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Horizontal canal fistulas are not uncommon in patients with cholesteatoma. Patients with canal wall down cavities and exposed horizontal canal fistulas develop significant dizziness with wind or suction exposure. Obliteration of mastoid cavities in patients with exposed fistulas can be challenging. We describe a patient with horizontal canal fistula and chronic dizziness from wind exposure who underwent successful mastoid cavity obliteration with preservation of hearing. Patients with horizontal canal fistulas in a canal wall down cavity can be managed with mastoid obliteration for relief of dizziness.

Endolymphatic sac tumour (ELST) is a rare low-grade locally aggressive neoplasm arising from the endolymphatic duct or sac. It presents mostly with vestibulo-cochlear symptoms either sporadically or as part of von Hippel–Lindau (VHL) syndrome. Micro-neurosurgical excision remains the cornerstone of therapy with the role of radiotherapy (RT) being controversial. This is a clinico-pathological analysis of consecutive ELST patients presenting to a single-institution in India. Neuropathology database of a tertiary-care comprehensive cancer centre was searched electronically to identify consecutive patients with histopathological diagnosis of ELST registered at the institute over last one decade. Data regarding demographic profile, clinical presentation, histopathological features, treatment details and outcomes were retrieved from electronic medical records for this retrospective analysis. Electronic search identified seven unique patients with biopsy-proven ELST registered at the institute between 2009 and 2020. Median age of the study cohort was 39 years (range 24–65 years) with strong male predilection (5:2 ratio) and left-sided preponderance (71%). Most common presenting symptoms were hearing loss (86%) and earache (71%) on affected side followed by headache (43%). All patients underwent maximal safe resection at initial diagnosis and were followed-up closely with periodic surveillance imaging. Two patients underwent salvage RT using high-precision conformal techniques at recurrence/progression. ELST is a rare low-grade locally aggressive neoplasm that arises generally as part of VHL syndrome or sometimes sporadically. Gross total resection provides the best chance of cure with RT being reserved for unresectable disease, large residue, medical inoperability, or as salvage therapy for recurrent/progressive tumor.

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.

Acute unilateral audiovestibulopathy is often caused by inflammatory disorders involving the labyrinth.

Intratympanic (IT) therapies have been explored to address several side effects that could be caused by systemic administration of steroids to treat inner ear diseases. For effective drug delivery to the inner ear, an IT delivery system was developed using poly(lactic-co-glycolic acid) (PLGA) nanoparticles (NPs) and thermosensitive gels to maintain sustained release. Dexamethasone (DEX) was used as a model drug. The size and zeta potential of PLGA NPs and the gelation time of the thermosensitive gel were measured. In vitro drug release was studied using a Franz diffusion cell. Cytotoxicity of the formulations was investigated using SK-MEL-31 cells. Inflammatory responses were evaluated by histological observation of spiral ganglion cells and stria vascularis in the mouse cochlea 24 h after IT administration. In addition, the biodistribution of the formulations in mouse ears was observed by fluorescence imaging using coumarin-6. DEX-NPs showed a particle size of 150.0 ± 3.2 nm in diameter and a zeta potential of −18.7 ± 0.6. The DEX-NP-gel showed a gelation time of approximately 64 s at 37 °C and presented a similar release profile and cytotoxicity as that for DEX-NP. Furthermore, no significant inflammatory response was observed after IT administration. Fluorescence imaging results suggested that DEX-NP-gel sustained release compared to the other formulations. In conclusion, the PLGA NP-loaded thermosensitive gel may be a potential drug delivery system for the inner ear.

Congenital hearing loss is a common disorder worldwide. Heterogeneous gene variation accounts for approximately 20-25% of such patients. We investigated a five-generation Chinese family with autosomal-dominant nonsyndromic sensorineural hearing loss (SNHL). No wave was detected in the pure-tone audiometry, and the auditory brainstem response was absent in all patients. Computed tomography of the patients, as well as of two sporadic SNHL cases, showed bilateral inner ear anomaly, cochlear maldevelopment, absence of the osseous spiral lamina, and an enlarged vestibular aqueduct. Such findings were absent in nonaffected persons. We used linkage analysis and exome sequencing and uncovered a heterozygous missense mutation in the PI4KB gene (p.Gln121Arg) encoding phosphatidylinositol 4-kinase β (PI4KB) from the patients in this family. In addition, 3 missense PI4KB (p.Val434Gly, p.Glu667Lys, and p.Met739Arg) mutations were identified in five patients with nonsyndromic SNHL from 57 sporadic cases. No such mutations were present within 600 Chinese controls, the 1000 genome project, gnomAD, or similar databases. Depleting pi4kb mRNA expression in zebrafish caused inner ear abnormalities and audiosensory impairment, mimicking the patient phenotypes. Moreover, overexpression of 4 human missense PI4KB mutant mRNAs in zebrafish embryos resulted in impaired hearing function, suggesting dominant-negative effects. Taken together, our results reveal that PI4KB mutations can cause SNHL and inner ear malformation. PI4KB should be included in neonatal deafness screening.

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 is0.75 mm.Investigate the prevalence of radiographic cochlear basal turn patency.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.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.Radiographic patency of the cochlear basal turn may be present in patients with hearing loss and normal hearing, but patency0.75 mm (i.e. risk for PLG) is rare.

To quantitatively analyze corrective saccade (CS) gain and further characterize the specific relationship between vestibulo-ocular reflex (VOR) gain and CS gain in patients with vestibular loss and healthy controls.Prospective combined with retrospective study.Affiliated Sixth People's Hospital, Shanghai Jiao Tong University.Forty patients with unilateral vestibular loss and 40 participants with normal vestibular function were subjected to video head impulse testing (vHIT). The analysis of the horizontal semicircular canal VOR and CS gains was based on individual head impulses.The patient group had significantly higher CS gain and lower VOR gain than the control group (In healthy participants, CS was not correlated with VOR gain, suggesting that CS is not due to VOR hypofunction. In patients with unilateral vestibular loss, CS was closely associated with VOR gain and can almost correct gaze position errors required for visual stabilization. CS gain could be an important indicator to diagnose vestibular loss and help physicians identify abnormal vHIT curves caused by artifacts and irregular practices.

ObjectiveThe diagnostic value of exploratory tympanotomy in sudden sensorineural hearing loss remains controversial. This study and review were performed to identify the incidence of perilymphatic fistula in patients with sudden sensorineural hearing loss. The effectiveness of tympanotomy for sealing of the cochlear windows in cases with perilymphatic fistula was evaluated.MethodsA search in common databases was performed. Overall, 5034 studies were retrieved. Further, a retrospective analysis on 90 patients was performed.ResultsEight publications dealing with tympanotomy in patients with sudden sensorineural hearing loss were identified. In 90 patients diagnosed with sudden sensorineural hearing loss and undergoing exploratory tympanotomy, 10 patients (11 per cent) were identified with a perilymphatic fistula, and this corresponds to the results obtained from our review (13.6 per cent).ConclusionThere was no significant improvement after exploratory tympanotomy and sealing of the membranes for patients with a definite perilymphatic fistula.

A 69-year-old man presented to the Emergency Department with 1 day of acute spontaneous vertigo, left-sided tinnitus and hearing loss. He had no otalgia, previous vertigo or hearing problems but did have a background of diabetes and hypertension. On examination, with fixation, there was no nystagmus, but without visual fixation (using Frenzel’s goggles), there was primary position left-beating nystagmus. The horizontal head-impulse test was negative, but his left Dix-Hallpike positional test was positive, provoking a paroxysm of up-beating, torsional nystagmus with vertigo. Otoscopy was normal with no vesicles. Weber’s test was positive to the right and Rinne’s was bilaterally negative. Smooth pursuit, saccades and cerebellar examinations were normal. Audiometry identified a left-sided profound and high-frequency and moderate-to-severe mid-frequency sensorineural hearing loss; right-ear audiometry identified only a mild high-frequency sensorineural hearing loss (figure 1A). He had acoustic reflexes in both ears at normal thresholds. Figure 1 (A) Asymmetric audiogram at initial presentation. Right ear shows a mild high-frequency sensorineural hearing loss, while the left ear shows a sloping mild-to-profound sensorineural hearing loss. (B) Left vestibular loss. Three-dimensional video head impulse at initial presentation shows isolated reduced left posterior semicircular canal vestibulo-ocular reflex gain (0.19) with catch-up saccades. (Gain values are listed to the top right of each individual head-impulse test.) (C) Repeat audiometry at 2 months. In comparison with the original audiogram, the right-ear results showed new upsloping severe-to-mild sensorineural hearing loss below 1 kHz and mild hearing loss above 1 kHz. The left-sided hearing has returned to normal at the low-to-mid frequencies after corticosteroid treatment. (D) Home audiometry over 45 days showing marked fluctuations in the right ear across the low-to-mid frequencies (≤1 kHz, red and orange traces). (E) The left ear shows little fluctuation in hearing loss. (F) Timeline of treatment. There was marked response in the left hearing loss, as …

Background Superior semicircular canal dehiscence (SSCD) is a rare inner ear disorder; currently, it is unknown whether the etiopathology underlying this structural irregularity affects neighboring structures. The goal is to investigate the prevalence of bone thinning in areas of the middle cranial fossa (MCF) floor in SSCD and non-SSCD patients. Methods This retrospective study analyzed 100 patients from March 2011 to June 2017 at a tertiary referral center. 100 patients undergoing 118 SSCD repair surgeries (18 bilateral) were identified. 12 SSCD ears were excluded due to lack of pre-operative computed tomography (CT) scans or history of prior SSCD repair at an outside facility. Non-SSCD ears were identified from routinely-obtained CT scans for temporal bone fracture (fractured sides excluded) for a total of 101 ears; 26 non-SSCD ears were excluded due to lack of high-resolution imaging. Results Univariate analyses reveal that SSCD diagnosis is associated with higher rates of geniculate ganglion (GG) dehiscence compared with non-SSCD controls (42.7 vs. 24%; χ 2 (1) = 9.69, P = 0.008). Individuals with SSCD depicted significantly thinner bone overlying the geniculate ganglion (GG) (0.23 ± 1.2 mm) compared to controls (0.28 ± 1.8 mm, (t(1 6 4)) = 2.1, P = 0.04). SSCD patients presented thinner bone overlying the internal auditory canal (IAC) (0.33 ± 1.3 mm) compared to patients without SSCD (0.46 ± 1.6 mm, (t(2 5 7) = 6.4, P Conclusions The increased prevalence of dehiscence of the MCF in this cohort of SSCD patients compared to non-SSCD patients suggests that the etiology underlying SSCD affects surrounding structures.

Labyrinthine fistula is the most common complication of middle ear cholesteatoma.To explore the postoperative hearing changes and surgical results of repairing middle ear cholesteatoma with labyrinthine fistula using the 'sandwich technique'.We retrospectively studied the clinical data of 36 patients (36 ears) who underwent surgical treatment for cholesteatoma with labyrinthine fistula. All patients were treated by completely removing the cholesteatoma matrix and repairing the fistula using the 'sandwich technique'. The hearing, clinical features, radiological data, intraoperative findings, and surgical results were respectively analyzed.Most labyrinthine fistulas were located in the lateral semicircular canal (94%). Fifty percent of fistulas were of medium size. Based on Dornhoffer classification, 17 cases of labyrinthine fistulas were classified as I. In 34 patients, the average bone conduction threshold improved or did not change after surgery. Two patients had preoperative facial paralysis. During follow-up (3-60 months), all patients had no postoperative vertigo symptoms and disease recurrence.Following the removal of the cholesteatoma matrix, the fistula is repaired using the "sandwich technique", which preserves or increases hearing and achieves an anti-vertiginous effect.This finding suggests that, 'sandwich technique' is a feasible procedure to treat cholesteatoma with labyrinthine fistula.

INTRODUCTION Ocular vestibular evoked myogenic potential (oVEMP) is a diagnostic test employed in the evaluation of superior semicircular canal dehiscence (SSCD) syndrome. Previous work showed that the presence of the n10 component of oVEMP at 4000 Hz was diagnostic of SSCD with perfect sensitivity and specificity of 1.0 in a series of 22 patients. This study sought to validate the diagnostic accuracy of high-frequency oVEMP with comparison to 500 Hz oVEMP and cervical vestibular evoked myogenic potential (cVEMP) in a larger series of patients. METHODS Retrospective chart review of 171 patients with clinical symptoms consistent with SSCD who underwent oVEMP and cVEMP testing. Dehiscence of the superior semicircular canal (SCC) on high-resolution computed tomography (CT) imaging of the temporal bone was used to identify cases of likely SSCD. The presence or absence of 4000 Hz oVEMP n10 responses, increased amplitude of 500 Hz oVEMP responses, and reduced threshold of 500 Hz cVEMP responses were identified for each patient. RESULTS SCC dehiscence was identified by CT imaging in 48 of 171 patients with symptoms consistent with SSCD. High-frequency oVEMP testing at 4000 Hz elicited a n10 response in 40 of 48 (83.3%) of patients and was present in 48 of 171 (28.1%) patients overall. The corresponding sensitivity was 0.83, specificity was 0.93, positive predictive value was 0.83, and negative predictive value was 0.93. oVEMP and cVEMP testing at 500 Hz was less accurate with sensitivity of 0.62 and 0.64, respectively, and specificity of 0.73 and 0.73, respectively. CONCLUSION The presence of a 4000 Hz oVEMP n10 response was predictive of SSC dehiscence on CT imaging among patients with symptoms consistent with SSCD with sensitivity of 0.83, specificity of 0.93, positive predictive value of 0.83, and negative predictive value of 0.93. A negative finding strongly rules out SSCD. High-frequency oVEMP was more accurate than 500 Hz oVEMP or cVEMP.

Recurrent dizziness, vertigo, and imbalance, as well as nausea or vomiting, can be induced by central or peripheral vestibular pathology. Vestibular migraine (VM) is a central pathology in which migraines reciprocally interact with vestibular nuclei. Vestibular neuritis and labyrinthitis (VN/L) are peripheral diseases involving inflammation of structures in the vestibular nerve or labyrinth. Because VM and VN/L in early stages can produce similar symptoms, diagnosis may require prolonged clinical evaluation. It has been suggested that differences in a patient's Vestibulo-Ocular Reflex (VOR) measured during the rotary chair step rotation test (RCS Test) can differentiate VM from peripheral pathologies, allowing for faster diagnosis. We sought to compare VOR time constants (VORTRetrospective chart review of patients seen at a hospital balance center between January 2010 and June 2019.Tertiary referral center.68 patients (mean age = 58.15, 65 % female, 35 % male) were placed into two groups based on clinical diagnosis codes: 1) VM or 2) VN/L.Primary variable of VORTPatients in the VM group demonstrated statistically significant elevated VOR TOur results support the conclusion that elevated VOR T

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.

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. 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. 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.

We investigated whether cholesteatoma is associated with sensorineural hearing loss (SNHL) and the effects of a lateral semicircular canal (LSCC) fistula, destruction of stapes, localization of cholesteatoma, and air–bone gap (ABG) size on SNHL. The charts of 159 patients who had received surgery for unilateral cholesteatoma were examined retrospectively. In all patients, air conduction and bone conduction (BC) thresholds in both ears were measured at 500, 1000, 2000, and 4000 Hz. Differences in BC thresholds between ears with cholesteatoma and contralateral ears were calculated. Demographics, localization of cholesteatoma, presence of LSCC fistula, condition of stapes, and ABG size on the affected ear were evaluated. There were significantly greater BC thresholds in ears with cholesteatoma than in normal ears for each frequency. Comparing the average BC differences at the different cholesteatoma locations, there were significant differences between the tympanic cavity + all mastoid cell group and attic, attic + antrum, and tympanic cavity + antrum groups. The BC differences at 4000 Hz were significantly high in patients with LSCC fistula. There were no significant relationships between the condition of the stapes and BC differences at any frequencies. There were significant correlations between average ABG and BC threshold differences at all frequencies. A significant relationship was found between cholesteatoma and SNHL. Patients with advanced cholesteatoma had significantly higher levels of SNHL. The BC threshold differences increased with increases in the ABG.

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.

Posterior semicircular canal dehiscence is a rare condition and can cause a variety of symptoms. We report three cases of children between 5 and 12 years of age with a PSCD. They all presented with different complaints as follows: Tullio phenomenon in the first case, conductive hearing loss in the second and, conductive hearing loss and pulsatile tinnitus in the third. Imaging showed in all cases a PSCD on the right side, caused by a prominent jugular bulb (high riding bulb). We describe the clinical, audiometric and radiological findings, and discuss the management and therapy. A conservative “wait and see” approach is recommended, especially with children, because of the possible complications of surgery and the possibility that the symptoms will lessen with the skull base osseous maturation.

Aim To report the effect of oval and round window reinforcement surgery performed in two patients with the Tullio phenomenon. Case 1: A male with bilateral superior canal dehiscence syndrome. Downbeat nystagmus with leftward horizontal eye movement was recorded in an electronystagmogram using a pure-tone sound of 110 dB at 2000 or 4000 Hz in the right ear. Case 2: A female who had undergone stapes surgery. Computed tomography revealed an ossicular prosthesis in the vestibule. An audiogram indicated mild to moderate hearing impairment in the right ear. Leftward horizontal nystagmus was recorded in an electronystagmogram using a pure-tone sound of 110 dB at 500 or 1000 Hz in the right ear. Surgical findings indicated that the prosthesis was inserted deep into the oval window, which was closed with thin connective tissue. Outcomes After oval and round window reinforcement surgery was performed in the right ear, and loud, pure-tone sounds elicited neither nystagmus nor dizziness in either patient.

Circulating microRNAs (miRNAs) have been suggested as non‑invasive biomarkers for the diagnosis of several autoimmune diseases. However, to the best of our knowledge, no studies have yet examined the miRNA expression profiles in autoimmune inner ear disease (AIED). The present study aimed to use an miRNA sequencing assay to detect the miRNA expression profiles of serum samples from 3 control mice and 3 antigen‑induced AIED model mice. Differentially expressed miRNAs (DE‑miRNAs) were screened using a t‑test. miRNA target prediction was performed using TargetScan Mouse. Then, the miRNA‑target gene interaction network was constructed and visualized using Cytoscape software. The underlying functions of the target genes of the DE‑miRNAs were predicted using the clusterProfiler package. As a result, 22 miRNAs were identified as DE‑miRNAs between AIED and control mice, including 10 upregulated and 12 downregulated genes. Based on the TargetScan Mouse prediction, 1,958 genes were identified as the targets for the 22 DE‑miRNAs. Functional analysis indicated that only the target genes of 8 miRNAs were respectively enriched for Gene Ontology terms and Kyoto Encyclopedia of Genes and Genomes pathways, among which miR‑10b‑3p, let‑7j and miR‑8112 were shared between the two pathway analyses. These 3 miRNAs may be involved in AIED by affecting inflammatory chemokine (miR‑10b‑3p‑C‑C motif chemokine 12), Wnt signaling (miR‑8112‑Wnt9b/Wnt 3a/Wnt2b) and Mucin type O‑glycan biosynthesis pathways (let‑7j‑Galnt2/Galnt12). In conclusion, miR‑10b‑3p, miR‑8112 and let‑7j may be underlying biomarkers for diagnosing AIED.

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.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.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.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.There was no significant change in bone thickness with advancing age. CBCT is the best method of assessing SSCD.

To describe a series of patients with iatrogenic inner ear dehiscence after lateral skull base surgery and discuss treatment options.Three patients with history of recent lateral skull base surgery, symptoms consistent with inner ear dehiscence, and radiographically proven bony dehiscence on postoperative imaging.All patients were initially managed conservatively with serial outpatient visits. Two patients with large cochlear and vestibular dehiscences had round window reinforcement procedures. One patient had transmastoid resurfacing for repair of an iatrogenic posterior semicircular canal dehiscence.Anatomical location of dehiscences; treatment options; subjective auditory and vestibular symptoms pre-dehiscence, post-dehiscence and after dehiscence repair; pre- and post-audiogram when available.Patient ages were 46, 52, and 60 with two of three being women. None of the patients had subjective auditory or vestibular symptoms of inner ear dehiscence before initial skull base surgery, but they all had development of these symptoms afterwards. All patients were initially managed conservatively, but all ultimately required a surgical procedure. The two patients who elected for round window reinforcements, and the one patient who required transmastoid resurfacing, had significant improvement of symptoms.Iatrogenic inner ear dehiscence after skull base surgery is best dealt with and repaired intraoperatively. Should intraoperative repair not be possible, transcanal round window reinforcement is a minimally invasive option for medial otic capsule dehiscence, although long-term outcomes are unclear. For lateral otic capsule dehiscence, a transmastoid approach is recommended.IV.

Objective To analyze surgical results on hearing and vestibular symptoms in patients with barotraumatic perilymphatic fistula (PLF) according to diagnostic criteria. Methods A total of 39 patients (41 ears) who underwent surgery on suspicion of barotraumatic PLF from January 2005 to December 2017 were included. Pure tone audiometry and videonystagmography (VNG) recording for spontaneous nystagmus and positional tests were performed preoperatively and postoperatively at 1 week and 1 month. Surgical outcomes were analyzed based on hearing results, subjective dizziness, and change of nystagmus. Results Preoperative hearing level was 75.5 ± 28.7 dB for definite PLF and 88.5 ± 22.8 dB for probable PLF, and levels were not significantly different between groups. Preoperatively, subjective dizziness was present in 18 (94.7%) and 19 (95%) in each group. Among 39 patients, 24 had VNG recordings. Positional nystagmus was recorded in 87.5% (7/8) and 87.5% (14/16) of the definite and probable PLF groups, respectively. Postoperatively, hearing was improved in 65% (13/20 ears) of definite PLF and 61.9% (13/21 ears) of probable PLF. There was no significant difference between the two groups. Hearing gain was significantly correlated with the time interval between symptom onset and surgical timing. Subjective dizziness was improved immediately after surgical repair in 97.4% (17/18) of definite PLF patients and 100% (19/19) of probable PLF patients. Even though dizziness was improved in most patients, some had persistent positional nystagmus and recurrent dizziness. Conclusions Surgical sealing of both windows in suspected barotraumatic PLF is an effective treatment to improve hearing and subjective dizziness.

OBJECTIVE To evaluate the association between mastoid encephalocele or cerebrospinal fluid (CSF) otorrhea and concurrent superior semicircular canal dehiscence (SSCD). STUDY DESIGN Retrospective case-control study with chart and imaging review. SETTING University-affiliated tertiary referral center. PATIENTS A chart review was conducted of all patients greater than 18 years of age who had primary surgery for CSF otorrhea or encephalocele at our university-affiliated tertiary center from 2000 to 2016. Eighty-three patients matched inclusion criteria for case subjects, and 100 patients without CSF otorrhea or encephalocele were included for controls. MAIN OUTCOME MEASURE High-resolution computed tomography (CT) scans were reviewed to assess superior semicircular canal roof integrity. Student's t test was used to determine significance of continuous variables. Odds ratio (OR) and χ test was used to determine the association between SSCD and concurrent mastoid encephalocele or CSF otorrhea compared with the control population. RESULTS The mean age of the 83 case subjects was 54 years, and 73% were women. In patients with confirmed encephalocele and CSF otorrhea, the prevalence of SSCD was 35%, which was significantly greater than controls (2%) (OR = 26.1, p

To investigate the clinical validity of the mathematical measured VVOR test results to detect horizontal semicircular canal hypofunction.Prospective, nonrandomized, observational study.Tertiary referral center, hospital.Consecutive patients on first time visit to otoneurology unit.Diagnostic.Values of statistical indicators of clinical validity for VVOR test to detect horizontal canal hypofunction were used considering vHIT horizontal aVOR gain values as gold standard.Area under curve of ROC curve for quantified VVOR testing was 0.92. Head movement frequency on VVOR test and contralateral VVOR gain were identified as significant influence factors of (unilateral) VVOR gain (p 0.0001) on statistical linear model.According to the obtained results, the quantified VVOR test has excellent clinical validity for detecting angular horizontal VOR hypofunction.

Objective The purpose of this study was to investigate the development of speech skills in young children with Mondini dysplasia and age-matched deaf children with radiologically normal inner ears over a period of 5 years after cochlear implantation (CI). Methods In total, 700 congenitally severely to profoundly deaf children (281 girls and 419 boys) participated in this study. All of the participants had undergone unilateral CI surgery before 36 months of age. The participants were categorized into two groups based on the absence or presence of Mondini dysplasia in the implanted ear, as assessed via high-resolution, thin-slice computerized tomography or magnetic resonance imaging: group A comprised 592 children with radiologically normal inner ears and group B comprised 108 children with Mondini dysplasia. The Meaningful Use of Speech Scale (MUSS) and Speech Intelligibility Rating (SIR) were used to evaluate the speech performance of all young children at various time points: pre-surgery and at 1, 3, 6, 12, 24, 36, 48, and 60 months after switch-on programming. Results The mean scores of SIR and MUSS in children from both group A and group B showed significant improvements over time. No significant differences were found in the mean scores of SIR between the two groups at any time interval during the 5-year follow-up. The mean score of MUSS was significantly different between group A and group B at 12, 24, and 36 months after implantation, whereas no obvious differences were noted pre-surgery, and at 1, 3, 6, 48, and 60 months post-operation. Conclusions Young children with Mondini dysplasia develop their speech skills at a fast rate and achieve similar speech acquisition compared to age-matched children with radiologically normal inner ears 5 years post-operation. Therefore, CI is an effective intervention method for young children with Mondini dysplasia.

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.

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.

ObjectiveTo characterise subjective symptoms in patients undergoing surgical repair of superior semicircular canal dehiscence.MethodsQuestionnaires assessing symptom severity and impact on function and quality of life were administered to patients before superior semicircular canal dehiscence surgery, between June 2011 and March 2016. Questionnaire sections included general quality of life, internal amplified sounds, dizziness and tinnitus, with scores of 0–100 points.ResultsTwenty-three patients completed the questionnaire before surgery. Section scores (mean±standard deviation) were: 38.2 ± 25.2 for general quality of life, 52.5 ± 23.9 for internal amplified sounds, 35.1 ± 28.8 for dizziness, 33.3 ± 30.7 for tinnitus, and 39.8 ± 22.2 for the composite score. Cronbach's α statistic averaged 0.93 (range, 0.84–0.97) across section scores, and 0.83 for the composite score.ConclusionThe Gopen–Yang Superior Semicircular Canal Dehiscence Questionnaire provides a holistic, patient-centred characterisation of superior semicircular canal dehiscence symptoms. Internal consistency analysis validated the questionnaire and provided a quantitative framework for further optimisation in the clinical setting.

Objectives: This is a qualitative study to explore the utility of gray-scale inversion or the “invert” function of high-resolution computed tomography (HRCT) scans in the diagnosis of temporal bone anatomy and pathology. Methods: This is a case series describing an innovative application of an existing image processing tool to visualize temporal bone anatomy and pathology. Illustrative patients at a tertiary referral center with otologic symptoms and findings leading to HRCT scans of the temporal bone were included. Diagnostic HRCT scans were evaluated utilizing the gray-scale inversion function (invert function). Results: Nine illustrative cases which demonstrate conditions such as persistent stapedial artery, membranous stapes footplate, total ossicular prosthesis migration into the vestibule, third window syndrome such as superior semicircular canal dehiscence (SSCD) and cochlea-facial nerve dehiscence, otosclerosis, and ossicular chain discontinuity are included. The enhanced visualization was confirmed surgically in 3 cases, and 1 had physiological confirmation using cervical vestibular evoked myogenic potentials (cVEMP). Conclusions: Gray-scale inversion can be used to improve visualization of temporal bone anatomy and pathologic changes when diagnoses are in doubt. The invert function is a useful adjunct in the armamentarium of both radiologists and otologists when evaluating HRCT of the temporal bone.

Purpose Intralabyrinthine schwannomas (ILS) are rare, benign, slow-growing tumors arising from schwann cells of the cochlear or vestibular nerves within the bony labyrinth. This study provides insight into the management of this rare tumor through a large case series. Materials and methods After Institutional Review Board approval, a retrospective chart review was performed of all ILS patients treated at our institution between 2007 and 2019. Results 20 patients (9 male, 11 female) with ILS were managed at our institution. The right ear was affected in 9 patients (45%) and the left in 11 (55%). Subjective hearing loss was endorsed by all 20 patients. Average pure tone average at presentation was 72 dB nHL. Nine tumors (45%) were intravestibular, 6 (30%) were intracochlear, 4 (20%) were transmodiolar and 1 (5%) was intravestibulocochlear. Hearings aids were used in 3 patients (15%), BiCROS in 2 (10%), CI in 2 (10%), and bone conduction implant in 1 (5%). Vestibular rehabilitation was pursued in 5 patients. Surgical excision was performed for one patient (5%) via translabyrinthine approach due to intractable vertigo. No patients received radiotherapy or intratympanic gentamicin injections. Conclusion ILS presents a diagnostic and management challenge given the similarity of symptoms with other disorders and limited treatment options. Hearing loss may be managed on a case-by-case basis according to patient symptoms while vestibular loss may be mitigated with vestibular therapy. Surgical excision may be considered in patients with intractable vertigo, severe hearing loss with concurrent CI placement, or in other case-by-case situations.

Purpose Middle ear disease is increasingly being managed via transcanal endoscopic ear surgery (TEES). A limitation of TEES is that it restricts the surgeon to single-handed dissection. One solution to this would be an endoscope holder to facilitate two-handed dissection. Current endoscope holders are stationary, and can cause potential damage from endoscope contact with the ossicles or ear canal if unintended head motion occurs from inadequate anesthetic. A dynamic device that could detect and react to patient motion would mitigate these concerns, but currently there is little formal characterization of the frequency, velocity and acceleration of unintended patient head motion during otologic procedures performed under general anesthesia. The present study aims to characterize intraoperative patient head motion kinematics during cases utilizing TEES. Materials and methods This is a prospective study of adults undergoing otologic procedures performed with general anesthesia and without paralysis. Head motion was characterized using a nine-axis inertial measurement unit (IMU), (LPMS-B2, Life Performance Research) mounted to each patient's forehead for the procedure duration. Results Data was collected across 10 cases; 50% of patients were female and mean age was 50 ± 14 years. There was observed patient head motion in 40% of cases with maximum linear acceleration of 0.75 m/s2 and angular velocity of 12.50 degrees/s. Conclusions Patient movement during otologic procedures was commonly observed, demonstrating the need for a dynamic holder to allow two-handed TEES. Results from this study are the first objective characterization of patient head motion kinematics during otologic procedures performed under general anesthesia.

Objectives We investigated the incidence and characteristics of pseudo-spontaneous nystagmus (PSN) in benign paroxysmal positional vertigo involving the lateral semicircular canal (LC-BPPV) and evaluated the correlation between PSN and the bow and lean test. Methods We examined nystagmus in the sitting position using video-oculography goggles in 131 LC-BPPV patients. The positioning test and bow and lean test were also performed. Patients were divided into canalolithiasis and cupulolithiasis groups according to the character of nystagmus. In each group, the incidence and direction of PSN, correlation with the bow and lean test, and treatment outcome were analyzed. Results PSN was observed in 25 cases (19.1%) in LC-BPPV patients, 7 of which were canalolithiasis and 18 of which were cupulolithiasis (p = 0.098). Of the 25 patients with PSN, 21 (84%) exhibited nystagmus consistent with the lean test whereas 4 (16%) exhibited nystagmus consistent with the bow test. In patients with PSN, nystagmus was observed in the bow and lean test in all cases (23/23), but in patients without PSN, no nystagmus was observed in 13 cases (13/87) in the bow and lean test (p = 0.048). The number of barbecue maneuvers performed until the end of treatment was 1.4 ± 0.7 in patients with PSN and 1.4 ± 0.9 in those without PSN (p = 0.976). Conclusion We identified PSN in patients with LC-BPPV irrelevant of subtype. Moreover, all patients with PSN showed nystagmus in the bow and lean test. The direction of PSN was mostly consistent with that of the lean test (21/25, 84%). The presence of PSN was not related to the treatment outcome in this study.

OBJECTIVES/HYPOTHESIS Internal auditory diverticula in adults have been found to exist independent of otosclerosis, and in the presence of otosclerosis. We sought to determine the prevalence of internal auditory canal (IAC) diverticula in a pediatric cohort, to assess whether IAC diverticula are a risk factor for hearing loss, and the co-occurrence of otic capsule hypoattenuation. STUDY DESIGN Retrospective review. METHODS A single-site retrospective review of high-resolution temporal bones computed tomography (CT) scans including the presence and size of diverticula and hypoattenuation of the otic capsule. Demographic, imaging, and audiometric data were collected and descriptively analyzed. Bivariate analysis of collected variables was conducted. Comparisons between sides in unilateral cases were also performed. RESULTS 16/600 (2.7%; 95% CI [2.0%, 3.4%]) were found to have IAC diverticula. Six were bilateral. Thirty-one patients (5.2%) were found to have hypoattenuation of the otic capsule. There were no coincident cases of IAC diverticulum and hypoattenuation of the otic capsule. There was no association between the presence of IAC diverticula and age (P = .13). In six patients with unilateral diverticula, pure tone average (P = .42), and word recognition (P = .27) scores were not significantly different when compared to the normal, contralateral side. CONCLUSIONS The prevalence of IAC diverticula in children is lower than the prevalence in adults. IAC diverticula in children likely represent congenital variants of temporal bone anatomy. Similar to adult populations, there is evidence that IAC diverticula in children are likely not an independent risk factor for hearing loss. LEVEL OF EVIDENCE 4 Laryngoscope, 131:E1683-E1687, 2021.

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.Retrospective study.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.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.Results demonstrated that a length3.65 mm and an angle71.6° 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.4 Laryngoscope, 131:E1301-E1307, 2021.

Objective:The aim of this study was to determine the incidence of inner ear involvement in various forms of acute otitis media (AOM) in ear, nose, and throat (ENT) practices in Germany.Methods:Patients who had been diagnosed with various forms of AOM in the years 2010 to 2017 were enrolled in the study from a nationwide, representative practice database (Disease Analyzer, IQVIA). In these patients, the incidence of simultaneous or subsequent inner ear disorders (IED) was determined within 7 days and within 12 months from the date of an AOM diagnosis.Results:A total of 286 186 patients with AOM were enrolled. The most frequent diagnoses were “nonsuppurative otitis media, unspecified” (47.6%) and “otitis media, unspecified” (39.0%). The diagnoses of hemorrhagic bullous myringitis (BM) or influenza-induced AOM were very rarely found in the database. The highest incidence of IED after 7 days and 12 months was found in “nonsuppurative otitis media, unspecified” (7.7% and 15.9%, respectively), followed by “otitis media, unspecified” (5.6% and 13.5%, respectively). The incidences of the most frequent IED “hearing loss, unspecified” and “sensorineural hearing loss, unspecified” increased proportionally with increasing patient age, while the rare diagnoses of “labyrinthitis” and “ototoxic hearing loss” were evenly distributed among the age groups.Conclusion:In ENT practices in Germany, both various forms of AOM, as well as simultaneous or subsequent IED, are mostly being coded in an unspecific way, while specific forms such as hemorrhagic BM, influenza-induced AOM, and labyrinthitis are coded very rarely. Older patients have a higher risk of IED in AOM. A visit due to AOM seems to be a regular occasion for the initial diagnosis of hearing impairment in the elderly individuals. The highest risk of IED was found in nonsuppurative AOM.

ObjectiveTo determine the prevalence and distribution of inner-ear malformations in congenital single-sided deafness cases, as details of malformation type are crucial for disease prognosis and management.MethodsA retrospective study was conducted of 90 patients aged under 16 years with congenital single-sided deafness. Radiological findings were evaluated using computed tomography and magnetic resonance imaging. Inner-ear malformations were identified and cochlear nerve status was determined in affected ears.ResultsOut of 90 ears, 42 (46.7 per cent) were found to have inner-ear malformation. Isolated cochlear aperture stenosis was the most common anomaly (n = 18, 20 per cent), followed by isolated cochlear aperture atresia (n = 11, 12.2 per cent) and cochlear hypoplasia (n = 7, 7.8 per cent). Cochlear nerve deficiency was encountered in 41 ears (45.6 per cent). The internal auditory canal was also stenotic in 49 ears (54.4 per cent).ConclusionInner-ear malformations, especially cochlear aperture anomalies, are involved in the aetiology of single-sided deafness more than expected. The cause of single-sided deafness differs greatly between congenital and adult-onset cases. All children with single-sided deafness should undergo radiological evaluation, as the prognosis and management, as well as the aetiology, may be significantly influenced by inner-ear malformation type.

BACKGROUND AND PURPOSE: Internal auditory canal diverticula are focal lucencies along the anterior-inferior aspect of the internal auditory canal fundus. Studies in adults report conflicting data on the etiology and clinical relevance of this finding. We would expect a pediatric study to help elucidate the significance of internal auditory canal diverticula. The primary goals of this study were to determine the temporal bone CT prevalence of diverticula among pediatric patients and to assess possible hearing loss and anatomic associations. MATERIALS AND METHODS: For this retrospective study including 283 pediatric temporal bone CTs, 4 neuroradiologists independently assessed for diverticula. Discrepancies were resolved by consensus. One neuroradiologist assessed for an enlarged vestibular aqueduct, labyrinthine dysplasia, cochlear cleft, and otospongiosis. Patient demographics, audiologic data, and pertinent clinical history were recorded. One-way analysis of variance and the Fisher exact test were used to assess possible associations between diverticula and specific patient characteristics. RESULTS: Diverticula were observed in 42/283 patients (14.8%) and were more commonly bilateral. There was no significant association with age, sex, hearing loss, enlarged vestibular aqueduct, labyrinthine dysplasia, or cochlear cleft. A statistically significant association was observed with otospongiosis (P = .013), though only 1 study patient had this disease. CONCLUSIONS: Internal auditory canal diverticula are a common finding on pediatric temporal bone CT. In the absence of clinical or imaging evidence for otospongiosis, diverticula likely fall within the range of a normal anatomic variation. Familiarity with these findings may prevent neuroradiologists from recommending unnecessary additional testing in pediatric patients with isolated internal auditory canal diverticula.

Number: 4 PMID: 32040717; 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.

Objectives To analyze the clinical characteristics of cochlear fistulas (CFs) and propose a new fistula classification system with regard to the cochlea. Materials and methods A retrospective chart review was conducted between January 2008 and December 2015 to identify patients who had undergone surgery for cholesteatoma with an associated CF. The following data were collected: preoperative symptoms, findings of temporal bone computed tomography (TBCT), fistula stage, cholesteatoma classification, surgical technique, and pre- and postoperative pure-tone audiometry. Results We analyzed a total of 159 patients, out of which 9 (5.7%) were diagnosed with a CF. The average duration of the chronic otitis media was 19.8 years. Cholesteatomas that induced CF rarely existed in the nonaggressive state; recurrent otorrhea was observed in all but one of our subjects. All the patients with CF had a distinct origin of cholesteatoma that developed from the retraction of posterior pars tensa; further, 88.9% cholesteatomas extended to and filled the sinus tympani. Preoperative audiometry revealed total hearing loss in 4 (44.4%) patients. Further, five patients with residual hearing before surgery had stage I fistulas, and the bone conduction thresholds remained stable after surgery. Conclusion Cochlear fistulas were often detected in patients with (1) a history of chronic otitis media (exceeding 10 years), (2) frequently recurring otorrhea, and (3) pars tensa cholesteatomas that extended to the posterior mesotympanum and filled the sinus tympani. Such patients can suffer from potentially severe and irreparable sensorineural hearing loss.

Interleukin-1β (IL-1β) is a key proinflammatory cytokine involved in the progression of many autoinflammatory and autoimmune diseases, including autoimmune inner ear disease (AIED). IL-1β inhibition has been shown to result in clinical hearing improvement in a small cohort of corticosteroid-resistant patients with AIED. Canonical processing of pro-IL-1β by caspase-1 generates an active 17-kDa fragment, capable of instigating a proinflammatory microenvironment. However, in response to LPS, PBMCs from patients with AIED uniquely express a 28-kDa IL-1β fragment, as compared with PBMCs from control subjects. We synthesized and compared the biologic activity of the 28-kDa fragment to the 17-kDa IL-1β product and the pro-IL-1 31-kDa protein. The 28-kDa IL-1β fragment induces IL-6, TNF-α, and CCL3 in PBMCs. Uniquely, only caspase-7 treatment showed a dose- and time-dependent increase in 28-kDa band generation. Mass spectrometry confirmed the putative caspase-7 cleavage site of pro-IL-1β, which was used to generate the 28-kDa fragment used for PBMC stimulation studies. Collectively, these results provide insight into the function of a poorly understood, processed 28-kDa form of IL-1β in patients with AIED that is uniquely generated by caspase-7 and is capable of activating further downstream proinflammatory cytokines. Further investigation may provide novel pharmacologic targets for the treatment of this rare disease.

International audience; 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.

Air-bone gaps (ABGs) are commonly found in patients with conductive or mixed hearing loss generally due to outer- and/or middle-ear diseases such as otitis externa, tympanic membrane perforation, interruption or fixation of the ossicular chain, and chronic suppurative otitis media. ABGs can also be found in correlation with inner-ear disorders, such as endolymphatic hydrops, enlarged vestibular aqueduct syndrome, semicircular canal dehiscence, gusher syndrome, cochlear dehiscence, and Paget disease's as well cerebral vascular anomalies including dural arteriovenous fistula. The typical clinical presentation of inner-ear conditions or cerebral vascular anomalies causing ABGs includes audiological and vestibular symptoms like vertigo, oscillopsia, dizziness, imbalance, spinning sensation, pulsatile or continuous tinnitus, hyperacusis, autophony, auricular fullness, Tullio's phenomenon, and Hennebert's sign. Establishing a definitive diagnosis of the underlying condition in patients presenting with an ABG is often challenging to do and, in many patients, the condition may remain undefined. Results from an accurate clinical, audiological, and vestibular evaluation can be suggestive for the underlying condition; however, radiological assessment by computed tomography and/or magnetic resonance imaging is mandatory to confirm any diagnostic suspicion. In this review, we describe and discuss the most recent updates available regarding the clinical presentation and diagnostic workup of inner-ear conditions that may present together with ABGs.

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.

The present case was a 38-year-old male who presented with progressive hearing loss, resulting in profound bilateral hearing loss. He had a past history of childhood medulloblastoma, which was treated with posterior fossa craniotomy and radiotherapy. A ventriculoperitoneal (VP) shunt was put in place to manage the hydrocephalus. Cochlear implantation (CI) was carried out on his right ear by a standard procedure. At CI activation, the electric impedance of the electrode was very high, and computed tomography revealed that there was no area of liquid density, suggesting depletion of the perilymph in the cochlea and vestibule. Eight months later, the impedance improved gradually, and the cochlea was filled with perilymph. Consequently, one of the causes of the pneumolabyrinth in the present case was that a scarred stenotic cochlear canaliculus secondary to surgery or radiation therapy might have prevented the CSF from filling the scala. In addition, it is also possible that the VP shunt might have altered the CSF pressure, leading to depletion of the perilymph.

Objective To describe the incidence of intralabyrinthine schwannoma (ILS) utilizing the Rochester Epidemiology Project (REP), a unique medical consortium in the United States that covers a complete population of all ages in a well-defined geographic area over the past half-century. Patients All patients with ILS diagnosed between January 1, 1966 and December 31, 2016 in Olmsted County, Minnesota, identified using the REP medical records-linkage system. Main outcome measures Incidence of ILS. Results Fourteen incident cases of ILS were identified in Olmsted County since 1966-a period spanning 5.9 million person-years. Over the past decade, the incidence rate of ILS was 0.81 per 100,000 person-years and 1.1 over the last 5 years. Since 1966, the cumulative incidence rate of ILS over the past 50 years was 0.26 per 100,000 person-years. The median age at diagnosis was 60 years (interquartile ranges, 39-70). Incidence increased with age: over the last 5 years, those aged 60 to 69 exhibited an incidence rate of 2.7 per 100,000 person-years and those 70+ displayed a rate of 4.1. All patients had varying levels of asymmetrical sensorineural hearing loss with 64% of patients presenting with class D hearing. Four (29%) patients had a history of sudden sensorineural hearing loss, five (36%) reported imbalance and/or vertigo, and four (29%) reported aural fullness. Three of the 14 (21%) patients had neurofibromatosis type 2. Conclusion The incidence of ILS exceeds 1 per 100,000 person-years with modern diagnostic imaging. The rising incidence of ILS in recent years most likely reflects improved capacity for disease detection rather than a true increase in tumor development. Given the reputability of the REP consortium, these data suggest that ILS comprises a more common entity than previously considered.