CLINICAL STUDIES

TOPIC Clinical Studies

Natural History of Hearing Deterioration in

Intracanalicular Vestibular Schwannoma
Ronald J. E. Pennings, MD,
PhD*
David P. Morris, MBBS, FRCS
(ORL-HNS)*
Linda Clarke, RN†
Stefan Allen,†
Simon Walling, MBChB,
FRCSC*
Manohar L. Bance, MB, MSc,
FRCSC*
*Division of Otolaryngology – Head &
Neck Surgery, Department of Surgery,
Dalhousie University, Halifax, Nova
Scotia, Canada †Division of Neurosur-
gery, Department of Surgery, Dalhousie
University, Halifax, Nova Scotia, Canada
Reprint requests:
Ronald Pennings, MD, PhD,
Dalhousie University,
Department of Surgery,
Division of Otolaryngology – Head &
Neck Surgery,
1278 Tower Road,
3184 Dickson Building,
VGH Site, QEII HSC, Capital Health,
B3H 2Y9, Halifax, Nova Scotia, Canada.
E-mail: r.pennings@kno.umcn.nl
BACKGROUND: Intracanalicular vestibular schwannomas have a range of treatment
options that can preserve hearing: microsurgery, stereotactic radiotherapy, and con-
servative observation.
OBJECTIVE: To evaluate the natural course of hearing deterioration during a period of
conservative observation.
METHODS: A retrospective case review was performed on 47 patients with a unilateral
intracanalicular vestibular schwannoma. Evaluation of growth was monitored by repeat
MRI scanning. Repeated pure-tone and speech audiometry results were evaluated for
subgroups of patients showing growth or no growth and by subsite location of tumor in
the internal auditory canal.
RESULTS: Patients had a mean follow-up of 3.6 years. Over the entire population, the
pure-tone average thresholds at 0.5, 1, 2, and 3 kHz and the word recognition scores
both significantly deteriorated from 38 to 51 dB HL, and from 66% to 55%, respectively.
Overall, 74% of subjects with good hearing, according to the 50/50 rule, maintained
hearing above this rule. There were no significant differences in hearing loss by subsite in
the internal auditory canal (porus, fundus, central) or by growth status (stable, growing,
shrinking). Only 6 patients showed a large hearing change. This happened early during
follow-up, with relatively stable hearing after this.
CONCLUSION: Hearing will deteriorate in some intracanalicular vestibular schwanno-
mas, regardless of tumor growth. Hearing deterioration, if on a large scale, most likely
occurs early in follow-up. The present results using conservative management in these
tumors appear similar to those reported for stereotactic radiotherapy or microsurgery.
KEY WORDS: Acoustic neuroma, Hearing, Hearing preservation, Intracanalicular, Microsurgery, Stereotactic
radiotherapy, Vestibular schwannoma

Received, September 9, 2009. Neurosurgery 0:1–10, 2010 DOI: 10.1227/NEU.0b013e3181fc60cb www.neurosurgery-online.com

Accepted, April 30, 2010.

A
Copyright ª 2010 by the vestibular schwannoma (often used syn-
Congress of Neurological Surgeons
onymously with the term acoustic neu-
roma) is a benign tumor arising from the
Schwann cell covering of the eighth cranial
nerve. These tumors often first present with
asymmetric hearing loss. Other symptoms may be
tinnitus, aural fullness, poor speech discrimination,
imbalance, or, rarely, vertigo. Varied explanations
for the etiology of hearing deterioration in patients
with vestibular schwannomas, including tumor-
ABBREVIATIONS: ANOVA, analysis of variance; HL,
hearing level; IAC, internal auditory canal; PTA,
pure-tone average; SRT, stereotactic radiotherapy;
WRS, word recognition score
induced expansion with subsequent increase of
pressure in the internal auditory canal, cochlear
hair cell loss, vascular compromise, and a change in
inner ear fluid composition, have been reported.1,2
As accessibility to MRI scanning has im-
proved, the number of patients with a diagnosis
of vestibular schwannoma has increased, and the
size of tumor at diagnosis appears to be smaller.
In recent years, the management of vestibular
schwannomas has become controversial, because
several different treatment strategies have been
proposed.
Intracanalicular tumors present several valid
management choices. Their small size allows for
the possibility of hearing preservation after sur-
gery, but also for policies of conservative

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PENNINGS ET AL
observation (‘‘wait-and-scan’’) and of stereotactic radiotherapy.
Larger tumors offer more circumscribed options, because hearing
preservation following surgery is much less likely with larger
tumors, and size alone may dictate surgical intervention to pre-
vent immediate risk to the patient. In intracanalicular tumors, the
management decisions are more complex, and the impact of the
available treatment options on hearing outcomes becomes of
much greater importance.
This report documents the auditory consequences of conser-
vative management in a cohort of patients with intracanalicular
tumors to aid this decision-making and management strategy
comparison process.
To date, very few studies3-5 have examined the natural course
of auditory function in purely intracanalicular vestibular
schwannomas. Only one study3 reported the results on more than
20 patients. We discuss comparisons with the hearing results
obtained after intervention with surgical and stereotactic radio-
therapeutic options.
PATIENTS AND METHODS
Informed consent is taken at first presentation to enter all patient
details into a database for follow-up and research purposes. Ethics ap-
proval for this study was obtained from the Capital Health Research
Ethics Board #CDHA-RS/2009-270.
Conservative Management Protocol
Our policy is that purely intracanalicular vestibular schwannomas are
followed by a conservative observational ‘‘wait-and-scan’’ protocol,
which is systematically followed. After the first MRI scan, the second
MRI scan is performed at 6 months. If no growth is seen, annual MRI
scans are performed up to 5 years. Between 5 and 10 years, scanning is
performed every 2 years, and after this period every 5 years.
Sample characteristics
The present cohort consists of patients with an intracanalicular ves-
tibular schwannoma identified on MRI scanning who presented to our
Skull Base Clinic between 1998 and 2007. Exclusion criteria included
intralabyrinthine lesions (n = 2), neurofibromatosis type 2 (n = 1), and
cases (n = 17) with fewer than 3 consecutive MRI scans. In addition, one
more patient was excluded because of insufficient data. After a 2-year
follow-up, significant growth of tumor was seen and he was referred for
stereotactic radiotherapy (SRT). However, only one pre-SRT audiogram
was made; therefore, it was decided to exclude this patient. In total, 47
(28 female and 19 male) patients with purely intracanalicular vestibular
schwannomas were included in the study. The median age at the first
audiogram was 55.7 years with a range of 26.3 to 80.0 years. The mean
follow-up time (difference in time between the first and last audiogram)
was 3.6 years with a range of 0.7 to 7.0 years.
Tumor Designations and Nomenclature
Significant growth of tumor was defined as an increase in size of more
than 2 mm in diameter between the first and last MRI scan.6 Using this
criterion, tumors were labeled as ‘‘growing’’ (40% or 19/47), ‘‘stable’’
(51% or 24/47), or, if they shrank by more than 2 mm, as ‘‘shrinking’’
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(9% or 4/47). In 18 patients growth of tumor was seen into the cer-
ebellopontine angle. Details of growth will be published separately.
Tumors were also characterized by location in the internal auditory canal
(IAC). Lateral ones were designated as ‘‘fundus’’ (n = 16), midportion as
‘‘central’’ (n = 24), and medial as ‘‘porus’’ (n = 7). In fundus tumors there
was no cerebrospinal fluid (CSF) between tumor and cochlea, in central
tumors there was CSF on both sides of the tumor, or the tumors filled
the entire IAC, and in porus tumors there was CSF between tumor and
cochlea.1 There was no significant difference in size at the time of
diagnosis in these 3 subsite locations of tumors. The average follow-up
times for the subtypes of tumors (years in parentheses) were growing
(3.2), stable (3.7), shrinking (5.1), fundus (4.0), central (3.7), and porus
(2.5). These were not significantly different on analysis of variance
(ANOVA) testing.
Hearing Assessment
All patients monitored in our clinic undergo audiometric testing at the
same time that their MRI scans are repeated. Pure-tone audiogram and
speech recognition scores are tested in standard sound-attenuating au-
diology booths, following standard American National Standards In-
stitute calibration and sound-level protocols. The pure-tone average
(PTA) of the hearing thresholds at index frequencies of 0.5, 1, 2, and 3
kHz (PTA0.5,1,2,3 kHz) was used according to the recommendations of
the Committee on Hearing and Equilibrium of the American Academy
of Otolaryngology-Head and Neck Surgery.7 If patients underwent
treatment, pre- and posttreatment hearing was also categorized according
to the American Academy of Otolaryngology and Head and Neck
Surgery (AAOHNS) classification. The first audiogram at diagnosis and
the last audiogram (before treatment) were used to evaluate deterioration
of hearing caused by the policy of conservative management. Hearing
results were analyzed for the whole group (n = 47), and separately for the
subgroups of growing, stable, and shrinking tumors, and for subsite
location in the IAC.
In those cases where MRI scanning showed tumor growth, an in-
dividualized decision was made about rescanning or referral for SRT
based on size of tumor, degree of growth, and patient factors such as age
and medical condition. The decrease in hearing after treatment was
evaluated separately and is not reported as part of the conservative
management strategy.
Statistical Analyses
For comparisons between the groups, paired t tests, Pearson corre-
lation tests, and one-way ANOVA followed by the Tukey multiple
comparison test were performed after testing for a Gaussian distribution
with the normality test. Criteria for significant differences was P , .05.
Because the distribution is often not Gaussian, nonparametric tests were
also used. Actuarial survival curves were also generated for hearing re-
tention. The computer software program Prism version 3.03 (GraphPad
Software Inc., San Diego, California) was used for statistical analysis.
RESULTS
Overall Results
Table 1 shows the deterioration in hearing for all 47 patients
with an intracanalicular vestibular schwannoma. The mean
PTA0.5,1,2,3 kHz threshold at the initial audiogram was 37.5 decibel
hearing level (dBHL). After follow-up, the mean threshold
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TABLE 1. Results of Evaluation of hearing in 47 Patients with An
Intracanalicular Vestibular Schwannoma
First Last
Audiogram Audiogram
All tumors PTA0.5,1,2,3kHz 37.5 (3.3) 50.9 (4.3)
(n = 47) in dB HL
WRS in % 66.2 (5.1) 54.5 (5.9)
PTA0.5,1,2,3 kHz, pure-tone average of thresholds at 0.5, 1, 2, and 3 kHz; HL, hearing
level; WRS, word recognition score. Numbers in parentheses indicate SEM. Bold
figures indicate significant deterioration of hearing.
decreased significantly to 50.9 dB at the last audiogram. This
represents a mean drop of 13.4 dB. The mean word recognition
score decreased significantly from 66.2% to 54.5%, a mean drop
of 11.7%. Figure 1 shows the mean audiogram of the affected ear
in all patients at their first and last visit. Figures 2 and 3 subdivide
this by tumor location and growth characteristics. Both one-way
ANOVA and Kruskal-Wallis testing, however, did not show any
significant differences in hearing loss between the 3 subtypes of
tumor location, or by growth characteristics, possibly because of
a lack of statistical power. Correlation testing on all patients and
on the different tested subgroups showed only one significant
correlation, between hearing at the first audiogram and the initial
tumor size. No other correlations (including those of growth
rates) were found. There was also no correlation between the
deterioration of hearing and age at diagnosis. Hearing in the
contralateral ear did not deteriorate during follow-up, with the
exception of a few older patients in whom the deterioration could
be attributed to presbyacusis (results not shown).
FIGURE 1. Mean change in audiograms in all patients.
Squares indicate mean first audiogram, triangles represent
mean last audiogram, and error bars indicate standard error
of the mean. HL, hearing level.
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HEARING IN INTRACANALICULAR VESTIBULAR SCHWANNOMA
However, further analysis showed that the mean
deterioration of hearing is not an adequate descriptor of the
data. When examining deterioration of the PTA and the word
recognition score (WRS), only 6 patients developed severe
P deterioration of hearing, whereas the remaining patients all had
relatively stable hearing or mildly progressive hearing loss. This
, .0001
is shown in the frequency distribution of hearing loss in Figure
.0060 4, which is clearly skewed toward most patients having no or
small losses, with a few large losses. In addition, one patient
with a stable tumor experienced hearing improvement during
follow-up. After a follow-up time of 1.8 years, the PTA im-
proved from 42.5 to 22.5 dB HL and the WRS improved from
88% to 96%.
When examining the time course of the hearing loss, it be-
comes clear that most of the significant drops in hearing oc-
curred early in the follow-up period, with relatively stable
hearing after the first 2 years of follow-up (Figure 5). The
characteristics of the 6 patients (gray symbols in Figure 5) who
developed significant hearing loss are shown in Table 2. A
Mann-Whitney nonparametric test showed no differences in
size, age, and location between the 6 patients who had severe
progression of hearing loss and the remaining 41 patients. There
is no one defining characteristic for these patients. To evaluate
whether this is also the case in the 31 patients with useful
hearing (50/50), a separate analysis was performed. Hazards
were calculated on the basis of the number of patients at risk in
the time period as the denominator, with the number losing
hearing (a drop of more than 40% in WRS) over the time
period. Results were: 0 to 1 year, 1/31 (3.2%); 1 to 2 years, 2/30
(6.7%); and 2 to 5 years, 1/72 (1.3%). A related survival curve
of this analysis is shown in Figure 6.
A common way to define hearing status is by a combination
of PTA0.5,1,2,3 kHz and WRS, such as the 50/50 rule, ie, greater
than 50% WRS and a PTA0.5,1,2,3 kHz of less than 50 dB
(AAOHNS classes A and B). An overview of all hearing results
specified per patient is given in Table 3. Of those patients (n =
31) whose initial hearing was better than the 50/50 hearing
rule, 74% (n = 23) remained better than the 50/50 rule at the
end of follow-up. Looking at an actuarial survival analysis of
hearing status according to this rule (Figure 7), it appears
initially that there is an ongoing risk of hearing loss throughout
the follow-up period. However, this graph should be in-
terpreted with caution, because there are few data points at 7
years, and the marked drop in the later years is because of one
patient crossing the 50/50 boundary line. When only class A is
used to evaluate hearing preservation, ie, greater than 70%
WRS and a PTA0.5,1,2,3 kHz of less than 30 dB HL, the hearing
preservation rate is 65% (13/20). Finally, we evaluated hearing
deterioration in patients with 100% WRS at diagnosis. Nine
patients had this and 8 (89%) of these patients managed to
keep the WRS above 70%; one patient had complete loss of
hearing in the affected ear. Sixteen patients had a WRS of 90%
or more and 13 (81%) kept this above a WRS of 70% during
follow-up.
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FIGURE 2. Mean change in audiogram subdivided by tumor growth subtype. Squares indicate first audiogram, triangles
represent last audiogram, and error bars indicate standard error of the mean. HL, hearing level; VS, vestibular schwannoma.

Treatment and Subsequent Hearing Change in
Growing Tumors
Based on individual evaluation it was decided that 8 of the 19
patients with a growing intracanalicular vestibular schwannoma
needed treatment (17% of all patients). Most of those patients
that showed growth of tumor have not (as of yet) been referred
for radiation, because they have relatively small tumors and
the decision was made to continue to closely follow them. One
patient opted for surgery using a translabyrinthine approach. She
had no useful hearing in that ear before surgery. The 7 remaining
patients underwent SRT. Three of them did not have any useful
hearing before treatment. One patient, who moved out-of-
province after SRT, had no follow-up audiometry. The remaining
3 patients experienced deterioration of hearing from a mean pre-
SRT PTA0.5,1,2,3 kHz value of 36.7 dB to a post-SRT value of 52.9
dB. The mean word recognition score decreased from 83.3% to
68.0% over a mean follow-up time of 2.5 years post-SRT.
DISCUSSION
The present study reports on the natural course of auditory
function in 47 patients with a purely intracanalicular vestibular
schwannoma, with the use of conservative management (‘‘wait-
and-scan’’ policy). Taken as a whole group, hearing deteriorated
significantly in this group of patients from a mean PTA0.5,1,2,3 kHz
of 37.5 dB HL at the first audiogram to 50.9 dB HL at the last-
visit audiogram. The word recognition score deteriorated sig-
nificantly from 66.2% to 54.5%. ANOVA testing showed there
was no significant difference in hearing deterioration between
growing, stable, or shrinking tumors nor between central, fundus,
and porus tumors (acknowledging that there is a decrease in
power with smaller numbers in each subgroup). Eight patients
(17% of all patients) eventually required intervention (surgery or
SRT) because of tumor growth.
Conservative management in even this small tumor population
has some risks to hearing. Analysis of the deterioration of hearing,
however, showed that only a few patients experienced severe
deterioration of hearing and this appears to occur relatively early,
with relatively stable hearing following this. This is an important
message, and one that has not been previously emphasized in
the literature. This early-loss effect can also be seen in the data of
Caye-Thomasen et al3 whose data show that hearing is mainly
lost during the very first years after diagnosis. Early hearing loss
is also seen in the data sample of Warrick et al,8 although not

FIGURE 3. Mean change in audiogram subdivided by tumor subsite. Squares indicate first audiogram, triangles represent last
audiogram, and error bars indicate standard error of the mean. HL, hearing level; VS, vestibular schwannoma.

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FIGURE 4. Frequency distribution of the deterioration of PTA0.5,1,2,3 kHz (A)
and WRS (B). The deterioration in hearing is plotted against the number of
patients that have a specific amount of deterioration. HL, hearing level; PTA,
pure-tone average; WRS, word recognition score.
explicitly acknowledged. The hearing loss data shown in Figure 6
imply a continual deterioration, but this is because an arbitrary
line (the 50/50 rule) is chosen as a ‘‘pass/fail’’ criterion. Hence,
ongoing small deteriorations in hearing in the order of 10 to 20
dB push some patients past this 50/50 rule if they were close to
it to begin with. The lack of data points at longer follow-up
intervals also magnifies the effect of change in even one patient,
because the percentage of change is calculated in actuarial analysis
as a percentage of remaining data points. Appreciating that most
large changes in hearing occur early during the follow-up period
has important clinical implications. If a patient has been moni-
tored for more than 3 years and has good hearing, the risk
of catastrophic hearing loss is probably small at that time. This
should be factored into the equation if choosing differing man-
agement options in patients who have been monitored for some
time, such as when taking over care from other centers.
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HEARING IN INTRACANALICULAR VESTIBULAR SCHWANNOMA
FIGURE 5. Deterioration of PTA0.5,1,2,3 kHz and WRS plotted against follow-up
time. Black and white symbols indicate the difference (deterioration) between the
first and last audiogram for all individuals during follow-up. White symbols
indicate patients with more than 70 dB HL on the first audiogram. Black
symbols present the deterioration of hearing in all remaining patients. Gray
symbols represent longitudinal hearing results in 6 patients with severe (.45 dB
HL PTA or .35% WRS) deterioration of hearing (the characteristics are
described in more detail in Table 2). HL, hearing level; PTA, pure-tone average;
WRS, word recognition score.
Definition of Useful Hearing and Hearing Preservation
In the past decade, there has been much discussion about
whether hearing preservation surgery in small intracanalicular
vestibular schwannomas with useful hearing should be the fa-
vored strategy for preserving this hearing, compared with a wait-
and-scan policy or SRT. The reported hearing results for these
treatments are variable, and this is muddied by the different
criteria applied for defining useful hearing and by the various
definitions of what hearing preservation means after surgery.
Most studies describe useful hearing as a PTA0.5,1,2,3 kHz of less
than 50 dB HL with a word recognition score higher than 50%
(50/50 rule). This equates to classes A and B by the AAOHNS,7
based on the classes I and II as defined by Gardner and
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TABLE 2. Patient Characteristics of Six Patients Who Had More Than 40 dB Deterioration of the PTA0.5,1, 2, 3 kHz or More Than 35 % Loss of WRS.
Patient First PTA Last PTA Deterioration First Last Deterioration Subsite Growth Size at First Size at Last
No. Age (y) (dB HL) (dB HL) (dB HL) WRS (%) WRS (%) (%) Location Status Diagnosis (mm) Diagnosis (mm)
32 37 23 64 41 100 0 100 Central Growth 13.8 19.7
35 69 52 110 58 80 0 80 Central Growth 8.9 13.7
38 51 34 60 26 88 4 84 Central Growth 9.7 14.7
42 56 31 55 24 92 48 44 Central Stable 8.8 10.4
45 37 62 111 49 38 0 38 Central Stable 3.1 3.5
58 50 21 110 81 80 0 80 Fundus Stable 0.9 1.2

PTA0.5,1,2,3 kHz, pure-tone average of thresholds at 0.5, 1, 2, and 3 kHz; HL, hearing level; WRS, word recognition score

Robertson.9 Sanna et al10 describe useful hearing as a PTA0.5,1,2,3 kHz since 1998) and therefore included more patients that were
better than 30 dB and a word recognition score of more than identified in a timeframe when MRI scanning was less available,
70% (70/30 rule). In 2006, Meyer et al11 suggested use of only and hence tumor size or location less exact. In addition, their
the word recognition scores to evaluate hearing in vestibular mean follow-up time is longer (4.6 years) than reported in our
schwannoma. study (3.6 years). A pass-fail rule like the 50/50 rule is very
A previous study by our group demonstrated that there was sensitive to how many patients start off close to the 50/50 border;
no ‘‘magic change’’ in ability to hear in background noise at the two studies with equal median deterioration can report very
50/50 or 70/30 rule when measured objectively, but rather a different preservation rates if in one study most patients were well
continuous decrease in speech comprehension as the PTA0.5,1,2,3 kHz above or below the 50/50 rule, and in another they were close
decreased.12 This is particularly relevant to Figure 6, because this to it. Because our study appears to have started out with more
kind of ‘‘pass/fail’’ plot is based on the assumption that there is better-hearing subjects, not many will cross the 50/50 line with
some nonlinear change in hearing ability when the 50/50 rule line small changes. Both studies have shown that hearing deteriorates
is crossed. Our previous study showed no such nonlinearity, but when comparing the first and last audiogram. However, in the
rather a gradual decline as WRS and PTA in the tumor ear fell. In present study this effect seems to be related mainly to the few
addition, the 50/50 rule may not be ideal in all cases. Some patients who had severe deterioration of hearing early in the
patients with more than 50 dB HL loss and near to normal speech follow-up period.
perception may still benefit from amplification. Finally, locali- In another article from the same Danish group, evaluation of
zation of sound can still benefit from hearing levels below the their large series of patients with vestibular schwannomas dem-
50/50 rule. onstrated that the main predictive factor is the initial speech
discrimination.15 A more recent article demonstrated that, of
Comparison of Present Results With the Literature those patients with 100% speech discrimination at diagnosis,
Only a few studies reported both PTA0.5,1,2,3 kHz and WRS
results after management of purely intracanalicular vestibular
schwannomas. Table 4 shows the hearing results from the present
study compared with results reported by other studies. Because of
the variations in criteria used to define hearing preservation, some
studies with large samples such as those reported by Samii
and Matthies,13 and more recently, Iwai et al,14 could not be
included.
The hearing results in our study are different from those re-
ported by Caye-Thomasen et al3 (Table 4), who reported pres-
ervation rates (using the 50/50 rule) of 47% (21/45) and (using
the 70/30 rule) of 47% (9/19), compared with our preservation
rate of 74% (23/31) and 65% (13/20), respectively. Their study
has so far reported on the largest group of patients with the
longest follow-up of intracanalicular vestibular schwannomas
managed with a wait-and-scan policy. In general, their patients FIGURE 6. Cumulative risk of losing less than 40% in
had worse hearing on initial testing than the patients reported in WRS during follow-up in all patients with initial useful
hearing according to the 50/50 rule. WRS, word recognition
the present study. This might be related to the fact that their
score; FU, follow-up.
study started much earlier than ours (since 1975; present study,

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 HEARING IN INTRACANALICULAR VESTIBULAR SCHWANNOMA

TABLE 3. Hearing Results for Patients with Growing, Stable, and Shrinking Tumors
Diagnostic Evaluation Follow-up Evaluation
Patient No. PTA0.5,1,2,3 WRS AAOHNS PTA0.5,1,2,3 WRS AAOHNS F.U.
Growing Tumors
1 30.00 84 A 42.50 72 B 6.96
2 27.50 96 A 41.25 64 B 5.63
4 45.00 80 B 45.00 96 B 4.04
6 98.75 0 D 110.00 0 D 5.16
11 43.75 92 B 53.75 100 C 6.54
14 45.00 0 D 52.50 0 D 4.93
21 96.25 0 D 110.00 0 D 1.50
25 43.75 52 B 53.75 36 D 4.03
32 22.50 100 A 63.75 0 D 2.87
33 12.50 100 A 23.75 90 A 1.63
34 12.50 96 A 13.75 100 A 4.28
35 52.50 80 C 110.00 0 D 1.55
38 33.75 88 B 60.00 4 D 3.09
39 20.00 84 A 47.50 56.00 B 1.18
50 16.25 92 A 15.00 96 A 1.32
51 17.50 100 A 18.75 96 A 2.37
57 21.25 100 A 23.75 100 A 0.96
60 47.50 22 D 56.25 41 D 1.61
65 20.00 90 A 37.50 100 B 1.93
Stable Size Tumors
8 46.25 60 B 58.75 28 D 5.09
9 55.00 12 D 63.75 0 D 6.11
10 32.50 40 D 58.75 48 D 6.29
13 10.00 100 A 28.75 100 A 5.61
19 67.50 68 C 66.25 72 C 4.97
20 30.00 70 A 58.75 64 C 5.57
23 47.50 16 D 61.25 16 D 4.59
24 63.75 4 D 98.75 0 D 6.02
26 66.25 20 D 76.25 0 D 3.06
28 25.00 76 A 26.25 68 B 3.00
29 7.50 100 A 13.75 100 A 5.65
30 61.25 44 D 63.75 56 C 2.35
36 15.00 100 A 18.75 100 A 3.49
40 37.50 96 B 47.50 100 B 2.65
41 38.75 76 B 43.75 56 B 1.78
42 31.25 92 B 55.00 48 D 3.02
43 52.50 56 C 58.75 44 D 2.92
45 62.50 38 D 111.25 0 D 2.15
48 7.50 100 A 10.00 100 A 1.92
49 25.00 68 B 22.50 76 A 0.73
54 10.00 100 A 13.75 92 A 0.65
58 21.25 80 A 110.00 0 D 4.41
62 30.00 88 A 33.75 92 B 4.21
63 42.50 88 B 22.50 96 A 1.84
Shrinking Tumors
5 80.00 0 D 76.25 0 D 5.82
12 30.00 68 B 33.75 80 B 4.39
15 7.50 96 A 8.75 100 A 4.59
74 53.75 0 D 72.50 20 D 5.75

PTA0.5,1,2,3 kHz, pure-tone average of thresholds at 0.5, 1, 2 and 3 kHz in dB HL; HL, hearing level; WRS, word recognition score in percent; AAOHNS, the classification according to
the American Academy of Otolaryngology and Head and Neck Surgery; F.U., follow-up in years.

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PENNINGS ET AL

after more than 10 years observation, 69% maintain good

hearing, compared with 38% of the patients with even a small
discrimination loss at diagnosis.16 These two studies on the same
patient groups deal with all types of vestibular schwannomas,
whereas we only present data on intracanalicular vestibular
schwannomas. The present study shows similar results with
a 88% preservation rate for patients with 100% speech dis-
crimination at diagnosis, although our follow-up needs to be
extended for many more years to confirm these data.

Comparison With Other Treatments for Intracanalicular

FIGURE 7. Cumulative risk of maintaining hearing above 50 dB for
PTA0.5,1,2,3 kHz and 50% for WRS (the 50/50 rule) and maintaining hearing
above 70% WRS. n, the number of patients; WRS, word recognition score; FU,
follow-up.
Vestibular Schwannomas
On the balance of reported results (Table 4), hearing preser-
vation surgery and SRT appear to be equally capable of preserving
hearing in small tumors (approximately 60%). Recently, an
overview of the hearing preservation results of microsurgery were
published by Noudel et al.17 They reported a hearing preservation
rate of 58% for the retrosigmoid approach and 62% for the
middle fossa approach (not significantly different). Most hearing
preservation microsurgical studies state that hearing preservation
surgery is especially important in young patients, because they
have more to lose and can handle extensive surgery better. Our
study shows there is no correlation between hearing deterioration
and age at diagnosis. Because the present result of 74% hearing
preservation with wait-and-scan appears better than the ap-
proximately 60% reported for microsurgery, we feel that younger
patients also deserve the option of a wait-and-scan policy, as an
alternative to microsurgery. A disadvantage in most studies of the
microsurgical treatment of intracanalicular vestibular schwan-
nomas is that the follow-up time is relatively short. Only a few
studies have reported long-term hearing results after hearing
preservation surgery. A study by Shelton et al18 demonstrated
that 56% of the patients who had had operations experienced
a decline in hearing quality during a follow-up time of 8 to 20
years. In another study by Chee et al19 the direct postoperative
serviceable hearing rate was 77%; however, this dropped to 57%
at a late evaluation with a mean follow-up of 9.4 years. Tran
Ba Huy reported on the results of a questionnaire he sent out
to French nonotologist ears, nose, and throat doctors and

TABLE 4. Overview of Some of the Reported Results in Literature on the Evaluation of Useful Hearing and Preservation of Hearing for
Different Types of Treatment for Intracanalicular Vestibular Schwannomas
%Hearing Preservation Mean/Median Follow-Up
Study Year No. of Patients Approach Using 50/50 Time (in Years)
Noudel et al17 2009 Review RSA/MCFA 58-62 Variable, usually
not long-term
Niranjan et al22 2008 96 Radiotherapy 61 3.5/2.3
Caye-Thomasen et al3 2007 156 Wait-and-scan 47 4.6/NA
Present study 47 Wait-and-scan 74 3.6/3.6

PTA, pure-tone average; WRS, word recognition score; 70/30, PTA #30 dB and WRS $70%; 50/50, PTA #50 dB and WRS $50%; NA, not available; RSA, retrosigmoid approach;
MCFA, middle cranial fossa approach.

8 | VOLUME 0 | NUMBER 0 | MONTH 2010 www.neurosurgery-online.com

Copyright © Congress of Neurological Surgeons. Unauthorized reproduction of this article is prohibited.


questioned them whether they would choose surgery or a wait-
and-scan policy. There was a very clear answer: 87% favored
follow-up over surgery.20
With regard to the third option, SRT, in the past few years
many reports had emerged on hearing preservation by Gamma
knife radiotherapy or fractionated radiation therapy. So far, only
one group has published results specifically on intracanalicular
vestibular schwannomas.21,22 Niranjan et al21 first published
their results on 15 patients with purely intracanalicular vestibular
schwannomas and found improved results for hearing preserva-
tion when the tumor was smaller (4 mm vs 5-7 mm) and when
small beam parameters were used (4 mm isocenters vs 4 and 8
mm isocenters). A second, more recent study by the same group
described the auditory performance after Gamma knife radio-
therapy in a larger group of patients (n = 96) with purely in-
tracanalicular vestibular schwannomas.22 This study showed that,
after a mean follow-up of 3.5 years, the hearing preservation rate
was 61% (using the 50/50 rule) and therefore lower than that
reported in our study (74%), which has a similar follow-up time.
In our study, 2 of the 3 patients who had residual hearing before
fractionated linear accelerator (LINAC)-based SRT showed de-
terioration of hearing after treatment.
Massager et al23 evaluated the hearing outcome of patients with
vestibular schwannomas after Gamma knife radiotherapy. They
noted that the volume of the intracanalicular part of the vestibular
schwannoma is significantly associated with the hearing outcomes
after radiotherapy. A higher volume of the tumor in the IAC sig-
nificantly increases the risk of deterioration of hearing after radio-
therapy. Vermeulen et al24 noticed that patients with intracanalicular
vestibular schwannomas are more prone to develop complications
after Gamma knife radiotherapy, especially vestibulocochlear nerve
injury. However, their high complication rate might also be caused
by the higher dose of radiation they used in their patients (18 Gy vs
,14 Gy in more recent series). The Pittsburgh group also reported
their results on Gamma knife radiotherapy of vestibular schwan-
nomas and reported a hearing preservation rate of almost 80%
(classes A and B according to AAOHNS).25 Recently, however, they
reported long-term hearing preservation results and noticed an
ongoing deterioration in serviceable hearing after treatment. The
actuarial rate of keeping the same Gardner and Robertson level
declined to 44% after 10 years, with very few tumors exhibiting
growth, indicating that the deterioration was not due to continued
tumor growth.26 It is possible that the wait-and-scan results in our
cohort will show similar deterioration with time, but there does not
appear to be evidence that SRT ‘‘protects’’ against hearing loss
with time.
Because the current results with a wait-and-scan policy and
a similar follow-up time appear as good as or better than those
reported for SRT, there is no evidence that SRT stabilizes hearing
in these tumors, and a wait-and-scan policy may offer equivalent
hearing results with less risk of complications. We acknowledge that
complications of expertly performed SRT are quite low, in partic-
ular, for intracanalicular tumors, but these complications do include
the risks of facial nerve paresis and vestibular loss, and the rare risk of
NEUROSURGERY
Copyright © Congress of Neurological Surgeons. Unauthorized reproduction of this article is prohibited.
HEARING IN INTRACANALICULAR VESTIBULAR SCHWANNOMA
development of malignancy with time. Any definitive treatment
recommendation would have to await truly long-term comparisons
(.10 y) between SRT and conservative management.
CONCLUSION
In our cohort of patients with purely intracanalicular vestibular
schwannomas, most major hearing losses occurred early in the
follow-up period. Hearing was relatively stable after this period,
although smaller hearing losses did occur. In comparing our
results with other treatment modalities available to this patient
group, the wait-and-scan policy seems to offer hearing results that
are as good as, and possibly better than, surgery or SRT. Hence,
we continue to recommend a conservative management approach
in intracanalicular vestibular schwannomas.
We acknowledge our relatively short follow-up time. However,
compared with the published literature, our follow-up is similar to or
longer than many other studies describing different modalities of
treatment. Hearing will continue to decrease with time, and final
comparisons at 10 years will be much more important. However,
because both SRT and surgery have been described to have long-
term deterioration in hearing, it is unlikely that the wait-and-watch
results will decline in isolation compared with the other two
modalities in the long term. We offer a comparison with other
modalities of treatment at a relatively early window of observation.
Disclosure
This research was funded in part by a Niels Stensen stipendium (to R.P.).
The other authors have no personal financial or institutional interest in any of the
drugs, materials, or devices described in this article.
REFERENCES
1. Kobayashi T, Aslan A, Chiba T, Takaska T, Sanna M. Measurement of endo-
cochlear DC potentials in ears with acoustic neuromas: a preliminary report. Acta
Otolaryngol. 1996;116(6):791-795.
2. Prasher D, Tun T, Brookes G, Luxon L. Mechanisms of hearing loss in acoustic
neuroma: an otoacoustic emission study. Acta Otolaryngol. 1995;115(3):375-381.
3. Caye-Thomasen P, Dethloff T, Hansen S, Stangerup SE, Thomsen J. Hearing in
patients with intracanalicular vestibular schwannomas. Audiol Neurootol. 2007;
12(1):1-12.
4. Graamans K, van Dijk JF, Janssen LW. Hearing deterioration in patients with
a non-growing vestibular schwannoma. Acta Otolaryngol. 2003;123(1):51-54.
5. Massick DD, Welling DB, Dodson EE, et al. Tumor growth and audiometric change
in vestibular schwannomas managed conservatively. Laryngoscope. 2005;115:292-296.
6. Solares CA, Panizza B. Vestibular schwannoma: an understanding of growth
should influence management decisions. Otol Neurotol. 2008;29(6):829-834.
7. Committee on Hearing and Equilibrium. Committee on Hearing and Equilib-
rium guidelines for the evaluation of hearing preservation in acoustic neuroma
(vestibular schwannoma). Otolaryngol Head Neck Surg. 1995;113(3):179-180.
8. Warrick P, Bance M, Rutka J. The risk of hearing loss in nongrowing, conser-
vatively managed acoustic neuromas. Am J Otol. 1999;20(6):758-62.
9. Gardner G, Robertson J. Hearing preservation in bilateral acoustic neuroma
surgery. Am J Otol. 1993;14(6):562-565.
10. Sanna M, Khrais T, Russo A, Piccirillo E, Augurio A. Hearing preservation surgery
in vestibular schwannoma: the hidden truth. Ann Otol Rhinol Laryngol.
2004;113(2):156-163.
11. Meyer TA, Canty PA, Wilkinson EP, Hansen MR, Rubinstein JT, Gantz BJ.
Small acoustic neuromas: surgical outcomes versus observation or radiation. Otol
Neurotol. 2006;27(3):380-392.
VOLUME 0 | NUMBER 0 | MONTH 2010 | 9
PENNINGS ET AL
12. Lee A, Clarke L, Bance M. Vestibular schwannoma: how much residual hearing is
useful? J Otolaryngol Head Neck Surg. 2008;37(3):399-410.
13. Samii M, Matthies C. Management of 1000 vestibular schwannomas (acoustic
neuromas): hearing function in 1000 tumor resections. Neurosurgery.
1997;40(2):248-262.
14. Iwai Y, Yamanaka K, Kubo T, Aiba T. Gamma knife radiosurgery for intra-
canalicular acoustic neuromas. J Clin Neurosci. 2008;15(9):993-997.
15. Stangerup SE, Caye-Thomasen P, Tos M, Thomsen J. Change in hearing during
!wait and scan" management of patients with vestibular schwannoma. JLO.
2008;122(7):673-681.
16. Stangerup S-E, Thomsen J, Tos M, Caye-Thomasen P. Long-term hearing
preservation in vestibular schwannoma. Otol Neurotol. 2010;31(2):271-275.
17. Noudel R, Gomis P, Duntze J, Marnet D, Bazin A, Roche PH. Hearing pres-
ervation and facial nerve function after microsurgery for intracanalicular vestibular
schwannomas: comparison of middle fossa and retrosigmoid approaches. Acta
Neurochir. 2009;151(8):935-944.
18. Shelton C, Hitselberger WE, House WF, Brackmann DE. Hearing preservation after
acoustic tumor removal: long-term results. Laryngoscope. 1990;100(2 pt 1):115-119.
19. Chee GH, Nedzelski JM, Rowed D. Acoustic neuroma surgery: the results of long-
term hearing preservation. Otol Neurotol. 2003;24(4):672-676.
10 | VOLUME 0 | NUMBER 0 | MONTH 2010
Copyright © Congress of Neurological Surgeons. Unauthorized reproduction of this article is prohibited.
20. Tran Ba Huy P. Operating on intracanalicular vestibular schwannoma – what
would ENT doctors chose for themselves? Lancet. 1998;351(9113):1406.
21. Niranjan A, Lunsford LD, Flickinger J, Maitz A, Kondziolka D. Dose reduction
improves hearing preservation rates after intracanalicular acoustic tumor radio-
surgery. Neurosurgery. 1999;45(4):753-762.
22. Niranjan A, Mathieu D, Flickinger JC, Kondziolka D, Lunsford LD. Hearing
preservation after intracanalicular vestibular schwannoma radiosurgery. Neuro-
surgery. 2008;63(6):1054-1063.
23. Massager N, Nissim O, Delbrouck C, et al. Role of intracanalicular volumetric and
dosimetric parameters on hearing preservation after vestibular schwannoma
radiosurgery. Int J Radiat Oncol Biol Phys. 2006;64(5):1331-1340.
24. Vermeulen S, Young R, Posewitz A, et al. Stereotactic radiosurgery toxicity in
the treatment of intracanalicular acoustic neuromas: the Seattle Northwest Gamma
Knife experience. Stereotact Funct Neurosurg 1998;70(Suppl 1):80–87.
25. Lunsford LD, Niranjan A, Flickinger JC, Maitz A, Kondziolka D. Radiosurgery
of vestibular schwannomas: summary of experience in 829 cases. J Neurosurg.
2005;102(Suppl):195-199.
26. Chopra R, Kondziolka D, Niranjan A, Lunsford LD, Flickinger JC. Long-term
follow-up of acoustic schwannoma radiosurgery with marginal tumor doses of 12
to 13 Gy. Int J Radiat Oncol Biol Phys. 2007;68(3):845-851.
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