[Dolichoectasia of the basilar artery caused by cystic medial degeneration, as a cause of neurovascular conflict with damage to the trigeminal, facial and vestibulocochlear nerves]. / Dolikhoektaziya bazilyarnoi arterii, obuslovlennaya kistoznoi medial'noi degeneratsiei, kak prichina neirovaskulyarnogo konflikta s porazheniem troinichnogo, litsevogo i vestibulokokhlearnogo nervov.

Cystic medial degeneration (Gsell-Erdheim syndrome, cystic medial necrosis) is considered to be a nonspecific histological manifestation of a group of diseases characterized by degenerative changes in the media, affecting primarily the aorta and adjacent branches, which leads to destruction of the vessel wall, followed by its expansion and, possibly, rupture. The authors describe a case of a 65-year-old female patient with a neurovascular conflict of the three cranial nerves with dolichoectatic basilar artery due to cystic medial degeneration. As a result, the patient has clinical manifestations in the form of hemifacial spasm, trigeminal neuralgia and vestibular paroxysmia. Data from instrumental studies and treatment provided are presented. Neurovascular conflict can be identified in various diseases and is characterized by the complex etiology. The most common clinical manifestations of neurovascular conflict are trigeminal neuralgia, hemifacial spasm, glossopharyngeal neuralgia, and vestibular paroxysmia.

Central myelin-peripheral myelin junction in trigeminal, facial, and vestibulocochlear nerve: A histo-morphometric study.

BACKGROUND: The study aimed to locate the central myelin and peripheral myelin junction (CNS PNS Junction, CPJ) in trigeminal, facial and vestibulocochlear nerves. METHODS: The cisternal segments of the nerves were cut from the brainstem to the proximal margin of trigeminal ganglia (trigeminal nerve) and internal acoustic meatus (facial and vestibulocochlear nerve) from cadavers. Horizontal sections of H&E stained slides were analysed and histo morphometry was performed. The CPJ was confirmed by immunohistochemistry using monoclonal myelin basic protein antibody. RESULTS: The mean length of the trigeminal, facial and vestibulocochlear nerves were 13.6 ± 3.1 mm, 12.4 ± 1.9 mm and 11.5 ± 2.0 mm respectively; mean length of the centrally myelinated segment at the point of maximum convexity was 4.1 ± 1.5 mm, 3.7 ± 1.6 mm, 3.6 ± 1.4 mm respectively. Six different patterns were observed fortheCPJ.Utilizing the derived values, the CPJwas located at a distance of 18 - 48% and 17 - 61% of the total length of the nerve in all the cases in trigeminal and facial nerve respectively. In vestibulocochlear nerve, it was located at a distance of about 13 - 54% of the total length of the nerve. CONCLUSIONS: The location of the CPJ in the vestibulocochlear nerve was midway between the brainstem and internal acoustic meatus which is a novel observation.For all the nerves, the CPJ was located either at or before the half way along the length of the nerve in huge majority (97%); never crossing the 60% of the nerve length.

Diffusion MRI of the facial-vestibulocochlear nerve complex: a prospective clinical validation study.

OBJECTIVES: Surgical planning of vestibular schwannoma surgery would benefit greatly from a robust method of delineating the facial-vestibulocochlear nerve complex with respect to the tumour. This study aimed to optimise a multi-shell readout-segmented diffusion-weighted imaging (rs-DWI) protocol and develop a novel post-processing pipeline to delineate the facial-vestibulocochlear complex within the skull base region, evaluating its accuracy intraoperatively using neuronavigation and tracked electrophysiological recordings. METHODS: In a prospective study of five healthy volunteers and five patients who underwent vestibular schwannoma surgery, rs-DWI was performed and colour tissue maps (CTM) and probabilistic tractography of the cranial nerves were generated. In patients, the average symmetric surface distance (ASSD) and 95% Hausdorff distance (HD-95) were calculated with reference to the neuroradiologist-approved facial nerve segmentation. The accuracy of patient results was assessed intraoperatively using neuronavigation and tracked electrophysiological recordings. RESULTS: Using CTM alone, the facial-vestibulocochlear complex of healthy volunteer subjects was visualised on 9/10 sides. CTM were generated in all 5 patients with vestibular schwannoma enabling the facial nerve to be accurately identified preoperatively. The mean ASSD between the annotators' two segmentations was 1.11 mm (SD 0.40) and the mean HD-95 was 4.62 mm (SD 1.78). The median distance from the nerve segmentation to a positive stimulation point was 1.21 mm (IQR 0.81-3.27 mm) and 2.03 mm (IQR 0.99-3.84 mm) for the two annotators, respectively. CONCLUSIONS: rs-DWI may be used to acquire dMRI data of the cranial nerves within the posterior fossa. CLINICAL RELEVANCE STATEMENT: Readout-segmented diffusion-weighted imaging and colour tissue mapping provide 1-2 mm spatially accurate imaging of the facial-vestibulocochlear nerve complex, enabling accurate preoperative localisation of the facial nerve. This study evaluated the technique in 5 healthy volunteers and 5 patients with vestibular schwannoma. KEY POINTS: • Readout-segmented diffusion-weighted imaging (rs-DWI) with colour tissue mapping (CTM) visualised the facial-vestibulocochlear nerve complex on 9/10 sides in 5 healthy volunteer subjects. • Using rs-DWI and CTM, the facial nerve was visualised in all 5 patients with vestibular schwannoma and within 1.21-2.03 mm of the nerve's true intraoperative location. • Reproducible results were obtained on different scanners.

The Vestibulocochlear Nerve: Anatomy and Pathology.

The vestibulocochlear nerve is the eighth cranial nerve, entering the brainstem in the medullopontine sulcus after crossing the internal auditory canal and cerebellopontine angle cistern. It is a purely sensitive nerve, originating from the Scarpa's and spiral ganglions, responsible for balance and hearing. It has 6 nuclei located in the lower pons. Magnetic resonance imaging (MRI) is useful for evaluating the vestibulocochlear nerve, although computed tomography may have a complementary role in assessing bone lesions. A heavily T2-weighted sequence, such as fast imaging employing steady-state acquisition (FIESTA) or constructive interference steady state (CISS), is crucial in imaging exams to depict the canalicular and cisternal segments of the vestibulocochlear nerve, as well as the fluid signal intensity in the membranous labyrinth. The vestibulocochlear nerve can be affected by several diseases, such as congenital malformations, trauma, inflammatory or infectious diseases, vascular disorders, and neoplasms. The purpose of this article is to review the vestibulocochlear nerve anatomy, discuss the best MRI techniques to evaluate this nerve and demonstrate the imaging aspect of the main diseases that affect it.

Is the cochleovestibular nerve function affected in patients with hemifacial spasm?

Hemifacial spasm (HFS) is a motor disorder caused by the vascular compression of the facial nerve in the posterior fossa. The cochleovestibular nerve is close to the facial nerve and shares the same entry to the periphery, also has disorders caused by vascular compression. We evaluated the cochleovestibular nerve function in patients with HFS based on the hypothesis that vascular compression, which causes HFS, can also affect the nearby cochleovestibular nerve function. The medical charts of 49 patients with surgically confirmed HFS were reviewed retrospectively. The results of the pure-tone threshold, auditory brainstem response (ABR), video head impulse test (vHIT), and magnetic resonance imaging were analyzed. In each patient, the HFS side and the unaffected side were compared in the paired manner. The anterior inferior cerebellar artery was the major offending vessel (69.4%). There were no significant differences in the pure-tone threshold, properties of ABR waves, and vHIT gain. There was no evidence of cochleovestibular nerve compression syndrome in all patients. The angulation of the nerve by the offending vessel was more frequently identified in the HFS side than in the unaffected side (p = 0.040). The effect of HFS on cochleovestibular nerve function is limited.

Límites de estabilidad en los distintos patrones posturográficos. Estudio de cohortes / Stability limits in different posturography patterns. Cohort study

Introducción y objetivo: El desarrollo de las plataformas dinamométricas computerizadas ha significado una evolución trascendental en la medición del equilibrio en bipedestación estática. El objetivo principal de nuestro estudio es determinar la relación entre los límites de estabilidad y el tipo de información que utilizan estos pacientes para mantener el equilibrio.Método: Estudio de cohortes retrospectivo de una muestra de 70 pacientes a partir del análisis de los resultados obtenidos en las posturografías dinámicas computerizadas (PDC) realizadas en la Unidad de Otoneurología del Servicio de Otorrinolaringología y Cirugía de Cabeza y Cuello del Complejo Asistencial Universitario de Salamanca.Resultados: No hay ningún tipo de déficit en 28 de los 70 pacientes a los que se realizó una PDC. La media de los límites de estabilidad en los pacientes con un valor compuesto patológico es menor que los pacientes con un compuesto normal, siendo esta relación estadísticamente significativa (p<0,05). Los pacientes con mayor afectación de los límites de estabilidad son aquellos que no hacen un buen uso de la información vestibular y visual.Discusión / Conclusiones: Es importante analizar si el paciente usa correctamente la información vestibular para mantener el equilibrio y de no ser así, promover estrategias de rehabilitación para reducir el riesgo de caídas. (AU)

Correlación clínico-radiológica del asa vascular del ángulo pontocerebeloso / Vascular loop in the cerebellopontine angle: clinical-radiological correlation

Antecedentes: El tronco encefálico, situado en la fosa posterior, conecta el cerebro con la médula espinal. Debido a su ubicación, sus componentes nerviosos guardan una estrecha relación con estructuras vasculares. Objetivos: Describir una relación clínico-radiológica del asa vascular del ángulo pontocerebeloso en pacientes con síntomas indicativos de afectación vestibulococlear mediante evaluación por neuroimagen. Materiales y métodos: Se realizó un estudio retrospectivo y descriptivo. Se incluyeron todos los pacientes evaluados entre 2011 y 2017 con indicios de asa vascular del ángulo pontocerebeloso e historial clínico y estudios de diagnóstico por imagen disponibles. Resultados: 102 pacientes (63 mujeres y 39 hombres) presentaban afectación vestibulococlear. La indicación clínica más frecuente fue mareos (41,18%). Se halló asa vascular unilateral en 43 pacientes (derecho: 21,57%, izquierdo: 20,59%) y bilateral en 59 pacientes (57,84%). El tipo de asa vascular más frecuente fue el tipo II (derecho: 69,14%; izquierdo: 58,75%). El origen más frecuente fue la arteria cerebelosa anteroinferior (ACAI) (derecha: 66,67%, izquierda: 65,00%). No se observó ninguna asociación entre asas vasculares y pérdida de audición neurosensitiva, nistagmo o vértigo. Se halló una asociación con acúfenos. Conclusiones y significación: La presencia de asas vasculares no se asocia a la mayoría de los síntomas auditivos. No obstante, deben notificarse todos los hallazgos de los estudios por imagen. La interpretación de los hallazgos de los estudios por imagen debe correlacionarse con los síntomas clínicos después de excluir otras causas más frecuentes que puedan explicar la sintomatología.(AU)

Background: The brainstem, situated in the posterior fossa, connects the brain to the spinal cord. Owing to its location, the nerves of the brainstem are closely related with vascular structures. Objectives: To correlate the finding of vascular loops in the cerebellopontine angle on imaging with symptoms indicative of vestibulocochlear involvement. Materials and methods: This retrospective descriptive study included all patients evaluated between 2011 and 2017 with findings suggestive of vascular loops in the cerebellopontine angle for whom the clinical history and imaging studies were available. Results: A total of 102 patients (63 women and 39 men) had vestibulocochlear involvement. The most common clinical indication was dizziness (41.18%). A unilateral vascular loop was found in 43 patients (right: 21.57%, left: 20.59%) and bilateral loops were found in 59 (57.84%) patients. The most common type of vascular loop was type II (right: 69.14%; left: 58.75%). The most common origin of vascular loops was the anterior inferior cerebellar artery (right: 66.67%, left: 65.00%). No associations were observed between vascular loops and sensorineural hearing, nystagmus, or vertigo. There was an association with tinnitus. Conclusions and significance: The presence of vascular loops is not associated with most auditory symptoms. Nevertheless, all findings on imaging studies must be reported. The interpretation of the findings of imaging studies must be correlated with the clinical symptoms after other more common causes that can explain the symptoms have been ruled out.(AU)

Application of ABR in pathogenic neurovascular compression of the 8th cranial nerve in vestibular paroxysmia.

PURPOSE: To investigate the clinical value of electrophysiological tests in indicating pathogenic vascular contact of the 8th nerve in definite vestibular paroxysmia (VP) cases to provide a reference for decompression surgery. METHODS: We retrospectively analyzed patients who had vertigo, unilateral tinnitus, or hearing loss and exhibited vascular contact of the 8th cranial nerve by MRI. Participants were classified into the VP or non-VP group according to the criteria of the Bárány Society in 2016. The demographic characteristics and audiological and electrophysiological test results of the two groups were compared. Receiver operating characteristic (ROC) curves were calculated for ABR to determine the best parameters and cutoff values to predict the existence of pathological neurovascular contact in VP. RESULTS: Thirteen patients in the VP group and 66 patients in the non-VP group were included. VP patients had longer interpeak latency (IPL) I-III and wave III latency compared to non-VP patients (p < 0.001; p < 0.001). According to the ROC analyses, IPL I-III and wave III latency were the best indicators for the diagnosis of VP. The optimal cutoff for IPL I-III was 2.3 ms (sensitivity 84.6%, specificity 95.5%), and that for wave III latency was 4.0 ms (sensitivity 92.3%, specificity 77.3%). There were no differences in the PTA, caloric test, o-VEMP, or c-VEMP results between the two groups. CONCLUSION: Prolonged IPL I-III and the wave III latency of ABR strongly suggested that vascular contact of the 8th cranial nerve was pathological, which may provide some references for microvascular decompression surgery of VP.

The Impact of Vascular Loops in the Cerebellopontine Angle on Audio-Vestibular Symptoms: A Systematic Review.

It has been suggested that vascular loops in the cerebellopontine angle and internal auditory canal are involved in the etiology of audio-vestibular symptoms. Several studies have focused on the compression of the eighth cranial nerve by vascular loops but have yielded contradictory results regarding their clinical significance. The aim of this study was to investigate whether vascular loops in this region correlate with audio-vestibular symptoms and which loop features - if any - can potentially lead to symptom manifestation. This systematic review was conducted according to the PRISMA guidelines. We performed on PubMed a literature search from November 2005 to October 2020. The search strategy included the following keywords ("vascular loops" OR "AICA loops" OR "vascular compression syndrome") AND ("hearing loss" OR "tinnitus" OR "vertigo"). Fifteen studies were eligible and included in the analysis. Overall, the studies encompassed a total of 11,788 patients included in this review. The significantly larger group of patients (70%), in which no correlation of symptoms with vascular loops was found, suggests that vascular loops are probably anatomic variations in a substantial majority of cases with an uncommon subset causing some audio-vestibular symptoms. Even within the papers claiming a correlation, there is a multitude of symptoms that did not correlate with vascular loops. It has been suggested by most authors that magnetic resonance imaging should be performed to exclude the role of a vascular loop in the etiology of audio-vestibular symptoms only when vascular compression syndrome is suspected based on clinical indications and not routinely. Further studies would be useful in order to detail the relationship between the vascular structures and the nervous system.

Can Imaging Predict Hearing Outcomes in Children With Cochleovestibular Nerve Abnormalities?

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.

Genetic alterations associated with malignant transformation of sporadic vestibular schwannoma.

INTRODUCTION: Malignant peripheral nerve sheath tumor of the vestibulocochlear nerve (VN-MPNST) is exceedingly rare and carries a poor prognosis. Little is known about its underlying genetics and in particular the process of malignant transformation. There is an ongoing debate on whether the transformation is initiated by ionizing radiation. We present here the analysis and comparison of two post-radiation VN-MPNST and one undergoing spontaneous transformation. METHODS: Four tumors from three patients (radiation-naïve vestibular schwannoma before (VS) and after (VN-MPNST) malignant transformation in addition to two post-radiation VN-MPNST) were subjected to DNA whole-genome microarray and whole-exome sequencing and tumor-specific mutations were called. Mutational signatures were characterized using MuSiCa. RESULTS: The tumor genomes were characterized predominantly by copy-number aberrations with 36-81% of the genome affected. Even the VS genome was grossly aberrated. The spontaneous malignant transformation was characterized by a near-total whole-genome doubling, disappearance of NF2 mutation and new mutations in three cancer-related genes (GNAQ, FOXO4 and PDGFRB). All tumors had homozygous loss of the tumor suppressor CDKN2A. Neither mutational signature nor copy number profile was associated with ionizing radiation. CONCLUSION: The VN-MPNST genome in our cases is characterized by large copy-number aberrations and homozygous deletion of CDKN2A. Our study demonstrates a VS with genetic alterations similar to its malignant counterpart, suggesting the existence of premalignant VS. No consistent mutational signature was associated with ionizing radiation.

[Macroscopic and microscopic changes of the vestibulocochlear nerve after Gamma Knife treatment]. / Makroskopische und mikroskopische Veränderungen des N. vestibulocochlearis nach Gamma-Knife-Therapie.

We report on a case in which macroscopic and microscopic changes of the vestibulocochlear nerve could be observed after radiosurgery of an intrameatal vestibular schwannoma. This case shows for the first time a morphological correlate for undesirable effects after radiosurgical treatment of a vestibular schwannoma and indicates that despite a certain distance to the actual tumor, degenerative changes in neural structures can be expected.

Vestibular paroxysmia: Clinical features and imaging findings; a literature review.

According to the definition of neurovascular compression syndromes (NVCS), a vascular structure in direct contact with a cranial nerve is causing mechanical irritation of the neural tissue producing correlating symptoms. Vestibular paroxysmia is an example of a neurovascular compression which is caused by neurovascular contact between the eighth cranial nerve and a vessel. It is crucial to understand the unique anatomy of the vestibulocochlear nerve in order to study the syndrome which is the result of its compression. More specifically, the long transitional zone between central and peripheral myelin plays a central role in clinical significance, as the transitional zone is the structure most prone to mechanical injury. Imaging techniques of the eighth cranial nerve and the surrounding structures are substantial for the demonstration of clinically significant cases and potential surgical decompression. The goal of the current review is to present and study the existing literature on vestibular paroxysmia and to search for the most appropriate imaging technique for the syndrome. An extensive literature search of PubMed database was performed, and the studies were ranked based on evidence-based criteria, followed by descriptive statistics of the data. The present analysis indicates that 3D CISS MRI sequence is superior to any other sequence, in the most studies reviewed, regarding the imaging of neurovascular compression of the eighth cranial nerve.

Vestibular paroxysmia caused by contralateral tortuous vertebral artery: A case report.

RATIONALE: Vestibular paroxysmia (VP) is characterized by spontaneous, recurrent, short, paroxysmal attacks of vertigo with or without tinnitus. PATIENT CONCERNS: We report a case of paroxysmal recurrent vertigo accompanying clicking tinnitus on the left side in a 61-year-old patient. He had undergone microvascular decompression to treat the left-side hemifacial spasm 6 years prior. The patient first developed vertigo attacks about 3 years after microvascular decompression, and the attacks increased in frequency over the last 4 months. Video-nystagmography revealed a background right-beating nystagmus which was reversed every 55 seconds, to left-beating nystagmus for 17 seconds. DIAGNOSIS: Brain magnetic resonance imaging and angiography demonstrated a compression of the cisternal segment of the left vestibulocochlear nerve between the tortuous right vertebral artery and the posterior wall of the left porus acusticus internus. INTERVENTIONS AND OUTCOMES: Under the diagnosis of VP, 300 mg oxcarbazepine was administered daily, which relieved the symptoms dramatically. LESSON: The neurovascular cross-compression of the vestibulocochlear nerve by the contralateral vertebral artery tortuosity can cause VP. Periodic paroxysms of right-beating nystagmus accompanying the left-side tinnitus during vertigo attacks in our patient can be explained by secondary central hyperactivity in both vestibular and cochlear nuclei following long-standing neurovascular cross-compression.

Correlation between Cranial Nerve Microstructural Characteristics and Vestibular Schwannoma Tumor Volume.

BACKGROUND AND PURPOSE: Vestibular schwannomas are common cerebellopontine angle tumors arising from the vestibulocochlear nerve and can result in cranial nerve dysfunction. Conventional MR imaging does not provide information that could correlate with cranial nerve compression symptoms of hearing loss or imbalance. We used multitensor tractography to evaluate the relationship between the WM microstructural properties of cranial nerves and tumor volume in a cohort of patients with vestibular schwannomas. MATERIALS AND METHODS: A retrospective study was performed in 258 patients with vestibular schwannomas treated at the Gamma Knife clinic at Toronto Western Hospital between 2014 and 2018. 3T MR images were analyzed in 160 surgically naïve patients with unilateral vestibular schwannomas. Multitensor tractography was used to extract DTI-derived metrics (fractional anisotropy and radial, axial, and mean diffusivities of the bilateral facial and vestibulocochlear nerves [cranial nerves VII/VIII]). ROIs were placed in the transition between cisternal and intracanalicular segments, and images were analyzed using the eXtended Streamline Tractography reconstruction method. Diffusion metrics were correlated with 3D tumor volume derived from the Gamma Knife clinic. RESULTS: DTI analyses revealed significantly higher fractional anisotropy values and a reduction in axial diffusivity, radial diffusivity, and mean diffusivity (all P < .001) within the affected cranial nerves VII and VIII compared with unaffected side. All specific diffusivities (axial, radial, and mean diffusivity) demonstrated an inverse correlation with tumor volume (axial, radial, and mean diffusivity, P < .01). CONCLUSIONS: Multitensor tractography allows the quantification of cranial nerve VII and VIII WM microstructural alterations in patients with vestibular schwannomas. Our findings support the hypothesis that tumor volume may cause microstructural alterations of the affected cranial nerves VII and VIII. This type of advanced imaging may represent a possible avenue to correlate diffusivities with cranial nerve function.

Long-Term Hearing Outcomes From Gamma Knife Treatment for Vestibulocochlear Nerve Schwannomas in a Large, Tertiary Care, Academic Hospital.

OBJECTIVE: Describe long-term hearing outcomes with audiologic data with modern stereotactic radiosurgery techniques for vestibular schwannoma tumors. BACKGROUND: Since the mid-20th century, stereotactic radiosurgery has been an option for central nervous system tumors. Due to the non-invasive manner of treatment, this was extended to treatment for benign vestibular schwannomas without intracranial surgery. Modern advances have localized radiation and reduced dosage, but data are still lacking in the long-term hearing outcomes of this method of treatment. As one of the national leaders in this procedure, we present our full database of these outcomes over the full time period of our institutions utility of this modality. METHODS: A retrospective review was performed of all patients undergoing stereotactic radiotherapy for vestibular schwannomas within the study period of 1998 to 2019 and their audiograms analyzed along with patient data. Laterality Gardner-Robertson hearing score changes were the primary outcome analyzed for each patient; and controls were placed to accommodate for patient demographic data. RESULTS: Long-term, multi-year audiometric evaluation showed statistically significant loss of serviceable hearing and reduction in hearing ability with the use of stereotactic radiosurgery for treatment of vestibular schwannomas. CONCLUSIONS: Little long-term data exists on the audiometric outcomes related to stereotactic radiosurgery treatment for vestibular schwannomas. Our institution has performed more than 300 stereotactic radiosurgery treatments showing a continued reduction over time in serviceable hearing. Practitioners should advise patients undergoing treatment for vestibular schwannomas with this treatment of long-term results.

Automated facial-vestibulocochlear nerve complex identification based on data-driven tractography clustering.

Small size and intricate anatomical environment are the main difficulties facing tractography of the facial-vestibulocochlear nerve complex (FVN), and lead to challenges in fiber orientation distribution (FOD) modeling, fiber tracking, region-of-interest selection, and fiber filtering. Experts need rich experience in anatomy and tractography, as well as substantial labor costs, to identify the FVN. Thus, we present a pipeline to identify the FVN automatically, in what we believe is the first study of the automated identification of the FVN. First, we created an FVN template. Forty high-resolution multishell data were used to perform data-driven fiber clustering based on the multishell multitissue constraint spherical deconvolution FOD model and deterministic tractography. We selected the brainstem and cerebellum (BS-CB) region as the seed region and removed the fibers that reach other brain regions. We then performed spectral fiber clustering twice. The first clustering was to create a BS-CB atlas and separate the fibers that pass through the cerebellopontine angle, and the other one was to extract the FVN. Second, we registered the subject-specific fibers in the space of the FVN template and assigned each fiber to the closest cluster to identify the FVN automatically by spectral embedding. We applied the proposed method to different acquirement sites, including two different healthy datasets and two tumor patient datasets. Experimental results showed that our automatic identification results have ideal colocalization with expert manual identification in terms of spatial overlap and visualization. Importantly, we successfully applied our method to tumor patient data. The FVNs identified by the proposed method were in agreement with intraoperative findings.

Resection of a Dumbbell-Shaped Facial Nerve Schwannoma With Preservation of Facial Nerve Function Through the Extended Middle Fossa Approach: 2-Dimensional Operative Video.

Facial nerve schwannomas are rare and can arise from any segment along the course of the facial nerve.1 Their location and growth patterns present as distinct groups that warrant specific surgical management and approaches.2 The management challenge arises when the facial nerve maintains good function (House-Brackmann grade I-II).3 Hence, a prime goal of management is to maintain good facial animation. In large tumors, however, resection with facial nerve function preservation should be sought and is achievable.4,5 While tumors originating from the geniculate ganglion grow extradural on the floor of the middle fossa, they may extend via an isthmus through the internal auditory canal to the cerebellopontine angle forming a dumbbell-shaped tumor. Despite the large size, they may present with good facial nerve function. These tumors may be resected through an extended middle fossa approach with preservation of facial and vestibulocochlear nerve function. The patient is a 62-yr-old man who presented with mixed sensorineural and conductive hearing loss and normal facial nerve function. Magnetic resonance imaging (MRI) revealed a large tumor involving the middle fossa, internal auditory meatus, and cerebellopontine angle. The tumor was resected through an extended middle fossa approach with a zygomatic osteotomy and anterior petrosectomy.6 A small residual was left at the geniculate ganglion to preserve facial function. The patient did well with hearing preservation and intact facial nerve function. He consented to the procedure and publication of images. Image at 1:30 © Ossama Al-Mefty, used with permission. Images at 2:03 reprinted from Kadri and Al-Mefty,6 with permission from JNSPG.

Retro-labyrinthine Lesion Site Detected by Galvanic Vestibular Stimulation Elicited Vestibular-evoked Myogenic Potentials in Patients with Auditory Neuropathy.

OBJECTIVE: Auditory neuropathy (AN) is a unique pattern of hearing loss with preservation of hair cell function. The condition is characterized by the presence of otoacoustic emissions (OAE) or cochlear microphonic (CM) responses with severe abnormalities of the auditory brainstem response (ABR). The vestibular branches of the VIII cranial nerve and the structures innervated by it can also be affected. However, the precise lesion sites in the vestibular system are not well characterized in patients with AN. METHODS: The air-conducted sound (ACS) vestibular-evoked myogenic potentials (VEMPs) and galvanic vestibular stimuli (GVS)-VEMPs were examined in 14 patients with AN. RESULTS: On examination of VEMPs (n=14, 28 ears), the absent rates of ACS-cervical VEMP (cVEMP), ACS-ocular VEMP (oVEMP), GVS-cVEMP, GVS-oVEMP and caloric test were 92.9% (26/28), 85.7% (24/28), 67.9% (19/28), 53.6% (15/28), and 61.5% (8/13), respectively. Impaired functions of the saccule, inferior vestibular nerve, utricle, superior vestibular nerve, and horizontal semicircular canal were found in 25.0% (7/28), 67.9% (19/28), 32.1% (9/28), 53.6% (15/28) and 61.5% (8/13) patients, respectively. On comparing the elicited VEMPs parameters of AN patients with those of normal controls, both ACS-VEMPs and GVS-VEMPs showed abnormal results in AN patients (such as, lower presence rates, elevated thresholds, prolonged latencies, and decreased amplitudes). CONCLUSION: The study suggested that patients with AN often have concomitant vestibular disorders. Retro-labyrinthine lesions were more frequently observed in this study. GVS-VEMPs combined with ACS-VEMPs may help identify the lesion sites and facilitate detection of areas of vestibular dysfunction in these patients.

Reliability and toxicity of bevacizumab for neurofibromatosis type 2-related vestibular schwannomas: A systematic review and meta-analysis.

BACKGROUND: The anti-angiogenic agent bevacizumab is currently the only drug used clinically for neurofibromatosis type 2-related vestibular schwannomas (NF2-VS). Though benefits have been demonstrated in several cases, the standardized dosage remains unclear. OBJECTIVE: Our meta-analysis was performed to systematically and comprehensively investigate the reliability and toxicity of bevacizumab in the treatment of NF2-VS, with particular emphasis on the impact of dosage. METHODS: The literature search was conducted for studies providing data on patients treated with bevacizumab for NF2-VS across PubMed, Embase, and Cochrane Library until December 31, 2020. Two reviewers extracted the incidence rate of results independently. Then we calculated and pooled unadjusted incidence rate with 95% CIs for each study. The subgroups analyzed were conducted. RESULTS: Fourteen citations (prospective or retrospective observational cohort studies) were eligible based on data from a total of 247 patients with NF2 and 332 related VSs. The pooled results showed that the radiographic response rate (RRR) was 30% [95% CI (20%-42%)], the hearing response rate (HRR) was 32% [95% CI (21%-45%)]. The incidence of major complications was: hypertension 29% [95% CI (23%-35%)], proteinuria 30% [95% CI (18%-44%)], menstrual disorders 44% [95% CI (16%-73%)], hemorrhage 14% [95% CI (4%-26%)], grade3/4 events 12% [95% CI (4%-22%)]. CONCLUSIONS: Nearly one-third of NF2-VS patients may benefit significantly from bevacizumab due to hearing improvement and tumor reduction. Menstrual disorders were the most common adverse events. The high-dose regimen didn't show better efficacy, but results varied considerably according to age.