[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.

Culprit vascular patterns and surgical outcomes of hemifacial spasm caused by an AICA segment passing between cranial nerve VII and VIII: A series of 25 cases.

OBJECTIVE: To report the vascular anatomic characteristics and surgical outcomes of hemifacial spasm (HFS) caused by an anterior inferior cerebellar artery (AICA) segment passing between cranial nerve VII (CN VII) and cranial nerve VIII (CN VIII). PATIENTS AND METHODS: This case series study retrospectively reviewed records of 1040 consecutive patients treated with MVD for HFS in our hospital in 10 years. 25 patients had the culprit vessel recorded as an AICA segment passing between CN VII and CN VIII. Vascular anatomic characteristics were reviewed from intraoperative microscopic videos. The clinical outcomes were followed up at 3-month and 1-year time points. RESULTS: The culprit AICA segments feature 3 discrete anatomic patterns. The patterns denoted as pattern A, B, and C were identified in 19(76%), 3(12%), and 3 (12%) of the 25 patients respectively. Postoperative spasm relief were achieved in 19(76%), 22(88%), and 23 (92%) of the patients at immediately after surgery, 3-month, and 1-year follow-up respectively. 3(12%) of them have permanent postoperative cranial nerve deficits, including one patient with hearing loss and 2 patients with vocal cord palsy. CONCLUSIONS: Though an AICA segment passing between CN VII and CN VIII is common, very rarely it was deemed the culprit for HFS in our patients. We used fREZ centered definition and operation. We found the culprit AICA segments feature 3 discrete anatomic patterns. We observed good spasm relief outcome and relatively fewer complications with CN VII and CN VIII. Identifying the 3 anatomic patterns may help with a smooth decision-making when vascular compression by an AICA segment passing between CN VII and CN VIII is suspected.

Applying artificial intelligence to longitudinal imaging analysis of vestibular schwannoma following radiosurgery.

Artificial intelligence (AI) has been applied with considerable success in the fields of radiology, pathology, and neurosurgery. It is expected that AI will soon be used to optimize strategies for the clinical management of patients based on intensive imaging follow-up. Our objective in this study was to establish an algorithm by which to automate the volumetric measurement of vestibular schwannoma (VS) using a series of parametric MR images following radiosurgery. Based on a sample of 861 consecutive patients who underwent Gamma Knife radiosurgery (GKRS) between 1993 and 2008, the proposed end-to-end deep-learning scheme with automated pre-processing pipeline was applied to a series of 1290 MR examinations (T1W+C, and T2W parametric MR images). All of which were performed under consistent imaging acquisition protocols. The relative volume difference (RVD) between AI-based volumetric measurements and clinical measurements performed by expert radiologists were + 1.74%, - 0.31%, - 0.44%, - 0.19%, - 0.01%, and + 0.26% at each follow-up time point, regardless of the state of the tumor (progressed, pseudo-progressed, or regressed). This study outlines an approach to the evaluation of treatment responses via novel volumetric measurement algorithm, and can be used longitudinally following GKRS for VS. The proposed deep learning AI scheme is applicable to longitudinal follow-up assessments following a variety of therapeutic interventions.

A Study of Outcome of Pediatric Cochlear Implantation in Patients with Cochleovestibular Nerve Deficiency.

OBJECTIVES: A cochleovestibular nerve deficiency (CVND) could compromise stimulation of nerve by electrical pulses delivered from a cochlear implant, thereby hindering activity along auditory pathway. The evaluation of children with congenital hearing loss with a high-resolution magnetic resonance imaging is presently the investigative modality of choice to diagnose CVND. The aim of this study was to determine the outcomes in pediatric cochlear implant recipients with a diagnosis of CVND. The objectives included (1) to study the prevalence of CVND among children with prelingual congenital severe to profound hearing loss; (2) to assess post cochlear implantation (CI) outcomes in children with CVND using categories of auditory performance (CAP), speech intelligibility rating (SIR), and cortical auditory evoked potentials (CAEPs); and (3) to propose a management protocol for these children. MATERIALS AND METHODS: All CI procedures performed during the study period in children 5 years or younger were included in study. All patients who were older than 5 years or had syndromic associations, multiple disabilities, second side or revision CI were excluded from the study. Children with unilateral cochleovestibular nerve aplasia and all other cases of CVND (type IIa and IIb) were advised to undergo CI on side with more radiologically robust nerve and/or cochlea anatomy. Children with bilateral CVND were included in group A, and age-matched cochlear implant candidates with normal cochleovestibular nerve anatomy were included in group B for statistical comparison of outcomes. RESULTS: In group A, post CI CAP and SIR, CAEP amplitude and latency at 12 months showed statistically significant difference (p<0.05) compared with preoperative values. However, mean score of CAEP latency and amplitude and SIR score was worse for group A compared with group B at 12 months, which was statistically significant (p<0.05). CONCLUSION: This study supports the fact that CI is a viable option to be offered in children with CVND (type IIa and IIb) for the development of auditory perception and speech.

MRI surveillance following concurrent cochlear implantation in cases of vestibular schwannoma resection.

OBJECTIVE: Cochlear nerve preserving translabyrinthine vestibular schwannoma (VS) resection enables concurrent cochlear implantation. Implantation in patients with VS raises important concerns including the ability to undergo postoperative magnetic resonance imaging (MRI) monitoring of residual tumor growth or tumor recurrence, specifically with a retained magnet. We aim to assess the feasibility of MRI monitoring and the impact on image quality with retained cochlear implant (CI) magnets. METHODS: Retrospective review of post-operative head MRI scans in CI recipients with a retained CI magnet, after cochlear nerve preserving translabyrinthine excision of VS. The ability to visualize the ipsilateral and contralateral internal auditory canal (IAC) and cerebellopontine angle (CPA) were assessed. RESULTS: A total of eight surveillance head MRI were performed in six patients. In one case, in which the receiver was positioned lower, the view of the ipsilateral IAC and CPA was distorted. In all other cases, the views of both the ipsilateral and contralateral IAC and CPA were overall unimpaired. DISCUSSION: Imaging artifact only very rarely impedes adequate visualization of the ipsilateral IAC or CPA in CI recipients. In anticipation of the need for further IAC and CPA imaging, it would be advisable to place the receiver in an exaggerated superior-posterior position to further decrease obscuring artifact. Thus, serial monitoring of VS tumors can be performed safely with preservation of image quality with a retained receiver magnet. CONCLUSIONS: When placing the CI receiver-stimulator farther posterior-superiorly, excellent visualization of the IAC and CPA can be accomplished without significantly impairing the image quality.

Sporadic and NF2-associated vestibular schwannoma surgery and simultaneous cochlear implantation: a comparative systematic review.

PURPOSE: Cochlear implantation (CI) in patients with sensorineural hearing loss caused by a vestibular schwannoma (VS) represents a unique subtype of hearing rehabilitation, as the outcome may be compromised by vestibulocochlear nerve injury as part of the natural VS history or due to iatrogenic trauma induced by surgical tumor removal. This paper aims to review and report contemporary knowledge and practice regarding feasibility and outcomes of simultaneous vestibular schwannoma resection and cochlear implantation to serve as a reference and guide for future surgery and studies. METHODS: The current literature was searched systematically according to the PRISMA guidelines and after criteria-based selection, 29 studies were identified, including a total of 86 patients who had undergone surgical resection of a vestibular schwannoma and subsequent cochlear implantation in a single procedure. RESULTS: The postoperative outcomes were reported with a high degree of heterogeneity, hindering a proper meta-analysis. However, pooling those cases with reported speech discrimination outcomes demonstrated mean scores equivalent to moderate-to-high performance. A few cases had no audibility. A positive cochlear nerve test result was not a secure positive predictor of success. Complications were rare. CONCLUSION: NF2-associated and sporadic VS had good and comparable postoperative outcomes despite significant differences in tumor size, location and surgical approach.

Radiosurgery treatment is associated with improved facial nerve preservation versus repeat resection in recurrent vestibular schwannomas.

BACKGROUND: Vestibular schwannomas (VSs) are benign neoplasms of the Schwann cells of cranial nerve VIII, and treatment of VS typically involves surgical resection. However, tumor recurrence may necessitate reintervention, and secondary treatment modalities include repeat surgical resection or adjuvant radiosurgery. The purpose of this study is to examine the scientific literature in order to determine whether surgical resection or radiosurgery for recurrent VS results in better tumor control, hearing preservation, and preservation of facial nerve function. METHODS: The PubMed, Scopus, Embase, Cochrane, and Web of Science databases were searched for studies reporting on patients undergoing either radiosurgery or repeat surgical resection after primary surgical resection for recurrent VS. Statistical analyses were performed on the compiled data, primarily outcome data involving tumor control, hearing preservation, and preservation of facial nerve function. RESULTS: We analyzed the data of 15 individual studies involving 359 total patients, and our results reveal that tumor control rates are comparable between adjuvant radiosurgery (91%, CI: 88-94%) and secondary resection (92%, CI 75-98%). However, adjuvant radiosurgery was shown to preserve good facial nerve function better (94%, CI 84-98%) compared to secondary surgical resection (56%, CI 41-69%). CONCLUSION: With comparable tumor control rates and better preservation of good facial nerve function, this study suggests that secondary radiosurgery for recurrent VS is associated with both optimal tumor control and preservation of good facial nerve function.

Correlation of Preoperative Cranial Nerve Diffusion Tensor Tractography with Intraoperative Findings in Surgery of Cerebellopontine Angle Tumors.

OBJECTIVE: Imaging to visualize nerves around tumors preoperatively could help the surgeon to plan the surgery and excise the tumor with preservation or improvement in patients' quality of life. In the present prospective study, we attempted to correlate the preoperative cranial nerves (i.e., trigeminal, abducens, facial, and vestibulocochlear diffusion tensor tractography and anatomic magnetic resonance imaging [MRI] findings) with intraoperative findings during cerebellopontine angle (CPA) tumor surgery. METHODS: The 40 enrolled patients had CPA tumors (31 vestibular schwannomas, 5 epidermoid tumors, 2 meningiomas, 2 trigeminal schwannomas). All the patients were undergoing microsurgery for the first time. They underwent preoperative MRI for anatomic and diffusion tensor imaging (DTI) data. The imaging series were postprocessed, and 3-dimensional images were obtained. The location of the involved nerves around the tumors was recorded during surgery by the senior surgeon, who was kept unaware of the results of the fiber tracking, and a correlation was performed. RESULTS: We evaluated the correlation between the DTI and intraoperative findings for the nerve location in relation to the tumor in the CPA. The correlation between the DTI and intraoperative findings for the facial, trigeminal, and vestibulocochlear nerves was 85% (34 of 40), 85% (34 of 40), and 75% (12 of 16), respectively. CONCLUSIONS: The modified technical parameters proposed could lead to better preservation of cranial nerve function, especially for patients with large tumors. To the best of our knowledge, the present study is the first systematic study of relevant cranial nerves in 40 patients with demonstration of the vestibulocochlear bundle by preoperative MRI in the most common CPA tumors using advanced MRI sequences such as diffusion tensor tractography and capturing the data in a multiplanar format.

Management of Vestibular Schwannomas for the Radiologist.

Vestibular schwannomas are the most common tumor of the cerebellopontine angle. The history of their management has driven advances in imaging, lateral skull base surgery, as well as radiosurgery. With these advances, a shift has occurred from life-saving treatment for late-stage disease to quality of life focused management of smaller tumors. The complicated treatment paradigms involving observation, stereotactic radiosurgery and surgery require close communication between the treatment and neuroradiology teams.

Comparison of Clinical, Histopathological, and Genomic Features Between Malignant Peripheral Nerve Sheath Tumors and Cellular Schwannomas of the Eighth Cranial Nerve: A Case Series.

BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) and cellular schwannomas (CSs) of the eighth cranial nerve are exceedingly rare. The purpose of the present study was to evaluate clinical and genetic characterization of these rare tumors. METHODS: The clinical and radiological features were analyzed retrospectively. The histopathological characteristics were assessed by hematoxylin and eosin staining and immunohistochemistry. Genomic abnormalities were evaluated using array comparative genomic hybridization. RESULTS: Of the 1287 surgeries for vestibular schwannomas from 2014 to 2017, 2 were for MPNSTs and 5 were for CSs. The mean age at diagnosis was older for patients with MPNSTs (57.0 ± 4.2 years) than that of patients with CS (35.8 ± 9.4 years; P = 0.03). Two patients with MPNST died of tumor recurrence. None of the patients with CS died. The 2-year overall and progression-free survival of patients with MPNSTs were worse than those for patients with CSs (overall survival, 50.0% ± 35.4% vs. 100%, P = 0.027; progression-free survival, 0% vs. 100%; P = 0.012). The Ki-67 index for the MPNSTs (29.0% ± 3.5%) was greater than that for the CSs (10.3% ± 3.1%; P = 0.001). The common alterations in MPNSTs mainly included gains of chromosomes 7p, 8p, 9q, 12, and 17 and loss of heterozygosity of 1p, 6 and 9p. The common alterations in CSs included gain of 4p16.3, loss of heterozygosity of 2p15-14, and 22q11.1-13.3. CONCLUSIONS: To the best of our knowledge, the present study is the first high-resolution genomic analysis of MPNSTs and CSs of the eighth cranial nerve and has shown a significant difference that might be more accurate to distinguish between these 2 types of rare tumors.

Glioneuronal Heterotopia Presenting as Cerebellopontine Angle Tumor of Cranial Nerve VIII.

BACKGROUND: Vestibular schwannomas and meningiomas account for the great majority of lesions arising in the cerebellopontine angle. In this report, we present a case of glioneuronal heterotopia, also known as glioneuronal hamartoma, arising from cranial nerve VIII, which is an extremely uncommon lesion. Important radiologic and surgical aspects are reviewed, which may help in early recognition and intraoperative decision making when these lesions are encountered. CASE DESCRIPTION: A healthy 29-year-old female presented with intermittent right facial numbness. Magnetic resonance imaging showed an incidental, minimally enhancing cerebellopontine angle lesion on the right cranial nerve VII-VIII complex. The patient declined serial observation and opted for operative intervention for resection. Intraoperatively, the lesion resembled neural tissue and was continuous with the VIII cranial nerve. Pathologic analysis demonstrated mature glioneuronal tissue consistent with hamartomatous brain tissue. The patient maintained normal hearing and facial nerve function after surgery. Radiologic, surgical, and pathologic characteristics are described. CONCLUSIONS: Ectopic glioneuronal tissue of cranial nerve VIII is a rare non-neoplastic lesion and should be considered in the differential diagnosis of unusual-appearing intracanalicular and cerebellopontine angle lesions. The congenital and benign nature of this entity makes observation a valid option for these cases, although they are so infrequent that they are often presumptively managed as vestibular schwannomas. Attempts to radically resect these lesions may result in higher rates of hearing loss or facial palsy due to their continuity with cranial nerves.

Microvascular Decompression of the VII/VIII Cranial Nerve Complex for the Treatment of Intermediate Nerve Neuralgia: A Retrospective Case Series.

BACKGROUND: Intermediate nerve neuralgia (INN) is a rare and often overlooked form of primary otalgia. The pathophysiological mechanism is unknown, although one of the possible contributing factors is a neurovascular conflict at the root entry zone of the intermediate nerve. The pain can be severely debilitating, and the palette of treatment options is small. OBJECTIVE: To assess the outcome of microvascular decompression (MVD) of the VII/VIII cranial nerve complex for treating INN. METHODS: We retrospectively reviewed the records of a group of 8 consecutive patients with INN who underwent MVD of the VII/VIII cranial nerve complex in the period 1994 to 2015. RESULTS: In total, 7 of the 8 patients experienced almost immediate and complete relief of pain, which remained at long-term follow-up (mean 35 mo ± 24 mo, range 8-84 mo). Postoperatively, 1 patient had a cerebrospinal fluid (CSF) leak, 3 patients experienced permanent ipsilateral hearing loss, and 3 patients had temporary complaints associated with excessive drainage of CSF. CONCLUSION: This study suggests MVD as a valid treatment for medically refractory INN. MVD carries surgical risk, but given the severity of complaints of these patients, we believe the treatment benefits outweigh the associated complications.

Cochleovestibular Nerve Compression Syndrome Caused by Intrameatal Anterior Inferior Cerebellar Artery Loop: Synthesis of Best Evidence for Clinical Decisions.

INTRODUCTION: Intrameatal cochleovestibular neurovascular conflict is a rare condition with specific clinical and therapeutic implications. Although surgery is commonly indicated in other neurovascular conflicts, for this subset of patients there is little evidence to guide treatment decisions. Moving from a case description, we performed a review of the literature on this topic to systematically present the best available evidence to guide clinical decisions. METHODS: We performed a literature review on reported cases of surgically treated intrameatal anterior inferior cerebellar artery (AICA)-cochleovestibular nerve neurovascular conflict, analyzing preoperative clinical data, surgical techniques, and outcomes. We analyzed the levels of evidence using the King's College guidelines. DISCUSSION: We found 35 studies of neurovascular compression of the cochleovestibular nerve by AICA for 536 patients operated on to resolve their neurovascular conflict. Only 4 of these studies describe intrameatal AICA neurovascular conflicts, for 9 cases, including our own. Tinnitus was the most frequent symptom (9/9), followed by vertigo (2/9). Our case was the only one showing unilateral hearing loss. Surgery consisted of microsurgical mobilization of the AICA loop performed through a retrosigmoid craniotomy. Tinnitus and vertigo resolved after surgery in all cases, whereas hearing loss did not improve after surgery. CONCLUSIONS: Surgical treatment offers the best results for tinnitus and vertigo, but it seems to have no effect on hearing loss, not even at long-term follow-up. Microvascular decompression should be proposed to intrameatal symptomatic patients before the onset of hearing loss.

An allograft mouse model for the study of hearing loss secondary to vestibular schwannoma growth.

Vestibular schwannoma is a benign neoplasm arising from the Schwann cell sheath of the auditory-vestibular nerve. It most commonly affects both sides in the genetic condition Neurofibromatosis type 2, causing progressive high frequency sensorineural hearing loss. Here, we describe a microsurgical technique and stereotactic coordinates for schwannoma cell grafting in the vestibular nerve region that recapitulates local tumor growth in the cerebellopontine angle and inner auditory canal with resulting hearing loss. Tumor growth was monitored by bioluminescence and MRI in vivo imaging, and hearing assessed by auditory brainstem responses. These techniques, by potentially enabling orthotopic grafting of a variety of cell lines will allow studies on the pathogenesis of tumor-related hearing loss and preclinical drug evaluation, including hearing endpoints, for NF2-related and sporadic schwannomas.

Microvascular decompression for typewriter tinnitus-case report.

BACKGROUND: Microvascular decompression has been tested as a treatment for tinnitus. METHODS: However, only a fraction of patients appear to benefit from surgery if the combination of findings such as paroxysmal vertigo, ABR changes and tinnitus is used to select patients for microvascular decompression. RESULTS: Instead, a more specific syndrome of staccato or "typewriter" tinnitus, which is highly responsive to carbamazepine, was suggested to be caused by a neurovascular conflict. CONCLUSION: We present the first case of typewriter tinnitus with complete long-term symptom relief following microvascular decompression of the vestibulocochlear nerve. We suggest that this specific syndrome is caused by a neurovascular conflict and treatable by microvascular decompression.

Surgical treatment of jugular foramen meningiomas.

OBJECT: We present our experience with surgery of jugular foramen meningiomas with special consideration of clinical presentation, surgical technique, complications, and outcomes. METHODS: This retrospective study includes three patients with jugular foramen meningiomas treated by the senior author between January 2005 and December 2010. The initial symptom for which they sought medical help was decreased hearing. In all of the patients there had been no other neurological symptoms before surgery. The transcondylar approach with sigmoid sinus ligation at jugular bulb was suitable in each case. RESULTS: No death occurred in this series. All of the patients deteriorated after surgery mainly due to the new lower cranial nerves palsy occurred. The lower cranial nerve dysfunction had improved considerably at the last follow-up examination but no patient fully recovered. Two of three patients with preoperatively impaired yet functional hearing deteriorated after surgery with no subsequent cranial nerve VIII function improvement. In one case postoperative stereotactic radiosurgery was performed due to non-radical tumour resection (Simpson Grade IV) and tumour remnant proved stable in the 4-year follow-up. None of the patients have shown signs of tumour recurrence in the mean follow-up period of 56 months. CONCLUSIONS: Jugular foramen meningiomas represent one of the rarest subgroups of meningiomas and their surgical treatment is associated with significant risk of permanent cranial nerve deficits.

Hemorrhagic vestibular schwannoma: review of the literature.

BACKGROUND: Clinically significant intratumoral hemorrhage historically has been reported in only a small fraction of vestibular schwannomas (VS). Patients with hemorrhagic VS are more likely to present with neurologic deficits and have worse outcomes than patients with nonhemorrhagic VS. The purpose of this study is to analyze characteristics that may predispose VS to hemorrhage and that may prove helpful in the management and treatment of VS. METHODS: A literature search was conducted using National Library of Medicine and National Institutes of Health databases to identify articles pertaining to intratumoral hemorrhage in VS. The authors selected 39 cases, described in 18 published articles, to review. RESULTS: Average patient age and tumor size in hemorrhagic cases of VS did not differ significantly from nonhemorrhagic cases of VS. Facial nerve dysfunction at presentation occurred with greater frequency in cases of hemorrhagic VS (33.3%) than in nonhemorrhagic VS (6.0%). Death occurred much more frequently in cases of hemorrhagic VS (10.0%) than in nonhemorrhagic VS (0.2%). Abnormality of tumor-associated vasculature was noted histologically in many cases, and a large number of the cases reported prior treatment by stereotactic radiosurgery. CONCLUSIONS: Understanding the origins and clinical implications of intratumoral hemorrhage in VS could potentially assist in clinical decision making and patient counseling.