantation., Methods: 146 patients were enrolled. MR hydrography and spiral CT examinations for the intracranial auditory canal were performed before surgery, and all imaging results were statistically analyzed in order to explore the application value of MR hydrography of the inner ear in cochlear implantation., Results: 146 patients (292 ears) were examined. Among these patients, 13 were diagnosed with abnormal vestibular aqueducts (20 ears) by MR hydrography, while five were diagnosed with this disease by CT; 15 patients were diagnosed with inner ear malformation (19 ears) by MR hydrography, while 11 were diagnosed by CT (four were misdiagnosed); five patients were diagnosed with internal acoustic canal stenosis (eight ears) by MR hydrography, while two were diagnosed by CT (three were misdiagnosed); and four patients were diagnosed with cochlear fibrosis (five ears) by MR hydrography, while four were diagnosed by CT (four ears). The correct rate of diagnosis was 77.40% (113/146) based on CT, while the rate was 93.84% (137/146) based on MR hydrography., Conclusions: MR hydrography imaging technique can be applied to the preoperative evaluation of cochlear implantation, providing accurate and reliable anatomic information on the inner membranous labyrinth and nerves in the internal acoustic canal and an accurate basis for the diagnosis of cochlear fibrosis and nerve development. This has a guiding significance for the selection of treatment schemes.

This case report describes a 53-year-old woman, who presented with symptoms of a perilymphatic fistula (PLF), developed 12 years after most recent surgery. The diagnosis was confirmed by a combination of clinical observations, temporal computed tomography, audiometry and video head impulse test. PLF normally occurs after traumatic lesions or substantial infections of the middle ear, often including cholesteatomas, and should be suspected when patients with the relevant clinical history present with sudden onset of unilateral hearing loss, dizziness, tinnitus and aural fullness.

Objective: In the diagnosis of superior semicircular canal dehiscence (SSCD), computed tomography (CT) is the only imaging method. The aims of the study were to show that reformat images are more accurate than standard planes for diagnosis of SSCD and to determine the prevalence of SSCD., Methods: The retrospective review yielded 1309 temporal CTs performed in our radiology department for any reason. Two radiologist interpreted CTs in standard planes collaboratively. Patients with SSCD were reinterpreted in Pöschl and Stenvers planes by 2 radiologists separately., Results: Statistical analysis was made by accepting that 2 radiologists diagnosis were accurate in Pöschl plane. Coronal plane sensitivity 86%, specificity 64%, Stenvers plane sensitivity 96%, and specificity 52% have been found in the mean result of 2 observers (P < 0.001)., Conclusions: In the diagnosis of SSCD, standard and Stenvers planes can cause false-negative and false-positive diagnoses. Interpretation in Pöschl plane can significantly increase sensitivity, specificity, negative, and positive predictive values for diagnosing dehiscence.

X-linked stapes gusher syndrome is a genetic form of deafness with distinct radiographic features on temporal bone CT. Hypothalamic hamartoma is a congenital glioneuronal anomaly of the hypothalamus. We report a potential association between these two rare anomalies that, to our knowledge, has not been reported. Two brothers presented with sensorineural hearing loss and almost identical inner ear and hypothalamic abnormalities, consistent with a diagnosis of X-linked stapes gusher syndrome and hypothalamic hamartoma. Genetic testing revealed identical mutations in the POU3F4 gene associated with X-linked stapes gusher syndrome. Furthermore, multiple vestibular diverticula were seen in both brothers, which have also not been reported with X-linked stapes gusher syndrome. This case suggests that POU3F4 mediated X-linked stapes gusher syndrome may also lead to multiple vestibular diverticula and hypothalamic hamartoma and, therefore, brain magnetic resonance imaging (MRI) could be considered in patients presenting with these inner ear findings.

Objectives: The aim of the present study is to assess the impact of different slice thicknesses in computed tomography for labyrinthine fistula evaluation and to determine the appropriate slice thickness., Methods: A total of 258 patients who underwent mastoidectomy and tympanoplasty for chronic otitis media with cholesteatoma from 2010 to 2014 were reviewed. The radiological diagnoses were compared with intraoperative findings. Sensitivity and specificity of 2.0-, 1.5-, 1.0-, and 0.75-mm-thick computed tomographic (CT) images for the evaluation of labyrinthine fistulae were calculated. Cohen's κ coefficient was also calculated., Results: The sensitivities of 2.0-, 1.5-, 1.0-, and 0.75-mm-thick CT images for the evaluation of labyrinthine fistulae were 76.9, 86.5, 90.4, and 93.3% (observer 1) and 67.3, 76.0, 79.8, and 87.5% (observer 2), respectively. The specificities of 2.0-, 1.5-, 1.0-, and 0.75-mm-thick CT images for labyrinthine fistula evaluation were 96.1, 94.8, 95.5, and 95.5% (observer 1) and 99.4, 97.4, 95.5, and 94.8% (observer 2), respectively. Cohen's κ coefficients were 0.790, 0.788, 0.876, and 0.911 in 2.0-, 1.5-, 1.0-, and 0.75-mm-thick CT images, respectively., Conclusions: The sensitivity of CT for labyrinthine fistula evaluation increases with decreasing slice thickness, while the specificity does not improve., (© 2019 S. Karger AG, Basel.)

Introduction: Autoimmune inner disease (AIED) is an uncommon cause of sensorineural hearing loss and poses a diagnostic challenge. The present study aims to review the existing knowledge on the clinicopathological aspects, the diagnostic challenges, and therapeutic interventions in AIED., Discussion: The incidence of AIED is less than five cases per 100,000 population. There are no definite seromarkers which make diagnosis of AIED difficult. Even though various markers have been studied, their sensitivity and specificity have not been replicated in the clinical scenario. The treatment of the condition is also an enigma. Corticosteroids are the drug of choice and require long-term use to prevent relapse. Various other therapeutic agents have been studied in a small cohort of patients, but the efficacy of these drugs needs to be validated in a large multicentric trial., Conclusion: Timely intervention can restore hearing loss in AIED patients, but the clinician has to find a delicate balance between the hearing outcome and the potential side effects resulting from long-term use of the drugs. Treatment of steroid resistant AIED is a challenge and there are no universal guidelines for the same. AIED being an uncommon diagnosis, multicentric trials and collaboration are required to formulate diagnostic criteria and therapeutic guidelines.

A four-year-old female French bulldog was presented for evaluation of acute, left-sided peripheral vestibular syndrome. Computed tomographic (CT) examination of the head revealed the presence of air within the left cochlea and vestibule, consistent with pneumolabyrinth. This was concurrent with ipsilateral otitis media and externa. Pneumolabyrinth is an uncommon finding in humans and is most frequently due to head trauma and temporal bone fracture. This is the first report describing pneumolabyrinth in a dog, apparently of nontraumatic origin in this case., (© 2017 American College of Veterinary Radiology.)

Superior semicircular canal dehiscence (SSCD) is a rare inner ear disorder with variable amounts of auditory and vestibular dysfunction. In addition to the absence of bone overlying the superior semicircular canal, thinning of bone in this area can also initiate the vestibulocochlear symptoms of SSCD. We evaluated normal bone thickness overlying the course of the semicircular canal using computed tomography (CT) scans and assessed correlations between bone thickness and age, gender, and location of the thinnest bone. A single-institution retrospective chart review was conducted on 133 high-resolution CT scans from 76 healthy, asymptomatic patients between ages 9 and 96 years. These CT scans of the temporal bone were obtained between January 2012 and August 2017. The superior semicircular canal dome thickness at the apex was reported with a mean of 1.25 mm for all 76 patients; the 10th percentile was 0.60 mm, and the 90th percentile was 2.08 mm. The thinnest area of bone at any location yielded a mean of 0.86 mm. The normal bone thickness overlying the superior semicircular canal does not depend on gender or age. The thinnest location was evenly distributed across the superior semicircular canal. A bone thickness of 0.40 mm or greater was present in 90% of normal patients based on CT scan measurements at the thinnest location., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Third window defects have increasingly been identified as a cause of vertigo. These defects are bony dehiscences that occur in the bony labyrinth, resulting in abnormal pressure gradient in the inner ear fluids leading to sound (Tullio's phenomenon) or pressure (Hennebert's sign) induced vertigo. The superior semicircular canal dehiscence syndrome is a well-described entity in this regard, however defects of the posterior semicircular canal are rare and may have overlapping symptomatology. We describe the history, clinical profile and management of a patient who had importunate symptoms despite being on conservative management for a year and had resolution of vestibular symptoms following surgical management., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Superior semicircular canal dehiscence syndrome (SSCD) is a current diagnosis that is due to a loss of bone covering the superior semicircular canal (SSC). This results in pressure-/sound- induced vertigo and oscillopsia., Objective: To find the variation of the thickness of the bone that covers the Superior Semicircular Canal with relation to age and gender among the Chinese descents., Materials and Methods: Three hundred and eleven temporal bone Cone Beam Computed Tomography (CBCT) images of patients who attended Otology clinic at Second Hospital of Shandong University from January, 2017 to April, 2018 were retrospectively studied. The images were reconstructed in the line of Poschl and the thinnest area of the bone covering the SSC was taken., Results: We included 172 (55.31%) females and 139 (44.69%) males. Mean age was 41 years. Overall mean difference in thickness was found to be -0.0210. There was no significant difference between the female and male bone thickness (p = .7113). With age the mean difference was 0.0801 (p = .1557) which was not statistically significant., Conclusion and Significance: There was no significant change in bone thickness with advancing age. CBCT is the best method of assessing SSCD.

Background: In sudden and asymmetrical progressive sensorineural hearing loss (SNHL), magnetic resonance imaging (MRI) is required to evaluate retrocochlear pathology and, with recent advances in MRI techniques, inner ear pathology. Given the limited literature regarding inner ear pathology associated with SNHL, we aimed to assess the incidence of retrocochlear and inner ear pathology, and congenital malformation on MRI in sudden SNHL (SSNHL) and progressive SNHL., Methods: A total of 987 acoustic neuroma (AN) protocol MRI internal acoustic meatus studies performed at our institution to investigate SNHL between January 2013 and December 2015 inclusive were identified. Following categorization for indication of SSNHL versus progressive asymmetrical SNHL, MRIs with retrocochlear or inner ear abnormality, congenital malformation or other otology-related abnormality were identified, and further data were collected for these patients including patient demographics, associated symptomatology, management and outcomes., Results: In SSNHL, aetiological abnormality on MRI was identified for 6.9% patients with AN present on 4% overall. 3.2% of MRIs for progressive asymmetrical SNHL identified a causative lesion with 2.3% of scans overall diagnosing AN. The incidence of congenital inner ear malformation on MRI in the setting of SSNHL and progressive asymmetrical SNHL are 1.7% and 0.6%, respectively., Conclusion: This is the first retrospective study of inner ear MRI abnormalities in both SSNHL and progressive asymmetrical SNHL in Australia and one of the largest cohorts published in the literature to date. MRI must be performed in the setting of SNHL to ensure aforementioned and rarer causative lesions are identified., (© 2018 Royal Australasian College of Surgeons.)

The aim of this study was to check the relevance of using in-vivo micro computed tomography (µCT) for the diagnosis of possible diseases of the middle and inner ear of the cat. Therefore, on the one hand, differences of the detail detectability between the two imaging methods conventional computed tomography (cCT) and in-vivo µCT were analyzed. Six healthy cat ears were dissected and scanned several times and the obtained images were compared with each other. On the other hand, histological slices of all ears were prepared and pictures of defined anatomical structures were taken and compared with the identical sectional plane of the µCT-images. This way it was possible to evaluate the quality and clinical limitations of the in-vivo µCT. The results show that an in-vivo µCT is suitable to analyze even the smallest osseous structures, such as the semicircular ducts, the spiral osseous lamina or the ossicles whereas with the help of cCT it is not possible to identify such small osseous structures because of their blurred and less detailed representation. Delicate soft tissue structures as the membranous labyrinth including hearing and vestibular organ cannot be differentiated with as well in-vivo µCT- as with cCT-images. In-vivo µCT represent a good possibility for more detailed diagnosis of extremely fine structures which cannot be detected with cCT. Histological slices can nonetheless not be replaced by in-vivo µCT due to a too low spatial resolution and the limitations of the in-vivo µCT with regard to the evaluation of soft tissue dense structures., (© 2019 Blackwell Verlag GmbH.)

Background and Purpose: Prior studies of radiologic superior semicircular canal dehiscence have suggested that CT may overcall dehiscence. However, many of those studies were performed before the advent of multichannel helical CT. Furthermore, there are limited data investigating the prevalence of radiologic superior semicircular canal dehiscence in asymptomatic individuals. The purpose of this study was to determine the rate of radiologic superior semicircular canal dehiscence in an asymptomatic population using 64-channel helical CT., Materials and Methods: We retrospectively enrolled 500 consecutive adult patients without symptoms of superior semicircular canal dehiscence who had undergone CT of the temporal bones in the emergency department of a tertiary academic center between February 2012 and June 2017. The superior semicircular canals were evaluated bilaterally by 2 dedicated head and neck radiologists and subjectively classified as either dehiscent or nondehiscent. A secondary group of 110 scans of patients with symptoms consistent with superior semicircular canal dehiscence was similarly interpreted, and the rate of radiologic superior semicircular canal dehiscence was calculated for each group., Results: Ten of the 500 asymptomatic patients (2.0%; 95% CI, 1.1%-3.6%) had CT evidence of superior semicircular canal dehiscence, compared with 15 of 110 symptomatic patients (13.6%; 95% CI, 7.8%-21.5%). There was excellent interobserver agreement (κ = 0.91)., Conclusions: Only 2% of asymptomatic patients had radiologic evidence of superior semicircular canal dehiscence on 64-detector row helical CT. This is markedly lower than previous reports and approaches the postmortem rate of asymptomatic superior semicircular canal dehiscence. We therefore recommend that asymptomatic patients with CT evidence of superior semicircular canal dehiscence undergo audiologic evaluation because the rate of false-positive scans is extremely low., (© 2019 by American Journal of Neuroradiology.)

Introduction: Labyrinthine fistula is one of the most common complications associated with cholesteatoma. It represents an erosive loss of the endochondral bone overlying the labyrinth. Reasons for cholesteatoma-induced labyrinthine fistula are still poorly understood., Objective: Evaluate patients with cholesteatoma, in order to identify possible risk factors or clinical findings associated with labyrinthine fistula. Secondary objectives were to determine the prevalence of labyrinthine fistula in the study cohort, to analyze the role of computed tomography and to describe the hearing results after surgery., Methods: This retrospective cohort study included patients with an acquired middle ear cholesteatoma in at least one ear with no prior surgery, who underwent audiometry and tomographic examination of the ears or surgery at our institution. Hearing results after surgery were analyzed according to the labyrinthine fistula classification and the employed technique., Results: We analyzed a total of 333 patients, of which 9 (2.7%) had labyrinthine fistula in the lateral semicircular canal. In 8 patients, the fistula was first identified on image studies and confirmed at surgery. In patients with posterior epitympanic and two-route cholesteatomas, the prevalence was 5.0%; and in cases with remaining cholesteatoma growth patterns, the prevalence was 0.6% (p=0.16). In addition, the prevalence ratio for labyrinthine fistula between patients with and without vertigo was 2.1. Of patients without sensorineural hearing loss before surgery, 80.0% remained with the same bone conduction thresholds, whereas 20.0% progressed to profound hearing loss. Of patients with sensorineural hearing loss before surgery, 33.33% remained with the same hearing impairment, whereas 33.33% showed improvement of the bone conduction thresholds' Pure Tone Average., Conclusion: Labyrinthine fistula must be ruled out prior to ear surgery, particularly in cases of posterior epitympanic or two-route cholesteatoma. Computed tomography is a good diagnostic modality for lateral semicircular canal fistula. Sensorineural hearing loss can occur post-surgically, even in previously unaffected patients despite the technique employed., (Copyright © 2018 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.)

Background: Superior semicircular canal dehiscence (SSCD) is a rare bony defect in the petrosal temporal bone, creating a new opening between the inner ear and middle cranial fossa. Ehlers-Danlos syndrome (EDS) is an inherited connective tissue disorder characterized by a defect in collagen synthesis. Patients with EDS are more likely to have bony abnormalities, which may predispose them to developing SSCD., Case Description: We report the cases of 2 women with history of EDS hypermobility type (HT). Both patients presented with bilateral auditory and vestibular symptoms, and computed tomography scan confirmed the presence of bilateral dehiscence in their superior semicircular canals. They underwent elective surgical repair via middle fossa craniotomy and report improvement in their symptoms., Conclusions: Because of the rarity of both diseases and their pathophysiology, a history of EDS may predispose an individual to developing SSCD. Although further research is necessary, this report aims to improve clinical screening of patients with EDS HT by suggesting an auditory and vestibular evaluation and assessment of pertinent bony abnormalities., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Hypothesis: Flat panel computed tomography (FPCT) provides more accurate measurements of dimensions for superior semicircular canal dehiscence (SCD) than multislice CT (MSCT)., Background: SCD syndrome occurs when a bony defect of the superior semicircular canal causes vestibular and auditory symptoms. MSCT can overestimate the size of the canal defect, with possible over-diagnosis of SCD and suboptimal selection of surgical approach. The higher resolution of FPCT should afford more accurate measurements of these defects., Methods: Radiographic and surgical measurements were compared in 22 patients (mean age 49.4) with clinical SCD syndrome and canal defects confirmed at surgery. Twenty second FPCT scans were acquired before surgery with parameters: 109Kv, small focus, 200 degrees rotation angle, and 0.4 degree per frame angulation step. Dehiscence dimensions were measured from orthogonal multiplanar reconstructions on a high-resolution liquid crystal display monitor and compared with actual measurements recorded during microsurgery., Results: SCD dimensions by FPCT (x) were 2.8 ± 1.6 mm for length and 0.72 ± 0.28 mm for width. The surgical measurements (y) were 2.8 ± 1.7 mm for length and 0.72 ± 0.34 mm for width. Linear fits between x and y yielded R values of 0.93 (length) and 0.66 (width). Our previous study using MSCT had R values of 0.28 (length) and 0.48 (width). The average difference between each FPCT and corresponding surgical measurement was not significantly different from zero, whereas the results for MSCT were significantly different., Conclusion: FPCT can provide more accurate measurements of SCD than MSCT. Clinicians should consider using FPCT for imaging suspected SCD.

Third window abnormalities are bony defects of the inner ear that enable abnormal communication with the middle ear and/or cranial cavity. Vestibular symptoms include vertigo and nystagmus induced by loud noises or increases in pressure. Auditory symptoms involve "pseudo-conductive" hearing loss with a low-frequency air-bone gap at audiometry, resulting from decreased air and increased bone conduction. High-resolution temporal bone computed tomography is the first-line imaging modality for evaluation of third window pathology and is critical for accurate diagnosis and management. This article reviews the fundamental mechanisms of the third window phenomenon and describes imaging findings and differential diagnosis., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: The etiology of symptoms following primary repair of superior canal dehiscence (SCD) may be due to a persistent third window. However, the extent of surgery cannot be seen on postoperative computed tomography (CT) since most repair materials are not radiopaque. We hypothesize that the extent of superior semicircular canal (SSC) occlusion following primary repair can be quantified based on postoperative magnetic resonance imaging (MRI) data., Study Design: Retrospective series., Setting: Tertiary care center., Patients: Adult patients with a history of SCD syndrome who 1) report persistent symptoms following primary SCD repair and 2) underwent heavily T2-weighted MRI postoperatively., Interventions: Analysis of SSC using 3D-reconstruction of CT co-registered with MRI data., Main Outcome Measures: Arc length of fluid void on MRI and quantification of persistent SCD based on CT/MRI co-registration., Results: We identified 9 revision cases from a cohort of 145 SCD repairs at our institution (2002-2017) with CT/MRI data. A fluid void on postoperative MRI (indicating occlusion of the SSC) was observed in all cases (anterior limb: 50.1 degrees [±21.8 SD] and posterior limb 48.1 degrees [±28.5 SD]). Co-registration of CT/MRI revealed a residual defect that was most commonly found along the posterior limb in most patients with persistent symptoms., Conclusions: The extent of SCD repair can be determined using reformatted or direct T2-weighted MRI sequences in the plane of Pöschl. Co-registration of CT/MRI may be useful to determine the location of a residual superior canal defect and when present was found most commonly along the posterior limb.

Introduction: Intralabyrinthine schwannoma is a rare, benign tumor that affects the most terminal portions of the vestibular and cochlear nerves. This tumor can be classified into 10 subtypes, according to its inner ear location., Objective: To carry out a comprehensive review of the most frequent auditory manifestations secondary to the intralabyrinthine schwannoma, describing the possible underlying pathophysiological mechanisms., Methods: Systematic review of the literature until October 2017 using the PubMed, Web of Science and Scopus databases. The inclusion criteria were clinical manifestations of the intralabyrinthine schwannoma. Three researchers independently assessed the articles and extracted relevant information. The description of a case of an intravestibular subtype intralabyrinthine schwannoma with multiple forms of clinical presentations was used as an example., Results: Twenty-seven studies met our inclusion criteria. The most common intralabyrinthine schwannoma subtype was the intracochlear, followed by the intravestibular type. All the cases demonstrated hearing loss, usually progressive hearing loss., Conclusion: The diagnosis of intralabyrinthine schwannomas is based on high-resolution magnetic resonance imaging and should be included in the differential diagnosis of patients with vestibulocochlear complaints. Although there are approximately 600 cases in the literature, we still lack a detailed description of the clinical evolution of the patients, correlating it with MRI findings of temporal bones and tumor subtype., (Copyright © 2018 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.)

Objective: To determine the prevalence of radiographic cochlear-facial nerve dehiscence (CFD)., Study Design: Retrospective radiological study., Setting: Two tertiary-referral centers., Patients: Two hundred six temporal-bone computed tomography (CT) scans (405 total ears) of otology/neurotology patients from two academic institutions between the years 2014 and 2017., Intervention: Diagnostic., Main Outcome Measures: The cochlear-facial nerve partition width (CFPW) was measured on coronal CT sections and defined as the shortest distance between the cochlear basal turn and facial nerve (FN) labyrinthine segment. We used logistics regression analyses to determine positive predictors for radiographic evidence of CFD., Results: The overall prevalence of radiographic CFD was 5.4% (22/406 ears). 9.2% of patients (19/206) had CFD. Of these 19 patients, only one patient had mixed hearing loss that could not be explained by any other vestibular or auditory etiology. Three out of 206 patients had dehiscence in both ears (1.4%). The average CFPW was 0.6 ± 0.2 mm, and fallopian canal width was 1.1 ± 0.02 mm (n = 405). Older age, use of traditional CT scans, and thinner CT slice thickness were significant predictors for radiographic CFD., Conclusions: The radiographic prevalence of CFD is higher than what is reported in histologic studies, and may over-estimate the true prevalence of CFD. The clinician should keep this in mind when considering this as potential cause for third-window symptoms.

Background and Purpose: High-resolution T2-weighted sequences are frequently used in magnetic resonance imaging (MRI) studies to assess the cerebellopontine angle and internal auditory canal (IAC) in sensorineural hearing loss patients but have low yield and lengthened examinations. Because image content in the Wavelet domain is sparse, compressed sensing (CS) that uses incoherent undersampling of k-space and iterative reconstruction can accelerate MRI acquisitions. We hypothesized that an accelerated CS T2 Sampling Perfection with Application optimized Contrasts using different flip angle Evolution (SPACE) sequence would produce acceptable diagnostic quality for IAC screening protocols., Material and Methods: Seventy-six patients underwent 3 T MRI using conventional SPACE and a CS T2 SPACE prototype sequence for screening the IACs were identified retrospectively. Unilateral reconstructions for each sequence were separated, then placed into mixed folders for independent, blinded review by 3 neuroradiologists during 2 sessions 4 weeks apart. Radiologists reported if a lesion was present. Motion and visualization of specific structures were rated using ordinal scales. McNemar, Wilcoxon, Cohen κ, and Mann-Whitney U tests were performed for accuracy, equivalence, and interrater and intrarater reliability., Results: T2 SPACE using CS reconstruction reduced scan time by 80% to 50 seconds and provided 98.7% accuracy for IAC mass detection by 3 raters. Radiologists preferred conventional images (0.7-1.0 reduction on 5-point scale, P < 0.001), but rated CS SPACE acceptable. The 95% confidence for reduction in any cerebellopontine angle, IAC, or fluid-filled inner ear structure assessment with CS SPACE did not exceed 0.5., Conclusions: Internal auditory canal screening MRI protocols can be performed using a 5-fold accelerated T2 SPACE sequence with compressed sensing while preserving diagnostic image quality and acceptable lesion detection rate.

Objectives: To highlight superior semicircular canal dehiscence (SSCD) involving the superior petrosal sinus (SPS), and to propose a novel classification system for SPS associated SSCD with potential surgical implications., Study Design: Multicenter retrospective review., Setting: Three tertiary referral centers., Patients: All patients diagnosed with SPS associated SSCD (1/2000 to 8/2016). Radiographic findings and clinical symptoms were analyzed., Intervention: Surgical repair or observation., Main Outcome Measure: Radiographic findings and clinical symptoms were analyzed., Results: Thirty-three dehiscences (30 patients) involving the SPS were identified. The average age at the time of presentation was 52.5 years (median, 56.9; range, 4.9-75.3 yr), and 53.3% of patients were men. Three patients had bilateral SPS associated SSCD. The most common associated symptoms at presentation were episodic vertigo (63.6%), subjective hearing loss (60.6%), and aural fullness (57.6%). Four distinct types of dehiscence were identified: class Ia. SSCD involving a single dehiscence into an otherwise normal appearing SPS; class Ib. SSCD involving a single dehiscence into an apparent venous anomaly of the SPS; class IIa. SSCD involving two distinct dehiscences into the middle cranial fossa and the SPS; class IIb. SSCD involving a single confluent dehiscence into the middle cranial fossa and the SPS., Conclusions: SSCD involving the SPS represents a small but distinct subset of SSCD cases. This scenario can create a unique set of symptoms and surgical challenges when intervention is sought. Clinical findings and considerations for surgical intervention are provided to facilitate effective diagnosis and management.

Background: Humans detect motion and gravity via the labyrinthine system of the inner ear, which consists of the vestibule and the semicircular canals. The vestibular system provides three major functions for maintaining balance and orientation. First, it maximizes the effectiveness of the visual system. Second, it provides orientational information necessary for performing both skilled and reflexive motor activities. Third, in the absence of vision, the vestibular system provides a reasonably accurate perception of motion and position. Although these organs provide important cues for basic orientation on the ground, they often provide misleading information during flight. A superior semicircular canal dehiscence can cause an individual to experience disorientation and vertigo-like symptoms., Case Report: A 30-yr-old male student pilot experienced airsickness on his first several flights in the T-6A Texan II aircraft. Airsickness is common among student pilots in the early stages of flight training; however, the subject pilot's symptoms lasted well beyond the normal 24 h, and included vertiginous symptoms lasting days after his last flight. His persistent symptoms required he be held out of training for further evaluation. Comprehensive vestibular and audiological testing were normal; however, a CT scan of the temporal bones showed a left superior semicircular canal dehiscence, and he was diagnosed with left labyrinthine fistula as the cause of his vertigo. He was medically eliminated from pilot training and permanently disqualified from U.S. Air Force flying duties., Discussion: Spatial disorientation remains one of the deadliest factors related to aircraft mishaps every year. In this case, an abnormal presentation of airsickness that prompted further evaluation may have prevented a dangerous situation in the air. In standard airsickness, the goal is to keep the pilot flying to expedite adaptation, so this case highlights the importance of distinguishing between normal airsickness and a spatially disorienting medical condition.Dreibelbis JA, Organ BE. Semicircular canal dehiscence syndrome and vestibular dysfunction disqualify a military student pilot. Aerosp Med Hum Perform. 2018; 89(10):923-926.

Objective: High-resolution flat panel computed tomography (FPCT) is useful for the evaluation of temporal bone pathologies. While radiation exposure remains a concern, efforts have been devoted to reduce dose, while maintaining image quality. We hypothesize that removing the antiscatter grid (ASG) results in decreased radiation exposure, while maintaining diagnostic image quality for the evaluation of superior semicircular canal dehiscence (SSCD)., Methods: Ten patients with clinical suspicion for SSCD participated in this prospective study. Two sequential collimated 20-second FPCT acquisitions were performed (first: grid in; second: grid removed) in all patients. Secondary reconstructions were created by manually generating the volume of interest to include the middle ear using a voxel size of 0.1 mm and 512 × 512 matrix. Radiation dose parameters (air kerma (Ka,r) in mGy and dose area product (DAP) in μGym) were recorded. Three reviewers analyzed images for the ability to diagnose SSCD, to identify the stapes crurae, and to determine if an ASG was present., Results: The average Ka,r and DAP for the grid-in acquisitions were 246.7 mGy (SD 47.9) and 2838.0 μGym (SD 862.8), versus 160.2 mGy (SD 33.2) and 2026.3 μGym (SD 644.8) for the grid-out acquisitions, respectively (p<0.001 for both Ka,r and DAP). Radiation exposure was reduced by approximately 30% solely by removing the ASG. All reviewers correctly identified all patients with SSCD (confirmed at surgery), with mean AUC of 0.99 (κ = 0.90)., Conclusion: Removing the antiscatter grid during FPCT imaging of the temporal bones is a simple and effective way to reduce radiation exposure while maintaining diagnostic image quality for the evaluation of SSCD.

Introduction: To evaluate the utility of 18 F-FDG PET/TC as an imaging tool for the characterization of immune-mediated inner ear disease (IMIED), providing measurements of the inner ear region activity as well as detecting possible involvement of other organs., Material and Methods: The study included 28 patients with IMIED and 4 sex-matched and age-matched control subjects with no history of ear disease. Eighteen patients were considered to be suffering from primary IMIED and 10 patients from secondary. PET/CT scans with 18 F-FDG were performed to assess systemic involvement as well as inner ear region activity. Interpretation of PET/CT scans was performed independently by 2nuclear medicine physicians blinded to clinical history. In order to assess inter-rater agreement before performing the analysis of the inner ear, different Bland & Altman plots and the intraclass correlation coefficients were estimated., Results: Different metabolically active foci findings were reported in 13 patients. Four patients diagnosed as primary IMIED showed thyroid and aorta activity. Regarding the inner-ear semiquantitative analysis, the inter-rater agreement was not sufficiently high. Comparisons between groups, performed using Mann-Whitney test or Kruskal-Wallis tests, showed no differences., Conclusions: The study showed 18 F-FDG PET/TC could be an important tool in the evaluation of IMIED as it can support the characterization of this entity providing the diagnosis of unknown or underestimated secondary IMIED. Nevertheless, we consider PET is not an adequate tool to approach the inner ear because of the small size and volume of the cochlea which makes the assessment very difficult., (Copyright © 2018 Sociedad Española de Medicina Nuclear e Imagen Molecular. Publicado por Elsevier España, S.L.U. All rights reserved.)

Objectives/hypothesis: To demonstrate imaging of labyrinthine fistula due to cholesteatoma and illustrate outcome following repair with bone pate., Study Design: Retrospective review., Methods: Patients with labyrinthine fistula due to cholesteatoma were identified, and pre- and postoperative imaging was assessed. Method of repair was recorded. The fistula site was examined for patency and bone density after repair., Results: Twenty-nine fistulae were seen in a cohort of 375 cholesteatoma cases (8%). Preoperative computed tomography (CT) imaging for fistula detection showed sensitivity of 96% and specificity of 90%. The lateral semicircular canal was the most common site of dehiscence occurring in all cases. Facial nerve dehiscence is observed in 91% of fistula cases and stapes erosion in 69%. Severe hearing loss was present preoperatively in four (14%) cases. Bone pate was used to repair the fistula with CT-documented restoration of the otic capsule in 11/13 cases with postoperative imaging. When bone pate was not used, the otic capsule defect persisted., Conclusions: Spontaneous repair of the otic capsule following treatment of cholesteatoma is expected to be an uncommon occurrence. Repair of the fistula with bone can result in long-lasting restoration of the otic capsule., Level of Evidence: 4. Laryngoscope, 128:1643-1648, 2018., (© 2017 The American Laryngological, Rhinological and Otological Society, Inc.)

An elderly male patient diagnosed with a right-sided cystic vestibular schwannoma (CVS) at our center underwent a translabyrinthine approach with a subtotal excision to preserve the facial nerve (FN). The tumor grew slowly for the first 9 years but in the subsequent 2 years grew rapidly, with the patient developing a FN paralysis. Using the previous approach, a second surgery was done and the tumor was excised, leaving behind a sheath of tumor on the facial and lower cranial nerves. This case demonstrates that CVSs show unpredictable growth patterns and need to be followed up for a longer period of time. Laryngoscope, 128:1649-1652, 2018., (© 2017 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To evaluate the presence of endolymphatic hydrops in patients with immune-mediated inner-ear disease., Methods: The presence of endolymphatic hydrops was prospectively evaluated in 17 patients clinically diagnosed with secondary (n = 5) or primary (n = 12) immune-mediated inner-ear disease, who attended the ENT department of a tertiary care centre for evaluation or treatment over the previous year. All patients underwent magnetic resonance imaging of the temporal bone., Results: Intratympanic gadolinium three-dimensional magnetic resonance imaging diagnosed hydrops in 11 of 12 patients with primary immune-mediated inner-ear disease (92 per cent). Of these, seven patients (64 per cent) presented only cochlear (n = 5) or predominantly cochlear (n = 2) hydrops. A positive magnetic resonance imaging result was observed in only one of five patients with secondary immune-mediated inner-ear disease (20 per cent)., Conclusion: This study confirms the presence of endolymphatic hydrops in immune-mediated inner-ear disease patients. The virtual absence of hydrops in patients with secondary immune-mediated inner-ear disease is remarkable, although firm conclusions cannot be drawn; this should be explored in a multicentre study with a larger sample of patients. A different immune reaction without development of endolymphatic hydrops should not be ruled out in secondary immune-mediated inner-ear disease patients.