Professional Documents
Culture Documents
www.uptodate.com
© 2022 UpToDate, Inc. and/or its affiliates. All Rights Reserved.
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Dec 2021. | This topic last updated: Dec 22, 2020.
INTRODUCTION
EPIDEMIOLOGY
The incidence appears to be increasing, due at least in part to the incidental diagnosis of
asymptomatic lesions with the widespread use of magnetic resonance imaging (MRI) and
computed tomography (CT) [3,4]. A retrospective analysis of 46,000 MRI scans done for other
reasons identified eight unsuspected vestibular schwannomas (0.02 percent) [3], and autopsy
studies suggest that the prevalence may be even higher [5,6].
The median age at diagnosis is approximately 50 years [4]. The tumors are unilateral in more
than 90 percent of cases [7], affecting the right and left sides with equal frequency. They occur
equally in both genders [1]. Bilateral vestibular schwannomas are primarily observed in patients
with neurofibromatosis type 2 (NF2) [6]. (See "Neurofibromatosis type 2", section on 'Vestibular
schwannomas'.)
In addition to NF2, other risk factors that have been associated with the development of
vestibular schwannomas in epidemiologic studies include the following:
● Childhood exposure to low-dose radiation for benign conditions of the head and neck has
been associated with an increased risk of vestibular schwannoma [9,10]. In a series of
2311 children irradiated between 1939 and 1962, 41 vestibular schwannomas were
identified with a latent period of 20 to 55 years [10]. The increase in risk was proportional
to the dose of radiation to the cerebellopontine angle (CPA; relative risk per Gy 1.14). (See
"Risk factors for brain tumors", section on 'Ionizing radiation'.)
● Some but not all studies have shown an increased incidence of various brain tumors,
including vestibular schwannomas, associated with the use of cellular telephones. Meta-
analyses of case-control studies have found a possible slight increase in risk of brain
tumors associated with cell phone use in higher-quality studies, with an induction period
of 10 years or longer [11,12]. Subsequent studies have cast doubt on this association,
however, as the incidence of cell phone use has markedly increased without a
complementary increase in vestibular schwannoma incidence [13,14].
● There are conflicting data on noise exposure as a risk factor for vestibular schwannomas
[7,15-18], despite experimental studies supporting biological plausibility [19,20]. Most
studies finding a positive association have relied on self-reported measures of noise
exposure [7,16,17], making it difficult to exclude recall bias as an alternative explanation
for the findings. In two population-based case-control studies that used a job exposure
matrix to assign noise exposure levels, occupational noise exposure was not associated
with an increased risk of vestibular schwannoma [1,21]. Other studies, however, have
found a positive association between vestibular schwannoma and self-reported exposure
to loud noise in leisure activities without hearing protection (odds ratio [OR] 1.5) [1,22].
HISTOPATHOLOGY
Vestibular schwannomas arise from perineural elements of the Schwann cell and are similar
pathologically to peripheral schwannomas found in other parts of the body. They occur with
equal frequency on the superior and inferior branches of the vestibular nerve ( figure 1); only
rarely are they derived from the cochlear portion of the VIII nerve.
Microscopically, zones of alternately dense and sparse cellularity, called Antoni A and B areas,
respectively, are characteristic of vestibular schwannomas ( picture 1). Malignant
degeneration is extremely rare, with only six cases having been reported.
Immunohistochemical staining for S100 protein is usually positive in both the benign and the
rare malignant forms of this tumor [23].
CLINICAL PRESENTATION
Symptoms associated with vestibular schwannoma can be due to cranial nerve involvement,
cerebellar compression, or tumor progression.
The clinical presentations of these tumors are illustrated by a series of 1000 vestibular
schwannomas treated at a single institution [24]. Clinical manifestations in this series included
the following:
The most nondescript vertiginous sensations, such as brief tilting or veering, can suggest
the presence of a vestibular schwannoma. The decision whether to obtain a magnetic
resonance image (MRI) for a patient with these symptoms depends upon clinical
judgment, with no good data in the literature about the likelihood that someone with such
symptoms harbors a schwannoma. (See "Causes of vertigo".)
● Facial nerve – The facial nerve was involved in 6 percent of patients [24]. The primary
symptoms were facial paresis and, less often, taste disturbances (due to nervus
intermedius impairment). Xerophthalmia, paroxysmal lacrimation, and xerostomia can
also be seen [25].
● Tumor progression – Other presenting signs can be the result of tumor progression,
leading to pressure on adjacent posterior fossa structures. Very large tumors can press on
the cerebellum or brainstem and result in ataxia. Brainstem compression, cerebellar tonsil
herniation, hydrocephalus, and death can occur in untreated cases. The functions of the
lower cranial nerves can also become impaired, leading to dysarthria, dysphagia,
aspiration, and hoarseness.
DIAGNOSIS
The differential diagnosis includes meningioma, which accounts for 4 to 10 percent of cases.
Other less common causes of such lesions include facial nerve schwannomas, gliomas,
cholesterol cysts, cholesteatomas, hemangiomas, aneurysms, arachnoid cysts, lipomas, and
metastatic tumor.
Further neurologic examination may reveal other cranial nerve deficits. A decreased or absent
ipsilateral corneal reflex and facial twitching or hypesthesia may occur as cranial nerves V and
VII become affected. Other cranial nerve deficits are uncommon unless the tumor is large.
Romberg, Hall-Pike, and other common office balance tests are typically normal.
Audiometry — Audiometry is the best initial screening laboratory test for the diagnosis of
vestibular schwannoma, since only 5 percent of patients will have a normal test. Pure tone and
speech audiometry should be performed in an acoustically shielded area. Test results typically
show an asymmetric sensorineural hearing loss, usually more prominent in the higher
frequencies. Hearing loss does not necessarily correlate with tumor size. The speech
discrimination score is usually markedly reduced in the affected ear and out of proportion to
the measured hearing loss. (See "Evaluation of hearing loss in adults", section on 'Formal
audiologic assessment'.)
Many other auditory tests have been used historically to try to diagnose vestibular
schwannomas. These include acoustic reflex testing, impedance audiometry, and Bekesy
audiometry. They have limited accuracy and diagnostic value, and their utility has diminished
with the advent of brainstem-evoked response audiometry (AER/ABR).
Vestibular testing — Vestibular testing has limited utility as a screening test for the diagnosis
of vestibular schwannoma because of the accuracy of evoked response audiometry. When
testing is performed, a decreased or absent caloric response on the affected side may be seen.
When the tumor is small, though, a normal response is often seen.
Vestibular schwannomas are seen on MRI and CT scans as enhancing lesions in the region of
the internal auditory canal with variable extension into the cerebellopontine angle (CPA). CT
scans with bone windows can also be of prognostic significance as the extent of widening of the
internal auditory canal (IAC) and the extent of tumor growth anterior and caudal to the IAC are
predictive of postoperative hearing loss [29].
Fast spin echo MRI may be useful as a screening test due to its low cost compared with
gadolinium MRI, noninvasiveness, and high sensitivity and specificity. In one study of 25
patients and 50 ears, there were 11 true positives and 39 true negatives on gadolinium MRI.
There were no false positives or false negatives with fast spin echo MRI [30]. This test is useful
when performed specifically for evaluation for a vestibular schwannoma, not as a general
screen.
TREATMENT
The three major treatment options for patients with a vestibular schwannoma are surgery,
radiation therapy, and observation. Management decisions take into account the size of the
tumor, signs and symptoms, patient age and comorbidities, and patient preferences [31].
Where available, patients may benefit from multidisciplinary specialty review with input from
neurosurgery, otolaryngology, and radiation oncology.
● Retromastoid suboccipital (retrosigmoid) – The suboccipital approach can be used for any
size tumor with or without attempted hearing preservation.
● Middle fossa – The middle fossa approach is suitable for small (<1.5 cm) tumors when
hearing preservation is a goal.
Outcomes — Complete tumor removal is feasible in almost all patients and there are few if
any recurrences when tumor removal is complete [35-38]. However, the outcome is less
favorable in patients who undergo subtotal removal in an attempt to preserve anatomic
continuity of the facial or acoustic nerves. Regrowth and/or recurrence, which is usually
asymptomatic, occurs in up to 15 to 20 percent of cases when initial resection is incomplete
[39,40].
The likelihood of surgical morbidity, which includes hearing loss, facial weakness, and vestibular
disturbances, depends upon tumor size. Facial nerve function can be preserved in most
patients even with large tumors [38,41,42], and serviceable hearing can be preserved in many
patients. However, only rarely does hearing improve after vestibular schwannoma surgery.
Intraoperative facial nerve and auditory monitoring have alerted surgeons to potential injury,
thereby improving the final outcome [41].
The outcomes that are achievable with an experienced team can be illustrated by the following
observations:
● In one series of 179 patients, 97 to 99 percent of patients had complete tumor removal
without evidence of recurrence at a mean follow-up duration of 70 months [35]. Normal or
near normal facial movement was present in 94 to 97 percent of patients with small
tumors and 28 to 57 percent with large tumors; in addition, 45 to 82 percent of patients
with good hearing and small tumors retained their hearing. Treatment complications
consisted mainly of cerebrospinal fluid leakage, which occurred in 15 percent of patients;
there were two deaths (1 percent).
● Similar results were noted in another report of 1000 tumors treated at a single institution
[36]. Ninety-eight percent of tumors were completely removed; in the other 21 patients,
deliberate partial removal was performed in severely ill patients for decompression of the
brainstem or in an attempt to preserve hearing in the last hearing ear. Anatomic
preservation of the facial nerve and the cochlear nerve was achieved in 93 and 68 percent,
respectively. Major neurologic complications included tetraparesis in one patient,
hemiparesis in 1 percent, lower cranial nerve palsies in 5.5 percent, and cerebrospinal fluid
fistulas in 9.2 percent. There were 11 deaths (1.1 percent) occurring at 2 to 69 days
postoperatively.
● Cerebrospinal fluid leaks were observed in 8.5 percent, of which one-third resolved with
conservative treatment. This complication was significantly more frequent in patients who
underwent a translabyrinthine approach, although at least one report has suggested that
technical modifications can decrease this complication with this approach [37]. The
frequency of cerebrospinal fluid leaks was not related to tumor size.
Compared with younger adults, older adults undergoing surgery for vestibular schwannoma
may be at substantially higher risk for in-hospital complications [46,47]. In a retrospective study
using the National Inpatient Sample database in the United States for vestibular schwannoma
surgeries from the years 2002 to 2010 (n = 4147), adults ≥65 years of age were at increased risk
for medical complications such as acute cardiac events, renal failure, and infection (adjusted
odds ratio [OR] 1.8) as well as in-hospital mortality (OR 13) [46].
Radiation therapy — Radiation therapy approaches that have been used in patients with
vestibular schwannoma include stereotactic radiosurgery (SRS), stereotactic radiotherapy (SRT),
and proton beam therapy, as well as conventional fractionated radiation therapy.
There are no randomized trials comparing different radiation therapy approaches, and data are
only available from observational studies [48]. A critical review of the literature concluded that
equivalent local control can be achieved with each of these approaches and that treatment
decisions could be based upon the availability of expertise and technology, as well as patient-
specific factors [49].
Risk factors for worse hearing outcomes include older age, larger tumor volume, and greater
degree of baseline hearing loss [57,59,60]. The impact of cochlear radiation dose alone as a
predictive parameter for hearing outcomes remains controversial. While several retrospective
studies have reported improved hearing preservation with maximum cochlear dose <4 Gy [61],
or central cochlear dose <4.2 Gy [62], others have not found cochlear dose to be a significant
factor on multivariate analysis [63].
● Cystic degeneration – Delayed cyst formation has been reported in 2 percent of patients,
occurring at a median of six years after SRS and requiring craniotomy for symptomatic
management in a small minority [56].
● Local tissue scarring – There is a concern that scarring following SRS may complicate
subsequent microsurgery should the tumor recur. In a series of 20 cases in which surgical
salvage was performed following recurrence after radiosurgery, approximately one-half
were determined to have greater difficulty for resection or facial nerve preservation [70].
Multiple series have demonstrated the general safety and efficacy of this approach [49,71-76].
● In one retrospective study, 200 patients with 202 vestibular schwannomas were treated
with SRT (n = 172) or SRS (n = 30) [71]. For patients treated with SRT, the median dose was
57.6 Gy, given in 1.8 Gy fractions. At a median follow-up of 75 months, the 5- and 10-year
local control rates were both 96 percent and there was no significant difference between
the two treatment techniques. In patients with serviceable hearing prior to SRT, the
hearing preservation rate was 78 percent at five years.
● In another single institution series using a different schedule, 496 patients were treated
between 1995 and 2007 [75]. A dose of 25 Gy in five fractions or 30 Gy in 10 fractions was
used (89 and 11 percent of cases, respectively). Analysis of the 385 patients with imaging
follow-up at least 18 months after diagnosis found a therapeutic failure rate of 3 percent,
as defined by progressive tumor growth requiring surgical intervention. However, 30
percent of these patients experienced some tumor growth, and 9 percent had a long-term
twofold or greater increase in tumor volume after treatment. Patients with a small tumor
(volume less than 1 cm3) were more likely to experience tumor growth than those with
larger tumors.
Proton beam therapy — Proton beam therapy may provide maximal local tumor control while
minimizing cranial nerve injuries. The physical characteristics of the beam result in the majority
of the energy being deposited at the end of a linear track (the Bragg peak), with the dose falling
rapidly to zero beyond the Bragg peak. Thus, the use of proton beam therapy permits the
delivery of high doses of radiation therapy to the target volume while limiting the "scatter" dose
received by surrounding tissues.
In one report of 88 patients who were treated with fractionated proton beam therapy, the two-
and five-year local control rates were 95 and 94 percent, respectively [77]. Salvage therapy was
necessary in five (radiosurgery in one, craniotomy in one, and shunting for hydrocephalus in
three). Seven of 21 patients with functional hearing retained serviceable hearing ability, while
facial and trigeminal nerve function was preserved in 91 and 89 percent of patients,
respectively.
The growth rate of untreated tumors during observation was illustrated by a single-institution
series of 386 patients who were initially managed with observation between 1990 and 2005
[78]. Patients were reimaged at one year and subsequently followed based upon rate of growth.
Indications for conservative management varied but included age >60 years, significant
comorbidity, small tumor size, lack of symptoms, risk of further hearing loss, or patient
preference. Overall, 59 percent of patients had an annual tumor growth rate <1 mm/year,
although 39 patients (12 percent) had tumor growth ≥3 mm at one year.
A review of the literature identified 21 studies that included 1345 patients who were managed
conservatively [79]. With an average follow-up of 3.2 years, 43 percent of tumors showed
evidence of growth, 51 percent remained stable, while 6 percent had evidence of regression
without treatment. Hearing loss occurred in one-half of the 347 individuals in whom it was
assessed longitudinally. In 15 of the studies, 20 percent of 1001 individuals eventually required
treatment with either surgery or radiation, due to either tumor growth on imaging or symptom
progression.
Observation may be particularly appropriate in older adult patients who are poor candidates for
immediate intervention. However, if there are signs or symptoms of tumor progression, age
should not be considered an absolute contraindication to either surgery or radiation [80,81].
While several series have found that neurologic complications and surgical outcomes are
comparable to those in younger patients undergoing surgery, older patients are at increased
risk for medical complications, including in-hospital mortality [46]. (See 'Other complications'
above.)
Studies on the natural history of small vestibular schwannomas have helped to define the risk
factors for further hearing loss. A review of the literature identified 34 studies that included 982
patients with vestibular schwannomas <25 mm in diameter, who had functional hearing and
were managed with observation [83]. Those patients with a tumor growth rate ≤2.5 mm/year
had a significantly higher rate of hearing preservation compared with those with a higher
tumor growth rate (75 versus 32 percent) [83].
A single-institution prospective study identified 59 patients over a 22-year period who had
vestibular schwannomas with serviceable hearing and were managed with serial observation
from presentation until hearing loss developed [84]. None of these patients underwent either
surgery or radiation therapy during the period of observation. For patients whose tumors
exhibited rapid growth (ie, >2.5 mm/year) at any time during the observation period, the
median time to hearing loss was seven years, and all of these patients had lost hearing in less
than 10 years. Hearing loss generally developed within one to two years of the onset of rapid
tumor growth. By contrast, the median time to hearing loss was 15 years in those with tumors
that never exhibited rapid growth.
For patients with vestibular schwannomas in whom preservation of residual functional hearing
on the involved side is important, these data suggest that careful serial imaging is needed, and
therapeutic intervention is indicated if there is evidence of rapid tumor growth (ie, >2.5
mm/year), regardless of tumor size.
HEARING REHABILITATION
Unfortunately, many patients with vestibular schwannoma lose some or all of their hearing
either from the tumor or from intervention to remove or control the tumor (either surgery or
radiation therapy). This loss can be immediate or delayed over years.
● Loss of ability to hear sounds coming from the deaf side (head-shadow effect)
Treatment options — While the focus tends to be on the diagnosis and treatment of vestibular
schwannomas, options for rehabilitation of the hearing deficit are slowly increasing [85]. If
surgery is planned, preoperative discussion of these options is important, as a titanium screw
or a cochlear implant can be placed at the time of surgery when hearing is gone or expected to
be lost. Rehabilitation can then begin much earlier in the postoperative course without
requiring a second operation.
Therapies range from supportive, compensatory strategies to hearing aids and implantable
devices.
● Get used to the loss. Individuals can function with only one hearing ear. Positioning
oneself so that the good ear is always toward the action and having important
conversations in quiet settings can minimize the impact of only having one hearing ear.
● Use a hearing aid in the ear if any serviceable hearing is left or consider a contralateral
routing of signal (CROS) hearing aid if it is not. A CROS aid sends the sound from the bad
ear to the good ear. This eliminates the head-shadow effect, but since all the sound is still
going through one ear it does not eliminate the other two problems.
● A bone-anchored hearing aid (BAHA) is a device where a titanium screw is placed in the
skull on the deafened side. A percutaneous hearing aid is attached to the screw. Sound is
then sent through the skull to the good side by bone vibration. This option eliminates the
head-shadow effect and may do a little for sound localization because of the sensation of
the skull vibration, but it does not help in background noise. All of the sound is still going
through one ear.
● Cochlear implants are devices where an electrode array is threaded into the cochlea to
stimulate the auditory nerve directly. This requires that the auditory nerve is still intact.
This is the case for patients undergoing observation for growth, for patients that have had
radiation, and for postsurgical patients where the auditory nerve was left intact no matter
what the approach. Some case reports show that the nerve can still be stimulated to help
restore some of the lost hearing.
Questions remain as to how much hearing can be restored; however, limited data now
show it is a viable option. The advantage of this option is that it can potentially improve all
three of the problems caused by the hearing loss. New implants are now magnetic
resonance imaging (MRI) compatible, so that continued monitoring of the posterior fossa
and internal auditory canal is possible without risk of damage to the implant or injury to
the patient [86-89].
● Brainstem implants have been available for many years. Electrodes can be placed next to
the cochlear nucleus to stimulate the area directly and bypass the inner ear and cochlear
nerve directly. The success of these implants is limited because of the small number of
electrodes available and the placement and contact of the electrodes with the cochlear
nucleus. They are usually done when the patient has loss of hearing in both ears and no
auditory nerve to stimulate [90].
POST-TREATMENT FOLLOW-UP
The optimal approach for follow-up studies after diagnosis and treatment is uncertain, and
there are no data to support specific recommendations. Because of the potentially slow growth
of these tumors, prolonged follow-up is necessary. The following represents an empiric
approach:
● For patients being managed with observation, yearly scans for 10 years, with scans
subsequently every three to five years if there has been no growth. Audiometry should
also be performed on a regular basis, if preservation of hearing is an important
consideration.
● For patients who underwent surgery, yearly scans for 8 to 10 years, and less frequently
thereafter if no residual tumor is present.
● For patients treated with radiation therapy, yearly scans for 10 years and then every two
years if no growth seen.
Links to society and government-sponsored guidelines from selected countries and regions
around the world are provided separately. (See "Society guideline links: Primary brain tumors".)
UpToDate offers two types of patient education materials, "The Basics" and "Beyond the
Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade
reading level, and they answer the four or five key questions a patient might have about a given
condition. These articles are best for patients who want a general overview and who prefer
short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more
sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading
level and are best for patients who want in-depth information and are comfortable with some
medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print
or e-mail these topics to your patients. (You can also locate patient education articles on a
variety of subjects by searching on "patient info" and the keyword(s) of interest.)
● Basics topic (see "Patient education: Vestibular schwannoma (acoustic neuroma) (The
Basics)")
• Both surgery and various radiation therapy techniques represent acceptable options
for patients undergoing treatment. The choice of therapy should be based upon the
availability of teams with appropriate expertise in treating vestibular schwannomas, as
well as patient-specific factors. (See 'Surgery' above and 'Radiation therapy' above.)
● For older patients with small tumors and limited hearing loss, we suggest observation
with serial imaging and audiometry (Grade 2C). However, such patients should be
monitored at least annually for evidence of tumor progression. Rapid tumor growth (>2.5
mm/year), rather than the absolute size of the tumor, may serve as a useful indicator for
therapy. (See 'Observation' above and 'Post-treatment follow-up' above.)
● Options for rehabilitation of hearing deficits are slowly increasing. If surgery is planned,
preoperative discussion of these options is important, as a titanium screw or a cochlear
implant can be placed at the time of surgery when hearing is gone or expected to be lost.
(See 'Hearing rehabilitation' above.)
ACKNOWLEDGMENT
The UpToDate editorial staff acknowledges Peter Black, MD, PhD, who contributed to an earlier
version of this topic review.
REFERENCES
1. Fisher JL, Pettersson D, Palmisano S, et al. Loud noise exposure and acoustic neuroma. Am J
Epidemiol 2014; 180:58.
2. Koo M, Lai JT, Yang EY, et al. Incidence of Vestibular Schwannoma in Taiwan from 2001 to
2012: A Population-Based National Health Insurance Study. Ann Otol Rhinol Laryngol 2018;
127:694.
3. Lin D, Hegarty JL, Fischbein NJ, Jackler RK. The prevalence of "incidental" acoustic neuroma.
Arch Otolaryngol Head Neck Surg 2005; 131:241.
4. Propp JM, McCarthy BJ, Davis FG, Preston-Martin S. Descriptive epidemiology of vestibular
schwannomas. Neuro Oncol 2006; 8:1.
5. Tos M, Stangerup SE, Cayé-Thomasen P, et al. What is the real incidence of vestibular
schwannoma? Arch Otolaryngol Head Neck Surg 2004; 130:216.
6. Eldridge R, Parry D. Vestibular schwannoma (acoustic neuroma). Consensus development
conference. Neurosurgery 1992; 30:962.
7. Edwards CG, Schwartzbaum JA, Lönn S, et al. Exposure to loud noise and risk of acoustic
neuroma. Am J Epidemiol 2006; 163:327.
8. Sughrue ME, Yeung AH, Rutkowski MJ, et al. Molecular biology of familial and sporadic
vestibular schwannomas: implications for novel therapeutics. J Neurosurg 2011; 114:359.
9. Schneider AB, Ron E, Lubin J, et al. Acoustic neuromas following childhood radiation
treatment for benign conditions of the head and neck. Neuro Oncol 2008; 10:73.
10. Shore-Freedman E, Abrahams C, Recant W, Schneider AB. Neurilemomas and salivary gland
tumors of the head and neck following childhood irradiation. Cancer 1983; 51:2159.
11. Prasad M, Kathuria P, Nair P, et al. Mobile phone use and risk of brain tumours: a
systematic review of association between study quality, source of funding, and research
outcomes. Neurol Sci 2017; 38:797.
12. Little MP, Rajaraman P, Curtis RE, et al. Mobile phone use and glioma risk: comparison of
epidemiological study results with incidence trends in the United States. BMJ 2012;
344:e1147.
13. Momoli F, Siemiatycki J, McBride ML, et al. Probabilistic Multiple-Bias Modeling Applied to
the Canadian Data From the Interphone Study of Mobile Phone Use and Risk of Glioma,
Meningioma, Acoustic Neuroma, and Parotid Gland Tumors. Am J Epidemiol 2017; 186:885.
14. Röösli M, Lagorio S, Schoemaker MJ, et al. Brain and Salivary Gland Tumors and Mobile
Phone Use: Evaluating the Evidence from Various Epidemiological Study Designs. Annu Rev
Public Health 2019; 40:221.
15. Preston-Martin S, Thomas DC, Wright WE, Henderson BE. Noise trauma in the aetiology of
acoustic neuromas in men in Los Angeles County, 1978-1985. Br J Cancer 1989; 59:783.
16. Schlehofer B, Schlaefer K, Blettner M, et al. Environmental risk factors for sporadic acoustic
neuroma (Interphone Study Group, Germany). Eur J Cancer 2007; 43:1741.
17. Hours M, Bernard M, Arslan M, et al. Can loud noise cause acoustic neuroma? Analysis of
the INTERPHONE study in France. Occup Environ Med 2009; 66:480.
18. Edwards CG, Schwartzbaum JA, Nise G, et al. Occupational noise exposure and risk of
acoustic neuroma. Am J Epidemiol 2007; 166:1252.
19. Ryals BM, Rubel EW. Hair cell regeneration after acoustic trauma in adult Coturnix quail.
Science 1988; 240:1774.
20. Hamernik RP, Turrentine G, Roberto M, et al. Anatomical correlates of impulse noise-
induced mechanical damage in the cochlea. Hear Res 1984; 13:229.
21. Aarhus L, Kjærheim K, Heikkinen S, et al. Occupational Noise Exposure and Vestibular
Schwannoma: A Case-Control Study in Sweden. Am J Epidemiol 2020; 189:1342.
22. Chen M, Fan Z, Zheng X, et al. Risk Factors of Acoustic Neuroma: Systematic Review and
Meta-Analysis. Yonsei Med J 2016; 57:776.
23. Rouleau GA, Merel P, Lutchman M, et al. Alteration in a new gene encoding a putative
membrane-organizing protein causes neuro-fibromatosis type 2. Nature 1993; 363:515.
24. Matthies C, Samii M. Management of 1000 vestibular schwannomas (acoustic neuromas):
clinical presentation. Neurosurgery 1997; 40:1.
25. Noonan KY, Rang C, Callahan K, et al. Nervus Intermedius Symptoms following Surgical or
Radiation Therapy for Vestibular Schwannoma. Otolaryngol Head Neck Surg 2016; 155:657.
26. Doyle KJ. Is there still a role for auditory brainstem response audiometry in the diagnosis of
acoustic neuroma? Arch Otolaryngol Head Neck Surg 1999; 125:232.
27. Koors PD, Thacker LR, Coelho DH. ABR in the diagnosis of vestibular schwannomas: a meta-
analysis. Am J Otolaryngol 2013; 34:195.
28. Wilson DF, Hodgson RS, Gustafson MF, et al. The sensitivity of auditory brainstem response
testing in small acoustic neuromas. Laryngoscope 1992; 102:961.
34. Barker FG 2nd, Carter BS, Ojemann RG, et al. Surgical excision of acoustic neuroma: patient
outcome and provider caseload. Laryngoscope 2003; 113:1332.
35. Gormley WB, Sekhar LN, Wright DC, et al. Acoustic neuromas: results of current surgical
management. Neurosurgery 1997; 41:50.
36. Samii M, Matthies C. Management of 1000 vestibular schwannomas (acoustic neuromas):
surgical management and results with an emphasis on complications and how to avoid
them. Neurosurgery 1997; 40:11.
37. Falcioni M, Mulder JJ, Taibah A, et al. No cerebrospinal fluid leaks in translabyrinthine
vestibular schwannoma removal: reappraisal of 200 consecutive patients. Am J Otol 1999;
20:660.
38. Darrouzet V, Martel J, Enée V, et al. Vestibular schwannoma surgery outcomes: our
multidisciplinary experience in 400 cases over 17 years. Laryngoscope 2004; 114:681.
39. Ohta S, Yokoyama T, Nishizawa S, Uemura K. Regrowth of the residual tumour after
acoustic neurinoma surgery. Br J Neurosurg 1998; 12:419.
46. Sylvester MJ, Shastri DN, Patel VM, et al. Outcomes of Vestibular Schwannoma Surgery
among the Elderly. Otolaryngol Head Neck Surg 2017; 156:166.
47. Hatch JL, Bauschard MJ, Nguyen SA, et al. National Trends in Vestibular Schwannoma
Surgery: Influence of Patient Characteristics on Outcomes. Otolaryngol Head Neck Surg
2018; 159:102.
48. Muzevic D, Legcevic J, Splavski B, Cayé-Thomasen P. Stereotactic radiotherapy for vestibular
schwannoma. Cochrane Database Syst Rev 2014; :CD009897.
49. Murphy ES, Suh JH. Radiotherapy for vestibular schwannomas: a critical review. Int J Radiat
Oncol Biol Phys 2011; 79:985.
50. Kondziolka D, Lunsford LD, McLaughlin MR, Flickinger JC. Long-term outcomes after
radiosurgery for acoustic neuromas. N Engl J Med 1998; 339:1426.
51. Flickinger JC, Lunsford LD, Linskey ME, et al. Gamma knife radiosurgery for acoustic
tumors: multivariate analysis of four year results. Radiother Oncol 1993; 27:91.
52. Foote RL, Coffey RJ, Swanson JW, et al. Stereotactic radiosurgery using the gamma knife for
acoustic neuromas. Int J Radiat Oncol Biol Phys 1995; 32:1153.
53. Mendenhall WM, Friedman WA, Buatti JM, Bova FJ. Preliminary results of linear accelerator
radiosurgery for acoustic schwannomas. J Neurosurg 1996; 85:1013.
54. Yang I, Sughrue ME, Han SJ, et al. A comprehensive analysis of hearing preservation after
radiosurgery for vestibular schwannoma. J Neurosurg 2010; 112:851.
55. Roos DE, Potter AE, Brophy BP. Stereotactic radiosurgery for acoustic neuromas: what
happens long term? Int J Radiat Oncol Biol Phys 2012; 82:1352.
56. Hasegawa T, Kida Y, Kato T, et al. Long-term safety and efficacy of stereotactic radiosurgery
for vestibular schwannomas: evaluation of 440 patients more than 10 years after treatment
with Gamma Knife surgery. J Neurosurg 2013; 118:557.
57. Carlson ML, Jacob JT, Pollock BE, et al. Long-term hearing outcomes following stereotactic
radiosurgery for vestibular schwannoma: patterns of hearing loss and variables influencing
audiometric decline. J Neurosurg 2013; 118:579.
58. Johnson S, Kano H, Faramand A, et al. Long term results of primary radiosurgery for
vestibular schwannomas. J Neurooncol 2019; 145:247.
59. Roos DE, Potter AE, Zacest AC. Hearing preservation after low dose linac radiosurgery for
acoustic neuroma depends on initial hearing and time. Radiother Oncol 2011; 101:420.
60. Mousavi SH, Niranjan A, Akpinar B, et al. Hearing subclassification may predict long-term
auditory outcomes after radiosurgery for vestibular schwannoma patients with good
hearing. J Neurosurg 2016; 125:845.
61. Yomo S, Carron R, Thomassin JM, et al. Longitudinal analysis of hearing before and after
radiosurgery for vestibular schwannoma. J Neurosurg 2012; 117:877.
62. Kano H, Kondziolka D, Khan A, et al. Predictors of hearing preservation after stereotactic
radiosurgery for acoustic neuroma: clinical article. J Neurosurg 2013; 119 Suppl:863.
63. Jacob JT, Carlson ML, Schiefer TK, et al. Significance of cochlear dose in the radiosurgical
treatment of vestibular schwannoma: controversies and unanswered questions.
Neurosurgery 2014; 74:466.
64. Pollock BE. Management of vestibular schwannomas that enlarge after stereotactic
radiosurgery: treatment recommendations based on a 15 year experience. Neurosurgery
2006; 58:241.
65. Niu NN, Niemierko A, Larvie M, et al. Pretreatment growth rate predicts radiation response
in vestibular schwannomas. Int J Radiat Oncol Biol Phys 2014; 89:113.
66. Link MJ, Cohen PL, Breneman JC, Tew JM Jr. Malignant squamous degeneration of a
cerebellopontine angle epidermoid tumor. Case report. J Neurosurg 2002; 97:1237.
72. Williams JA. Fractionated stereotactic radiotherapy for acoustic neuromas. Int J Radiat
Oncol Biol Phys 2002; 54:500.
73. Koh ES, Millar BA, Ménard C, et al. Fractionated stereotactic radiotherapy for acoustic
neuroma: single-institution experience at The Princess Margaret Hospital. Cancer 2007;
109:1203.
74. Andrews DW, Suarez O, Goldman HW, et al. Stereotactic radiosurgery and fractionated
stereotactic radiotherapy for the treatment of acoustic schwannomas: comparative
observations of 125 patients treated at one institution. Int J Radiat Oncol Biol Phys 2001;
50:1265.
75. Kapoor S, Batra S, Carson K, et al. Long-term outcomes of vestibular schwannomas treated
with fractionated stereotactic radiotherapy: an institutional experience. Int J Radiat Oncol
Biol Phys 2011; 81:647.
76. Combs SE, Engelhard C, Kopp C, et al. Long-term outcome after highly advanced single-
dose or fractionated radiotherapy in patients with vestibular schwannomas - pooled results
from 3 large German centers. Radiother Oncol 2015; 114:378.
77. Weber DC, Chan AW, Bussiere MR, et al. Proton beam radiosurgery for vestibular
schwannoma: tumor control and cranial nerve toxicity. Neurosurgery 2003; 53:577.
78. Bakkouri WE, Kania RE, Guichard JP, et al. Conservative management of 386 cases of
unilateral vestibular schwannoma: tumor growth and consequences for treatment. J
Neurosurg 2009; 110:662.
79. Smouha EE, Yoo M, Mohr K, Davis RP. Conservative management of acoustic neuroma: a
meta-analysis and proposed treatment algorithm. Laryngoscope 2005; 115:450.
80. Pulec JL. Acoustic neuroma surgery in geriatric patients. Ear Nose Throat J 1999; 78:429.
81. Samii M, Tatagiba M, Matthies C. Acoustic neurinoma in the elderly: factors predictive of
postoperative outcome. Neurosurgery 1992; 31:615.
82. Akpinar B, Mousavi SH, McDowell MM, et al. Early Radiosurgery Improves Hearing
Preservation in Vestibular Schwannoma Patients With Normal Hearing at the Time of
Diagnosis. Int J Radiat Oncol Biol Phys 2016; 95:729.
83. Sughrue ME, Yang I, Aranda D, et al. The natural history of untreated sporadic vestibular
schwannomas: a comprehensive review of hearing outcomes. J Neurosurg 2010; 112:163.
84. Sughrue ME, Kane AJ, Kaur R, et al. A prospective study of hearing preservation in
untreated vestibular schwannomas. J Neurosurg 2011; 114:381.
85. Snapp HA, Ausili SA. Hearing with One Ear: Consequences and Treatments for Profound
Unilateral Hearing Loss. J Clin Med 2020; 9.
89. Roberts S, Levin B, Sanli H, et al. Simultaneous cochlear implantation and removal of
acoustic neuroma: implications for hearing. J Laryngol Otol 2020; 134:519.
90. Wong K, Kozin ED, Kanumuri VV, et al. Auditory Brainstem Implants: Recent Progress and
Future Perspectives. Front Neurosci 2019; 13:10.
Topic 5222 Version 30.0
GRAPHICS
Acoustic neuroma. Low and high power magnification of acoustic neuroma with
zones of alternately dense and sparse cellularity.
This 30-year-old female presented with hearing loss on the right and
has findings consistent with an acoustic neuroma on the following
MRI images. The T1-weighted image without contrast (A) shows a
mass centered in the right internal auditory canal (arrow). It is
isointense to the brain parenchyma. Following gadolinium, the
schwannoma enhances homogeneously and has the classic "ice
cream cone" shape (short arrow). The high-resolution T2-weighted
image shows loss of the normal fluid signal in the internal auditory
canal, which can be appreciated on the left side. The mass
(arrowhead; green overlay) projects into the cerebellopontine angle
cistern (dashed arrow).
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must
conform to UpToDate standards of evidence.