Development of Air Cell System Following Canal Wall Up Mastoidectomy for Pediatric Cholesteatoma.

Background: The development of temporal bone pneumatization is related to the postnatal middle ear environment, where the development of air cells is suppressed with otitis media in early childhood. However, whether air cell formation restarts when mastoidectomy is performed during temporal bone pneumatization remains unclear. Herein, we evaluated temporal bone pneumatization after canal wall up (CWU) tympanomastoidectomy for middle ear cholesteatoma in children. Methods: In total, 63 patients, including 29 patients with congenital cholesteatoma (CC) and 34 patients with acquired cholesteatoma (AC), were assessed using a set of pre- and postoperative computed tomography images. The air cells of the temporal bone were divided into five areas: periantral (anterior), periantral (posterior), periantral (medial), peritubal, and petrous apex. The number of areas with air cells before and after surgery was compared to evaluate temporal bone pneumatization after surgery. Results: A total of 63 patients, comprising 29 with CC and 34 with AC (pars flaccida; 23, pars tensa; 7, unclassified; 4), were evaluated. The median age of patients (18 males and 11 females) with CC was 5.0 (range, 2-15 years), while that of the AC group (23 males and 11 females) was 8 (range, 2-15 years). A significant difference in air cell presence was identified in the CC and AC groups after surgery (Mann-Whitney U, p < 0.001 and p = 0.003, respectively). Between the two groups, considerably better postoperative pneumatization was observed in the CC group. A correlation between age at surgery and gain of postoperative air cell area development was identified in the CC group (Spearman's rank-order correlation coefficient, r = -0.584, p < 0.001). In comparison with the postoperative pneumatization rate of each classified area, the petrous apex area was the lowest in the CC and AC groups. Conclusions: Newly developed air cells were identified in the temporal bones after CWU mastoidectomy for pediatric cholesteatoma. These findings may justify CWU tympanomastoidectomy, at least for younger children and CC patients, who may subsequently develop air cell systems after surgery.

Superior semicircular canal dehiscence: a radiological and clinical study.

Aim: to assess radiologically the prevalence of SSCD with its clinical presentations and its relationship with age. Methods: a prospective cohort study carried out on 200 consecutive patients (400 temporal bones). Radiological evaluation was performed using High Resolution Computed Tomography (HRCT) with measurement of thickness of bone covering superior semicircular canal (SCC), height and diameter of SSC. Results: Two hundred patients (400 temporal bones) were involved. The mean thickness ± SD, the mean diameter ± SD and the mean height ± SD were 1.38 ± 0.80 mm, 0.94 ± 0.26 mm and 10.91 ± 2.39 mm respectively. The prevalence of SSCD and predehiscence were 1% and 14% respectively. The commonest symptom encountered was autophony (48.3%). When the SSC thickness, diameter and height were compared with the age of patients, statistically significant differences were detected. The highest diameter, lowest height and lowest thickness were found in patients aged from 54 to 72 years. Thickness of bony layer covering SSC was found to be the most validated measurement for differentiation between cases with positive and negative symptoms with the highest sensitivity and specificity. Conclusion: The prevalence of SSCD and predehiscence varied among the studies. Autophony is the commonest symptom usually encountered. The condition is acquired rather than congenital. The thickness of bone covering SCC is the most validated measurement in differentiation between cases with positive and negative symptoms.

Temporomandibular Joint Herniation into the External Auditory Canal.

Teaching point: Always ask a patient to open and close their mouth when you see a mass protruding into the external auditory canal, as to not miss this rare anomaly.

Computed tomography approximated superior semicircular canal dehiscence size and location and their association with clinical presentation.

OBJECTIVE: This study investigates the relationship of superior semicircular canal dehiscence (SSCD) size and location with patient symptomatology and audiometry. METHODS: We retrospectively reviewed SSCD cases presented to a tertiary institution between 2011 and 2022. Dehiscence length and width measured on high-resolution temporal bone computed tomography imaging were used to calculate an approximate dehiscence area (ADA). Dehiscence location was classified employing a six-grade system. Multivariable regression analyses were performed with symptomatology and audiogram metrics as outcome variables and ADA and dehiscence location as the primary covariates of interest. RESULTS: A total of 402 SSCD were included in the analysis. Controlling for patient age, sex, dehiscence laterality, and location, greater ADA was independently associated higher rates of autophony (aOR 1.39; 95% C.I. 1.14-1.71; P = 0.004) and hyper-amplification (aOR 1.39; 1.14-1.70; P = 0.004). Additionally, dehiscences with greater ADA exhibited significantly lower bone conduction threshold at 500 Hz (adjusted ß -1.75, P = 0.006) and 1000 Hz (adjusted ß -1.61, P = 0.018) and significantly wider air-bone gap at 500 Hz (adjusted ß 2.22, P < 0.001) and 1000 Hz (adjusted ß 1.00, P = 0.039). Dehiscence location was not independently associated with any outcome variable examined when accounting for dehiscence size. CONCLUSION: The size of dehiscence is more independently related to clinical presentations than the anatomical location of the dehiscence in the superior semicircular canal. Greater dehiscence size is associated with symptomatology and audiometry consistent with more severe bone conduction hyperacusis.

Application of UNETR for automatic cochlear segmentation in temporal bone CTs.

OBJECTIVE: To investigate the feasibility of a deep learning method based on a UNETR model for fully automatic segmentation of the cochlea in temporal bone CT images. METHODS: The normal temporal bone CTs of 77 patients were used in 3D U-Net and UNETR model automatic cochlear segmentation. Tests were performed on two types of CT datasets and cochlear deformity datasets. RESULTS: Through training the UNETR model, when batch_size=1, the Dice coefficient of the normal cochlear test set was 0.92, which was higher than that of the 3D U-Net model; on the GE 256 CT, SE-DS CT and Cochlear Deformity CT dataset tests, the Dice coefficients were 0.91, 0.93, 0 93, respectively. CONCLUSION: According to the anatomical characteristics of the temporal bone, the use of the UNETR model can achieve fully automatic segmentation of the cochlea and obtain an accuracy close to manual segmentation. This method is feasible and has high accuracy.

An Unusual Pattern of Skull Base Pneumatization.

OBJECTIVE: Our aim is to describe an unusual pattern of craniocervical pneumatization resulting in a spontaneous fracture in a patient, who presented with tinnitus. METHODS: Temporal CT was ordered in a patient with tinnitus lasting over a year. His audiogram was normal. No history of previous trauma, recent flight, diving, or weight lifting was present. Further questioning revealed his habitual Valsalva maneuvers to relieve symptoms of nasal congestion. RESULTS: Temporal CT showed abnormal pneumatization of the occipital bone and C1 vertebra. A definite cortical defect resulted with the presence of air around the neighboring soft tissues. The emphysema was most prominent in the suboccipital fossa but it was also seen in the epidural space of the spinal canal. CONCLUSION: Abnormal pneumatization of the skull base and cervical vertebra can be symptomatic due to its tendency to fracture. The presumed cause of pneumatization in this patient is habitual Valsalva maneuvers.

Otological Manifestations in Adults with Primary Ciliary Dyskinesia: A Controlled Radio-Clinical Study.

Primary ciliary dyskinesia (PCD) is a rare genetical disease characterized by an abnormal structure or function of the cilia, causing sinusitis, otitis, and bronchiectasis. Hearing loss affects 60% of PCD patients, but data are lacking concerning hearing and temporal bone imaging in adults. Our aim was to describe clinical and radiological ear disease in adults with genetically confirmed PCD. Data were recorded from January 2018 to December 2019. PCD patients were compared with controls with bronchiectasis without PCD. Clinical examination included otomicroscopy and auditory tests. A temporal bone CT scan (TBCT) was systematically performed. Seventeen patients (34 ears) were included in each group. The eardrums were abnormal in 25 (74%) PCD ears versus 8 (24%) ears in the controls (p < 0.05). Conductive hearing loss was more frequent in the PCD group (24% vs. 12% in controls). TBCT were abnormal in 94% PCD patients vs. 32% in the controls (p < 0.05). The Main CT-scan images in PCD were middle ear inflammation (65%), mastoid condensation (62%), or ossicular anomalies (35%). With its excellent sensitivity, TBCT gives typical arguments for PCD diagnosis, adding otological signs to the usual sinus CT signs (hypoplasia, aplasia). Systematic TBCT could be useful in the initial evaluation of patients with suspicion of PCD.

A novel mutation of X-linked recessive deafness gene POU3F4 in a boy with congenital deafness.

Purpose: To report an interstitial deletion of Xq21.1 in chromosome X in a boy with congenital deafness. Methods: The proband underwent a thorough physical examination and a detailed audiological and temporal bone computed tomography (CT) scan. Cochlear implantation was performed on the proband, and follow-up was conducted. High throughput sequencing and copy number analysis was made of peripheral blood samples from the proband, family members, and control subjects. Results: Sensorineural hearing loss was present in the boy and temporal bone CT scan showed a bilateral incomplete partition type III anomaly (IP-III). Q21.1 (79.40-83.32 Mb) of chromosome X in the proband had a copy number deletion with a fragment size of about 3.92 Mb. Categories of auditory performance scores and SIR scores of the cochlea in this child improved after surgery. Conclusion: Through the analysis of POU3F4, a novel mutation site with potentially pathogenic significance was found.Level of Evidence: 5.

A geometric alignment for human temporal bone CT images via lateral semicircular canals segmentation.

PURPOSE: Due to the different posture of the subject and settings of CT scanners, the CT images of the human temporal bone should be geometrically aligned with multiplanar reconstruction to ensure the symmetry of the bilateral anatomical structure. Manual alignment is a time-consuming task for radiologists and an important preprocessing step for further computer-aided CT analysis. We propose a fully automatic alignment algorithm for temporal bone CT images via lateral semicircular canals (LSCs) segmentation. METHODS: The LSCs are segmented with our proposed multifeature fusion network as anchors at first. Then, we define a standard 3D coordinate system and propose an alignment procedure. RESULTS: The experimental results show that our LSC segmentation network achieved a higher segmentation accuracy. The acceptable rate is achieved 85% over 910 raw temporal bone CT sequences. The alignment speed is reduced from 10 min by manual to 60s. CONCLUSIONS: Aiming at the problem of bilateral asymmetry in the raw temporal bone CT images, we propose an automatic geometric alignment method. Our proposed method can help to perform alignment of temporal bone CT images efficiently.

CT scan findings impact on hearing thresholds in otosclerosis: A study of 108 patients.

Background: The relationship between the location of otosclerotic zones and hearing thresholds has been evaluated in several studies and has generated different conflicting reports. This study was carried out in order to evaluate the relationship between otosclerotic zones extension on CT scan and pure tone audiometry (PTA) thresholds, before and after stapedotomy. Materials and Methods: 108 patients with a positive surgical diagnosis of otosclerosis, operated by the same surgeon, were enrolled in this retrospective study, performed in a tertiary referral hospital between 2015 and 2018. Results: PTA thresholds were significantly poorer in cases of extensive otosclerosis (peri cochlear, peri vestibular, or internal auditory canal hypodensities, p = 0,001). However, for cases with hypodensity extending to the endosteum of cochlea (Type III), we have noted a significant improvement in postoperative PTA thresholds (Mean AC (air conduction) = 32,8 ± 8,16/62,97 ± 12,28 dB), Mean BC (bone conduction) = 18,3 ± 8,56/26,25 ± 15,93 dB). Conclusions: In our study, extensive and multifocal otosclerosis lesions had a statistically significant negative impact on postoperative AC and BC threshold; however, type III lesions tend to be associated with a very good prognosis.

Fully automated segmentation in temporal bone CT with neural network: a preliminary assessment study.

BACKGROUND: Segmentation of important structures in temporal bone CT is the basis of image-guided otologic surgery. Manual segmentation of temporal bone CT is time- consuming and laborious. We assessed the feasibility and generalization ability of a proposed deep learning model for automated segmentation of critical structures in temporal bone CT scans. METHODS: Thirty-nine temporal bone CT volumes including 58 ears were divided into normal (n = 20) and abnormal groups (n = 38). Ossicular chain disruption (n = 10), facial nerve covering vestibular window (n = 10), and Mondini dysplasia (n = 18) were included in abnormal group. All facial nerves, auditory ossicles, and labyrinths of the normal group were manually segmented. For the abnormal group, aberrant structures were manually segmented. Temporal bone CT data were imported into the network in unmarked form. The Dice coefficient (DC) and average symmetric surface distance (ASSD) were used to evaluate the accuracy of automatic segmentation. RESULTS: In the normal group, the mean values of DC and ASSD were respectively 0.703, and 0.250 mm for the facial nerve; 0.910, and 0.081 mm for the labyrinth; and 0.855, and 0.107 mm for the ossicles. In the abnormal group, the mean values of DC and ASSD were respectively 0.506, and 1.049 mm for the malformed facial nerve; 0.775, and 0.298 mm for the deformed labyrinth; and 0.698, and 1.385 mm for the aberrant ossicles. CONCLUSIONS: The proposed model has good generalization ability, which highlights the promise of this approach for otologist education, disease diagnosis, and preoperative planning for image-guided otology surgery.

Ambient Pressure Tympanometry Wave Patterns in Patients With Superior Semicircular Canal Dehiscence.

Importance: Superior semicircular canal dehiscence (SSCD) is a treatable condition, but current diagnostic modalities have numerous limitations. Clinicians would benefit from an additional tool for diagnostic workup that is both rapid and widely available. Objective: To assess the utility of ambient pressure tympanometry (APT) in the diagnostic workup of SSCD by determining the sensitivity and specificity of APT for SSCD in comparison to other diagnostic modalities. Design: Retrospective cohort study of patients who underwent APT and temporal bone computerized tomography (CT) scans from May 2017 to July 2018. Setting: Tertiary referral center. Participants: APT was performed as part of routine audiological testing on adult patients. We retrospectively analyzed all patients who received both APT and temporal bone CT scans, and divided ears into SSCD and non-SSCD groups based on the presence or absence of radiographic SSCD. Ears with other radiographic findings that could affect tympanic membrane compliance were excluded. Exposures: All patients in this study underwent APT and temporal bone CT scans. Some patients also underwent pure tone audiometry and vestibular evoked myogenic potentials (VEMPs). Main Outcomes and Measures: The primary outcome measures were sensitivity, specificity, and risk ratio of APT for SSCD. Secondary outcome measures include sensitivity of VEMPs and supranormal hearing thresholds. Results: We describe 52 patients (70 ears) who underwent APT and CT imaging (mean age 47.1 years, 67.1% female). APT detected SSCD with 66.7% sensitivity and 72.1% specificity. In symptomatic patients, sensitivity was 71.4% and specificity was 75%. VEMPs performed best at detecting SSCD when defining a positive test as oVEMP amplitude >17 µV, with a sensitivity of 68.2%, similar to APT (p > 0.99). The combination of APT and VEMPs increased sensitivity to 88.9%, better than APT alone (p = 0.031) and trending toward better than VEMPs alone (p = 0.063). Conclusions and Relevance: Rhythmic wave patterns on APT are associated with SSCD and may raise suspicion for this condition in conjunction with consistent results on other diagnostic modalities. Although clinical utility requires confirmation in a larger prospective study, APT is a simple, rapid, and widely available tool warranting further study.

Improved image quality of temporal bone CT with an ultrahigh-resolution CT scanner: clinical pilot studies.

PURPOSE: Ultrahigh-resolution CT (UHRCT) with slice collimation of 0.25 mm × 160 and matrix size of 1024 × 1024 has become clinically available. We compared the image quality of temporal bone CT (TBCT) between UHRCT and conventional multidetector CT (MDCT). MATERIALS AND METHODS: We retrospectively enrolled 20 patients who underwent TBCT by MDCT (matrix size, 512 × 512) and subsequently by UHRCT (matrix size, 1024 × 1024). Two independent reviewers subjectively graded delineation of normal stapes, oval window, facial nerve canal, incudostapedial joint, and tympanic tegmen. We also quantified image noise in the cerebellar hemisphere. Between MDCT and UHRCT, we compared mean subjective grades using the Wilcoxon signed-rank test and the image noise using paired t test. RESULTS: Grades were significantly higher with UHRCT than with MDCT for all the anatomies (P < 0.001), whereas noise was significantly higher with UHRCT than with MDCT (P = 0.002). CONCLUSION: For TBCT, UHRCT shows better delineation of the fine anatomical structures compared with MDCT.

[Clinical and diagnostic observations of a complicated course of chronic otitis media, cholesteatoma in children]. / Kliniko-diagnosticheskie nabliudeniia oslozhnennogo techeniia khronicheskogo gnoinogo kholesteatomnogo srednego otita u detei.

Formation of labyrinth fistulas and destruction of the facial nerve canal in children with middle ear cholesteatoma is rare. Data of children with cholesteatoma of the middle ear, operated in the ENT department of MONIKI, is analyzed. Several clinical observations of multiple-aged patients with the presence of cholesteatoma intra-temporal complications are presented. The preoperative computer tomography made it possible to diagnose the presence of the labyrinth fistula and the destruction of the facial nerve channel, which was confirmed intraoperatively.

Spontaneous Resolution of Cholesteatoma in a Patient on Long-Term Infliximab.

OBJECTIVE:: To describe an observed case of spontaneous regression of cholesteatoma in a patient on chronic anti-tumor necrosis factor-alpha (TNF-a) therapy and inspire further research into the role of TNF-a in cholesteatoma. METHODS:: Clinical assessment of disease in a single-patient case report. RESULTS:: A 49-year-old woman suffered a severe case of Stevens-Johnson syndrome when she was 12 years old, leaving her with bilateral corneal opacification and tympanic membrane perforations with extensive cholesteatoma. For her corneal opacification, a corneal prosthesis was placed, which was complicated by a foreign body reaction necessitating long-term therapy with infliximab, a monoclonal antibody against TNF-a that is therapeutic in some chronic inflammatory diseases. She was otherwise healthy and took no other medications. While on infliximab, the patient had spontaneous and complete resolution of her cholesteatoma without any surgical intervention. CONCLUSIONS:: This surprising case suggests that there may be a prominent role of TNF-a in cholesteatoma pathophysiology and that TNF-a may be an effective target for nonsurgical therapy.

Radiation dose and image conspicuity comparison between conventional 120 kVp and 150 kVp with spectral beam shaping for temporal bone CT.

OBJECTIVE: The purpose of this article is to compare radiation doses and conspicuity of anatomic landmarks of the temporal bone between the CT technique using spectral beam shaping at 150 kVp with a dedicated tin filter (150 kVp-Sn) and the conventional protocol at 120 kVp. METHODS: 25 patients (mean age, 46.8 ±â€¯21.2 years) were examined using the 150-kVp Sn protocol (200 reference mAs using automated tube current modulation, 64 × 0.6 mm collimation, 0.6 mm slice thickness, pitch 0.8), whereas 30 patients (mean age, 54.5 ±â€¯17.8 years) underwent the 120-kVp protocol (180 mAs, 128 × 0.6 mm collimation, 0.6 mm slice thickness, pitch 0.8). Radiation doses were compared between the two acquisition techniques, and dosimetric data from the literature were reviewed for comparison of radiation dose reduction. Subjective conspicuity of 23 anatomic landmarks of the temporal bone, expressed by 5-point rating scale and objective conspicuity by signal-to-noise ratio (SNR) which measured in 4 different regions of interest (ROI), were compared between 150-kVp Sn and 120-kVp acquisitions. RESULTS: The mean dose-length-product (DLP) and effective dose were significantly lower for the 150-kVp Sn scans (0.26 ±â€¯0.26 mSv) compared with the 120-kVp scans (0.92 ±â€¯0.10 mSv, p < 0.001). The lowest effective dose from the literature-based protocols was 0.31 ±â€¯0.12 mSv, which proposed as a low-dose protocol in the setting of spiral multislice temporal bone CT. SNR was slightly superior for 120-kVp images, however analyzability of the 23 anatomic structures did not differ significantly between 150-kVp Sn and 120-kVp scans. CONCLUSION: Temporal bone CT performed at 150 kVp with an additional tin filter for spectral shaping markedly reduced radiation exposure when compared with conventional temporal bone CT at 120 kVp while maintaining anatomic conspicuity. The decreased radiation dose of the 150-kVp Sn was also lower in comparison to the previous literature-based low-dose temporal bone CT protocol.

Comparison of Diagnostic Methods of Secretory Otitis Media in Children / 听力学及言语疾病杂志

Objective To investigate the diagnostic value of endotoscopy,tympanometry,pure tone audiometry (PTA) and high resolution temporal bone CT in children with secretory otitis media (SOM).Methods The study comprised 414 ears in 207 children with adenoid hypertrophy.Endotoscopy,tympanometry,PTA and high resolution temporal bone CT were conducted to evaluate the function of middle ear.Based on the imaging findings of tympanic cavity fluid by high resolution temporal bone CT,the diagnostic accordance rate of endotoscopy,tympanograms and PTA for SOM were studied.Results The diagnostic accordance rate of endotoscopy,tympanometry and PTA for SOM were 98.79％(409/414),98.07％ (406/414) and 88.41％ (366/414),respectively.The sensitivity of the three examinations were 99.20％(124/125),100％ (125/125),63.20％ (79/125),respectively.The specificity of the three examinations were 98.62％ (285/289),97.23％ (281/289),99.31％ (287/289),respectively.The diagnostic accordance rate of endotoscopy combined with tympanometry for SOM was significantly higher than any single examination or any combined examinations.Conclusion Endotoscopy combined with tympanometry shows a great value in diagnosing SOM,which is accurate and safe.

Anatomic and Quantitative Temporal Bone CT for Preoperative Assessment of Branchio-Oto-Renal Syndrome.

We describe the temporal bone computed tomography (CT) findings of an unusual case of branchio-oto-renal syndrome with ectopic ossicles that are partially located in the middle cranial fossa. We also describe quantitative temporal bone CT assessment pertaining to cochlear implantation in the setting of anomalous cochlear anatomy associated with this syndrome.

A radiologic and anatomic study of the superior semicircular canal.

OBJECTIVES: The present study sought to determine whether there is a correlation between the prevalence of superior semicircular canal (SSC) dehiscence (SSCD) on temporal CT and population age. The secondary objective was to identify anatomic factors for SSCD by studying SSC diameter and its protrusion into the middle cranial fossa. The aim was to determine the acquired or congenital origin of SSCD (Minor's syndrome). MATERIAL AND METHOD: A single-center retrospective radiological and anatomic study included 180 CT scans of 354 petrous parts of the temporal bone taken between January and December 2011 in a university hospital center. Bone thickness above the SSC was measured and classified in 4 grades: grade 1, >2.5mm; grade 2, <2.5mm: grade 3, predehiscent; grade 4, dehiscent. SSC diameter was also measured, as was the height of SSC protrusion into the middle cranial fossa. RESULTS: SSCD was found in 0.8% of cases and predehiscence in 12%. Patients with dehiscence were older; patients with grade 3 or 4 were significantly older than those free of dehiscence (P<0.05). There was no significant difference in SSC diameter according to grade. In grade 1, protrusion was greater than in other subjects, with a significant correlation between age and reduced protrusion (P<0.05). CONCLUSION: The study demonstrated a correlation between aging and SSCD prevalence. Reduced SSC roof height with age suggests that SSCD may be an acquired phenomenon, related in some way to aging of the base of the skull.