[Vestibular Schwannoma: Factors in Therapy Decision-Making]. / Vestibularisschwannom: Faktoren bei der Therapieentscheidung.

The treatment of vestibular schwannomas (VS) has always posed a challenge for physicians. Three essential treatment principles are available: wait-and-scan, surgery, and stereotactic radiotherapy. In addition to the type of treatment, decisions must be made regarding the optimal timing of therapy, the combination of different treatment modalities, the potential surgical approach, and the type and intensity of radiation. Factors influencing the therapy decision include tumor location and size or stage, patient age, comorbidities, symptoms, postoperative hearing rehabilitation options, patient preferences, and, not least, the experience of the surgeons and the personnel and technical capabilities of the clinical site. This article begins with a brief overview of vestibular schwannomas, then outlines the fundamental interdisciplinary treatment options, and finally discusses the ENT (ear, nose, and throat)-relevant factors in the therapy decision.

Patient-preferred outcomes in patients with vestibular schwannoma: a qualitative content analysis of symptoms, side effects and their impact on health-related quality of life.

PURPOSE: During counseling and management of patients with vestibular schwannoma (VS), the emphasis is shifting from tumour control and nerve preservation towards maintaining or improving health-related quality of life (HRQoL). Understanding the patients' perspective and impact of VS is, therefore, of utmost importance. The current study aimed to identify treatment outcomes preferred by patients and to explore the patient-reported VS symptoms and management-related side effects and their impact on HRQoL. METHODS: Patients with VS were contacted through the Dutch VS association Stichting Hoormij and questioned using a semi-structured, cross-sectional online survey. Patients were asked to report and rank symptoms and side effects, with their impact on HRQoL and frequency of occurrence. Results were structured through qualitative content analysis. Coded symptoms, side effects, impacts, frequencies, and patient-preferred outcomes were analysed and summarized with descriptive statistics. RESULTS: Of the 231 respondents, 71% were actively treated. Hearing (symptoms vs. side effects: 78.8% vs. 63.6%), balance (62.3%; 48.8%), and energy issues (33.8%; 32.6%) were the most frequently mentioned symptoms and management-related side effects. Fatigue, deafness, headaches, and hearing loss had the highest impact on HRQoL. The majority of patients identified hearing preservation (61%), balance preservation (38.5%), and reduced tinnitus (34.6%) to be the patient-preferred outcomes. CONCLUSION: This qualitative study demonstrates that in this population many patients with VS encounter participation difficulties in their daily physical and social activities and value hearing and balance preservation, reduced tinnitus, and restored energy as preferred outcomes as they are hampered by symptoms and side effects related to hearing, balance, and energy. Healthcare professionals should consider these key points and use these and the patient-preferred outcomes in consultation, shared decision making, treatment, and follow-up to optimize patient-centred care.

Upfront Radiosurgery vs a Wait-and-Scan Approach for Small- or Medium-Sized Vestibular Schwannoma: The V-REX Randomized Clinical Trial.

Importance: Current guidelines for treating small- to medium-sized vestibular schwannoma recommend either upfront radiosurgery or waiting to treat until tumor growth has been detected radiographically. Objective: To determine whether upfront radiosurgery provides superior tumor volume reduction to a wait-and-scan approach for small- to medium-sized vestibular schwannoma. Design, Setting, and Participants: Randomized clinical trial of 100 patients with a newly diagnosed (<6 months) unilateral vestibular schwannoma and a maximal tumor diameter of less than 2 cm in the cerebellopontine angle as measured on magnetic resonance imaging. Participants were enrolled at the Norwegian National Unit for Vestibular Schwannoma from October 28, 2014, through October 3, 2017; 4-year follow-up ended on October 20, 2021. Interventions: Participants were randomized to receive either upfront radiosurgery (n = 50) or to undergo a wait-and-scan protocol, for which treatment was given only upon radiographically documented tumor growth (n = 50). Participants underwent 5 annual study visits consisting of clinical assessment, radiological examination, audiovestibular tests, and questionnaires. Main Outcomes and Measures: The primary outcome was the ratio between tumor volume at the trial end at 4 years and baseline (V4:V0). There were 26 prespecified secondary outcomes, including patient-reported symptoms, clinical examinations, audiovestibular tests, and quality-of-life outcomes. Safety outcomes were the risk of salvage microsurgery and radiation-associated complications. Results: Of the 100 randomized patients, 98 completed the trial and were included in the primary analysis (mean age, 54 years; 42% female). In the upfront radiosurgery group, 1 participant (2%) received repeated radiosurgery upon tumor growth, 2 (4%) needed salvage microsurgery, and 45 (94%) had no additional treatment. In the wait-and-scan group, 21 patients (42%) received radiosurgery upon tumor growth, 1 (2%) underwent salvage microsurgery, and 28 (56%) remained untreated. For the primary outcome of the ratio of tumor volume at the trial end to baseline, the geometric mean V4:V0 was 0.87 (95% CI, 0.66-1.15) in the upfront radiosurgery group and 1.51 (95% CI, 1.23-1.84) in the wait-and-scan group, showing a significantly greater tumor volume reduction in patients treated with upfront radiosurgery (wait-and-scan to upfront radiosurgery ratio, 1.73; 95% CI, 1.23-2.44; P = .002). Of 26 secondary outcomes, 25 showed no significant difference. No radiation-associated complications were observed. Conclusion and relevance: Among patients with newly diagnosed small- and medium-sized vestibular schwannoma, upfront radiosurgery demonstrated a significantly greater tumor volume reduction at 4 years than a wait-and-scan approach with treatment upon tumor growth. These findings may help inform treatment decisions for patients with vestibular schwannoma, and further investigation of long-term clinical outcomes is needed. Trial Registration: ClinicalTrials.gov Identifier: NCT02249572.

A Critical Overview of Targeted Therapies for Vestibular Schwannoma.

Vestibular schwannoma (VS) is a benign tumor that originates from Schwann cells in the vestibular component. Surgical treatment for VS has gradually declined over the past few decades, especially for small tumors. Gamma knife radiosurgery has become an accepted treatment for VS, with a high rate of tumor control. For neurofibromatosis type 2 (NF2)-associated VS resistant to radiotherapy, vascular endothelial growth factor (VEGF)-A/VEGF receptor (VEGFR)-targeted therapy (e.g., bevacizumab) may become the first-line therapy. Recently, a clinical trial using a VEGFR1/2 peptide vaccine was also conducted in patients with progressive NF2-associated schwannomas, which was the first immunotherapeutic approach for NF2 patients. Targeted therapies for the gene product of SH3PXD2A-HTRA1 fusion may be effective for sporadic VS. Several protein kinase inhibitors could be supportive to prevent tumor progression because merlin inhibits signaling by tyrosine receptor kinases and the activation of downstream pathways, including the Ras/Raf/MEK/ERK and PI3K/Akt/mTORC1 pathways. Tumor-microenvironment-targeted therapy may be supportive for the mainstays of management. The tumor-associated macrophage is the major component of immunosuppressive cells in schwannomas. Here, we present a critical overview of targeted therapies for VS. Multimodal therapy is required to manage patients with refractory VS.

[Vestibular schwannoma: Diagnosis-Therapy-Aftercare]. / Vestibularisschwannome: Diagnose ­ Therapie ­ Nachsorge.

Vestibular schwannomas can severely impair the quality of life of patients. Next to impaired hearing function, facial palsy is perceived as particularly disturbing in this context. Varying growth rates of these benign tumors complicate a prediction of functional impairment of cranial nerves. Therefore, a regular update on current therapeutic strategies and alternative treatment options is relevant for both physicians and patients.

Management of vestibular dysfunction and hearing loss in intralabyrinthine schwannomas.

PURPOSE: Intralabyrinthine schwannomas (ILS) are rare, benign, slow-growing tumors arising from schwann cells of the cochlear or vestibular nerves within the bony labyrinth. This study provides insight into the management of this rare tumor through a large case series. MATERIALS AND METHODS: After Institutional Review Board approval, a retrospective chart review was performed of all ILS patients treated at our institution between 2007 and 2019. RESULTS: 20 patients (9 male, 11 female) with ILS were managed at our institution. The right ear was affected in 9 patients (45%) and the left in 11 (55%). Subjective hearing loss was endorsed by all 20 patients. Average pure tone average at presentation was 72 dB nHL. Nine tumors (45%) were intravestibular, 6 (30%) were intracochlear, 4 (20%) were transmodiolar and 1 (5%) was intravestibulocochlear. Hearings aids were used in 3 patients (15%), BiCROS in 2 (10%), CI in 2 (10%), and bone conduction implant in 1 (5%). Vestibular rehabilitation was pursued in 5 patients. Surgical excision was performed for one patient (5%) via translabyrinthine approach due to intractable vertigo. No patients received radiotherapy or intratympanic gentamicin injections. CONCLUSION: ILS presents a diagnostic and management challenge given the similarity of symptoms with other disorders and limited treatment options. Hearing loss may be managed on a case-by-case basis according to patient symptoms while vestibular loss may be mitigated with vestibular therapy. Surgical excision may be considered in patients with intractable vertigo, severe hearing loss with concurrent CI placement, or in other case-by-case situations.

Patient-reported factors that influence the vestibular schwannoma treatment decision: a qualitative study.

PURPOSE: In cases of small- to medium-sized vestibular schwannomas, three management strategies can be opted for: active surveillance, surgery or radiotherapy. In these cases, the patient's preference is pivotal in decision-making. The aim of this study was to identify factors that influence a patient's decision for a particular management strategy. METHODS: A qualitative inductive thematic analysis was performed based on semi-structured interviews. Eighteen patients with small- to medium-sized vestibular schwannomas were interviewed. All patients were diagnosed or treated at one of the two participating university medical centers in the Netherlands. RESULTS: Ten themes were identified that influenced the decision, classified as either medical or patient-related. The medical themes that emerged were: tumor characteristics, the physician's recommendation, treatment outcomes and the perceived center's experience. The patient-related themes were: personal characteristics, anxiety, experiences, cognitions, logistics and trust in the physician. CONCLUSION: Knowledge of the factors that influence decision-making helps physicians to tailor their consultations to arrive at a true shared decision on vestibular schwannoma management.

[Management of transmodiolar and transmacular cochleovestibular schwannomas with and without cochlear implantation. German version]. / Management transmodiolärer und transmakulärer Vestibularis- und Cochlearisschwannome mit und ohne Cochleaimplantation.

INTRODUCTION: Hearing rehabilitation with cochlear implants has attracted increasing interest also for patients with cochleovestibular schwannoma. The authors report their experience with the surgical management of tumors with rare transmodiolar or transmacular extension and outcomes after cochlear implantation (CI). METHODS: This retrospective case series included nine patients with either primary intralabyrinthine tumors or secondary invasion of the inner ear from the internal auditory canal. The primary endpoint with CI, performed in six patients, was word recognition score at 65 dB SPL (sound pressure level). Secondary endpoints were intra- and postoperative electrophysiological parameters, impedance measures, the presence of a wave V in the electrically evoked (via the CI) auditory brainstem responses, the specifics of postoperative CI programming, and adverse events. RESULTS: Hearing rehabilitation with CI in cases of transmodiolar tumor growth could be achieved only with incomplete tumor removal, whereas tumors with transmacular growth could be completely removed. All six patients with CI had good word recognition scores for numbers in quiet conditions (80-100% at 65 dB SPL, not later than 6 to 12 months post CI activation). Four of these six patients achieved good to very good results for monosyllabic words within 1-36 months (65-85% at 65 dB SPL). The two other patients, however, had low scores for monosyllables at 6 months (25 and 15% at 65 dB SPL, respectively) with worsening of results thereafter. CONCLUSIONS: Cochleovestibular schwannomas with transmodiolar and transmacular extension represent a rare entity with specific management requirements. Hearing rehabilitation with CI is a principal option in these patients.

What factors influence treatment decision making in acoustic neuroma? Our experience on 103 cases.

Acoustic Neuromas (AN) are benign tumors of the vestibulocochlear nerve with symptomatology that includes unilateral sensorineural hearing loss, tinnitus, dizziness, facial and/or trigeminal neuropathy. There are different treatment options of AN: watchful waiting, microsurgical resection, stereotactic radiation and the choice depends by many variables such age, health and hearing of patients as size, location and growing status of tumor. The objective of this retrospective study is to better understand the differences in demographic, hearing status, symptoms, tumor characteristics in patients affected by AN presenting at our clinic and analyze the factors that influence the therapeutic choice. One-hundred three patients affected by AN were included in the study. All subjects underwent a detailed clinical interview and audio-vestibular examination, and Magnetic Resonance Imaging (MRI). Tumor status, growing or stable was estimated comparing new size to any previous MRI with at least a 6-month interval. Descriptive statistics were used for clinical and demographic features of patients. Therapeutic choices related to subjective symptoms were assessed with the chi-square test. Treatment options in our sample included watchful waiting, surgical resection and stereotactic radiosurgery. Overall, 17 patients (16.5%) pursued surgical resection via the retrosigmoid approach, 3 patients (2.9%) were treated with gamma knife stereotactic radiosurgery and 83 patients (80.6%) underwent watchful waiting. The decision-making process for AN treatment was based on size of tumor, age, and hearing loss; a statistically significant difference was found at Z test about size of tumor and PTA of patients that underwent retrosigmoid surgery. No statistically significant difference was found at chi-square test between the type of treatment and symptoms (p=0.719). The analysis of the data showed that the main elements taken into consideration for surgery were the size of tumor (p<0.000004) and, secondly, the PTA threshold; the latter may be due to the fact that patients with bigger tumors had greater hearing impairment (p<0.001). Disease progression influenced the therapeutic decision making with a positive correlation between tumor progression and surgery (p<0.001). In our sample, active surveillance was the most adopted option for small tumor, slow growth and old age. Microsurgical resection was the preferred treatment in patients with large tumors, hearing deterioration and rapid growth. Stereotactic radiation has been proposed in a few cases of elderly patients with slow growing tumor and mild hearing loss.

Quality of life related to symptomatic outcomes in patients with vestibular schwannomas: A Canadian Centre perspective.

Patients with vestibular schwannomas (VS) typically present with hearing loss and tinnitus as well as variable cranial nerve dysfunctions. Surgical resection, stereotactic radiotherapy and/or conservative management employing serial magnetic resonance or computed tomography imaging serve as the main treatment options. Quality of life (QoL) may be impacted by the extent of tumour burden and exacerbated or relieved by treatment. Subjective assessment and quality of life inventories provide valuable information in client centered approaches with important implications for treatment. The intention of QoL measurements affecting VS patients within a clinical setting is to facilitate discussions regarding treatment options and objectively evaluate patient- centered clinical outcomes in a naturalistic setting.

An international comparison of diagnostic and management strategies for vestibular schwannoma.

OBJECTIVE: To compare international diagnostic and management strategies for vestibular schwannoma (VS). METHODS: A web-based questionnaire was sent to 130 otolaryngologists, mainly identified through the European Skull Base Society. It contained questions on general information including guideline usage as well as questions on diagnosis (focussing on selection of patients for MRI) and management of VS, including case scenarios. Descriptive statistics were reported. RESULTS: Thirty-six otolaryngologists working in 11 different countries completed the questionnaire (response rate: 28%). Guidelines for diagnosis and management of VS are used by 44% and 42% of respondents, respectively. In the diagnostic strategy for VS, different types and combinations of audiovestibular function tests are used when deciding whether a patient should undergo an MRI. Respondents apply 18 different definitions of asymmetrical hearing loss. Variation was also apparent from reported considerations on management of VS. Most respondents (84%) prefer a wait-and-scan strategy in case of a small intrameatal VS (Koos 1). Variety in management strategies increases for patients with a medium to large sized VS (Koos 2, 3 and 4). The details of each management strategy (wait-and-scan, microsurgery, stereotactic radiosurgery and fractionated radiotherapy) also differ among respondents. CONCLUSIONS: A large variation in diagnostic and management strategies for VS was identified between respondents. More evidence and/or consensus seem warranted to reduce uncertainties for patients, and differences in outcome and costs that might result from the variety of strategies currently being applied.

[Management of vestibular schwannomas]. / Management des Vestibularisschwannoms.

Vestibular schwannomas are benign neoplasms originating from the Schwann cells of the vestibular vestibular nerve of the vestibulocochlear nerve, and rarely from the pars cochlearis. These are tumors that are in contact with the nerve but do not bind the fibers. Benign neoplasms of the Schwann cells of the auditory and equilibrium nerves can also occur primarily in the inner ear and are referred to as intralabyrinthine schwannomas (ILS). Vestibular schwannomas represent 6-7 % of all intracranial and 90 % of cerebellopontine angle tumors. Bilateral occurrence occurs in < 5 % of cases, and then corresponds to type 2 neurofibromatosis. The first symptom is often a unilateral hearing loss. It may then lead to balance disorders, tinnitus, facial paralysis and other impairments. Diagnosis is audiological, vestibular and imaging. Magnetic resonance imaging currently represents the gold standard. Management chooses between an observational strategy and surgery, depending on tumor size, age, and other factors. The possible access routes offer different advantages and disadvantages; the potential complications include the liquorrhoea. Radiation therapy is possible in special cases, and drug therapies are also being tested. In the rehabilitation of the hearing function, in addition to a CROS or BICROS restoration, the cochlear implant has been used with good success. The impact on quality of life is largely determined by hearing impairment, balance disorders, tinnitus, and possibly headache, which must be considered in patient consultation and long-term care.

[Spontaneous involution of Vestibular Schwannomas]. / Schwannomes vestibulaires spontanément involutifs.

The majority of vestibular schwannomas have a stable evolution or a progression of size. Some publications report cases of spontaneous involution. We report here the clinical and radiological characteristics of this type of tumor observed in 14 patients showing a spontaneous tumor involution of their vestibular schwannoma. The results showed an incidence of 8 % with an average tumor regression of 50 %. Functionally, a hearing loss was found in all patients, without correlation with the duration of the observational follow-up. There are spontaneously involutive vestibular schwannomas, but tumor regression does not prevent the deterioration of hearing. Patients should be considered as a subset of those receiving observational management.

La majorité des schwannomes vestibulaires ont une évolution stable ou une progression de taille. Quelques publications rapportent des cas d'involution spontanée. Nous rapportons ici les caractéristiques cliniques et radiologiques de ce type de tumeur observée chez 14 patients montrant une involution tumorale spontanée de leur schwannome vestibulaire. Les résultats ont montré une incidence de 8 % avec une régression tumorale moyenne de 50 %. Sur le plan fonctionnel, une perte auditive a été mise en évidence chez l'ensemble des patients, sans corrélation avec la durée du suivi observationnel. Il existe des schwannomes vestibulaires spontanément involutifs, mais la régression tumorale n'empêche pas la dégradation de l'audition. Les patients doivent être considérés comme un sous-groupe de ceux bénéficiant d'une prise en charge observationnelle.

[Surgical approach to resection of vestibular schwannomas following stereotactic radiological treatment. Surgical outcomes and morphological changes in tumors after radiotherapy]. / Khirurgicheskaia taktika pri udalenii vestibuliarnykh shvannom posle stereotaksicheskogo radiologicheskogo lecheniia. Rezul'taty operatsii i morfologicheskie izmeneniia v opukholiakh posle oblucheniia.

INTRODUCTION: Radiation therapy of small vestibular schwannomas is quite often used as an effective alternative to surgical treatment. At the same time, 2-10% of patients are detected with radioresistant tumors progressing to varying degrees, which is associated with continued tumor growth. In these cases, a decision on surgical resection or re-irradiation of the tumor is made depending on the neurological symptoms, patient's somatic status, and neuroimaging data. Surgical outcomes and intraoperative findings in pre-irradiated patients have been poorly represented in the literature, for which reason we decided to conduct this study. The paper presents a series of patients with vestibular schwannomas who underwent surgical removal of the tumor after radiotherapy. MATERIAL AND METHODS: A total of 39 patients with vestibular schwannomas after radiotherapy underwent surgery at the Burdenko Neurosurgical Institute in 2007-2017. Of these, 22 patients had a tumor removed after a previously performed combined surgical and radiotherapy treatment (group I), and 17 patients underwent tumor resection after previous radiological treatment (group II). The surgical outcomes were studied depending on various factors, and an analysis of the morphological changes in vestibular schwannomas after radiological treatment was carried out. RESULTS: In group I, the tumor was resected totally in 18% of patients, almost totally in 5% of patients, subtotally in 68% of patients, and partially in 9% of patients. In group II, the tumor was resected totally in 6% of patients, almost totally in 12% of patients, subtotally in 76% of patients, and partially in 6% of patients. We found that post-radiation changes in patients undergoing surgery led to an increase in the response of neurovascular structures to surgical intervention, development of pronounced fibrosis around tumors, and changes in the structure of tumors that became more solid. As a result, surgical morbidity increased, and the patient's quality of life after surgery deteriorated.

Cerebellopontine angle tumors in young children, displaying cranial nerve deficits, and restricted diffusion on diffusion-weighted imaging: a new clinical triad for atypical teratoid/rhabdoid tumors.

Atypical teratoid/rhabdoid tumors (AT/RT) of the central nervous system (CNS) are rare, highly malignant neoplasms that carry a poor prognosis. Even with prompt diagnosis, gross total resection and early initiation of intensive adjuvant therapy, the majority of patients will succumb within 9-12 months of diagnosis. The CPA location in children harbors lesions along a wide spectrum varying from benign to highly malignant. Imaging features of lesions within the CPA that aid the diagnostic process will help to initiate early treatment in higher-grade lesions. We report three cases, in very young children, all with cranial nerve deficits, who displayed CPA lesions with restricted diffusion on diffusion-weighted imaging (DWI) with pathology confirming AT/RT. We propose that in young children with a CPA tumor diffusion-weighted imaging should be routinely evaluated to aid in prompt management. In addition, the diagnosis of AT/RT should be highly suggestive in infants presenting with cranial nerve findings as well as DWI restricted diffusion within the CPA.

Development of an evidence-based decision pathway for vestibular schwannoma treatment options.

OBJECTIVE: To integrate multiple sources of clinical information with patient feedback to build evidence-based decision support model to facilitate treatment selection for patients suffering from vestibular schwannomas (VS). METHODS: This was a mixed methods study utilizing focus group and survey methodology to solicit feedback on factors important for making treatment decisions among patients. Two 90-minute focus groups were conducted by an experienced facilitator. Previously diagnosed VS patients were recruited by clinical investigators at the University of Pittsburgh Medical Center (UPMC). Classical content analysis was used for focus group data analysis. Providers were recruited from practices within the UPMC system and were surveyed using Delphi methods. This information can provide a basis for multi-criteria decision analysis (MCDA) framework to develop a treatment decision support system for patients with VS. RESULTS: Eight themes were derived from these data (focus group + surveys): doctor/health care system, side effects, effectiveness of treatment, anxiety, mortality, family/other people, quality of life, and post-operative symptoms. These data, as well as feedback from physicians were utilized in building a multi-criteria decision model. DISCUSSION: The study illustrated steps involved in the development of a decision support model that integrates evidence-based data and patient values to select treatment alternatives. CONCLUSIONS: Studies focusing on the actual development of the decision support technology for this group of patients are needed, as decisions are highly multifactorial. Such tools have the potential to improve decision making for complex medical problems with alternate treatment pathways.

[Diagnosis and Management of Vestibular Schwannomas - An Interdisciplinary Challenge]. / Diagnostik und Therapie des Vestibularisschwannoms ­ eine interdisziplinäre Herausforderung.

Vestibular schwannomas expand slowly in the internal auditory canal, in the cerebellopontine angle, inside the cochlear and the labyrinth. Larger tumors can displace and compress the brainstem. With an annual incidence of 1:100 000 vestibular schwannoma represent 6-7% of all intracranial tumors. In the cerebellopontine angle they are by far the most neoplasm with 90% of all lesions located in this region. Magnetic resonance imaging (MRI), audiometry, and vestibular diagnostics are the mainstays of the clinical workup for patients harboring tumors. The first part of this paper delivers an overview of tumor stages, the most common grading scales for facial nerve function and hearing as well as a short introduction to the examination of vestibular function.Upholding or improving quality of life is the central concern in counseling and treating a patient with vestibular schwannoma. Preservation of neuronal function is essential and the management options - watchful waiting, microsurgery and stereotactic radiation - should be custom-tailored to the individual situation of the patient. Continuing interdisciplinary exchange is important to monitor treatment quality and to improve treatment results. Recently, several manuscripts and reviews have been published on the topic of vestibular schwanomma. On the occasion of the 88th annual meeting of the German Society of Oto-rhino-laryngology, Head and Neck surgery a special volume of the journal "HNO" will be printed. Hence this presentation has been designed to deviate from the traditional standard which commonly consists of a pure literature review. The current paper was conceptually woven around a series of interdisciplinary cases which outlines examples for every stage of the disease that show characteristic results for management options to date. Systematic clinical decision pathways have been deduced from our experience and from results reported in the literature. These pathways are graphically outlined after the case presentations. Important criteria for decision making are size and growth rate of the tumor, hearing of the patient and the probability of total tumor resection with preservation of hearing and facial nerve function, age and co-morbidity of the patient, best possible control of vertigo and tinnitus and last but not least the patient's preference and choice. In addition to this, the experience and the results of a given center with each treatment modality will figure in the decision making process.We will discuss findings that are reported in the literature regarding facial nerve function, hearing, vertigo, tinnitus, and headache and reflect on recent studies on their influence on the patient's quality of life. Vertigo plays an essential role in this framework since it is an independent predictor of quality of life and a patient's dependence on social welfare.Pathognomonic bilateral vestibular schwannoma that occur in patients suffering from neurofibromatosis typ-2 (NF2) differ from spontaneous unilateral tumors in their biologic behavior. Treatment of neurofibromatosis type-2 patients requires a multidisciplinary team, especially because of the multitude of separate intracranial and spinal lesions.Off-label chemotherapy with Bevacizumab can stabilize tumor size of vestibular schwannomas and even improve hearing over longer periods of time. Hearing rehabilitation in NF2 patients can be achieved with cochlea and auditory brainstem implants.

Diagnosis and Management of Hereditary Meningioma and Vestibular Schwannoma.

Bilateral vestibular schwannomata and meningiomata are the tumours most commonly associated with neurofibromatosis type II (NF2). These tumours may also be seen in patients with schwannomatosis and familial meningioma, but these phenotypes are usually easy to distinguish. The main diagnostic challenge when managing these tumours is distinguishing between sporadic disease which carries low risk of subsequent tumours or NF2 with its associated morbidities and reduced life expectancy. This chapter outlines some of the diagnostic and management considerations along with associated evidence.

Pediatric neurofibromatosis type 2: clinical and molecular presentation, management of vestibular schwannomas, and hearing rehabilitation.

OBJECTIVE: This study aims to describe the clinical and molecular presentation of pediatric neurofibromatosis type 2 (NF2) and the subsequent management of vestibular schwannomas (VS) and hearing rehabilitation. METHODS: This is a single-center retrospective study of neurofibromatosis type 2 diagnosed before the age of 18 years old from 1997. Natural history of vestibular schwannomas and surgical outcomes were evaluated using volumetric MRI, hearing, and facial nerve assessment. Patients included in chemotherapy protocols were excluded. RESULTS: From a database of 80 patients followed up for NF2 on a regular basis, 25 patients were eligible (11 sporadic cases, 14 inherited in five families). The mean age at diagnosis was 11.6 years old. The average clinical follow-up was 6.5 years. NF2 mutation was identified in 81 % of the probands. The average growth rate based on the maximum linear diameter (DGR) was 1.68 mm/year (n = 33, average follow-up 4.22 years) and 545 mm3/year in volumetric assessment (VGR) for VS larger than 1 cm (n = 21, average follow-up 3.4 years). In unoperated ears, hearing was stable in about 50 % of ears. The mean change in dB HL was 9.5 dB/year for pure-tone average and 3.5 for speech-recognition threshold (n = 34, 5.5 years 1-12). Eight children required removal through a translabyrinthine approach (mean follow-up was 4.5 years), six patients were operated on for hearing preservation (mean postoperative follow-up 4.3 years). Six patients were eligible for hearing rehabilitation with cochlear implantation (I), and five received placement of an auditory brainstem implant. CONCLUSION: Early diagnosis and treatment of small growing VS should be carefully discussed considering familial history and possible rehabilitation with a CI.