Highlights & Basics
- Vestibular schwannoma is a histologically benign, generally slow-growing tumor that may remain stable for long periods on follow-up magnetic resonance imaging scans after initial detection.
- Progressive unilateral sensorineural hearing loss is the most common presenting symptom, followed by tinnitus and intermittent dizziness; hemifacial numbness or pain may occur less frequently.
- Larger tumors may cause headaches, ataxia, and obstructive hydrocephalus and may rarely be life threatening.
- Diagnosis is confirmed by gadolinium-enhanced magnetic resonance imaging of the head.
- Management includes observation (active surveillance), focused radiation (stereotactic radiosurgery or radiation therapy), or surgery.
- Decision-making regarding tumor management is mainly based on the presenting tumor size, detection of growth during active surveillance, provider bias, baseline patient function (e.g., hearing status), and patient preference.
Quick Reference
History & Exam
Key Factors
asymmetric hearing loss
progressive episodes of dizziness
tinnitus
Other Factors
difficulty localizing sounds
headache
facial numbness
facial weakness
diplopia on lateral gaze
nystagmus
loss of balance and coordination difficulties
slower blink
swallowing difficulties
gait disturbances
hydrocephalus
papilledema
increased intracranial pressure
Diagnostics Tests
1st Tests to Order
audiogram
T1-weighted MRI using gadolinium-based contrast
contrast-enhanced head CT
Other Tests to consider
auditory brainstem reflexes
Treatment Options
ongoing
small tumor (Koos grades 1-2)
observation
focused radiation or surgery
salvage radiation or surgery
medium tumor (Koos grades 3-4, tumor size <3 cm)
focused radiation or surgery
salvage radiation or surgery
Definition
Classifications
Koos classification system
- Grade 1: intracanalicular tumor
- Grade 2: small tumor with protrusion into the cerebellopontine angle; no contact with the brainstem
- Grade 3: tumor occupying the cerebellopontine cistern with no brainstem displacement
- Grade 4: large tumor with brainstem and cranial nerve displacement.
American Academy of Otolaryngology-Head and Neck Surgery (AAOHNS) hearing classification system
- Class A: PTA ≤30 dB HL, WRS ≥70%
- Class B: PTA >30 and ≤50 dB HL, WRS ≥50%
- Class C: PTA >50 dB HL, WRS ≥50%
- Class D: PTA any level, WRS <50%.
House-Brackmann (HB) facial nerve grading scale
- Grade 1: normal facial movement
- Grade 2: slight facial weakness, only perceptible on close inspection
- Grade 3: obvious but not disfiguring weakness, forehead shows moderate movement, eye with complete closure on maximal effort
- Grade 4: disfiguring asymmetry, no forehead movement, incomplete eye closure
- Grade 5: only slightly perceptible facial movement
- Grade 6: complete facial paralysis, no perceptible facial movement.
Vignette
Common Vignette 1
Common Vignette 2
Other Presentations
Epidemiology
Etiology
Pathophysiology
Diagnostic Approach
History
- Personal or family history of other brain, spinal, or neurogenic tumors such as schwannomas or meningiomas
- Family history of neurofibromatosis-related schwannomatosis
- History of prior radiation exposure such as head or neck therapeutic radiation for cancer
- Most people with sporadic vestibular schwannomas do not have identifiable risk factors.
- Onset, duration, and laterality of hearing loss (e.g., sudden, slowly progressive over years) or tinnitus
- Presence and character of dizziness, imbalance, or vertigo
- History of facial spasm or facial nerve weakness; specifically asking about prior Bell palsy may be helpful in eliciting relevant history
- Presence, distribution, severity, and laterality of facial numbness or facial pain
- Presence and character of headaches or vision loss.
- Conditions associated with increased anesthetic or wound-healing risk should be elicited such as hypertension, diabetes, smoking, malnutrition, or cardiac disease
- Medications that increase risk of hemorrhage such as antiplatelet therapy or anticoagulation
- Pregnancy, as this impacts tumor management decision-making and may potentially impact natural history of disease.
Physical exam
- Eye examination to examine for spontaneous or gaze-induced nystagmus
- Examination of facial sensation and distribution of any potential facial hypoesthesia
- Presence of facial nerve weakness, spasm, or synkinesis; diminished fast or casual blink is among the most sensitive measures of early facial nerve weakness
- Otoscopy to examine for other visible causes of hearing loss
- Romberg and tandem gait to assess for cerebellar findings
- Fundoscopic examination for papilledema if concern for hydrocephalus exists.
Investigations
Risk Factors
History & Exam
Tests
Differential Diagnosis
Meningioma
Differentiating Signs/Symptoms
- Highly dependent on the location and size of the tumor.
- Hearing loss is a less prominent symptom.
Differentiating Tests
- Meningiomas of the cerebellopontine angle classically present eccentric to the porus acusticus, often have limited or tapering extension into the meatus, exhibit dural tails along the posterior petrous dura, typically exhibit more homogenous enhancement, and may acquire intratumoral bone formation or hyperostosis at the base.
- CT and MRI findings show a large angle between the tumor and dura not centered over the internal acoustic meatus. Also, imaging displays bone hyperostosis, no internal acoustic meatus enlargement, and no or less extension into the internal acoustic meatus.
Differentiating Signs/Symptoms
- Epidermoid cysts are congenital, noncancerous lesions that contain an outer cyst wall of keratinizing squamous epithelium and a core of keratin debris. Hearing loss is often a less prominent symptom.
Differentiating Tests
- Epidermoids have similar signal characteristics to arachnoid cysts on non-contrast T1- and T2-weighted MRI; however, they characteristically exhibit restricted diffusion on non-echo planar diffusion-weighted imaging sequences.
Differentiating Signs/Symptoms
- Facial nerve paresis or facial spasm is a prominent symptom, occurring in at least half of people; hearing loss also commonly occurs.
Differentiating Tests
- CT and MRI results are similar to those of vestibular schwannoma; however, nodular enhancement often extends into the labyrinthine segment of the facial nerve and geniculate ganglion on MRI; CT may show widening of the fallopian canal.
Differentiating Signs/Symptoms
- Clinically associated with more prominent facial numbness and less severe hearing loss.
Differentiating Tests
- CT and MRI display a dumbbell-shaped mass spanning the posterior and middle fossae and involving Meckel cave. The tumor enhancement does not extend into the internal auditory canal.
Treatment Approach
Management decisions
- Approximately half of small vestibular schwannomas exhibit growth following diagnosis within the first 3-5 years, while approximately half remain stable. Studies using volumetric analysis, a more sensitive measure for changes in tumor size, identify higher rates of growth compared to studies using linear diameter assessments.[40]
- There are no strong predictors for future growth, aside from larger tumor size at diagnosis.
- Progression of a patient's symptoms is not a reliable indicator of growth.
- Smaller tumors carry lower risk from treatment (fewer risks to facial nerve and hearing).
- Younger patients with fewer comorbidities may respond better to treatment.
- Tumor treatment is unlikely to improve hearing, tinnitus, headache, dizziness, or quality of life.
Radiation and surgery
Small tumor (Koos grades 1-2)
Medium tumor (Koos grades 3-4; tumor size <3 cm)
Large tumor (Koos grades 3-4; tumor size >3 cm)
Management of tumors in the only hearing ear
Treatment Options
small tumor (Koos grades 1-2)
observation
Comments
- Small, asymptomatic tumors with normal cranial nerve function can be monitored by observation. Observation is also considered the first option in patients with small tumors with complete hearing loss.
- Clinical observation is recommended if no growth appears on serial scans. At 6 months from presentation, patients who are being observed only should have an MRI, followed by yearly scans for several subsequent years, then scans every 2 years.[58] In select cases, patients with smaller growing tumors may still be observed if reliable follow-up can be assured.[57]
focused radiation or surgery
Comments
- Some patients with normal cranial nerve function may opt for focused radiation or surgery, but these treatments can deteriorate the nerve function and are usually not recommended.[13]
- Surgery is completed with a number of approaches tailored to the patient's tumor size and degree of hearing loss, and to the surgeon's experience. In general, a middle fossa approach is completed through an incision that begins in front of the tragus of the affected ear and extends superiorly onto the temporal scalp. A 5 x 5 cm craniotomy is made above the ear, essentially centered on the ear canal. This approach is typically reserved for smaller tumors confined to the internal auditory canal when hearing preservation is attempted.[64] The translabyrinthine approach is performed through an incision behind the ear, working through the mastoid portion of the temporal bone, and does not preserve hearing because the inner ear is opened when, as the name suggests, the labyrinthine bone is drilled away. Thus, this approach is often used for patients who already have poor hearing or sometimes in cases where hearing preservation is unlikely, such as with large tumors.[64] The retrosigmoid approach is completed through an incision behind the ear allowing a craniotomy or craniectomy inferior to the transverse sinus and posterior to the sigmoid sinus.[64] This approach can be used for any sized tumor, with or without the intent of hearing preservation. Almost always, the posterior aspect of the internal auditory canal must be removed with a high-speed drill to access the component of the tumor extending into this area.[65] [66] [67] Intraoperative neurophysiologic monitoring of cranial nerves, in particular the facial nerve, is considered standard of care. Monitoring of cranial nerves has changed the nature and success of surgery in terms of cranial nerve morbidity.[68] [69] [70] [71]
salvage radiation or surgery
Comments
- In approximately 5% to 10% of patients who opt for radiation therapy, tumors may grow despite treatment and may require salvage surgery.[72] Similarly, in patients selected for upfront surgical treatment, tumor recurrence may occur during follow-up, especially if less than a gross total resection is performed, and then may require follow-up salvage radiation. Generally speaking, if a person fails initial radiation treatment, then surgery is pursued. If a tumor recurs after surgery, then radiation is pursued.
- Recurrence after microsurgical resection of small tumors is approximately 1% to 3% but may be slightly higher in cases of attempted hearing preservation.
medium tumor (Koos grades 3-4, tumor size <3 cm)
focused radiation or surgery
Comments
- A majority of patients with medium-sized tumors may have vestibular/cochlear symptoms.[13] Upfront treatment with radiosurgery or microsurgery is typically recommended for such tumors, and long-term observation is usually not considered. The reason for this paradigm is that treatment outcomes are primarily driven by tumor size, and tumors >1.5 cm risk poorer outcomes if further growth occurs.[57] Stereotactic radiation carries lower risk than surgery. Nevertheless, surgery ensures complete resection of the tumor.[13]
- Surgery is completed with a number of approaches tailored to the patient's tumor size and degree of hearing loss, and to the surgeon's experience. In general, a middle fossa approach is completed through an incision that begins in front of the tragus of the affected ear and extends superiorly onto the temporal scalp. A 5 x 5 cm craniotomy is made above the ear, essentially centered on the ear canal. This approach is typically reserved for smaller tumors confined to the internal auditory canal when hearing preservation is attempted.[64] The translabyrinthine approach is performed through an incision behind the ear, working through the mastoid portion of the temporal bone, and does not preserve hearing because the inner ear is opened when, as the name suggests, the labyrinthine bone is drilled away. Thus, this approach is often used for patients who already have poor hearing or sometimes in cases where hearing preservation is unlikely, such as with large tumors.[64] The retrosigmoid approach is completed through an incision behind the ear allowing a craniotomy or craniectomy inferior to the transverse sinus and posterior to the sigmoid sinus.[64] This approach can be used for any sized tumor, with or without the intent of hearing preservation. Almost always, the posterior aspect of the internal auditory canal must be removed with a high-speed drill to access the component of the tumor extending into this area.[65] [66] [67] Intraoperative neurophysiologic monitoring of cranial nerves, in particular the facial nerve, is considered standard of care. Monitoring of cranial nerves has changed the nature and success of surgery in terms of cranial nerve morbidity.[68] [69] [70] [71]
salvage radiation or surgery
Comments
- In approximately 5% to 10% of patients who opt for radiation therapy, tumors may grow despite treatment and may require salvage surgery.[72] Similarly, in patients selected for upfront surgical treatment, tumor recurrence may occur during follow-up, especially if less than a gross total resection is performed, and then may require follow-up salvage radiation. Generally speaking, if a person fails initial radiation treatment, then surgery is pursued. If a tumor recurs after surgery, then radiation is pursued.
large tumor (Koos grade 4, tumor size >3 cm)
surgery
Comments
- With or without tumor growth on serial scans, surgery is recommended owing to the large size and potential damage to adjacent structures and resulting functional deficits. Surgery is recommended in most centers in preference to radiation, because of improvement in mass effect symptoms offered by surgery over radiation.[66] [75] Surgery to preserve hearing is not offered by most surgeons as the mass effect of such a large tumor often has already caused damage to hearing structures, although if the patient has useful hearing prior to surgery, a hearing preservation attempt is a reasonable undertaking, albeit not likely to succeed.[53] [67] Surgery should focus on improving or maintaining quality of life and attempting to preserve the nerve function.[76]
- Patients with large tumors may have modest brainstem compression and may present with facial nerve paresis and gait ataxia. Decompression of the brainstem and stretched cranial nerves is vital in such cases, and thus surgery is the preferred treatment option.
- Surgery is completed with a number of approaches tailored to the patient's tumor size and degree of hearing loss, and to the surgeon's experience. In general, a middle fossa approach is completed through an incision that begins in front of the tragus of the affected ear and extends superiorly onto the temporal scalp. A 5 x 5 cm craniotomy is made above the ear, essentially centered on the ear canal. This approach is typically reserved for smaller tumors confined to the internal auditory canal when hearing preservation is attempted.[64] The translabyrinthine approach is performed through an incision behind the ear, working through the mastoid portion of the temporal bone, and does not preserve hearing because the inner ear is opened when, as the name suggests, the labyrinthine bone is drilled away. Thus, this approach is often used for patients who already have poor hearing or sometimes in cases where hearing preservation is unlikely, such as with large tumors.[64] The retrosigmoid approach is completed through an incision behind the ear allowing a craniotomy or craniectomy inferior to the transverse sinus and posterior to the sigmoid sinus.[64] This approach can be used for any sized tumor, with or without the intent of hearing preservation. Almost always, the posterior aspect of the internal auditory canal must be removed with a high-speed drill to access the component of the tumor extending into this area.[65] [66] [67] Intraoperative neurophysiologic monitoring of cranial nerves, in particular the facial nerve, is considered standard of care. Monitoring of cranial nerves has changed the nature and success of surgery in terms of cranial nerve morbidity.[68] [69] [70] [71]
radiation or observation
Comments
- As complete resection may lead to deterioration of cranial nerve function, incomplete resection of the tumor followed by radiation or observation is recommended.[13]
Follow-Up Overview
Prognosis
Long-term sequelae
Monitoring
Complications
Citations
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Lin VY, Stewart C, Grebenyuk J, et al. Unilateral acoustic neuromas: long-term hearing results in patients managed with fractionated stereotactic radiotherapy, hearing preservation surgery, and expectantly. Laryngoscope. 2005 Feb;115(2):292-6.[Abstract]
Pollock BE. Management of vestibular schwannomas that enlarge after stereotactic radiosurgery: treatment recommendations based on a 15 year experience. Neurosurgery. 2006 Feb;58(2):241-8.[Abstract]
Darrouzet V, Martel J, Enee V, et al. Vestibular schwannoma surgery outcomes: our multidisciplinary experience in 400 cases over 17 years. Laryngoscope. 2004 Apr;114(4):681-8.[Abstract]
Roland JT Jr, Fishman AJ, Golfinos JG, et al. Cranial nerve preservation in surgery for large acoustic neuromas. Skull Base. 2004 May;14(2):85-91.[Abstract][Full Text]
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Key Articles
Other Online Resources
Referenced Articles
Guidelines
Diagnostic
Summary
Evidence-based guidelines for the diagnosis, management, and treatment of patients with vestibular schwannomas.Published by
Congress of Neurological Surgeons
Published
2018
Summary
Evidence-based guidelines for the diagnosis, management, treatment, and follow-up of patients with vestibular schwannomas.Published by
European Association of Neuro-Oncology
Published
2020
Treatment
Summary
Evidence-based guidelines for the diagnosis, management, and treatment of patients with vestibular schwannomas.Published by
Congress of Neurological Surgeons
Published
2018
Summary
This guideline contains expert consensus on the management of patients with vestibular schwannoma based on a systematic review of the literature.Published by
International Stereotactic Radiosurgery Society
Published
2017
Summary
Evidence-based guidelines for the diagnosis, management, treatment, and follow-up of patients with vestibular schwannomas.Published by
European Association of Neuro-Oncology
Published
2020