Neurosurg Clin N Am 19 (2008) 379–392

Guiding Patients Through the Choices for Treating

Vestibular Schwannomas: Balancing Options
and Ensuring Informed Consent
Douglas D. Backous, MD, FACSa,*, Huong T. Pham, MDb
a
Otology, Neurotology and Skull Base Surgery, Section of Otolaryngology-Head and Neck Surgery,
Virginia Mason Medical Center, 1100 Ninth Avenue, X10-0N, Seattle, WA 98111-0900, USA
b
Section of Radiation Oncology, Virginia Mason Medical Center, P O. Box 900, Seattle, WA 98111-0900, USA

Counseling patients who are diagnosed with patients should be encouraged to gather informa-
vestibular schwannomas (VS), formerly known as tion before making a treatment decision. For the
acoustic neuromas, can be challenging. These physicians managing these patients, information
benign neoplasms, which originate from either should be delivered in a balanced way to ensure
the superior or inferior vestibular nerves, have an patient understanding of their options leading to
average growth rate of 1 mm3 per year. Larger tu- adequate informed consent.
mors can cause brainstem compression with few Options for treatment include radiation ther-
noticeable symptoms, whereas smaller VS can apy, surgical excision, and observation with serial
cause vertigo, tinnitus, and hearing loss. MRI. Discerning treatment advantages from
Patients are confronted by the difficult task of a particular modality is made difficult because of
choosing a treatment method based on the advice nonstandardized definitions of tumor control and
of caregivers, currently available literature, and hearing preservation and varied posttreatment
Internet-based information sources. Patients often intervals presented in the medical literature.
visit the Internet either before or during their Surgical techniques and radiotherapy dosage par-
decision period, which can be helpful or even adigms have evolved considerably over the past
more confusing for them as they weigh their two decades. Currently, no randomized, prospec-
options. The health care provider has the re- tive clinical trial has compared the three treatment
sponsibility to explain, in understandable lan- options and there are no clearly accepted, evi-
guage, to the patient or legal representative dence-based, best practices for managing VS.
governing the patient’s care the proposed treat- The treatment of VS requires a multidisciplin-
ment options, risks and complications associated ary team not only to deliver the chosen therapy
with each form of treatment, and alternatives to but also to assist in the decision-making process.
treatment, including no therapy. The medical At our center, a nurse familiar with VS treatment
record must contain evidence of the patient’s coordinates appointments with a neurotologist,
informed consent, with the exception of emer- a neurosurgeon, and a radiotherapist. Patients are
gency situations in which a delay in intervention encouraged to take the amount of time necessary
could compromise outcomes in a life or limb- to make a decision with full understanding of
threatening situation. Aside from cases with potential risks and benefits. The amount of time
brainstem compression and hydrocephalus, necessary to come to a decision depends on the
needs of a particular patient.
A version of this article originally appeared in This article evaluates the English language
Otolaryngologic Clinics of NA, volume 40, issue 3. literature, dating back to 1994, to present long-
* Corresponding author. term results for tumor control and complication
E-mail address: otoddb@vmmc.org (D.D. Backous). rates for the treatment of VS, excluding cases of
1042-3680/08/$ - see front matter Ó 2008 Elsevier Inc. All rights reserved.
doi:10.1016/j.nec.2008.02.004 neurosurgery.theclinics.com
380 BACKOUS & PHAM

neurofibromatosis type II. The goal is to provide Although the tumor margin doses are similar to
a guide to otolaryngologists who provide initial the GK, LINAC radiosurgery typically uses fewer
counseling for patients with newly diagnosed VS. isocenters and the dose to the tumor is more ho-
mogeneous. Similar to the GK, a non-relocatable
invasive head frame is required for patient immo-
Stereotactic radiation therapy
bilization during treatment.
Radiation therapy was initially used as an Fractionated stereotactic radiation therapy is
adjunct to surgery in patients with incompletely the most recently developed technique for de-
resected VS. In a study reported from University livering high-dose and localized treatment. Unlike
of California San Francisco, Wallner and col- with GK or LINAC, a noninvasive relocatable
leagues [1] demonstrated that conventional frac- head frame or thermoplastic mask is used for
tionated radiation therapy to more than 45 Gy fractionated stereotactic radiation therapy. This
significantly reduced regrowth from 46% to 6% head frame increases patient comfort but may
in incompletely resected VS. In this series, 31 pa- result in less dose conformality when compared
tients treated between 1945 and 1983 were with other radiosurgery techniques. Subscribers of
followed from 2.6 to 40.7 years. The authors this technique believe that fractionation takes
concluded that postoperative radiation therapy advantage of radiobiologic principles to reduce
reduced the ‘‘local recurrence rate’’ of VSs that late toxicity while maintaining tumor control
were incompletely excised or only biopsied and [10,11]. The fractionated treatment regimens
demonstrated the effectiveness of radiation ther- range from doses given over several days to stan-
apy in the treatment of acoustic neuromas. dard fractionation given over 4 to 5 weeks, similar
Stereotactic radiosurgery, first developed in to the scheme originally used by Wallner.
1951 by Leksell [2], is a method of delivering Data regarding the outcomes GK, LINAC,
a highly conformal single dose of ionizing and FSRT are reviewed in the next section.
radiation with submillimeter accuracy to an intra- Because few centers in the world offer proton
cranial target. The goal of a single high-dose deliv- beam radiation therapy to treat VS, studies that
ery was to cause tumor necrosis and control of used proton-based treatments were excluded. All
growth as an alternative to surgery for patients pertinent papers published from 1994 to the
who were suboptimal candidates for excision. present that assess local control rates with a me-
The first stereotactic radiosurgery treatment for dian follow-up of at least 2 years were reported.
VS was performed in 1969 with the gamma knife Outcomes included tumor control, hearing pres-
(GK), also developed by Leksell [3]. The GK is ervation, facial neuropathy, and trigeminal neu-
a highly specialized radiation delivery system ralgia. In most patients, tumors were sporadic and
that uses 201 radioactive cobalt 60 sources to de- had a maximum diameter of %3 cm.
liver high-dose radiation accurately to tumor The goal of radiation therapy is to arrest
masses. A stereotactic frame is fixed to the skull tumor growth. Local control rate in radiotherapy
and attached to the treatment table to provide studies can be defined as the percentage of tumors
rigid immobilization of the patient’s head and that do not increase in size on follow-up imaging.
ensure accurate localization of the radiation dose. Many researchers define the local control rate as
Thousands of patients have been treated with the percentage of tumors that do not require
the GK. Initially, doses were high, and although salvage therapy. This determination could over-
tumor control was excellent, toxicity was signifi- estimate the control rate because some tumors
cant [4–6]. Several refinements, including dose re- may have progressed but are not symptomatic
duction, improved target definition, and treatment enough to require further treatment. All three
accuracy, have provided excellent tumor control techniques seem to achieve excellent local control
while minimizing toxicities of treatment [7,8]. with a range of 87% to 100% (Tables 1–3)
Lower radiation doses were shown to be effective [8,10,12–45].
in controlling tumor growth, whereas tumor Up to 50% of radiated tumors developed
shrinkage could take several years to document central necrosis that results in transient increase
radiographically. in tumor volume (23% of cases) [12]. This
The linear accelerator (LINAC) also can be phenomenon was observed up to 4 years after
adapted to perform stereotactic radiosurgery [9]. treatment and took from 6 months to 5 years to
Multiple beam positions or arcs are used to create disappear. If tumor progression was defined as
a conformal dose distribution around the target. tumor growth, then some patients may have
Table 1
Gamma knife radiosurgery outcomes (median marginal dose 12–14 Gy)
Number Local Hearing Facial Trigeminal Other
Author (year) of patients control rate preservation rate neuropathy rate neuropathy rate complications
Flickinger, 2004 [14] 313 98.6% @ 6 y 78%@ 6 y 0% @ 6 y 4.4% @ 6 y
Andrews, 2001 [10] 63 98% 33% 2% 5% hc 2.9% vertigo 1.4%
ataxia 1.4%
Massager, 2006 [15] 82 98% 65%

CHOICES FOR TREATING VESTIBULAR SCHWANNOMAS

Ottaviani, 2002 [16] 30 87% 73% 3% temp 16% mild symptoms
Wackym, 2004 [17] 29 94% 0% 3% temp
Prasad, 2000 [12] 153 92% 75% for tumors 2.3% 1.7% perm,
!1 cc, 57% for 2.5% temp
tumors O1 cc
Chung, 2005 [18] 195 95% @ 7 y 60% 1.4% temp 1.1% hc 3.5%
Wowra, 2005 [19] 111 95% @ 6 y 2.7% 11.5%
Lundsford, 2005 [8] 829 97% @10 y 78.6% 0% 4.4% ventriculoperitoneal shunt 0.8%,
tinnitus 0.2%, peritumoral
cyst 3.6%, no radiation-
induced malignancy
Petit, 2001 [20] 45 96% 88% 4% temp 0%
Hasegawa, 2005 [13] 317 92% @10 y 68% 1% temp 2% hc 4.1%, malignant
transformation 0.3%
Pollock, 2006 [21] 46 100% 63% 4% 2.1% hc 4%
Mysreth, 2005 [22] 103 89.2% 5.2% hc 3.9%
Paek, 2005 [23] 25 100% 46% 0% 5%
Litvack, 2003 [24] 134 97.7% 61.7% 2.2% temp 5.8% temp hc 3.0%
Hirato, 1996 [25] 29 93% 59% 10.3% temp 3% temp hc 6.8%
Muacevic, 2004 [26] 219 97% 49% 0.5% 5% temp
Iwai, 2003 [27] 51 96% 56% 0% 4% hc 8%
Kwon, 1998 [28] 88 95% 67% 4% temp, 4% perm 3.4% temp hc 3.5%
Hearing preservation rate is the percentage of patients who maintained or gained useful hearing (Gardner-Robertson class 1-2) after treatment.
Abbreviations: perm, permanent; hc, hydrocephalus; temp, temporary.

381
382 BACKOUS & PHAM

undergone surgery for salvage treatment unneces-

of cerebellum
radionecrosis
1 patient with
complications sarily [13].
Lundsford and colleagues [8] and Hasegawa and

hc 4.4%

hc 7.5%
colleagues [13] reported 10-year local control rates

hc 2%
Other

of more than 90% in separate series of patients

treated with GK. The median follow-up period in
Hasegawa’s study was 7.8 years. Partial or com-
plete radiographic response to treatment occurred
3.7% (0.7% w/dose

Hearing preservation rate is the percentage of patients who maintained or gained useful hearing (Gardner-Robertson class 1-2) after treatment.
dose %12.5 Gy)

in 62% of radiated tumors, and tumors !15 cm3

neuropathy rate

had a better progression-free survival than tumors

O15 cm3 (96% versus 57%, P ! .001). Tumors
7.5% perm

2.5% perm
9.5% temp
Trigeminal

not compressing the brainstem or obstructing the

18%

fourth ventricle had a better progression-free

8%

8%

survival (97% versus 74%, P ! .008). Tumor pro-

gression occurred within 3 years from the time of
treatment in most cases. Forster and colleagues
2.4% temp, 8% perm
5% perm, 15% temp

[46] documented local control rates for tumors

4.4% (0.7% w/dose

O3 cm, 2 to 3 cm, and !2 cm at 33%, 86%, and

neuropathy rate

89%, respectively. Similar findings were reported

%12.5 Gy)

11.8% temp
4.4% perm

2.5% perm

by Kondziolka and colleagues [4].

Friedman and colleagues [7] reviewed the
Facial

outcomes of 390 patients treated with LINAC

7%

radiosurgery for VS. With a median follow-up

of 40 months and a median dose of 12.5 Gy, the
5- and 10-year local control rates were 90% with
preservation rate

all patients deaf

only 1% of patients requiring surgery for treat-

ment failure during the follow-up period. With
before tx

a median follow-up period of 48.5 months, Combs

Hearing

and colleagues [30] also reported a local control

55%

75%
71%

82%

rate of 91% at 10 years.

LINAC stereotactic radiosurgery outcomes (median marginal dose 12–14 Gy)

The longest follow-up periods for the FSRT

Abbreviations: hc, hydrocephalus; perm, permanent; temp, temporary.

studies are from Combs and colleagues [35],

1% required surgery

Sawamura and colleagues [40], and Chan and

colleagues [42]. The median follow-up periods in
90% @ 5 y, only

these studies averaged 48 months, and the 5-year

local control rates in all three studies are more
91% @ 10 y
control rate

than 90% (see Table 3).

Unlike with use of GK, tumor volume or size
95.4%
100%

100%

100%
Local

98%

has not been shown to be of prognostic value in

predicting response to treatment with LINAC
radiosurgery or fractionated stereotactic radio-
therapy for acoustic neuromas. Different fraction-
of patients

ation regimens were used depending on tumor size

Number

[11,37].
390

149
45

26

49
44

42

Hearing preservation has not been documented

according to a consistent standard (Table 4), and
no randomized studies regarding tumor control
Speigelman, 2001 [32]

and hearing preservation have been reported to

Friedman, 2006 [7]

date. The use of pure tone audiometry and dis-

Combs, 2006 [30]
Chung, 2004 [29]

Meijer, 2003 [31]

Foote, 2001 [33]

crimination testing before and at a standardized

Lee, 2001 [34]

interval after treatment would be optimal. In

most of the radiotherapy papers reviewed, the
Table 2

Author
(year)

statistic that is most often reported is the per-

centage of patients who maintained or gained
Table 3
Fractionated stereotactic radiation therapy outcomes
Author Number Local Hearing Facial Trigeminal Other
(year) of patients Dose control rate preservation rate neuropathy rate neuropathy rate complications
Andrews, 2001 [10] 46 50 Gy/25 fx 97% 81% 2% 7% hc 3.6%, vertigo 1.7%,
ataxia 3.6%
Combs, 2005 [35] 106 57.6 Gy/32 fx 93% 98% 2.3% 3.4%

CHOICES FOR TREATING VESTIBULAR SCHWANNOMAS

Chung, 2004 [29] 27 45 Gy/25 fx 100% 57% 4% temp 7% temp hc 4%
Shirato, 1999 [36] 33 36–44 Gy/20–22 fx 98% 53% 5% temp 12% temp
Williams/Shokek, 375 25 Gy/5 fx 97% 59% 1.5% temp 1.2% temp
2004 [11,37] (!3 cm diam), 0.5% perm
30 Gy/10 fx (O3 cm
diam), 40 Gy/20 fx
Meijer, 2003 [31] 80 20–25 Gy/5 fx 94% @ 5 y 61% 3% 2%
Fuss, 2000 [38] 42 57.6 Gy/32 fx 97.5% @ 5 y 85% 0% 4.8%
Selch, 2004 [39] 48 54 Gy/30 fx 100% 91.4% 2.8% 3.8% Tinnitus 5%, hc 0%,
ataxia 2%
Sawamura, 2003 [40] 101 40–50 Gy/20–25 fx 97% 71 4% temp 14% hc 11% disequilibrium
16.8%
Poen, 1999 [41] 31 21 Gy/3 fx/24 h 97% 77% 3% 16%
Chan, 2005 [42] 70 54 Gy/30 fx 92% 84% 1% 4%
Chang, 2005 [43] 61 21 Gy/3 fx/24 h 98% 74% 0% perm 0%
Lederman, 1997 [44] 38 20 Gy/4 fx or 100% 93.5% (does not 2.6% temp 0%
20 Gy/5 fx distinguish useful
from no useful
hearing)
Ishihara, 2004 [45] 38 15–20.5 Gy/1–3 fx 94% 93% 2.6% temp 2.6%
Hearing preservation rate is the percentage of patients who maintained or gained useful hearing (Gardner-Robertson class 1-2) after treatment.
Abbreviations: fx, fraction; hc, hydrocephalus; perm, permanent; temp, temporary.

383
384 BACKOUS & PHAM

Table 4
Hearing classification scales
AAO-HNS classification
Pure tone average (0.5, 1, 2, Speech discrimination
Class 3 kHz measured in dB HL) score (%)
A 0–30 70–100
B 31–50 50–100
C O50 50–100
D Any !50
Gardner-Robertson Classification
Class Pure Tone/Speech Reception Speech Discrimination
Threshold (dB HL) Score (%)
1 0–30 70–100
2 31–50 50–69
3 51–90 5–49
4 O90 1–4
Word Recognition Scores
Class Word Recognition Score (%)
I 70–100
II 50–69
III 1–50
IV 0%
Data from Meyer TA, Canty PA, Wilkinson EP, et al. Small acoustic neuromas: surgical outcomes versus observa-
tion or radiation. Otol Neurotol 2006;27(3):380–92.

useful hearing (Gardner-Robertson classes 1 and using stereotactic radiosurgery to control VS

2). Intervals for testing hearing after treatment growth and maximize hearing preservation.
are not clear, and in some studies residual hear- Others attempt to minimize toxicity and im-
ing was defined by whether patients could talk prove hearing preservation rates by fractionating
on the telephone on the side of the affected the dose. This practice is based on a radiobiologic
ear. Hearing loss is rare, tends to occur over principle that there is a direct relationship between
6 to 24 months, and can continue to decline late normal tissue damage and dose per treatment
for years after treatment. Prasad [12] reported delivered to the tissue. Andrews and colleagues
a useful hearing preservation rate of 58% with [10] performed a prospective, nonrandomized
a median follow-up of 4.2 years. In most patients, study to compare the outcomes of treatment for
hearing decline occurred after 2 years and contin- acoustic neuromas with GK radiation to FSRT.
ued up to 8 years after treatment, with a hearing The GK dose was 12 Gy and the FSRT dose
preservation rate of 75% for tumors !1 cm3 com- was 50 Gy in 25 fractions given daily. They found
pared with 57% for tumors O1 cm3. Massager [15] no difference in local control rates, but the hearing
demonstrated better useful hearing preservation preservation rate was 33% for GK treatment com-
rates in tumors with intracanalicular volumes pared with 81% for fractionated stereotactic radio-
!100 mm3 compared with larger tumors (82.6% therapy. These results should be viewed with
versus 44.8%, P ¼ .045). caution because they had relatively short mean fol-
Improved hearing outcome has been demon- low-up of less than 3 years. Although most of the
strated at doses %13 Gy. In an early paper from FSRT studies reported excellent hearing preserva-
the University of Pittsburg, Kondziolka and tion rates, they have shorter follow-up than other
colleagues [4] reported a ‘‘useful’’ hearing preser- radiosurgery series. The different fractionation
vation rate of 46% at an average marginal dose regimens make it difficult to make any conclusions
of 16 Gy. Later studies documented hearing pres- regarding dose and hearing preservation.
ervation in up to 68% to 78% of cases treated in Facial neuropathy is a potential complication
the range of 12 to 13 Gy [13,14]. Because there with all three treatment options for VS. Kond-
were no differences in the local control rates ziolka and colleagues [4] performed a multivariate
with the lower doses, it is common practice to pre- analysis of 162 patients treated with GK and
scribe 12 to 13 Gy to the tumor margin when found that tumor volume and dose of radiation
 CHOICES FOR TREATING VESTIBULAR SCHWANNOMAS 385

to the tumor margin were associated with the risk transformation occurred in one patient 51 months
of neuropathy (P ! .001). With an average after radiotherapy treatment. The malignant
marginal dose of 16 Gy, the overall rate of facial transformation rate in this study was 0.3% among
neuropathy was 15%. In subsequent reports, patients who were followed longer than 5 years
when 12 to 13 Gy were prescribed to the margin, after GK [13]. Two other case reports of malig-
the facial neuropathy rate dropped to 0%. Fried- nant transformation have been reported with
man and colleagues [7] presented a similar finding GK [47,48]. One case was reported of a patient
when they lowered their LINAC radiosurgery who developed a glioblastoma multiforme adja-
dose to 12.5 Gy (4.4% versus 0.7%). As seen in cent to an acoustic neuroma that was treated
Tables 1 and 2, the risk of facial neuropathy with with GK 7.5 years earlier [49]. Another patient
radiosurgery is minimal and often temporary treated with FSRT developed a low-grade malig-
with a dose of 12 to 13 Gy. Similar rates of facial nant nerve sheath tumor 216 months after initial
neuropathy are reported with FSRT despite vari- treatment [50]. Delayed malignant transformation
able doses and fractionation regimens (see Table 3). or radiation-induced malignancies are rare. Long-
Unfortunately, no consistent facial nerve func- term, yearly follow-up of these patients provides
tion scale was used for reporting. a more accurate assessment of incidence.
After performing a multivariate analysis, Recent papers compared the outcomes of
Kondziolka and colleagues [4] documented that microsurgery to stereotactic radiosurgery to at-
tumor volume and radiation dosage to the tumor tempt to determine which treatment is better for
margin were associated with the risk of trigeminal sporadic VS %3 cm. Myrseth and colleagues [22]
neuropathy (P ! .001). With average marginal reported a retrospective study of 189 consecutive
doses of 16 Gy, the overall rate of trigeminal patientsd86 treated by microsurgery and 103 by
neuropathy was 16% compared with 4.4% at 12 GK. The mean follow-up period was 5.9 years.
to 13 Gy. There was no evidence of trigeminal In addition to local control and cranial nerve
nerve damage for intracanalicular tumors. Fried- preservation, they also evaluated quality of life
man and colleagues [7] noted a similar finding through standardized questionnaires. The overall
when they lowered their LINAC radiosurgery local control rates for microsurgery and GK
dose to 12.5 Gy (3.7% versus 0.7%). were 89.2% versus 94.2%, which was not statisti-
Meijer and colleagues [31] reported the out- cally significant. Facial nerve function (House-
comes of treatment with LINAC compared with Brackmann grade 1-2) was preserved in 79.8%
FSRT for their patients with VS. They found a sta- of the microsurgery group and in 94.8% of the
tistically significant increased incidence of trigemi- GK group (P ¼ .0026). The middle fossa ap-
nal neuropathy in patients treated with FSRT proach was not used in these patient cohorts.
(8%) versus LINAC radiosurgery (2%) (P ¼ .048). Overall, the quality-of-life scores were signifi-
Other potential side effects from radiation cantly lower in the microsurgery group compared
therapy include vertigo, tinnitus, ataxia, head- with the GK group. The authors concluded that
ache, hydrocephalus, cyst formation, radiation- these results favored GK as the treatment of
induced edema or necrosis, intratumoral bleeding, choice for this group of patients.
and malignant transformation (see Tables 1–3). Pollock and colleagues [21] presented a pro-
The reporting of these toxicities has not been spective cohort study of 82 patients with
consistent. The rate of hydrocephalus ranges unilateral, unoperated VS !3 cm in greatest di-
from 0 to 11% [10,29,39]. Sawamura and col- mension undergoing surgical resection (n ¼ 36)
leagues [40] reported the highest incidence of or GK (n ¼ 46). Other than age (patients undergo-
hydrocephalus, with 11% of 101 patients treated ing microsurgery were younger), all other pre-
with FSRT 40 to 50 Gy in 20 to 25 daily fractions treatment characteristics were matched in the
manifesting communicating hydrocephalus that two treatment groups. The mean follow-up period
requires ventriculoperitoneal shunting. The hy- was 42 months. There was no difference in the
drocephalus resolved in all patients with shunt local control rate between microsurgery and
placement and was assumed to be caused by cere- GK (100% versus 96%, P ¼ .50). They found
brospinal fluid (CSF) malabsorption associated that facial nerve preservation (96% versus 75%,
with VS. P ! .01) and serviceable hearing rates (63%
Delayed malignant transformation or radia- versus 5%, P ! .001) were better in the GK group
tion-induced malignancy may occur with radia- than microsurgery group. With regard to quality
tion therapy for acoustic neuromas. Malignant of life, patients who underwent microsurgery
386 BACKOUS & PHAM

had a decline in physical functioning and bodily and stroke. Harsha and Backous [55] performed
pain scores, whereas patients who had GK had a Medline search for all articles pertaining to VS
lower Dizziness Handicap Inventory scores. All surgery published from 1994 to 2004. They refined
of these differences in quality of life were statisti- the search to include only English-language arti-
cally significant. Although the authors concluded cles that reported hearing outcome using either
that radiosurgery should be considered the best the AAO-HNS hearing or Gardner-Robinson
treatment for this group of patients, they com- hearing outcome scales, facial nerve outcomes
mented on the necessity of longer follow-up. using the AAO-HNS (House-Brackmann) facial
nerve grading scale for facial nerve outcome,
and complication rates based on approach used.
Microsurgery
Of 1132 articles evaluated, 31 met the criteria
Three principal approaches are used to surgi- for inclusion and only 14 compared one or more
cally remove VS. The translabyrinthine (TL) route outcome measure for more than one approach
is used for tumors with either no hearing or in from the same institution. Two additional articles,
cases with little chance of preserving hearing. TL which specifically addressed hearing preservation
typically would be chosen in patients with less after middle fossa surgery, have been published
than 50% speech discrimination and 50 dB speech subsequent to this article (Table 5) [56–69].
reception threshold, in intracanalicular tumors Overall, hearing outcome was serviceable
extending to the lateral margin of the internal (AAO-HNS class A or B/Gardner-Robinson class
auditory canal, or in tumors O3 cm in largest 1 or 2) in 618 of 2034 (30%) of patients treated
dimension. Two approaches, the middle cranial with a hearing preservation approach. Results
fossa (MCF) and retrosigmoid (RS), are used in were considerably better with MCF (523/1017
cases with potential hearing preservation. MCF is [51%]) than with RS (95/304 [31%]). In six
ideal for intracanalicular tumors with minimal papers, MCF was compared with RS in the
extension into the cerebellopontine angle. The RS same institution. Forty-eight percent preserved
approaches the cerebellopontine angle with better serviceable hearing with MCF compared with
visualization of the cranial nerves, the pons, and 31% with RS [55]. The two most recent articles
brainstem. Anatomic limitations may foster that addressed hearing preservation and the
recidivism with the hearing preservation ap- MCF approach reported high levels of maintained
proaches. The falciform crest obscures the inferior hearing in the serviceable range. Arts and col-
portion of the lateral fundus when using the leagues [67] reviewed 73 consecutive patients
MCF. Inferior vestibular nerve tumors may excised via MCF. Of the 27 with class A hearing
extend into this region to an inaccessible space preoperatively, 62% remained class A, 18%
of 1.82 to 2.33 mm [51,52]. The RS approach deteriorated to class B, and 20% fell to class C.
leaves up to the lateral 3 mm of the internal audi- Overall, of the 62 patients presenting with class
tory canal underexposed. The addition of opera- A or B hearing before surgery, 73% remained in
tive endoscopy has proved useful in accessing class A or B. No patients improved their hearing
the lateral 30% of the internal auditory canal class with surgery [66].
[53]. Larger tumors with significant brainstem Meyer and colleagues [68] reported on 162 con-
compression may be accessed via an RS route in secutive VS resected by the MCF approach. Class
the setting of poor hearing because of the better A or B/Class 1 or 2 hearing was preserved in 66 of
visibility of brainstem structures. Prognostic 162 (41%) patients overall. Eight patients im-
factors that favor hearing preservation include proved to class 1 hearing with surgical removal
a small tumor, greater than 70% speech discrimi- of their VS. Of the 113 patients with word recog-
nation and a 30dB speech reception threshold, nition scores (WRS) more than 70% preopera-
auditory brainstem response with a normal wave tively, 56 (50%) maintained WRS of more than
V amplitude and latency, and no medical contra- 70% after excision. Tumor size ranged between
indications for surgery [54]. The choice of 0.2 and 2.5 cm in largest diameter. Smaller tumors
approach also depends on the preferences of the (0.2–1.0 cm) had 59% maintain WRS more than
surgical team. 70% and 9% improved to more than 70% when
The most commonly reported major compli- falling below preoperatively. For tumors that
cations from microsurgery include hearing loss, measured 1.1 to 1.4 cm, 39% maintained at least
facial nerve dysfunction, balance abnormalities, 70% WRS and 3 additional patients (9%) im-
cerebrospinal fluid leakage, headache, meningitis, proved to more than 70% postoperatively. Only
 CHOICES FOR TREATING VESTIBULAR SCHWANNOMAS 387

Table 5
Hearing outcomes using AAO-HNS/Gardner-Robinson classifications
Postoperative hearing classification [Number (%)]
Author (ref) A/1 B/2 C/3 D/4
Retrosigmoid approach
Colletti, 2003 [56] 2 (8%) 3 (32%) 4 (16%) 11 (44%)
Staecker, 2000 [57] 6 (40%) 1 (7%) 1 (7%) 7 (46%)
Hecht, 1997 [58] 4 (10%) 5 (12%) 4 (10%) 29 (68%)
Arriaga, 1997 [59] 8 (31%) 6 (23%) 1 (4%) 11 (42%)
Sanna, 2004 [60] 8 (18%) 7 (16%) 4 (9%) 29 (66%)
Magnan, 2002 [61] 17 (15%) 18 (16%) 23 (19%) 61 (51%)
Lassaletta, 2003 [62] 0 (0%) 5 (17%) 1 (3%) 23 (79%)
Total 45 (15%) 50 (16%) 38 (13%) 171 (56%)
Middle Cranial Fossa Approach
Colletti, 2003 [56] 3 (12%) 10 (40%) 3 (12%) 9 (36%)
Staecker, 2000 [57] 5 (33%) 3 (20%) 2 (13%) 5 (33%)
Hecht, 1997 [58] 4 (22%) 3 (17%) 1 (5%) 10 (56%)
Arriaga, 1997 [59] 15 (44%) 8 (24%) 2 (6%) 9 (26%)
Sanna, 2004 [60] 4 (7%) 15 (26%) 10 (17%) 30 (50%)
Slattery, 1997 [63] 35 (25%) 39 (27%) 5 (4%) 64 (45%)
Brackmann, 2000 [64] 109 (33%) 87 (26%) 16 (5%) 121 (36%)
Weber, 1996 [65] 5 (10%) 13 (27%) 3 (6%) 28 (57%)
Satar, 2002 [66] 30 (22%) 42 (31%) 18 (13%) 64 (34%)
Arts, 2006 [67] 21 (29%) 24 (33%) 6 (8%) 22 (30%)
Meyer, 2006 [68] 37 (23%) 29 (18%) 4 (2.5%) 83 (51%)
Total 268 (26%) 255 (25%) 64 (6%) 430 (42%)

33% of patients with tumors between 1.5 and 12-month visit, is the gold standard for reporting
2.5 cm maintained WRS more than 70%. In the facial nerve outcomes after VS treatment. In the
hands of the same surgical team, statistically sig- review by Harsha and Backous [55], overall grade
nificant improvement was noted in cases in which I/II function was 82%, with 92% of RS, 89% of
near field eighth nerve compound action potential MCF, and 73% of TL having a good outcome.
monitoring was used. With tumors !1.4 cm, Only one center reported results of all three
MCF done in experienced hands provided signifi- approaches within the same institution. Grade I/II
cant hearing preservation not previously reported. function was maintained as follows: RS (91%),
This article suggested changing the hearing MCF (88%), and TL (77%). Centers with more
classification to report only WRS because it than 100 cases with a single approach were ana-
more accurately reflects rehabilitation potential lyzed separately and good function (AAO-HNS
for ‘‘residual’’ hearing. I/II) was maintained as follows: RS (97%),
Hearing preservation rates reported in Table 5 MCF (93%), and TL (78%). These results were
address the number and percent of patients with not analyzed separately for tumor size, which
serviceable hearing when compared with all pa- may have contributed to the choice of surgical ap-
tients treated by approach. This rate does not proach. Meyer and colleagues [68] reported grade
take into account the number of patients who I/II function in 97% of 162 patients (86% grade I)
maintained or improved their hearing status after operated via the MCF, whereas Arts and col-
operative tumor removal. In the two studies that leagues [67] maintained 96% grade I/II function
specifically examined MCF for intracanalicular (85% grade I).
tumors, the results are much more favorable. No specific data were presented with standard-
Long-term (O5 years) hearing preservation in ized methodology to assess postoperative vestib-
this group of patients should be compared with ular and other balance dysfunction.
patients who received radiotherapy at matched CSF leakage is the most common complication
intervals. after VS resection. Typically, CSF leaks occur 2 to
The AAO-HNS (House-Brackmann) facial 3 days postoperatively (early) and at 10 to 14 days
nerve grading scale score, determined at the 6- to after surgery (late). Pooling the data from
388 BACKOUS & PHAM

19 studies revealed an incidence of 8% (360/4297) and regression in 8%. Growth patterns were vari-
for CSF leakage. Leakage rate stratified by able and unpredictable. Leeuwen and colleagues
approach was: 6% MCF (67/1038), 11% RS [75] found no correlation among tumor size, symp-
(42/380), and 8% TL (253/2881) [55]. Slattery toms, and patient age in 164 patients treated and
and colleagues [70,71] reported 1697 patients over a period of 13 years in Holland. Yoshimoto
from the House Ear Clinic and found a higher [76] reviewed 1340 patients from a Medline search
incidence of CSF leakage in the RS group (15%) of 26 studies. The overall growth of VS during
compared with TL (11%) and MCF (6%). Seles- a mean follow-up period of 38 months was 46%.
nick [72] performed a meta-analysis and found Growth averaged 1.2 mm/y. Raut and colleagues
the lowest CSF leakage rate in the TL group [77] reviewed conservative management of 72 pa-
(9.5%) when compared with the RSA and MCF tients in the United Kingdom. Growth was seen
groups (both 10.6%). Overall, the CSF leak rate in 38.9%, no growth in 41.7%, and regression in
is low for each of the three approaches and may 19.4% after a mean follow-up of 80 months. Pure
vary according to the preferences and experiences tone and speech discrimination scores deteriorated
of individual surgeons and skull base teams. regardless of tumor growth. In Aarhus County,
Headache is common in the first few days to Denmark, 162 VSs were diagnosed and 64 patients
weeks after VS resection. Prolonged postoperative opted for conservative management. Twenty-three
headachedbeyond 3 monthsdoccurs in roughly percent of tumors grew more than 1 mm/y, 55% did
10% of cases. Bone dust in contact with the CSF not grow, and 22% regressed. The observation
and meninges causing secondary aseptic meningi- period extended to 15 years (180 months) [78].
tis, entrapment of the occipital nerve in neck Unfortunately, no common predictors or ‘‘red
musculature and scar tissue, scarring of the neck flags’’ could be extracted from these studies to
muscles to the dura, and migraines are considered predict growth patterns for VS.
the four mechanisms for prolonged headaches [54]. Battaglia and colleagues [79] reviewed 164 pa-
No standardized reporting scheme has been devel- tients treated with radiation therapy from 1986
oped for postoperative headache, but pooled data to 2004 at the Southern California Permanente
from six articles revealed clinically significant head- Group and compared their long-term growth
ache in 21% of RS, 8% MCF, and 3% TL [55]. rates with the results of a meta-analysis of five
Staecker [57] found more than double the headache studies of cases managed conservatively. They re-
rate in RS (47%) when compared with MCF (20%). ported an average growth rate of 0.7 mm/y. They
The rate of bacterial meningitis was 2% to 3% determined that small intracanalicular tumors
for all three approaches [55]. Meningitis peaked at grew more slowly, if at all, when compared with
3 to 5 days postoperatively and was most com- tumors in the cerebellopontine angle. They
monly caused by Staphylococcus aureus. Aseptic concluded that to determine whether radiotherapy
meningitis can occur in up to 20% of patients and alters tumor growth rate, a direct comparison of
responds to intravenous or oral steroids. Seizure, small, medium, and large tumors is necessary.
hydrocephalus, and stroke rates caused by surgery The option of observation with serial MRI
are rare and occur in less than 2% of cases. scanning is a valid form of managing VS. Severity
of symptoms does not correlate with lesion size,
and determination of efficacy of radiotherapy in
Observation with serial neuroimaging
small tumors VS the natural history of intra-
An often undervalued treatment modality for canalicular lesions is unclear. If no brainstem
VS is observation with serial neuroimaging using compression or hydrocephalus is present, patients
MRI scanning at regular intervals. A report from should be offered an observation course with
Saudi Arabia retrospectively reviewed 205 patients an understanding that no clear risk factors
with intracanalicular tumors for an average of for growth have been defined. Our center
40 months (range 12–180 months). No growth considers observation and interval symptom
was found in 66.3% of patients, 23.9% showed review, physical examination, and neuroimaging
slow growth, and 4% had rapid growth. Six a form of treatment and document it as such.
patients (3%) had tumor regression [73]. Charabi
and colleagues [74] reported on 127 tumors in 123
Summary
patients followed from 1973 to 1993 in Denmark.
At a mean follow-up of 3.8 years, tumor growth Patients diagnosed with VS are faced with
was found in 82% of tumors, no growth in 12%, a difficult decision-making process based on
 CHOICES FOR TREATING VESTIBULAR SCHWANNOMAS 389

medical literature weakened by inconsistent re- intracanalicular VSs grow more slowly. The in-
porting of results. Radiotherapy protocols and terval for imaging may vary depending on tumor
surgical techniques have evolved in the past characteristics in individual patients.
30 years. Most reporting is retrospective and Stereotactic radiosurgery is a valid option for
does not adhere to consistent and validated tumors !3 cm in largest dimension. Tumors
standards for hearing preservation, facial nerve O3 cm are poorer candidates for radiotherapy
function, or balance disturbance. and should be considered for surgical removal
Confusion surrounding recidivism versus re- unless the patient is medically unstable. An intra-
currence of VS and no clear and validated canalicular VS that is !1.4 cm in greatest di-
definition of tumor control further clouds the mension with CSF between the lateral margin of
interpretation of results. The lack of randomized, the tumor and the labyrinth on T2-weighted MRI
blinded clinical trials has retarded the develop- imaging, has a normal wave V on auditory
ment of evidence-based clinical best practices for brainstem response, has more than 70% WRS,
recommending optimal treatment for patients and is in a patient with good health can be excised
dictated by such factors as tumor size, location, via MCF with hearing preservation approaching
and hearing level. Patients often review data on 70% and good facial nerve function in more than
the Internet, which is not peer reviewed and can 96% of cases.
complicate preoperative counseling. Longer term follow-up determines the longev-
The mandate for establishing and documenting ity of hearing preservation in surgical and radio-
informed consent in a format that is timely and therapy patients and catalogs late complications
understandable to individual patients remains. A possible after both. Most surgical complications
trend is developing in which neurosurgeons and occur within the first weeks after resection,
neurotologists are acquiring certification in GK whereas radiotherapy complications may occur
and other forms of radiosurgery. These surgeons many years after treatment is complete. The diffi-
provide a balanced informational process because cult cases are found in patients with VS !3 cm.
they are involved in either option selected by All three options are potentially useful, and
a patient. In other centers, a multidisciplinary unbiased provision of information to help
team of surgeons and radiotherapists individually them decide on a path of treatment is manda-
consults with patients within their own area of tory for proper informed consent.
expertise to present options to patients and foster At our institution, we use a multidisciplinary
appropriate informed consent. Whichever the team that includes a neurotologist, neurosurgeon,
approach taken to explain options to patients, and radiotherapist to explain the risks and
one physician should remain the gatekeeper of the benefits of each option for treatment. We give
patient’s care. Answering additional questions the patient a packet with selected articles, and
and ensuring the opportunity for interval clinical, a nurse-coordinator follows up to be sure all
audiometric, and radiographic follow-up remains questions are relayed to the appropriate physician
the responsibility of the care team. The choice to and answered. The process is designed to allow
follow surveillance is a patient choice. the patient a balanced presentation of information
Standardized quantification of tumor size to foster a timely decision that ensures adequate
(volumetric or greatest dimension), consistent informed consent.
adherence to the AAO-HNS facial nerve report-
ing scale, consistent acceptance of one reporting
Acknowledgments
standard for hearing and agreed-upon intervals
for testing, and follow-up for longer intervals This work was supported by the Listen for Life
improve data and foster outcomes-based best Foundation Fund at the Virginia Mason Medical
practices for reporting results and advising pa- Center.
tients for treatment.
Currently, in patients without significant
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