Objective: To describe a new case of intralabyrinthine lipoma and review all previously published cases in the literature.

OBJECTIVES/HYPOTHESIS:To identify the imaging characteristics associated with better hearing outcomes found in cochleovestibular nerve (CVN) abnormalities treated with hearing aids and/or cochlear implantation (CI).STUDY DESIGN:Retrospective review.METHODS:A retrospective review was undertaken of 69 ears with CVN abnormalities seen on magnetic resonance imaging (MRI) treated at a tertiary referral academic center analyzing the clinical features, imaging characteristics, and hearing data. We searched for associations among the hearing and imaging data, hypothesizing that the imaging data was not a good indicator of hearing function.RESULTS:In univariable analysis of all those who underwent aided testing (hearing aid and CI), health status (P = .016), internal auditory canal (IAC) midpoint diameter (P < .001), and number of nerves in the IAC (P < .001) were predictors of positive hearing outcome. Modiolar abnormalities, cochlear aperture diameter, cochlear malformations, vestibular malformations, and nerves in the cerebellar cistern did not predict hearing outcome (P = .79, .18, .59, .09, .17, respectively). For patients who received CI, health status (P = .018), IAC midpoint (P = .024), and number of nerves in the IAC (P = .038) were significant. When controlling for health status, IAC midpoint diameter (P < .001) and number of nerves in the IAC (P < .001) remained significant. In our cohort, one out of the eight ears (13%) with Birman class 0 or 1 exhibited responses to sound compared to nine out of 13 ears (70%) with Birman class 2-4.CONCLUSIONS:Current imaging modalities cannot accurately depict the status of the cochleovestibular nerve or predict a child's benefit with a CI. Cochlear implantation should be considered in children with abnormal cochleovestibular nerves.LEVEL OF EVIDENCE:3 Laryngoscope, 132:S1-S15, 2022.

A known complication of cholesteatoma is tegmen dehiscence, and meningocele and encephalocele can ensue from the disease process or its treatment. Radiologic findings in patients with far-advanced cholesteatoma with tegmen dehiscence can be misleading and are critical in the decision to prepare and consent for middle fossa surgery in addition to tympanomastoidectomy. Otologic surgery for cholesteatoma, often performed in outpatient settings, should be converted to an inpatient setting if the need for middle fossa is anticipated. Those patients should be specifically counseled about risks and benefits of middle fossa surgery and the likelihood that it will be necessary.

Imaging plays an important role in identifying the cause of the much less common secondary headaches . Such headaches may be caused by a variety of pathologic conditions which can be categorized as intracranial and extracranial. Idiopathic intracranial hypertension imaging findings include "empty sella," orbital changes, and dural venous sinus narrowing. Intracranial hypotension (ICH) is frequently caused by CSF leaks . Imaging findings include loss of the CSF spaces, downward displacement of the brain , as well as dural thickening and enhancement. Severe cases of ICH may result in subdural hematomas . A variety of intracranial and skull base tumors may cause headaches due to dural involvement. Extracranial tumors and lesions that frequently present with headaches include a variety of sinonasal tumors as well as mucoceles . Neurovascular compression disorders causing headaches include trigeminal and glossopharyngeal neuralgia. Imaging findings include displacement and atrophy of the cranial nerve caused by an adjacent arterial or venous structure.

Posterior reversible encephalopathy syndrome (PRES) represents a unique clinical entity with non-specific clinical symptoms and unique neuroradiological findings. This syndrome may present with a broad range of clinical symptoms from headache and visual disturbances to seizure and altered mentation. Typical imaging findings include posterior-circulation predominant vasogenic edema. Although there are many well-documented diseases associated with PRES, the exact pathophysiologic mechanism has yet to be fully elucidated. Generally accepted theories revolve around disruption of the blood-brain barrier secondary to elevated intracranial pressures or endothelial injury. In this article, we will review the clinical, typical, and atypical radiological features of PRES, as well as the most common theories behind the pathophysiology of PRES. Additionally, we will discuss some of the treatment strategies for PRES related to the underlying disease state.

Technologic advances have resulted in the expansion of web-based conferencing and education. While historically video-conferencing has been used for didactic educational sessions, we present its novel use in virtual radiology read-outs in the face of the COVID-19 pandemic. Knowledge of key aspects of set-up, implementation, and possible pitfalls of video-conferencing technology in the application of virtual read-outs can help to improve the educational experience of radiology trainees and promote potential future distance learning and collaboration. (C) 2020 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.

OBJECTIVES:There are significant variations across centers on how to acquire and interpret imaging of children with congenital sensorineural hearing loss and cochleovestibular abnormalities. This study assesses the quality of imaging, sequences included, and accuracy of official radiology reports, to determine if these children are being assessed appropriately. METHODS:This study is retrospective review of CTs and MRIs from 40 pediatric patients diagnosed with profound sensorineural hearing loss and cochleovestibular structure/nerve abnormalities presenting to a tertiary referral academic center. Images were reviewed by two experienced neuroradiologists and a neurotologist. Findings were compared to official reports, when available. RESULTS:Twelve (30%) patients had an MRI only, while 28 (70%) had both an MRI and a CT. There were 3 (10.7%) CTs and 7 (17.5%) MRIs noted to be of poor quality. Children received an average of 6.8 (±2.7) CT acquisitions and 10.9 (±5.7) MRI acquisitions. There was non-concordance between the official report and expert review for 27 (71.1%) ears on CT and 27 (56.3%) ears on MRI. CONCLUSIONS:These data demonstrate high variability in protocols and quality of medical imaging of children with sensorineural hearing loss. Interpretation of images is highly discordant between official reports and tertiary review. Given these results, we recommend that these children be imaged and evaluated at centers with neuroradiologists who are experienced in interpreting congenital abnormalities of the cochleovestibular system.

Posterior Reversible Encephalopathy Syndrome (PRES) represents a unique clinical entity with non-specific clinical symptoms and unique neuroradiological findings. This syndrome may present with a broad range of clinical symptoms from headache and visual disturbances to seizure and altered mentation. Typical imaging findings include posterior-circulation predominant vasogenic edema. Although there are many well documented diseases associated with PRES, the exact pathophysiologic mechanism has yet to be fully elucidated. Generally accepted theories revolve around disruption of the blood-brain barrier secondary to elevated intracranial pressures or endothelial injury. In this article, we will review the clinical, typical and atypical radiological features of PRES, as well as the most common theories behind the pathophysiology of PRES. Additionally, we will discuss some of the treatment strategies for PRES related to the underlying disease state.

Objective: A small subset of children with congenital hearing loss have abnormal cochleovestibular nerves (i.e., absent, aplastic, or deficient cochlear nerves), with largely unknown etiology. Our objective was to investigate the underlying pathways and identify novel genetic variants responsible for cochleovestibular malformations and nerve abnormalities. It is our hypothesis that several cochleovestibular nerve abnormalities might share common causative pathways. Design: We used a family-based exome sequencing approach to study 12 children with known rare inner ear and/or cochleovestibular nerve malformations. Results: Our results highlight a diverse molecular etiology and suggest that genes important in the developing otic vesicle and cranial neural crest, e.g.,MASP1, GREB1L, SIX1, TAF1, are likely to underlie inner ear and/or cochleovestibular nerve malformations. Conclusions: We show that several cochleovestibular nerve malformations are neurocristopathies, which is consistent with the fact that cochleovestibular nerve development is based on otic placode-derived neurons in close association with neural crest-derived glia cells. In addition, we suggest potential genetic markers for more severely affected phenotypes, which may help prognosticate individual cochlear implantation outcomes. Developing better strategies for identifying which children with abnormal nerves will benefit from a cochlear implantation is crucial, as outcomes are usually far less robust and extremely variable in this population, and current neuroimaging and electrophysiologic parameters cannot accurately predict outcomes. Identification of a suitable treatment early will reduce the use of multiple interventions during the time-sensitive period for language development.

Radiologists must convert the complex information in head and neck imaging into text reports that can be understood and used by clinicians, patients, and fellow radiologists for patient care, research, and quality initiatives. Common data elements in reporting, through use of defined questions with constrained answers and terminology, allow radiologists to incorporate best practice standards and improve communication of information regardless of individual reporting style. Use of common data elements for head and neck reporting has the potential to improve outcomes, reduce errors, and transition data consumption not only for humans but future machine learning systems.

A 9-month-old male with bilateral profound sensorineural hearing loss was referred to otolaryngology clinic for cochlear implantation (CI) evaluation. He had been using hearing aids since 5 months of age. There was no other known family history of hearing loss. The patient had a normal head and neck examination without syndromic features. Temporal bone computed tomography scan showed bilateral cochlear incomplete partition type I and enlarged vestibules (Figure 1). Magnetic resonance imaging (MRI) of the lateral internal auditory canals demonstrated 3 nerves on the right side and 4 nerves on the left side. Risks and benefits of a left CI were reviewed with the parents, and a higher risk of facial nerve aberration and cerebral spinal fluid (CSF) leak was reviewed. While undergoing CI evaluation, the patient had 2 hospitalizations for bacterial meningitis. After discharge, a repeat MRI was obtained which showed no postmeningitis cochlear ossification. Given the new history of recurrent meningitis in addition to the abnormal inner ear anatomy, bilateral external auditory canal (EAC) closures were planned along with the left CI to protect the patient from a higher risk of CSF leak. The patient underwent the planned procedures. The right EAC was transected at the bony cartilaginous junction, and the skin cuff was everted and closed in multiple layers. The EAC skin, tympanic membrane, malleus, and incus were removed. The right middle ear was dry. The left EAC was closed in a similar fashion. When the middle ear was entered, significant

BACKGROUND:Childhood hearing impairment affects language and cognitive development. Profound congenital sensorineural hearing impairment can be due to an abnormal cochleovestibular nerve (CVN) and cochleovestibular malformations, however, the etiology of these conditions remains unclear. METHODS:We used a trio-based exome sequencing approach to unravel the underlying molecular etiology of a child with a rare nonsyndromic CVN abnormality and cochlear hypoplasia. Clinical and imaging data were also reviewed. RESULTS:We identified a de novo missense variant [p(Asn174Tyr)] in the DNA-binding Homeodomain of SIX1, a gene which previously has been associated with autosomal dominant hearing loss (ADHL) and branchio-oto-renal or Branchio-otic syndrome, a condition not seen in this patient. CONCLUSIONS:SIX1 has an important function in otic vesicle patterning during embryogenesis, and mice show several abnormalities to their inner ear including loss of inner ear innervation. Previous reports on patients with SIX1 variants lack imaging data and nonsyndromic AD cases were reported to have no inner ear malformations. In conclusion, we show that a de novo variant in SIX1 in a patient with sensorineural hearing loss leads to cochleovestibular malformations and abnormalities of the CVN, without any other abnormalities. Without proper interventions, severe to profound hearing loss is devastating to both education and social integration. Choosing the correct intervention can be challenging and a molecular diagnosis may adjust intervention and improve outcomes, especially for rare cases.

Patients with blunt and penetrating traumatic injuries to the skull base and soft tissues of the neck present to the emergency department every day. Fortunately, truly life-threatening injuries to these regions are relatively uncommon. However, when encountered and not correctly diagnosed, these entities may result in severe morbidity or mortality. The radiologist plays a critical role recognizing these injuries, in which findings may often be subtle and the anatomy potentially challenging to identify. Multisection CT and CT angiography are commonly performed to assess these injuries in the emergency department. Vascular injury to the neck may result in dissection, occlusion, pseudoaneurysm formation, or frank extravasation resulting in stroke or death. Airway compromise may result from laryngotracheal injury. Injuries to the pharynx and esophagus may result in perforation. Injuries to the temporal bone may result in vascular injury to the internal carotid artery or facial nerve injury, which would require immediate surgery or intervention to prevent paralysis.©RSNA, 2019.

Objective: Cochlear fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) signal intensity has been shown to be elevated in patients with vestibular schwannomas (VS). This study evaluated the preoperative and postoperative cochlear signal on FLAIR sequences in patients undergoing hearing preservation surgery. Study Design: Retrospective chart review. Setting: Tertiary referral center. Patients: All patients undergoing middle cranial fossa or retrosigmoid craniotomy for VS at a single institution from September 2013 to January 2017 were screened. Hearing was graded according to the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) hearing classification. Inclusion criteria included preoperative AAO-HNS class A or B hearing and available preoperative and postoperative FLAIR sequences. Main Outcome Measure: Signal intensity of cochlear FLAIR signal was measured in the affected cochlea and normalized to the contralateral cochlea. Hearing preservation was defined as AAO-HNS class A or B postoperatively. Results: Eighteen patients met all inclusion criteria, and 12/18 experienced hearing preservation. There was no difference in preoperative FLAIR ratio between hearing preserved and nonpreserved groups (2.02 vs 2.32, p = 0.52). Postoperatively, FLAIR ratio was lower in the hearing preserved group compared to the nonpreserved group (1.19 vs 1.87, p = 0.033). Conclusions: The current study is the first to examine postoperative cochlear FLAIR changes in VS patients undergoing hearing preservation surgery. In our study population, abnormal hyperintense FLAIR signal normalized in patients experiencing successful hearing preservation, while those who lost hearing maintained abnormal signal. Future studies may investigate the role of FLAIR in guiding optimal timing of operative intervention in VS patients.

Objectives: Little evidence exists regarding the management of orbital meningioma causing vision loss in the setting of neurofibromatosis Type 2 (NF2). We review here our experience with patients at risk for blindness due to intra-orbital meningioma. Design/Setting/Participants/Main Outcome Measures: The charts of patients with NF2 presenting for evaluation of intra-orbital meningioma and vision impairment between 2008 and 2013 were retrospectively reviewed in accordance with institutional IRB policies. Patients with primarily extra-orbital tumors and minimal intra-orbital extension were excluded. Charts were analyzed for the presence and/or imaging progression of intra-orbital tumor, presence of other optic apparatus pathology, presence and/ or progression of vision impairment, and intervention performed (if any). Results: Seven patients with intra-orbital meningioma on MRI and bilateral vision loss and/or unilateral visual impairment due to tumor and contralateral blindness of any etiology were identified. Patients without salvageable vision in either eye were excluded (n = 3). Diagnosis of meningioma was obtained either by biopsy or based on imaging assessment. Conclusion: Conservative management of orbital tumors in NF2 may be preferred in asymptomatic patients but may not be acceptable in patients with progressive visual decline. Radiation is a reasonable option for meningiomas of the orbit and optic nerve sheath. Finally, although the benefit of cranial nerve decompression in NF2 for preservation of facial nerve and hearing has previously been established, the role of optic nerve decompression for preservation of vision in NF2 remains poorly defined.

Objective: Imaging characteristics and hearing outcomes in children with cochleovestibular or cochleovestibular nerve (CVN) abnormalities. Study Design: Retrospective, critical review. Setting: Tertiary referral academic center. Patients: Twenty-seven children with CVN abnormalities with magnetic resonance (MRI) and/or computed tomography (CT). Study Intervention(s): None. Main Outcome Measure(s): Determine the likely presence or absence of a CNV and auditory stimulation responses. Results: Two of 27 cases had unilateral hearing loss, and all others had bilateral loss. Eleven (46%) were identified with a disability or additional condition. Twenty-two (42%) ears received a cochlear implant (CI) and 9 ears (17%) experienced no apparent benefit from the device. MRI acquisition protocols were suboptimal for identification of the nerve in 22 (42%) ears. A likely CVN absence was associated with a narrow cochlear aperture and internal auditory canal and cochlear malformation. Thirteen (48%) children with an abnormal nerve exhibited normal cochleae on the same side. Hearing data were available for 30 ears, and 25 ears (83%) exhibited hearing with or without an assistive device. One child achieved closed set speech recognition with a hearing aid, another with a CI. One child achieved open set speech recognition with a CI. Conclusions: Current imaging cannot accurately characterize the functional status of the CVN or predict an assistive device benefit. Children who would have otherwise been denied a CI exhibited auditory responses after implantation. A CI should be considered in children with abnormal CVN. Furthermore, imaging acquisition protocols need standardization for clear temporal bone imaging.

Abnormal cochleovestibular nerves (i.e., as absent, aplastic, or deficient) are a rare congenital malformation that have a devastating impact on hearing and language development. To date, there have been no genes identified associated with this abnormality. Acknowledgment: The authors thank the family for participating in this study and Keri Ramsey for her help with sample collection.

A woman in her 70s was brought to the emergency department after an unwitnessed fall. She was found in her yard at the bottom of the stairs with blood noted in her ear canal. Patient history was limited by her altered mental status. Computed tomography (CT) of the patient’s head demonstrated a left parietal scalp laceration with a subjacent fracture extending throughout the left parietal and temporal bones, through the superior margin of the left mastoid air cells, internal auditory canal (IAC), and abutting the superior aspect of the left carotid canal. Otolaryngology was consulted to evaluate the patient’s temporal bone fracture. The physical exam was notable for a laceration of the patient’s left external auditory canal; the tympanic membrane could not be visualized because of the presence of blood. CT scans of the bilateral IACs showed an intact malleus on the right (figure 1, A) and a fracture of the malleus on the left (figure 1, B); they also showed an intact malleoincudal joint on the right (figure 2, A) and a widening of the malleoincudo joint on the left (figure 2, B), as well as a distraction injury of the incudostapedial joint. On CT scans, mild separations of the ossicles are described as subluxation, and frank separations are described as dislocation. While ossicular dislocation injuries are common in head trauma, fracture of the malleus is rare as the malleus is typically well stabilized by the tympanic membrane and the tensor tympani tendon compared to the incus, which is larger, heavier, and more exposed.1 Diagnosis of ossicular injuries is often challenging due to their subtle appearance on CT scans, especially in the presence of hemotympanum or opacification of the middle ear cavity. The incudomalleolar joint, with its characteristic appearance of an ice cream cone, can be easily assessed on axial CT images. The incudostapedial joint is usually evaluated on axial CT images by assessing the relative position of the long and lenticular processes of the incus with the capitulum, crura, and footplate of the stapes. Both axial and coronal images are essential in the evaluation of the ossicular chain.2 Blunt trauma to the temporal and parietal area, as in this case, usually causes longitudinal fractures of the temporal bone. It is more commonly associated with conductive hearing loss in contrast to the transverse or otic-capsule–involved fractures associated with sensorineural hearing loss.3 Individuals with malleolar fractures, such as this patient, can present with mild to moderate conductive hearing loss. In her case, audiometry could not be obtained due to her altered mental status. Patients with missed diagnoses of ossicular injury at the time of mild head injury may present OTOSCOPIC CLINIC

OBJECTIVE To evaluate the ability of ultra-high-field magnetic resonance imaging (UHF-MRI) at 11.7 T to visualize membranous structures of the human inner ear. SPECIMENS Three temporal bones were extracted from cadaveric human heads for use with small-bore UHF-MRI. INTERVENTION Ex vivo cadaveric temporal bone specimens were imaged using an 11.7 T magnetic resonance imaging (MRI) scanner via T1- and T2-weighted-imaging with and without contrast. MAIN OUTCOME MEASURE Qualitative visualization of membranous components of the inner ear compared with reports of UHF-MRI at lower field strengths. RESULTS The membranous anatomy of the inner ear was superbly visualized at 11.7 T. In the cochlea, Reissner's membrane, the scala media, and the basilar membrane were clearly shown on the scan. In the vestibular labyrinth, the wedge-shaped crista ampullaris and the maculae of both the saccule and utricle were visible. Details of the endolymphatic sac and duct were also demonstrated. CONCLUSION To our knowledge, this report presents the first images of the ex vivo human inner ear using 11.7 T UHF-MRI, offering near-histologic resolution. Increased field strength may be particularly useful when imaging the delicate membranous anatomy of the inner ear. Further research on the use of UHF-MRI in clinical and research settings could illuminate structural changes associated with inner ear disorders.

Purpose/Objectives: To educate on the potential pitfall of Streptococcus intermedius as a mimicker of brain metastases.Methods/Results: Case review of a 49-year-old male who presented with a lung mass and multiple brain lesions concerning for metastatic lung cancer, but ultimately found to represent an infection by Streptococcus intermedius. Multiple non-neoplastic pathologies that have been known to mimic metastatic brain tumors are reviewed.Conclusion: Streptococcus intermedius infection may mimic brain metastases. This poses a potential pitfall for radiation oncologists consulted to provide urgent empiric brain-directed radiotherapy in the setting of neurologic symptoms presumed to be from metastatic disease.