Vestibular schwannoma (acoustic neuroma)
A vestibular schwannoma is a benign growth on the balance and hearing nerve, deep in the skull. It is rarely dangerous, so many are simply watched with scans; growing tumours are treated with either an operation or a single precisely focused dose of radiation, and people with the inherited condition NF2, who develop tumours on both sides, can be helped by the drug bevacizumab.
Overview
Vestibular schwannoma is a WHO grade 1 nerve sheath tumour arising from Schwann cells of the vestibular division of the eighth cranial nerve in the internal auditory canal and cerebellopontine angle. Sporadic tumours are unilateral and carry somatic NF2 inactivation; bilateral tumours define NF2-related schwannomatosis, the germline condition renamed in 2022 from neurofibromatosis type 2, which also causes meningiomas, spinal schwannomas and ependymomas. Presentation is progressive unilateral hearing loss, tinnitus and imbalance; large tumours compress the brainstem and cause hydrocephalus. Diagnosis is by MRI; biopsy is not needed.
Management has three arms. Observation with serial MRI is appropriate for small tumours because many do not grow, and hearing can be followed. Microsurgery through retrosigmoid, translabyrinthine or middle fossa approaches removes the tumour with facial nerve monitoring; it is favoured for large tumours with brainstem compression and in younger patients. Stereotactic radiosurgery at marginal doses of about 12 to 13 Gy controls the great majority of small and medium tumours with low facial nerve risk, and fractionated stereotactic radiotherapy or proton therapy is used for larger lesions. Leksell treated the first acoustic neuroma with the Gamma Knife in 1969, and radiosurgery has since displaced surgery for most small growing tumours. Hearing preservation is the outcome that most separates the options and depends on pre-treatment hearing and tumour size.
In NF2-related schwannomatosis the aim is to preserve hearing and function over a lifetime of multiple tumours. Bevacizumab produced tumour shrinkage and hearing improvement in progressive NF2 tumours (Plotkin and colleagues, NEJM 2009) and is used off label; brigatinib showed activity across NF2-related tumour types in the INTUITT-NF2 platform trial (2024), and cochlear or auditory brainstem implants restore hearing after bilateral loss. Radiosurgery is used more cautiously in NF2 because control rates are lower and there is a small concern about malignant change in a germline-predisposed nerve.
State of the art
- Radiosurgery controls most small vestibular schwannomas with a single outpatient treatment and has displaced surgery as the default for growing small tumours.
- Watchful waiting is legitimate first-line management because a large share of tumours never grow.
- Bevacizumab and, more recently, brigatinib are the first drugs to shrink NF2-related tumours, turning a surgical disease into one partly managed medically.
Red cards
From the labels and guidelines behind the standard of care. Your team's thresholds win.- Emergency services nowBowel perforation
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
- Emergency services nowFainting or palpitations
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
- Good to knowAnti-VEGF class toxicities (hypertension, proteinuria, bleeding, perforation)
The shared side effects of drugs that block blood vessel growth (bevacizumab, ramucirumab and VEGFR kinase inhibitors): high blood pressure, protein leaking into the urine, nosebleeds and more serious bleeding, slow wound healing, and rarely holes in the bowel.
- Good to knowRadiation necrosis (brain)
Death of brain tissue months to years after radiosurgery or high-dose brain radiotherapy, which can look exactly like tumour growing back on a scan.
- Good to knowVenous thromboembolism (VTE)
Blood clots in the leg veins or lungs. Cancer makes blood clot more easily and some treatments (IMiDs, anti-VEGF drugs, hormone therapy, central lines, surgery) add risk; clots are the second commonest cause of death in cancer patients after the cancer itself.
See all on the product pages:BevacizumabBrigatinib·Printable cards in the navigator
Anatomy and lymph node drainage
- Frontal lobe (glioblastoma commonest)
- Temporal lobe
- Corpus callosum (butterfly glioma)
- Lower-grade IDH-mutant glioma
- Cerebellum (medulloblastoma)
- Brainstem and spinal cord (diffuse midline glioma, cord tumours)
- Ventricles and ependymal lining (ependymoma)
- Sella and pituitary (pituitary tumours, craniopharyngioma)
- Deep periventricular tissue (CNS lymphoma)
- Meninges and convexity (meningioma)
- Grey-white junction (brain metastases)
- Cerebellopontine angle and eighth nerve (vestibular schwannoma)
- Pineal and suprasellar midline (germ cell tumours)
Gliomas infiltrate along white matter and can cross the corpus callosum, medulloblastoma sits in the cerebellum, and CNS lymphoma favours deep periventricular tissue; none spread through lymph nodes.
- Frontal lobe (glioblastoma commonest)
- Temporal lobe
- Corpus callosum (butterfly glioma)
- Lower-grade IDH-mutant glioma
- Cerebellum (medulloblastoma)
- Brainstem and spinal cord (diffuse midline glioma, cord tumours)Cerebellopontine angle schwannoma with brainstem compression
- Ventricles and ependymal lining (ependymoma)
- Sella and pituitary (pituitary tumours, craniopharyngioma)
- Deep periventricular tissue (CNS lymphoma)
- Meninges and convexity (meningioma)
- Grey-white junction (brain metastases)
- Cerebellopontine angle and eighth nerve (vestibular schwannoma)Sporadic unilateral vestibular schwannoma (somatic NF2 loss) · NF2-related schwannomatosis with bilateral vestibular schwannoma · Intracanalicular schwannoma (confined to the internal auditory canal) · Cerebellopontine angle schwannoma with brainstem compression · Cystic vestibular schwannoma (faster growth, less predictable radiosurgery response)
- Pineal and suprasellar midline (germ cell tumours)
No conventional lymphatics: gliomas spread along white matter tracts and, rarely, through cerebrospinal fluid; medulloblastoma can seed the spine.
Same organ: Glioma & glioblastoma, Primary CNS lymphoma, Medulloblastoma, Paediatric low-grade glioma, Diffuse midline glioma, H3 K27-altered (including DIPG), Atypical teratoid/rhabdoid tumour (ATRT), Ependymoma, Craniopharyngioma, Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma, Brain and spinal cord tumours (all types), Astrocytoma, IDH-mutant (grades 2 to 4), Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, Paediatric high-grade glioma (excluding diffuse midline glioma), Meningioma, Brain metastases (secondary brain tumours), Central nervous system germ cell tumours (germinoma and non-germinomatous), Spinal cord tumours (intramedullary and intradural)
A benign tumour found in roughly one in a thousand people over a lifetime, presenting with one-sided hearing loss; sporadic tumours are single, while NF2-related schwannomatosis causes bilateral tumours from young adulthood and is the hard form of the disease.
- MRIStandard of care
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Observation with MRI and audiometry, typically yearly; treatment on documented growth or hearing decline.
Stereotactic radiosurgery (Gamma Knife, CyberKnife or linac) at about 12 to 13 Gy, or fractionated stereotactic radiotherapy; microsurgery in younger patients or by preference.
Microsurgical resection with facial nerve monitoring, sometimes deliberately subtotal followed by radiosurgery to the remnant; shunting for hydrocephalus.
Bevacizumab (off label, phase 2 evidence) to shrink tumours and improve hearing; brigatinib on INTUITT-NF2 evidence; hearing rehabilitation with cochlear or auditory brainstem implants; surgery and radiosurgery used selectively.
Subtypes & biomarkers
top- Sporadic unilateral vestibular schwannoma (somatic NF2 loss)
- NF2-related schwannomatosis with bilateral vestibular schwannoma
- Intracanalicular schwannoma (confined to the internal auditory canal)
- Cerebellopontine angle schwannoma with brainstem compression
- Cystic vestibular schwannoma (faster growth, less predictable radiosurgery response)
- Tumour size and growth on serial MRI (Koos grade)
- Pure-tone audiometry and word recognition (hearing class)
- Germline NF2 testing when bilateral, young or with other schwannomas or meningiomas
- Facial nerve function (House-Brackmann grade) before and after treatment
How often this target appears
- 1894Ballance removes an acoustic neuroma
Among the first successful removals of a cerebellopontine angle tumour.
- 1917Cushing publishes his monograph on acoustic neuromas
- 1961House introduces the translabyrinthine microsurgical approach
The operating microscope transforms mortality and facial nerve outcomes.
- 1969Leksell treats the first acoustic neuroma with the Gamma Knife
- 1993NF2 gene cloned
Trofatter and Rouleau identify the merlin tumour suppressor on chromosome 22.
- 2009Bevacizumab shrinks NF2 vestibular schwannomas and improves hearing
Plotkin and colleagues (NEJM).
- 2022Neurofibromatosis type 2 renamed NF2-related schwannomatosis
- 2024INTUITT-NF2: brigatinib active across NF2-related tumours
Plotkin and colleagues (NEJM) report the first platform trial in the condition.
Dated changes read from the records linked to this cancer: approvals, regulatory steps, reported trials, guideline versions and milestones. Newest first; no date is inferred.
All 9 changes by month →- 2026-09-17This recordVestibular schwannoma (acoustic neuroma)Facts on this page last checked
When this page itself was last checked or edited.
- 2024MilestoneBrigatinibINTUITT-NF2: brigatinib active across NF2-related tumours
Plotkin and colleagues (NEJM) report the first platform trial in the condition.
- 2022MilestoneVestibular schwannoma (acoustic neuroma)Neurofibromatosis type 2 renamed NF2-related schwannomatosis
A milestone in how this cancer is treated.
- 2009MilestoneBevacizumabBevacizumab shrinks NF2 vestibular schwannomas and improves hearing
Plotkin and colleagues (NEJM).
- 1993MilestoneVestibular schwannoma (acoustic neuroma)NF2 gene cloned
Trofatter and Rouleau identify the merlin tumour suppressor on chromosome 22.
- 1969MilestoneGamma KnifeLeksell treats the first acoustic neuroma with the Gamma Knife
A milestone in how this cancer is treated.
What is in development for Vestibular schwannoma (acoustic neuroma), drawn from the whole corpus: 1 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Technologies being tested · 1
Open problems and what is being done
No approved drug for NF2-related schwannomatosis; bevacizumab is off label and its effect wanes.
Predicting which small tumours will grow, so that the rest can be left alone.
Preserving hearing: each option trades tumour control against hearing and facial nerve function.
Rare malignant transformation after radiosurgery in NF2 is not well quantified.
Trials
topTrials recruiting now
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Expert centres
topExpert centres
Houston · cancer center | United States | 0 | 6,724 | 95,007 | #2 | ||
Seoul · hospital | South Korea | none recorded | 0 | 1,312 | 17,172 | #3 | |
Rochester, MN · hospital | United States | 0 | 4,511 | 44,748 | #5 | ||
Villejuif · cancer center | France | none recorded | 0 | 1,855 | 31,182 | #6 | |
Seoul · hospital | South Korea | none recorded | 0 | 1,607 | 24,676 | #8 | |
Baltimore · cancer center | United States | 0 | 2,955 | 41,449 | #10 | ||
Berlin · university | Germany | none recorded | 0 | 1,563 | 17,749 | #12 | |
Boston · hospital | United States | 0 | 3,582 | 54,857 | #16 | ||
Cleveland · hospital | United States | 0 | 2,264 | 29,412 | #20 | ||
Seoul · hospital | South Korea | none recorded | 0 | 464 | 3,248 | #22 | |
Stanford · university | United States | 0 | 3,000 | 50,162 | #30 | ||
San Francisco · cancer center | United States | 0 | 2,800 | 46,704 | #33 | ||
Stockholm · university | Sweden | none recorded | 0 | 987 | 12,440 | #39 | |
London · hospital | United Kingdom | none recorded | 0 | 2,376 | 28,940 | - | |
Utrecht · cancer center | Netherlands | none recorded | 0 | 1,422 | 20,323 | - |
These are the things we can measure; they are not a ranking of quality. Each column is a field on the institution record or a count over what OnCo has linked; a centre that treats many patients with Vestibular schwannoma but is thinly recorded here will look small.
Not known: OnCo holds no case-volume or outcome figures for centres, so none are shown. Where a national audit or registry publishes them, the centre's page links to it. Default order: Newsweek rank, then trials for this cancer, then research output.
Questions to ask
topQuestions to ask your oncologist about Vestibular schwannoma
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Tumour size and growth on serial MRI, Pure-tone audiometry and word recognition, Germline NF2 testing when bilateral, young or with other schwannomas or meningiomas, Facial nerve functionbefore and after treatment), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Sporadic unilateral vestibular schwannoma, NF2-related schwannomatosis with bilateral vestibular schwannoma, Intracanalicular schwannoma.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Small tumour, stable or good hearing
- For my situation (small tumour, stable or good hearing), which of the standard options do you recommend and why?Why: Guideline options include: Observation with MRI and audiometry, typically yearly; treatment on documented growth or hearing decline.
Growing small or medium tumour
- For my situation (growing small or medium tumour), which of the standard options do you recommend and why?Why: Guideline options include: Stereotactic radiosurgery (Gamma Knife, CyberKnife or linac) at about 12 to 13 Gy, or fractionated stereotactic radiotherapy; microsurgery in younger patients or by preference.
Large tumour with brainstem compression or hydrocephalus
- For my situation (large tumour with brainstem compression or hydrocephalus), which of the standard options do you recommend and why?Why: Guideline options include: Microsurgical resection with facial nerve monitoring, sometimes deliberately subtotal followed by radiosurgery to the remnant; shunting for hydrocephalus.
NF2-related schwannomatosis, progressive tumour or falling hearing
- For my situation (nf2-related schwannomatosis, progressive tumour or falling hearing), which of the standard options do you recommend and why?Why: Guideline options include: Bevacizumab (off label, phase 2 evidence) to shrink tumours and improve hearing; brigatinib on INTUITT-NF2 evidence; hearing rehabilitation with cochlear or auditory brainstem implants; surgery and radiosurgery used selectively.
- Am I a candidate for Bevacizumab, Brigatinib, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Brigatinib, Bevacizumab, Stereotactic radiosurgery (Gamma Knife, CyberKnife, linac SRS)?Why: Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “No approved drug for NF2-related schwannomatosis; bevacizumab is off label and its effect wanes”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Predicting which small tumours will grow, so that the rest can be left alone”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
Newly diagnosed? Read the first 60 days with Vestibular schwannoma, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
10targets
2drugs
2companies
3terms
1Latest papers
topQuery for this cancer: (TITLE:"Vestibular schwannoma" OR ABSTRACT:"Vestibular schwannoma" OR TITLE:"acoustic neuroma" OR ABSTRACT:"acoustic neuroma" OR TITLE:"Acoustic neuroma" OR ABSTRACT:"Acoustic neuroma" OR TITLE:"NF2-related schwannomatosis" OR ABSTRACT:"NF2-related schwannomatosis" OR TITLE:"Eighth nerve tumour" OR ABSTRACT:"Eighth nerve tumour") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Vestibular schwannoma (acoustic neuroma), not a curated reading list.
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