Beyond the otoscope: an imaging review of congenital cholesteatoma.

Congenital cholesteatoma (CC) is a non-neoplastic lesion of keratin debris lined by epithelium found in the temporal bone. It is the lesser-known sibling of the acquired cholesteatoma and may be classified as congenital middle ear cholesteatoma and congenital petrous bone cholesteatoma. The incidence is rising, probably owing to increased recognition and advances in imaging modalities. Cone beam CT provides detailed anatomical information, highlighting quadrant location, ossicular involvement, and mastoid extension. MRI aids in lesion characterization and detection of complications. The classification systems for congenital middle ear and petrous bone cholesteatoma are helpful in the preoperative workup and have a role in predicting postoperative recurrence rates. Management almost invariably involves surgical intervention aimed at preserving middle and inner ear function. Follow-up of CC is mainly based on MRI together with otoscopic examination. Non-echo planar diffusion-weighted imaging, especially, has proven essential for detecting residual disease. This review article emphasizes the significance of imaging in the timely diagnosis and management of CCs. CLINICAL RELEVANCE STATEMENT: This article underscores the crucial role of imaging for prompt detection, preoperative assessment, and postoperative follow-up of CCs, a condition with rising incidence associated with potentially severe complications. KEY POINTS: Timely diagnosis of CCs is imperative for avoiding complications. Imaging is key in detection, preoperative evaluation, and postoperative management. Cone Beam CT and non-echo planar DWI represent state-of-the-art imaging techniques.

Two Cases of Atraumatic Laryngeal Fractures.

INTRODUCTION AND AIM: Atraumatic laryngeal fractures are extremely rare and are most commonly provoked by sneezing or coughing. Only seven cases have been described in medical literature, and only one case described a fracture after swallowing. We present two cases of atraumatic laryngeal fracture after swallowing. CASE REPORT: A 37-year-old male presented to the outpatient ENT clinic with severe dysphonia and odynophagia. He reported feeling a crack in the throat after swallowing with a flexed head. The patient's physical examination showed diffuse swelling and tenderness over the thyroid cartilage without subcutaneous emphysema. Flexible nasolaryngoscopy showed a large right true vocal fold hematoma with normal vocal fold movement. Computed tomography (CT) showed a fracture of the thyroid. Treatment consisted of corticosteroids and pantoprazole. Two years later he presented again at the emergency department with extreme odynophagia after suffering a knee punch on the larynx. CT showed a new fracture line, slightly off midline to the left in the thyroid cartilage. A 42-year-old male presented at the emergency department with odynophagia, dysphonia, and fever after feeling a crack in the throat during forceful swallowing in an extended neck position. Physical examination demonstrated a painful thyroid cartilage with subcutaneous emphysema. Flexible nasolaryngoscopy was normal but CT scan showed a slightly displaced fracture line of the median thyroid cartilage. Complaints gradually disappeared with conservative treatment with corticosteroids and antibiotics. CONCLUSION: Congenital anomalies by abnormal mineralization and ossification could lead to focal weakness of the thyroid cartilage and thus predispose to non-traumatic fractures. The double triad of odynophagia, dysphagia, and dysphonia after sneezing, coughing or swallowing should raise the physician's attention to the possibility of thyroid cartilage fracture, especially after feeling or hearing a crack. Further investigation is obligatory with high-resolution CT of the neck and examination by an ENT specialist.

3D-localisation of cochlear implant electrode contacts in relation to anatomical structures from in vivo cone-beam computed tomography.

Positioning of the cochlear implant (CI) electrode in relation to the anatomical structures is a key factor for the hearing outcome and the preservation of residual hearing after cochlear implantation. Determining the exact electrode's location is therefore expected to play an important role in optimisation of the electrode design, the surgical techniques and the post-operative device fitting. The aim of this study is the development and validation of a robust and efficient computerised algorithm for three-dimensional (3D) localisation of the CI-electrode contacts with respect to the relevant cochlear structures, such as the basilar membrane and the modiolus, from modern clinical in vivo cone-beam computed tomography (CBCT). In the presented algorithm, the pre- and post-implantation CBCT are spatially aligned. To localise the anatomical structures, a cochlear microanatomical template derived from lab-based X-ray computed microtomography (µCT) measurements is warped to match the patient-specific cochlear shape acquired from pre-implantation CBCT. The electrode-contact locations, determined from the post-operative CBCT, are superimposed onto the cochlear fine-structure of the microanatomical template to localise the array. The accuracy of this method was validated in a temporal bone study by comparing the distance of the electrode contacts from the modiolar wall, as derived by the algorithm from CBCTs, with the distance determined from synchrotron-radiation (SR) µCT on the same specimens. Due to the achievable spatial resolution, good tissue contrast and limited presence of metallic artifacts, the SRµCT technique is considered to be a golden standard in the proposed approach. In contrast to other approaches, this validation method allowed for the evaluation of the final electrode-to-modiolus distance (EMD) error, and covers the error in co-alignment of the images, in the determination of the electrode contact location and in the localisation of the cochlear structures. The absolute mean error on the EMD parameter was determined at 0.11 mm (max = 0.29 mm, SD = 0.07 mm) across five samples, slightly lower than the voxel size of the CBCT-scans. In a retrospective study, the algorithm was applied to identify scalar translocations of the electrode from clinical in vivo CBCT datasets of 23 CI-recipients, which showed perfect (100%) agreement with the blinded opinion of two experienced neuroradiologists.

Guidelines for magnetic resonance imaging in pediatric head and neck pathologies: a multicentre international consensus paper.

The use of standardized imaging protocols is paramount in order to facilitate comparable, reproducible images and, consequently, to optimize patient care. Standardized MR protocols are lacking when studying head and neck pathologies in the pediatric population. We propose an international, multicenter consensus paper focused on providing the best combination of acquisition time/technical requirements and image quality. Distinct protocols for different regions of the head and neck and, in some cases, for specific pathologies or clinical indications are recommended. This white paper is endorsed by several international scientific societies and it is the result of discussion, in consensus, among experts in pediatric head and neck imaging.

Evaluation of the transmastoid plugging approach for superior semicircular canal dehiscences: a retrospective series of 30 ears.

PURPOSE: The classical surgical approach for superior semicircular canal dehiscences (SSCD) is via the extradural middle cranial fossa. This pathway is used to resurface or to plug the SSC. In this paper, we present long-term data on an alternative route: the transmastoid pathway. The predictive factors for a successful surgery are equally presented in this paper. METHODS: Thirty reports of patients operated between September 2007 to January 2020 were analysed. SSCD was confirmed by the association of concordant complaints, audiometric data, cervical vestibular evoked myogenic potentials (cVEMP) responses and computerized tomography findings. Before and after surgery, the following factors were analysed: auditory and vestibular subjective symptoms, Tullio phenomenon, pure-tone audiometry thresholds for air and bone conduction, air-bone gap, cVEMP threshold, and computerized tomography data, for instance the size of the dehiscence. RESULTS: The follow-up is 21 months on average. The transmastoid approach significantly improves all symptoms (although there were less probing results for the vestibular symptoms). Objectively, we can observe, a closure of the audiometric air-bone gap on the low frequencies and an improvement in the cVEMP. The only correlation that was identified was between the preoperative cVEMP results and the postoperative air conduction. CONCLUSIONS: The originality of this study is the long postoperative follow-up. It allowed us to evaluate the symptoms in the long term and to determine a predictive factor of postoperative complication, which has not yet been described until today.The transmastoid plugging technique is safe and effective. Additional long-term data with a larger cohort are needed to confirm our results and correlation studies.

Comparison between 3D SPACE FLAIR and 3D TSE FLAIR in Menière's disease.

PURPOSE: Heavily T2-weighted 3D FLAIR (hT2w-3D-FLAIR) sequence with constant flip angle (CFA) has been reported as being more sensitive to low concentrations of gadolinium (Gd) enabling endolymphatic hydrops (EH) visualization. The purpose of this study was to compare signal-to-noise (SNR) ratio, detection rate of EH, and increased perilymphatic enhancement (PE) as well as diagnostic accuracy in diagnosing definite Menière's disease (MD), using 3D-SPACE FLAIR versus conventional 3D-TSE FLAIR. METHODS: This retrospective study included 29 definite MD patients who underwent a 4-h delayed intravenous (IV) Gd-enhanced 3D-TSE FLAIR and 3D-SPACE FLAIR MRI between February 2019 and February 2020. MR images were qualitatively and quantitatively analyzed twice by 2 experienced head and neck radiologists. Qualitative assessment included grading of cochlear and vestibular EH and visual comparison of PE. Quantitative assessment of PE was performed by placing a region of interest (ROI) and ratio calculation in the basal turn of the cochlea and the brainstem. RESULTS: The intra- and inter-reader reliability for grading of EH and PE was excellent (0.7 < kappa < 0.9) for 3D-SPACE FLAIR and exceeded the values for 3D-TSE FLAIR (0.5 < kappa < 0.9) The combination of EH and visual assessment of PE has the highest diagnostic accuracy in diagnosing definite MD on 3D-SPACE FLAIR with a sensitivity of 0.91 and a specificity of 0.98 resulting in a sensitivity raise of 6% compared to 3D-TSE FLAIR. CONCLUSION: Four-hour delayed IV Gd-enhanced 3D-SPACE FLAIR sequence has a higher sensitivity and reproducibility than 3D-TSE FLAIR for the visualization of EH and increased PE in definite MD patients.

Radiological Follow-up After the Bony Obliteration Tympanoplasty in Detecting Residual Cholesteatoma: Towards an Optimal Postoperative MR Imaging Protocol.

INTRODUCTION AND AIM: There is no consensus in literature on the most optimal follow-up imaging protocol for non-echoplanar diffusion-weighted magnetic resonance imaging (non-EP DW MRI) after the canal wall-up bony obliteration tympanoplasty. Clearly, no residual cholesteatoma should be missed but on the other hand, unnecessary MR controls should be avoided. The aim of this study is to evaluate the postoperative results of non-EP DW MRI after canal wall-up bony obliteration tympanoplasty surgery at our Institute and to propose an optimal postoperative MR imaging scheme based on our data. MATERIAL AND METHODS: Retrospective cohort study; all 271 patients who underwent the bony obliteration tympanoplasty between January 2010 and January 2016 with follow-up at our Institute were included. A postoperative MR imaging was systematically performed at 1 year after surgery and repeated at either 5 or both 3 and 5 years after surgery, based on the preferences of the surgeon. Variables of interest were retrieved from electronic patient records. RESULTS: The median follow-up time was 60 months (inter-quartile range 56-62 mo). Two hundred seventy-one patients (100%) received a 1-year MRI, 107 (39%) a 3-year MRI, and 216 (79.7%%) a 5-year MRI. Residual cholesteatoma was found in nine cases (3.3%), corresponding with an estimated residual rate at 5 years follow-up of 3.7% when using Kaplan-Meier analysis. Of these nine cases, six cases of residual cholesteatoma (66.7%) were detected at the 1-year MRI (12-14 mo postsurgery), two cases (22.2%) at the 3-year MRI (35-39 mo postsurgery), and one case (11.1%) at the 5-year MRI (51 mo postsurgery, in this patient no 3-year MRI was performed). An uncertain MRI result was found in 15 cases, presenting as relatively hyperintense lesions. However, subsequent follow-up scans did not show persistent evidence for residual disease in 14 of these 15 cases. CONCLUSIONS: A postoperative MRI scan after 1 and 5 years is essential to detect early and late residual cholesteatoma. In our cohort, 22.2% of residual cases were detected at the 3-year MRI. However, this percentage could potentially have been higher when all patients would have received a 3-year MRI. Therefore, in order to detect residual disease as soon as possible, we propose to perform an MRI scan at 1, 3, and 5 years after the bony obliteration tympanoplasty. In cases with an unclear MR result, we suggest a repeat MRI after 12 months.

The relationship between cochleovestibular function tests and endolymphatic hydrops grading on MRI in patients with Menière's disease.

PURPOSE: In this retrospective study the relationship between cochleovestibular function and a magnetic resonance imaging (MRI-) based classification system of endolymphatic hydrops was investigated. METHODS: Seventy-eight patients with unilateral definite Menière's disease who underwent MRI were included. The parameters of Pure Tone Audiometry (PTA), caloric irrigation test, cervical vestibular evoked myogenic potentials, and video Head Impulse Test were compared between the grades of endolymphatic hydrops (EH) and perilymphatic enhancement (PE) on MRI. RESULTS: The low-frequency PTA was significantly different between cochlear EH grades I and II (p = 0.036; Grade I: mean (Standard Deviation, SD) = 51 decibel Hearing Level (dB HL) (18 dB HL); Grade II: mean (SD) = 60 dB HL (16 dB HL)), and vestibular EH grades 0 and III (p = 0.018; Grade 0: mean (SD) = 43 dB HL (21 dB HL); Grade III: mean = 60 dB HL (10 dB HL)). The ipsilateral caloric sum of ears with vestibular EH grade I (n = 6) was increased with regards to vestibular EH grades 0 (p = 0.001), II (p < 0.001), and III (p < 0.001) (Grade 0: mean (SD) = 24°/s (15°/s); Grade I: mean (SD) = 47°/s (11°/s); Grade II: mean (SD) = 21°/s (13°/s); Grade III: mean (SD) = 16°/s (8°/s)). CONCLUSION: According to these results we can conclude that only the highest grades of cochlear and vestibular EH seem to be associated with decreased cochleovestibular functioning.

Synchrotron radiation X-ray microtomography for the visualization of intra-cochlear anatomy in human temporal bones implanted with a perimodiolar cochlear implant electrode array.

Recently, synchrotron radiation computed microtomography (SRµCT) has emerged as a promising tool for non-destructive, in situ visualization of cochlear implant electrode arrays inserted into a human cochlea. Histological techniques have been the `gold standard' technique for accurate localization of cochlear implant electrodes but are suboptimal for precise three-dimensional measurements. Here, an SRµCT experimental setup is proposed that offers the benefit of a high spatial and contrast resolution (isotropic voxel size = 4.95 µm and propagation-based phase-contrast imaging), while visualizing the soft-tissue structures and electrode array of the cochlear implant simultaneously. In this work, perimodiolar electrode arrays have been tested, which incorporate thick and closely spaced platinum-iridium contacts and wiring. These data can assist cochlear implant and hearing research, can be used to verify electrode segmentation techniques for clinical computed tomography or could be utilized to evaluate cochlear implant electrode array designs.

Labyrinthine enhancement on 3D black blood MR images of the brain as an imaging biomarker for cisplatin ototoxicity in (lung) cancer patients.

PURPOSE: Cancer patients treated with platinum-based chemotherapy can present with ototoxicity symptoms. The purpose of this work is to report the imaging features related to cisplatin ototoxicity. METHODS: Between December 2015 and March 2019, a cohort of 96 consecutive patients with lung cancer was selected. Only patients who received cisplatin chemotherapy and underwent an imaging protocol consisting of a Gd-enhanced 3D-BB and 3D-T1W sequence, as well as T2W sequence to exclude metastases, were included. Labyrinthine enhancement was assessed, and all findings regarding the auditory and vestibular function were retrieved from the clinical files. RESULTS: Twenty-one patients met the inclusion criteria. The Gd-enhanced 3D-BB images were used to divide them into the labyrinth enhancement group (LEG) and the labyrinth non-enhancement group (LNEG). None of these patients demonstrated enhancing regions on the 3D-T1W images. The labyrinthine fluid remained high on the T2 images in all patients, excluding metastases. The LEG consisted of 6 patients. The cochlea and semicircular canals were the most frequently affected regions. All the LEG patients that presented with hearing loss (4/6) had cochlear enhancement. Patients with normal hearing had no cochlear enhancement. Five patients (5/6) showed vestibular enhancement. Four of these patients had vestibular symptoms. CONCLUSION: Labyrinthine enhancement as an imaging feature related to cisplatin ototoxicity is unreported. This study demonstrates a correlation between hearing loss and cochlear enhancement and also between vestibular impairment and vestibular/semicircular enhancement on 3D-BB images, which remained invisible on the 3D-T1W images. The labyrinthine enhancement on 3D-BB images in the presence of normal signal intensity of the intralabyrinthine fluid can be used as an imaging biomarker for cisplatin toxicity in daily clinical practice and should not be mistaken for intralabyrinthine metastases.

Imaging Anatomy of the Jaw and Dentition with Cone Beam Computed Tomography.

Knowledge of dental, maxillary, and mandibular anatomy and the use of correct nomenclature is critical in the evaluation of a mandibulofacial and/or maxillofacial imaging data set. The use of the correct diagnostic imaging tool tailored to the patient's needs is of equal importance. This article highlights imaging anatomy and cross-sectional imaging modalities mainly focusing on cone beam computed tomography of the mandibulofacial and maxillofacial region.

Facial and Dental Trauma.

Dental and facial trauma are very common in a daily routine radiology practice. Knowledge of the functional anatomy of the mandibulofacial and maxillofacial regions forms the cornerstone of a thorough full radiologic evaluation of the patient with a dental and/or facial trauma. Cone beam computed tomography and multidetector computed tomography are currently considered the imaging modalities of choice. There are several patterns and classification systems for the various subtypes of mandibulofacial and maxillofacial fractures. They are discussed separately in this article with special attention to the types of dental trauma and orbital trauma.

Pediatric necrotizing soft tissue infection after elective surgery: A case report and literature review.

INTRODUCTION: Necrotizing soft tissue infections (NSTIs) are rare but often lethal. Early diagnosis and aggressive surgical debridement are essential to achieve the best possible outcome. CASE PRESENTATION: A 12-year old boy was diagnosed with a necrotizing soft tissue infection following elective revision surgery for functional impairment resulting from scar tissue of the neck. Fever and inflammation of the surgical wound manifested 24-36 hours postoperatively. Antibiotic therapy with amoxicillin-clavulanic acid was initiated, but the patient was unresponsive. Ultrasonography, a wound culture and surgical exploration confirmed the diagnosis. The culture was positive for a Streptococcus pyogenes infection and antibiotic treatment was switched to penicillin and clindamycin. Following the diagnosis, surgical debridement was performed subcutaneously, and only necrotic tissue was removed to preserve as much skin tissue as possible. After eradication of the infection, vacuum-assisted closure of the wound was used to close the subcutaneous space. The patient was discharged after 40 days. CONCLUSION: In this patient, we treated a necrotizing soft tissue infection with antibiotics, skin sparing surgeries and negative pressure wound therapy (NPWT). We used ultrasonography as imaging technique to help with the diagnosis. The extensiveness of surgical debridement was rather limited. We focused on opening all affected fascial layers. The surgical debridement was subcutaneous, and only necrotic tissue was removed. Because of the location in the neck, we tried to avoid an aggressive skin debridement to preserve as much skin tissue as possible. Negative pressure wound therapy is not frequently used in this context but it contributed to an enhanced wound healing. Ultrasonography for diagnosing NSTIs is useful, but the clinical findings and an explorative surgery will remain most important.

Intratemporal facial nerve schwannomas: multicenter experience of 80 cases.

PURPOSE: To provide more data on the clinical presentation and natural evolution of facial nerve schwannomas and to provide guidance for therapeutic decision making. METHODS: A retrospective case review of eighty patients diagnosed with a facial nerve schwannoma between 1990 and 2018 in ten tertiary referral centers in Europe was performed. Patients' demographics, symptomatology, audiometry, anatomical site (segments involved), size and whenever possible volume measurement were registered. RESULTS: At presentation, transient or persistent facial palsy was the most common symptom, followed by hearing loss. The schwannoma involved more than one segment in the majority of the patients with the geniculate ganglion being most commonly involved. Initial treatment consisted of a wait and scan approach in 67.5%, surgery in 30% and radiation therapy in 2.5% of the patients. Tympanic segment schwannomas caused mainly conductive hearing loss and were more prone to develop facial palsy at follow-up. Internal auditory canal or cerebellopontine angle schwannomas presented with significantly more sensorineural hearing loss. CONCLUSIONS: Although modern imaging has improved diagnosis of this tumor, choosing the best treatment modality remains a real challenge. Based on the literature review and current findings, more insights into the clinical course and the management of facial nerve schwannomas are provided.

The Sensitivity of the cVEMP Test in Detecting A Superior Semicircular Canal Dehiscence and the Influence of a Coexisting Incudal Lysis: A Case Report.

In this case report, the air-conducted cervical vestibular evoked myogenic potentials (AC cVEMP) test was only sensitive for the left superior semicircular canal dehiscence (SCD), even though the contralateral SCD was of equal length (2.5 mm). Furthermore, a lysis of the processus lenticularis incudis caused a real conductive hearing loss in the left ear. A diminished left AC cVEMP was thus expected, but the opposite was shown (increased corrected amplitude, lowered detection threshold). The patient only experienced hearing loss, so middle ear surgery was performed to repair the lysis. The postoperative AC cVEMP showed a further "uncovering" of the SCD with increased corrected amplitude on the left but no vestibular symptoms. The significance of an SCD should be interpreted with caution, even when the AC cVEMP and the imaging are significant. Furthermore, AC cVEMPs should not be considered as evidence for the absence or presence of conductive hearing loss.

The value of four stage vestibular hydrops grading and asymmetric perilymphatic enhancement in the diagnosis of Menière's disease on MRI.

PURPOSE: There is still a clinical-radiologic discrepancy in patients with Menière's disease (MD). Therefore, the purpose of this study was to investigate the reliability of current MRI endolymphatic hydrops (EH) criteria according to Baráth in a larger study population and the clinical utility of new imaging signs such as a supplementary fourth low-grade vestibular EH and the degree of perilymphatic enhancement (PE) in patients with Menière's disease (MD). METHODS: This retrospective study included 148 patients with probable or definite MD according to the 2015 American Academy of Otolaryngology, Head and Neck Surgery criteria who underwent a 4-h delayed intravenous Gd-enhanced 3D-FLAIR MRI between January 2015 and December 2016. Vestibular EH, vestibular PE, cochlear EH, and cochlear PE were reviewed twice by three experienced readers. Cohen's Kappa and multivariate logistic regression were used for analysis. RESULTS: The intra- and inter-reader reliability for the grading of vestibular-cochlear EH and PE was excellent (0.7 < kappa < 0.9). The two most distinctive characteristics to identify MD are cochlear PE and vestibular EH which combined gave a sensitivity and specificity of 79.5 and 93.6%. By addition of a lower grade vestibular EH, the sensitivity improved to 84.6% without losing specificity (92.3%). Cochlear EH nor vestibular PE showed added-value. CONCLUSIONS: MRI using vestibular-cochlear EH and PE grading system is a reliable technique. A four-stage vestibular EH grading system in combination with cochlear PE assessment gives the best diagnostic accuracy to detect MD.