Vestibular schwannoma-related increased labyrinthine post-gadolinium 3D-FLAIR signal intensity and association with hearing impairment.

BACKGROUND AND PURPOSE: Vestibular schwannomas (VSs) are benign neurogenic tumors commonly associated with progressive unilateral hearing loss, tinnitus, and vestibular symptoms. Growing evidence links signal changes in the VS-adjacent labyrinth with sensorineural hearing loss. This study seeks to quantify the association of labyrinthine signal on post-gadolinium 3D-FLAIR imaging correlates with hearing loss and to evaluate potential longitudinal changes over time. MATERIALS AND METHODS: Selected patients were identified from a prospectively maintained VS registry. Mean signal intensity ratios of the bilateral labyrinth and pons were measured on 3D-FLAIR post-gadolinium MRI. Correlations with paired audiometric data including pure tone average (PTA), word recognition score (WRS), and AAO-HNS hearing class within one year were evaluated. RESULTS: 125 studies obtained from 2015 to 2022 among 66 patients undergoing observational management for sporadic VS were analyzed. Increased signal intensity was noted of the VS-affected labyrinth/contralateral labyrinth (mean ratio 1.56, SD 0.58). Increased signal intensity was associated with increased PTA on both labyrinthine (correlation coefficient [CC] 0.20, p=0.03) and pontine comparisons (CC 0.24, p=0.006), and with decreased WRS on pontine comparisons (CC -0.18, p=0.04). Increased signal intensity was significantly associated with non-serviceable AAO-HNS C/D hearing when intensities were compared to the pons (p=0.01) but not the contralateral labyrinth (p=0.1). Among 44 patients with available follow-up, no statistically significant associations were identified between audiometric data and signal changes over the same interval. CONCLUSIONS: Increased 3D-FLAIR post-gadolinium labyrinthine signal is associated with sensorineural hearing loss; however, its relationship with hearing trajectory remains unclear. Overall findings suggest that while post-gadolinium 3D-FLAIR techniques are sensitive to inner ear involvement associated with VS, the driving mechanism and their temporal relationships with labyrinthine signal intensity and hearing impairment remain unknown. ABBREVIATIONS: AAO-HNS =American Academy of Otolaryngology -Head and Neck Surgery. BLB =blood-labyrinth barrier. CPA = cerebellopontine angle. IAC = internal auditory canal. PTA = pure tone average; SIR = signal intensity ratio. VS = vestibular schwannoma. WRS = word recognition score.

Stapedotomy Versus Cochlear Implantation for Far Advanced Otosclerosis: Insights From a Patient With Matched Preoperative and Postoperative Function.

OBJECTIVE: To examine patient preference after stapedotomy versus cochlear implantation in a unique case of a patient with symmetrical profound mixed hearing loss and similar postoperative speech perception improvement. PATIENTS: An adult patient with bilateral symmetrical far advanced otosclerosis, with profound mixed hearing loss. INTERVENTION: Stapedotomy in the left ear, cochlear implantation in the right ear. MAIN OUTCOME MEASURE: Performance on behavioral audiometry, and subjective report of hearing and intervention preference. RESULTS: A patient successfully underwent left stapedotomy and subsequent cochlear implantation on the right side, per patient preference. Preoperative audiometric characteristics were similar between ears (pure-tone average [PTA] [R: 114; L: 113 dB]; word recognition score [WRS]: 22%). Postprocedural audiometry demonstrated significant improvement after stapedotomy (PTA: 59 dB, WRS: 75%) and from cochlear implant (PTA: 20 dB, WRS: 60%). The patient subjectively reported a preference for the cochlear implant ear despite having substantial gains from stapedotomy. A nuanced discussion highlighting potentially overlooked benefits of cochlear implants in far advanced otosclerosis is conducted. CONCLUSION: In comparison with stapedotomy and hearing aids, cochlear implantation generally permits greater access to sound among patients with far advanced otosclerosis. Though the cochlear implant literature mainly focuses on speech perception outcomes, an underappreciated benefit of cochlear implantation is the high likelihood of achieving "normal" sound levels across the audiogram.

Imaging Findings Post-Stereotactic Radiosurgery for Vestibular Schwannoma: A Primer for the Radiologist.

Noninvasive tumor control of vestibular schwannomas through stereotactic radiosurgery allows high rates of long-term tumor control and has been used primarily for small- and medium-sized vestibular schwannomas. The posttreatment imaging appearance of the tumor, temporal patterns of growth and treatment response, as well as extratumoral complications can often be both subtle or confusing and should be appropriately recognized. Herein, the authors present an imaging-based review of expected changes as well as associated complications related to radiosurgery for vestibular schwannomas.

Magnetic Resonance Imaging Artifact Associated With Transcutaneous Bone Conduction Implants: Cholesteatoma and Vestibular Schwannoma Surveillance.

OBJECTIVE: To evaluate the magnetic resonance (MR) image artifact and image distortion associated with the two transcutaneous bone conduction implants currently available in the United States. STUDY DESIGN: Cadaveric study. METHODS: Two cadaveric head specimens (1 male, 1 female) were unilaterally implanted according to manufacturer guidelines and underwent MR imaging (General Electric and Siemens 1.5 T scanners) under the following device conditions: (1) no device, (2) Cochlear Osia with magnet and headwrap, (3) Cochlear Osia without magnet, and (4) MED-EL Bonebridge with magnet. Maximum metal mitigation techniques were employed in all conditions, and identical sequences were obtained. Blinded image scoring (diagnostic vs nondiagnostic image) was performed by experienced neuroradiologists according to anatomical subsites. RESULTS: All device conditions produced artifact and image distortion. The Osia with magnet produced diagnostic T1- and T2-weighted images of the ipsilateral temporal bone, however, non-echo planar imaging diffusion-weighted imaging (DWI) was nondiagnostic. The Osia without magnet scanned on the Siemens MR imaging demonstrated the least amount of artifact and was the only condition that allowed for diagnostic imaging of the ipsilateral temporal bone on DWI. The Bonebridge produced a large area of artifact and distortion with the involvement of the ipsilateral and contralateral temporal bones. CONCLUSION: In summary, of the three device conditions (Osia with magnet, Osia without magnet, and Bonebridge), Osia without magnet offered the least amount of artifact and distortion and was the only condition in which diagnostic DWI was available for the middle ear and mastoid regions on the Siemens MR imaging scanner.

Third window lesions of the inner ear: A pictorial review.

PURPOSE: Radiographic review of pathologies that associate with third window syndrome. METHODS: Case series and literature review. RESULTS: Eight unique third window conditions are described and illustrated, including superior, lateral, and posterior semicircular canal dehiscence; carotid-cochlear, facial-cochlear, and internal auditory canal-cochlear dehiscence, labyrinthine erosion from endolymphatic sac tumor, and enlarged vestibular aqueduct. CONCLUSION: The present study highlights the characteristic imaging features and symptoms to differentiate third window pathologies for expedient diagnosis and management planning.

Peritumoral Signal on Postcontrast FLAIR Images: Description and Proposed Biomechanism in Vestibular Schwannomas.

BACKGROUND AND PURPOSE: Anecdotally, postcontrast FLAIR images of vestibular schwannomas can show peritumoral hyperintense signal, hypothesized to represent gadolinium extravasation. This study assessed the incidence of this phenomenon in a cohort of patients with treatment-naïve sporadic vestibular schwannomas. MATERIALS AND METHODS: A retrospective review of 20 patients with presumed vestibular schwannoma based on characteristic imaging findings and with dedicated internal auditory canal imaging, including postcontrast T1W1 and postcontrast FLAIR, was performed. Tumor size and location were recorded, as was the presence or absence of a fundal fluid cleft. Images were reviewed for the presence of peritumoral hyperintense signal on FLAIR images (a "halo") and for both subjectively and objectively elevated signal in the ipsilateral cochlea and fundus. RESULTS: Patients were randomly selected from an institutional vestibular schwannoma registry. Eleven (55.0%) were women. A peritumoral halo was present in 90% of patients, averaging 1.0 (SD, 0.2) mm in thickness. The maximum mean FLAIR signal in the ipsilateral fundus (205.9 [SD, 110.2]) was significantly greater than on the contralateral side (121.6 [SD, 27.8]) (P = .02). Maximum mean ipsilateral intracochlear signal (167.8 [SD, 104.5]) was also significantly greater than on the contralateral side (113.4 [SD, 40.1]) (P = .04). CONCLUSIONS: A peritumoral halo on postcontrast FLAIR images was present in 90% of our cohort with randomly selected, treatment-naïve sporadic vestibular schwannomas. Although its mechanism is unknown, this signal is hypothesized to represent gadolinium extravasation, given an ipsilateral increased signal in the adjacent internal auditory canal fundus and cochlea.

Magnetic Resonance Imaging in Patients with Cochlear Implants without Magnet Removal: A Radiology-Administered Protocol to Enhance Operational Efficiency and Improve Workflow.

OBJECTIVE: To describe the development, implementation, and validation of a radiology-administered protocol to obtain magnetic resonance imaging (MRI) in patients with cochlear implants and auditory brainstem implants without magnet removal. STUDY DESIGN: Retrospective review and description of novel care pathway. METHODS: A radiology-administered protocol was designed based on careful input from the radiology safety committee and neurotology. Radiology technologist training modules, consent instructions, patient educational material, clinical audits, and other safeguards were implemented, with samples provided in this report. The primary outcomes measured included instances of magnet displacement during MRI and premature termination of MRI studies secondary to pain. RESULTS: Between June 19, 2018, and October 12, 2021, 301 implanted ears underwent MRI without magnet removal, including 153 devices housing diametric MRI-conditional magnets, and 148 implants with conventional axial (i.e., nondiametric) magnets. Among cases with diametric MRI-conditional magnets, all studies were completed without magnet dislodgement or need to terminate imaging early due to pain. Among cases with conventional axial (nondiametric) magnets, 29 (19.6%) MRI studies were stopped prematurely secondary to pain or discomfort; the overall rate of this event was 9.6% (29 of 301) among the entire study cohort. In addition, 6.1% (9 of 148) experienced confirmed magnet displacement despite headwrap placement; the overall rate among all cases was 3.0% (9 of 301). Eight of these patients received successful external magnet reseating through manual pressure on the external scalp without surgery, and one required surgical replacement of the magnet in the operating room. There were no documented instances of hematoma, infection, device or magnet extrusion, internal device movement (i.e., gross receiver-stimulator migration), or device malfunction in this cohort related to MRI. CONCLUSIONS: We present the successful implementation of a radiology-administered protocol designed to streamline care for cochlear implant and auditory brainstem implant recipients who require MRI and ease clinical demands for otolaryngology providers. Examples of resources developed, including a process map, radiology training modules, consent instructions, patient educational materials, clinical audit, and other procedural safety measures are provided so interested groups may consider adapting and implementing related measures according to need.

Natural history of facial paraganglioma with 2 decades of follow-up: A case report and literature review.

OBJECTIVE: To report 20 years of natural history data for a facial paraganglioma and provide a comprehensive review of the existing literature. PATIENT: 81-year-old female with a remote history of cardiac arrest while under anesthesia who elected to observe her facial paraganglioma for 20 years. INTERVENTIONS: Observation, clinical documentation, radiographic surveillance. MAIN OUTCOME MEASURES: Tumor progression, patient symptomatology, and review of management options. RESULTS: The initial presentation of the facial paraganglioma was facial spasm. Over the course of observation, symptoms progressed to include complete facial nerve paralysis, pulsatile tinnitus, and otalgia on the affected side. Radiologic surveillance demonstrated incremental growth and erosion of surrounding structures, including the posterior external auditory canal, stylomastoid foramen, and lateral semicircular canal with near-dehiscence. Twenty-four cases of facial paraganglioma were identified in the extended literature search and are summarized herein. CONCLUSIONS: This unique case contributes to the scarce literature surrounding facial paragangliomas by reporting the extended natural history of this disease.

Intraoperative diagnosis of facial schwannomas: a multicenter summation of clinical experience, preoperative avoidance, and intraoperative management protocol.

OBJECTIVE: Preoperative differentiation of facial nerve schwannoma (FNS) from vestibular schwannoma (VS) can be challenging, and failure to differentiate between these two pathologies can result in potentially avoidable facial nerve injury. This study presents the combined experience of two high-volume centers in the management of intraoperatively diagnosed FNSs. The authors highlight clinical and imaging features that can distinguish FNS from VS and provide an algorithm to help manage intraoperatively diagnosed FNS. METHODS: Operative records of 1484 presumed sporadic VS resections between January 2012 and December 2021 were reviewed, and patients with intraoperatively diagnosed FNSs were identified. Clinical data and preoperative imaging were retrospectively reviewed for features suggestive of FNS, and factors associated with good postoperative facial nerve function (House-Brackmann [HB] grade ≤ 2) were identified. A preoperative imaging protocol for suspected VS and recommendations for surgical decision-making following an intraoperative FNS diagnosis were created. RESULTS: Nineteen patients (1.3%) with FNSs were identified. All patients had normal facial motor function preoperatively. In 12 patients (63%), preoperative imaging demonstrated no features suggestive of FNS, with the remainder showing subtle enhancement of the geniculate/labyrinthine facial segment, widening/erosion of the fallopian canal, or multiple tumor nodules in retrospect. Eleven (57.9%) of the 19 patients underwent a retrosigmoid craniotomy, and in the remaining patients, a translabyrinthine (n = 6) or transotic (n = 2) approach was used. Following FNS diagnosis, 6 (32%) of the tumors underwent gross-total resection (GTR) and cable nerve grafting, 6 (32%) underwent subtotal resection (STR) and bony decompression of the meatal facial nerve segment, and 7 (36%) underwent bony decompression only. All patients undergoing subtotal debulking or bony decompression exhibited normal postoperative facial function (HB grade I). At the last clinical follow-up, patients who underwent GTR with a facial nerve graft had HB grade III (3 of 6 patients) or IV facial function. Tumor recurrence/regrowth occurred in 3 patients (16%), all of whom had been treated with either bony decompression or STR. CONCLUSIONS: Intraoperative diagnosis of an FNS during a presumed VS resection is rare, but its incidence can be reduced further by maintaining a high index of suspicion and undertaking further imaging in patients with atypical clinical or imaging features. If an intraoperative diagnosis does occur, conservative surgical management with bony decompression of the facial nerve only is recommended, unless there is significant mass effect on surrounding structures.

Absent Stapedial Tendon: Imaging Features of an Underrecognized Entity : Clinical Neuroradiology.

PURPOSE: Congenital absence of the stapedial tendon is a rare entity with characteristic imaging findings, which can go unrecognized due the scarcity of the diagnosis and limited previous description in the imaging literature. We aim to characterize the imaging features of this entity. METHODS: A series of 9 cases with surgical confirmation of stapedial tendon absence were retrospectively reviewed and the most common abnormalities on high resolution computed tomography (CT) of the temporal bone described. RESULTS: Congenital fixation of the stapes footplate was present in nearly all cases of stapedial tendon absence (n = 8, 89%), a clinically important association because the stapes footplate abnormality was not detectable on preoperative CT. Absence or hypoplasia of the pyramidal eminence and aperture was identified in almost all cases (n = 8, 89%), which may be the sole imaging finding to suggest stapedial tendon absence and associated stapes footplate fixation prior to surgery. CONCLUSION: The most reliable indicator of stapedial muscle absence on temporal bone CT is the absence or hypoplasia of the pyramidal eminence and aperture. Importantly, most patients had congenital stapes footplate fixation confirmed intraoperatively with a normal stapes footplate on CT, meaning the pyramidal eminence/aperture abnormality was the only preoperative imaging finding that could have suggested the footplate fixation.

Classifying the Large Vestibular Aqueduct: Morphometry to Audiometry.

OBJECTIVE: Large vestibular aqueduct (LVA) is the most common inner ear dysplasia identified in patients with hearing loss. Our objective was to systematically quantify LVA morphologies and correlate imaging findings with established audiometric outcomes. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: Patients with large vestibular aqueduct identified radiographically, with or without hearing loss. INTERVENTIONS: Diagnostic only. MAIN OUTCOME MEASURES: Vestibular aqueduct (VA) width at midpoint, width at external aperture, and length were measured on cross-sectional imaging. Morphology was classified as type I (borderline), type II (tubular), or type III (funneled). Audiometric endpoints included air/bone conduction, pure tone averages, and air-bone gaps at 250 and 500 Hz. Statistical associations were evaluated using linear regression models, adjusted for age at first audiogram and sex. RESULTS: One hundred seventeen patients (197 ears) were included, with mean age at first audiogram of 22.2 years (standard deviation, 21.7 yr). Imaging features associated with poor audiometric outcomes were increasing VA width at midpoint and external aperture, decreasing VA length, dilated extraosseous endolymphatic sac, cochleovestibular malformations, and increasing VA type (III > II > I). CONCLUSIONS: Quantitative LVA measurements and a standardized morphologic classification system aid in prediction of early audiometric endpoints.

Tympanic Membrane Pneumatocele from Auto-insufflation.

OBJECTIVE: The differential of an external auditory canal mass is broad. One rare potential cause is a pneumatocele of the tympanic membrane, which has only been described 1 other time in the literature. This report serves to describe the second case of this pathology, including its unique presentation, and benign clinical course. METHODS: Case report. RESULTS: A case is discussed in which a pneumatocele of the tympanic membrane was incidentally identified during evaluation of contralateral otologic pathology. The etiology was suspected to be habitual auto-insufflation. After cessation of this practice, the pneumatocele was noted to resolve without further intervention. CONCLUSION: A tympanic membrane pneumatocele represents a rare cause of an external auditory canal mass. The diagnosis can be made clinically via history, palpation, and otoscopy during auto-insufflation, potentially avoiding further diagnostic testing. Depending on the etiology, resolution can occur after lifestyle modification; however, further interventions may definitively treat the condition if so required.

Absent pyramidal eminence and stapedial tendon associated with congenital stapes footplate fixation: Intraoperative and radiographic findings.

OBJECTIVE: Report an association between congenital stapes footplate fixation (CSFF) and radiological absence of the pyramidal eminence and stapedial tendon. PATIENTS: Children and adults with intraoperatively confirmed CSFF and an absent stapedial tendon. INTERVENTIONS: Computed tomography (CT); exploratory tympanotomy with stapedotomy. MAIN OUTCOME MEASURES: Absence of a pyramidal eminence and stapedial tendon aperture identified on preoperative CT that was confirmed intraoperatively. RESULTS: Eight patients with intraoperative confirmation of CSFF and absent stapedial tendon were retrospectively identified. The average preoperative bone conduction and air conduction pure tone averages were 19.6 dB (SD 15.6 dB) and 55.9 dB (SD 23.6 dB), respectively. The average air-bone gap was 36.3 dB (SD 17.9 dB) preoperatively. In the seven patients who underwent preoperative CT, all were consistently identified to have an absent or hypoplastic pyramidal eminence and absent stapedial tendon aperture at the pyramidal eminence. In six cases, the stapedial footplate appeared normal, while in one case the footplate appeared abnormal which correlated with severe facial nerve prolapse observed intraoperatively. All eight cases underwent exploratory tympanotomy and demonstrated intraoperative stapes footplate fixation, absent stapedial tendon and either absent or hypoplastic pyramidal eminence, which correlated with preoperative CT findings. CONCLUSIONS: This study identifies a clinically pragmatic association between an absent pyramidal eminence identified on high-resolution CT and the diagnosis of CSFF. In a condition that otherwise generally lacks distinctive radiological features, the absence of a pyramidal eminence on CT in a patient with nonprogressive, congenital conductive hearing loss may strengthen clinical suspicion for CSFF.

Clinical 7-T MRI for neuroradiology: strengths, weaknesses, and ongoing challenges.

Since the relatively recent regulatory approval for clinical use in both Europe and North America, 7-Tesla (T) MRI has been adopted for clinical practice at our institution. Based on this experience, this article reviews the unique features of 7-T MRI neuroimaging and addresses the challenges of establishing a 7-T MRI clinical practice. The underlying fundamental physics principals of high-field strength MRI are briefly reviewed. Scanner installation, safety considerations, and artifact mitigation techniques are discussed. Seven-tesla MRI case examples of neurologic diseases including epilepsy, vascular abnormalities, and tumor imaging are presented to illustrate specific applications of 7-T MRI. The advantages of 7-T MRI in conjunction with advanced neuroimaging techniques such as functional MRI are presented. Seven-tesla MRI produces more detailed information and, in some cases, results in specific diagnoses where previous 3-T studies were insufficient. Still, persistent technical issues for 7-T scanning present ongoing challenges for radiologists.

Cholesteatoma Localization Using Fused Diffusion-Weighted Images and Thin-Slice T2 Weighted Images.

BACKGROUND: Fusion of diffusion-weighted imaging (DWI) to computed tomography (CT) has been touted as a possible technique to improve cholesteatomas localization. This study set out to assess the ability of DWI images fused with thin-slice heavily T2-weighted images to similarly localize surgically-confirmed cholesteatomas. MATERIALS AND METHODS: A retrospective review was completed of consecutive patients that underwent MR temporal bone imaging (9/2011-3/2020) with both DWI and thin-slice T2-weighted imaging. Included patients underwent surgical resection of primary or recidivistic cholesteatoma after preoperative MR imaging. A neuroradiologist, who was blinded to operative and clinical notes, localized each lesion on both DWI and fused DWI-T2 images in 11 anatomic subdivisions of the temporal bone. Surgical confirmation of cholesteatoma location was used as the gold standard for comparison. RESULTS: Of 24 included examinations, the average age at time of MR was 48.2 ± 24.7 years; 12/24 (50.0%) were female. Five of 24 had primary cholesteatoma, while the remainder had recidivistic disease. Sensitivity, specificity, and accuracy of unfused DWI images were 52.1%, 88.9%, and 75.8%, respectively. Sensitivity, specificity, and accuracy of fused DWI-T2 images were 57.1%, 94.8%, and 81.8%, respectively. CONCLUSION: Fused DWI-T2 images outperformed unfused DWI images in the anatomic localization of temporal bone cholesteatomas. This method represents a potential alternative to MR-CT fusion for pre-operative cholesteatoma evaluation, and warrants future investigations. Opposed to MR-CT fusion, this method only necessitates MR sequences and removes the need for additional CT acquisition. LEVEL OF EVIDENCE: 3 Laryngoscope, 131:E1662-E1667, 2021.

MRI of the Internal Auditory Canal, Labyrinth, and Middle Ear: How We Do It.

MRI is firmly established as an essential modality in the imaging of the temporal bone and lateral skull base. It is used to evaluate normal anatomic structures, evaluate for vestibular schwannomas, assess for inflammatory and/or infectious processes, and detect residual and/or recurrent cholesteatoma. It is also extensively used in pre- and postoperative evaluations, particularly in patients with vestibular schwannomas and candidates for cochlear implantation. Nevertheless, despite the widespread use of MRI for these purposes, many radiologists remain unfamiliar with the complex anatomy and expected imaging findings with such examinations. The purpose of this review is to provide an overview of the most useful MRI sequences for internal auditory canal and labyrinthine imaging, review the relevant anatomy, and discuss the expected appearances of the most commonly encountered pathologic entities. In addition, the features at pre- and postprocedural MRI will be discussed to help ensure that diagnostic radiologists may be of greatest use to the ordering physicians. © RSNA, 2020.