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J Neurosurg 121:554–563, 2014

©AANS, 2014

Retrosigmoid removal of small acoustic neuroma: curative


tumor removal with preservation of function

Clinical article
Iwao Yamakami, M.D.,1 Seiro Ito, M.D., 2 and Yoshinori Higuchi, M.D. 3
1
Neurosurgery, Chiba Central Medical Center, Chiba, Japan; 2Neurosurgery, Chiba Rosai Hospital, Ichihara,
Japan; and 3Neurosurgery, Chiba University Graduate School of Medicine, Chiba, Japan

Object. Management of small acoustic neuromas (ANs) consists of 3 options: observation with imaging follow-
up, radiosurgery, and/or tumor removal. The authors report the long-term outcomes and preservation of function after
retrosigmoid tumor removal in 44 patients and clarify the management paradigm for small ANs.
Methods. A total of 44 consecutively enrolled patients with small ANs and preserved hearing underwent ret-
rosigmoid tumor removal in an attempt to preserve hearing and facial function by use of intraoperative auditory
monitoring of auditory brainstem responses (ABRs) and cochlear nerve compound action potentials (CNAPs). All
patients were younger than 70 years of age, had a small AN (purely intracanalicular/cerebellopontine angle tumor ≤
15 mm), and had serviceable hearing preoperatively. According to the guidelines of the Committee on Hearing and
Equilibrium of the American Academy of Otolaryngology–Head and Neck Surgery Foundation, preoperative hearing
levels of the 44 patients were as follows: Class A, 19 patients; Class B, 17; and Class C, 8. The surgical technique for
curative tumor removal with preservation of hearing and facial function included sharp dissection and debulking of
the tumor, reconstruction of the internal auditory canal, and wide removal of internal auditory canal dura.
Results. For all patients, tumors were totally removed without incidence of facial palsy, death, or other compli-
cations. Total tumor removal was confirmed by the first postoperative Gd-enhanced MRI performed 12 months after
surgery. Postoperative hearing levels were Class A, 5 patients; Class B, 21; Class C, 11; and Class D, 7. Postopera-
tively, serviceable (Class A, B, or C) and useful (Class A or B) levels of hearing were preserved for 84% and 72%
of patients, respectively. Better preoperative hearing resulted in higher rates of postoperative hearing preservation (p
= 0.01); preservation rates were 95% among patients with preoperative Class A hearing, 88% among Class B, and
50% among Class C. Reliable monitoring was more frequently provided by CNAPs than by ABRs (66% vs 32%, p
< 0.01), and consistently reliable auditory monitoring was significantly associated with better rates of preservation of
useful hearing. Long-term follow-up by MRI with Gd administration (81 ± 43 months [range 5–181 months]; median
7 years) showed no tumor recurrence, and although the preserved hearing declined minimally over the long-term
postoperative follow-up period (from 39 ± 15 dB to 45 ± 11 dB in 5.1 ± 3.1 years), 80% of useful hearing and 100%
of serviceable hearing remained at the same level.
Conclusions. As a result of a surgical technique that involved sharp dissection and internal auditory canal recon-
struction with intraoperative auditory monitoring, retrosigmoid removal of small ANs can lead to successful curative
tumor removal without long-term recurrence and with excellent functional outcome. Thus, the authors suggest that
tumor removal should be the first-line management strategy for younger patients with small ANs and preserved hearing.
(http://thejns.org/doi/abs/10.3171/2014.6.JNS132471)

Key Words      •      acoustic neuroma      •      hearing preservation      •      oncology      •     


intraoperative auditory monitoring      •      microsurgery      •      tumor removal

T
echnological developments and the prevalence of curately, noninvasively, and repeatedly. Because tumor
MRI have increased the number of small acoustic growth of small ANs is usually slow, wait-and-scan has
neuromas (ANs) that are detected. Currently, the 3 become a common and valid alternative that allows for
management options for small ANs are observation with documentation of the benign natural history of small ANs
imaging follow-up (wait-and-scan), radiosurgery, and/ while offering the safety and feasibility of observation
or tumor removal.52 Magnetic resonance imaging can be management.2,44 Radiosurgery, which is much less in-
used to evaluate the size and volume of small ANs ac- vasive than tumor removal, offers the most control over
small ANs, although it does not eradicate the tumor. Ra-
Abbreviations used in this paper: ABR = auditory brainstem This article contains some figures that are displayed in color
response; AN = acoustic neuroma; CNAP = cochlear nerve com- on­line but in black-and-white in the print edition.
pound action potential.

554 J Neurosurg / Volume 121 / September 2014


Retrosigmoid removal of small acoustic neuroma

diosurgery achieves a good functional outcome; facial area near the porus acusticus. The restricted arachnoid
function is preserved for 95%–100% of patients7,35 and dissection prevented the cerebellum from sinking down-
useful hearing for 61%–78%.4,31,45 Under certain circum- ward from gravity, which has been shown to be the typi-
stances, a surgeon might be requested to perform curative cal cause of hearing loss because of longitudinal traction
tumor removal of a small AN, usually to preserve facial of the acoustic nerve.10
function and hearing. To achieve excellent functional
outcome after surgery, we have refined the microsurgi- Petrous Dura Flap to Protect the Cerebellopontine
cal technique and intraoperative auditory monitoring for Angle. Before the posterior wall of the internal auditory
retrosigmoid removal of small ANs. Our newly designed canal was drilled, the dura of the petrous bone that forms
intracranial electrode enables continuous and reliable the posterior wall was peeled off as a flap from the fun-
auditory monitoring by use of cochlear nerve compound dus side to the porus side. The petrous dura flap, secured
action potentials (CNAPs).49,51,54 We report long-term by using a brain retractor, protected the cerebellopontine
outcomes of curative tumor removal with preservation angle during posterior wall drilling (Fig. 1A).
of hearing and facial function; thus, we recommend that Posterior Wall Drilling. The petrous bone was drilled
tumor removal be the first-line management strategy for wide enough on both sides of the internal auditory canal
younger patients with small ANs and preserved hearing. to provide a sufficient extraction area; however, to pre-
serve the common crus, we limited drilling on the lateral
(fundus) end. We began drilling with a large (3-mm) steel
Methods bur head and then used a 3-mm diamond bur head to skel-
During the period of 1998–2012, we consecutively etonize the internal auditory canal dura. The last stage of
enrolled 44 patients (19 men and 25 women, 22–69 years internal auditory canal skeletonization comprised trim-
of age [mean age 52 ± 12 years]) with small ANs and pre- ming the sides and fundus area of the internal auditory
served hearing who underwent curative tumor removal in canal by using a smaller (2-mm) diamond bur head (Fig.
an attempt to preserve facial and hearing functions. To 1B). Sufficient drilling caused the meatal tumor to bulge
meet the study inclusion criteria, patients needed to have out by itself; however, at this time, the tumor was still
a small AN, have serviceable hearing preoperatively, and covered by the dura (Fig. 1C).
be younger than 70 years of age. A small AN was defined Harvesting of the Petrous Dura Flap for Internal Au-
as a purely intracanalicular AN without cerebellopontine ditory Canal Reconstruction. After completing adequate
angle extension (6 patients) or an AN with a cerebello- posterior wall drilling, we removed the petrous dura flap
pontine angle extension in which the maximal diameter and preserved it for subsequent internal auditory canal
of the cerebellopontine angle tumor was 15 mm or less reconstruction.
(38 patients). During the study period, we performed tu-
mor removal in 240 patients with previously untreated Wide Removal of the Internal Auditory Canal Dura.
ANs; therefore, these 44 study patients accounted for 18% To gain wide exposure of the intrameatal nerves and tu-
of our total AN experience. According to the guidelines mor, after cutting the sides and lateral end, we removed
of the Committee on Hearing and Equilibrium of the the exposed internal auditory canal dura in its entirety as
American Academy of Otolaryngology–Head and Neck a single sheet.
Surgery Foundation,11 serviceable hearing is defined as Sharp Tumor Dissection. We then dissected the tu-
Class A, B, or C. The preoperative hearing levels of the mor from the nerves (vestibular, cochlear, and facial) by
44 study patients were Class A, 19 patients; Class B, 17; using microscissors and microknives in the same way
and Class C, 8 (Table 1). Furthermore, those with a hear- that they would be used for dissecting the arachnoid
ing level of Class A or B were designated as the useful membrane and trabeculae.
hearing group; they had pure tone averages of 50 dB or
less and speech discrimination scores of 50% or more. Tumor Debulking. The tumor and the surrounding
nerves were tightly packed in the internal auditory canal.
Curative Tumor Removal With Preservation of Function Dissection of the tumor without first debulking it would
via the Retrosigmoid Approach have caused unexpected compression or damage to the
All patients underwent retrosigmoid tumor removal. preserved nerve. Therefore, intracapsular tumor debulk-
The goals were curative (total) tumor removal and preser- ing is always mandatory, even in patients with small
vation of hearing and facial function. ANs. Tumor debulking using ring or blunt dissectors can
compress and damage the nerves surrounding the tumor.
Surgical Technique for Functional Preservation Thus, we debulked the tumor by using microscissors and
microknives. Last, the tumor was removed piece by piece
Because the retrosigmoid AN removal surgical tech- by using microscissors throughout the dissection.
nique has been reported elsewhere, 53,54 herein we list sev-
eral points with regard to curative total tumor removal Tumor Removal Near the Fundus of the Internal Au-
and preservation of function, especially hearing. ditory Canal. Tumor excision near the fundus was the last
and most hazardous stage of tumor removal. With direct
Minimal Cerebellopontine Angle Arachnoid Dis- visualization of the fundus area, we cautiously and sharp-
sections. In patients with a small AN, especially those ly dissected the tumor out by using microscissors.
with a pure intracanalicular tumor, the cerebellopontine
angle arachnoid dissection was limited to the minimal Preservation of Nerve Function. For hemostasis, we

J Neurosurg / Volume 121 / September 2014 555


I. Yamakami, S. Ito, and Y. Higuchi
TABLE 1: Preoperative and postoperative hearing of 44 patients with small ANs

Postop Hearing Class* Rate of Postop Serviceable


Preop Hearing Class* A B C D Hearing Preservation Total
A 4 12 2 1 95%† 19
B 1 9 5 2 88%† 17
A&B 92%‡
C 0 0 4 4 50%§ 8
total 5 21 11 7 84% 44

*  American Academy of Otolaryngology–Head and Neck Surgery Classification.


†  p = 0.01 (chi-square test).
‡  p < 0.01 (chi-square test).
§  50% (4/8), the rate of postoperative serviceable hearing preservation among the 8 patients with preoperative Class C hearing, is
statistically significant (p = 0.01) compared with 95% (18/19), the rate of postoperative serviceable hearing preservation among the
patients with preoperative Class A hearing, and 88% (15/17), the rate of postoperative serviceable hearing preservation among the
patients with preoperative Class B hearing. Moreover, 50% (4/8) is statistically significant (p < 0.01) compared with 92% (33/36),
the rate of postoperative serviceable hearing preservation among the patients with Class A or B hearing.

used oxidized cellulose, surgical patties, saline irrigation, internal auditory canal roof separated the preserved co-
and, sometimes, an application of gentle pressure, which chlear and facial nerves from the grafts and restored the
was sufficient. space in the internal auditory canal (Fig. 2).
Internal Auditory Canal Reconstruction and Re- Intraoperative Monitoring of Auditory and Facial Function
storing the CSF Space in the Internal Auditory Canal.
As mentioned, the petrous dura flap was harvested for During surgery, we performed simultaneous and con-
internal auditory canal reconstruction (Fig. 1D).50 After tinuous monitoring of the auditory brainstem responses
completion of the tumor removal, the roof of the internal (ABRs) and CNAPs.51,54 In brief, ABRs were monitored
auditory canal was reconstructed by using the harvested throughout the duration of the surgery, and CNAPs were
dura flap. Thereafter, muscle or fat grafts were placed on monitored continuously throughout intradural micro-
this roof to fill up the removed posterior wall. The new surgery by using our newly developed intracranial elec-

Fig. 1.  A: The petrous dura flap (arrows) protecting the cerebellopontine angle.  B: Trimming both sides of the internal audi-
tory canal by using a small diamond bur head.  C: Sufficient internal auditory canal drilling caused meatal tumor bulging.   D:
Using the petrous dura flap (arrows) to reconstruct the new roof of the internal auditory canal.

556 J Neurosurg / Volume 121 / September 2014


Retrosigmoid removal of small acoustic neuroma

follow-up Gd-enhanced MRI every year thereafter. Data


analysis was performed by using StatView software (SAS
Institute Inc.), and statistical significance was defined as
p ≤ 0.05. Data are expressed as means and standard de-
viations.
This study adhered to the World Medical Association
Declaration of Helsinki and the guidelines for clinical re-
search published by the ethical committee of Chiba Cen-
tral Medical Center. All patients gave informed consent
before inclusion in this study.

Results
Outcomes of Tumor Removal and Preservation of Function
Total tumor removal was achieved for all patients and
confirmed by surgical records and the first postoperative
Gd-enhanced MRI (12 months after surgery). Postopera-
tive hearing levels were Class A, B, C, and D for 5, 21, 11,
and 7 patients, respectively (Table 1). Postoperative rates
of preservation of serviceable and useful hearing were
37/44 (84%) and 26/36 (72%), respectively. The postop-
erative rate for preservation of serviceable hearing was
95% among patients with preoperative Class A hearing,
88% among those with Class B hearing, and 50% among
those with Class C hearing (p = 0.01). Serviceable hearing
was preserved for 92% of patients with preoperative Class
A or B hearing and 50% of patients with preoperative
Class C hearing (p < 0.01). A mild facial palsy (House-
Brackmann Grade II) occurred in 2 patients (5%) during
the first postoperative week, but normal facial function
had been recovered (House-Brackmann Grade I) when
they were seen in the outpatient clinic. No deaths or other
postoperative complications occurred.

Intraoperative Auditory Monitoring (Fig. 3)


Monitoring of ABRs during tumor removal was of-
ten disrupted by artifacts from various surgical equip-
ment and procedures. Among the 44 patients, reliable
monitoring of ABRs with the distinct wave V was consis-
Fig. 2.  A patient with left small AN. Before tumor removal, the left tently obtained in 14 (32%), was intermittently obtained
internal auditory canal was filled with tumor (T1-weighted axial Gd-en- in 10 (23%), and was either unreliable or not obtained in
hanced image [A]; T2-weighted coronal image [B]). Tumor was totally 20 (45%) (Table 2). Consistently reliable ABR monitoring
removed via the retrosigmoid approach, and function was preserved; was obtained in 11 (58%) of 19 patients with preoperative
preoperative and postoperative hearing levels were 35 and 30 dB, re- Class A hearing, 3 (18%) of 17 with preoperative Class B
spectively. At 2 years after tumor removal, T2-weighted coronal image hearing, and 0 of 8 with preoperative Class C hearing (p
(C) showed that the intrameatal tumor was removed and that the left
internal auditory canal restored the cerebrospinal fluid space after inter- < 0.01). Useful hearing was preserved postoperatively in
nal auditory canal reconstruction. Arrows indicate left internal auditory 12 (86%) of 14 patients with consistently reliable ABRs,
canal. R = right. but in only 9 (45%) of 20 patients with unreliable/not ob-
tained ABRs (p < 0.05).
Cochlear nerve compound action potentials were
trodes. Facial function was monitored by intermittent not affected by artifacts of surgical equipment and pro-
electrical stimulation of the intracranial facial nerve with cedures. Reliable CNAPs were obtained consistently
the NIM nerve monitoring system (Medtronic). throughout microsurgical tumor removal in 29 (66%)
of the 44 patients, and reliable auditory monitoring was
Postoperative Follow-Up
achieved more frequently with CNAPs than with ABRs
After tumor removal, all patients were regularly (66% vs 32%, p < 0.01). Cochlear nerve compound ac-
examined at the outpatient clinic every 3–6 months. To tion potentials were intermittently reliable in 7 patients
confirm the extent of tumor removal, they underwent the and unreliable/not obtained in 8 patients (Table 2). Useful
first postoperative Gd-enhanced MRI 12 months after hearing was preserved postoperatively in 22 (76%) of 29
surgery; to evaluate tumor recurrence, they underwent patients with consistently reliable CNAPs and in 4 (27%)

J Neurosurg / Volume 121 / September 2014 557


I. Yamakami, S. Ito, and Y. Higuchi

of 15 patients with intermittently reliable or unreliable/


not obtained CNAPs (p < 0.01).
Long-Term Postoperative Follow-Up Results of
Gd-Enhanced MRI and Audiological Examinations
During the long-term Gd-enhanced MRI follow-up
period (81 ± 43 months [range 5–181 months], median 7
years), no tumor recurred in any patient. However, for 1
patient (a 42-year-old woman) hearing suddenly worsened
30 months after surgery. No tumor recurrence was dem-
onstrated on Gd-enhanced MRI, so the patient underwent
treatment for sudden deafness, which did not ameliorate
her hearing impairment (from 36 dB to 70 dB in pure
tone averages). No sudden deterioration of the preserved
hearing occurred in any other patient; postoperative au-
diological follow-up examinations for preserved hearing
showed a decline of pure tone averages from 39 ± 15 dB
to 45 ± 11 dB over 5.1 ± 3.1 years (range 2–13 years); that
is, the rate of annual hearing decrease was 1.2 dB per
year. Preserved useful hearing remained the same for 12
(80%) of 15 patients, and preserved serviceable hearing
was maintained for all 37 patients.

Discussion
Wait-and-Scan for Patients With Small ANs and Preserved
Hearing
Observation management with imaging follow-up
(wait-and-scan) has become the prevalent strategy for
managing small ANs. This approach has revealed that
the natural history of ANs as a whole is benign and that
the mean tumor growth rate is 1.0–3.0 mm per year.2,44,52
Fig. 3.  Intraoperative auditory monitoring of a 46-year-old man with a However, the wait-and-scan strategy has also revealed
small AN. This patient’s preoperative hearing level was Class A, and his that the growth rate and pattern of small ANs vary widely
hearing level was preserved after tumor removal. Continuously reliable among patients. Growth rates are less than 1 mm per year
monitoring of ABRs (left) and CNAPs (right) was obtained throughout for more than 60% of patients and more than 3 mm per
surgery. Black arrow indicates CNAP with latency of 3.2 msec. year for 12% of patients.2 Moreover, no significant associ-
ation has been found between tumor growth rate and fac-
tors such as sex, age, initial hearing status, or preliminary

TABLE 2: Results of intraoperative auditory monitoring versus preoperative and postoperative hearing among 44
patients who underwent retrosigmoid removal of small AN

Hearing Level*
No. of Preoperative Postoperative
Type of Monitoring Cases A B C A B C D
ABRs
  consistently reliable 14 11 3 0 4 8 1 1
  intermittently reliable 10 3 5 2 0 5 4 1
    unreliable/not obtained 20 5 9 6 1 8 6 5
  total 44 19 17 8 5 21 11 7
CNAPs
  consistently reliable 29 17 10 2 4 18 5 2
  intermittently reliable 7 2 4 1 0 3 4 0
    unreliable/not obtained 8 0 3 5 1 0 2 5
  total 44 19 17 8 5 21 11 7

*  American Academy of Otolaryngology–Head and Neck Surgery Classification.

558 J Neurosurg / Volume 121 / September 2014


Retrosigmoid removal of small acoustic neuroma

tumor grade.2,52 Because the tumor growth of each patient

  Monte 2012,13 (94) Friedman 2003,16 (97) Meyer


is unpredictable, the ideal interval for imaging follow-up

  Staechker 2000,42 (100) Magnan 2002,24 (100)


  2006,27 (99) Gjurić 2001,18 (100) Sameshima
(88) Hillman 2010,21 (89) Kutz 2012,23 (93) De-
studies is not fixed.12

(95) Rowe 2003,35 (95) Murphy 2011,30 (99)


Previous reports of the wait-and-scan approach for

(90) Samii 2006,37 (91) Colletti 2005,9 (92)

  Hasegawa 2013,19 (100) Chopra 20077


Rate of Good Facial Function Preservation
patients with small ANs and preserved hearing have in-
dicated that hearing progressively declines regardless of

(%) Author & Year


tumor growth. Specifically, 1 study showed that pure tone
averages among 47 patients with intracanalicular AN de-
teriorated from 38 dB to 51 dB and that 26% of useful
hearing was lost during the 3.6-year duration of the wait-

  Sameshima 201036
and-scan management strategy.33 Another study revealed
that over a follow-up period of more than a decade, most
patients with preserved useful hearing lost the useful
hearing regardless of tumor growth.43 These studies pro-

 201036
vide compelling evidence that, to avoid the progressive
hearing decline that has been reported for the prevalent
wait-and-scan approach, tumor removal should be the
first treatment option for younger patients with small ANs

88–100

95–100
88–100

90–100
%
and preserved hearing.

100
Long-Term Outcome of Curative Small AN Removal With
Preservation of Function

Chopra 2007,7 Hasegawa


Friedman 2003,16 Wood-

  2011,19 Roos 201234


In our study, retrosigmoid removal of small ANs in

Author & Year


44 patients with preserved hearing was accomplished

Progressive Hearing Decline

Mazzoni 201226
with excellent functional outcomes. Curative tumor re-

  son 201049
moval was confirmed by long-term MRI follow-up ex-
aminations.
One of the main and most challenging focuses in the
management of small ANs is preservation of hearing. In
our study, the rates of postoperative preservation of useful
Amount of Decline
no/rare/minimal
and serviceable hearing were 72% and 84%, respectively.

usual/definite
rare/minimal
Table 3 summarizes the outcome of hearing and facial
no/minimal

function after tumor removal and radiosurgery of small


ANs. Two surgical approaches (the retrosigmoid ap-
Hearing After Treatment

proach and the middle fossa approach) are currently used rare
for small AN removal in cases where hearing preserva-
TABLE 3: Outcome of hearing and facial function after treatment of small ANs*

tion is desired. Although it has previously been claimed

(61) Niranjan 2008,31 (68) Franzin 2009,15 (78)


  Mangham 2004,25 (77) Sameshima 201036

that a higher rate of hearing preservation can be accom-


  Colletti 2005,9 (73) Arts 2006,1 (73) De-
(52) Sanna 2004,39 (53) Satar 2002,40 (59)

(50) Mohr 2005,29 (57) Colletti 2005,9 (72)


  Hillman 2010,21 (62) Gjurić 2001,18 (66)

plished with the middle fossa approach than with the ret-
rosigmoid approach,22 recent publications have suggested
Rate of Useful Hearing Preservation

that there is no difference between the 2 approaches (that


(%) Author & Year

is, hearing is preserved for 50%–77% of patients after the


retrosigmoid approach9,25,29,36 and for 52%–73% after the
middle fossa approach9,13,21,39). By using the retrosigmoid
*  Data from a series of studies published in 2000 or later.

approach, we achieved the same level of preservation


  Tamura 200945

(72%) in the study reported here.


  Monte 201213

We showed that better preoperative hearing resulted


in a higher rate of postoperative hearing preservation:
95% preservation for patients with preoperative Class A
hearing, 88% for Class B, and 50% for Class C. There-
fore, hearing preservation after tumor removal is associ-
ated with the preoperative hearing level.4
50–77

50–77
middle fossa approach 52–73

61–78
%

Intraoperative auditory monitoring during this study


72

showed that reliable auditory monitoring was more fre-


quently obtained for patients with better preoperative
retrosigmoid approach
Treatment Modality

hearing. Moreover, reliable monitoring was also associ-


ated with higher rates of postoperative hearing preser-
current series

vation. In this study, intraoperative auditory monitoring


radiosurgery

with ABRs and CNAPs was performed simultaneously


surgery

and reliable auditory monitoring more frequently with


CNAPs than with ABRs (66% vs 32%, p < 0.01). Because

J Neurosurg / Volume 121 / September 2014 559


I. Yamakami, S. Ito, and Y. Higuchi

of their large amplitude, CNAPs can almost be moni- the internal auditory canal and results in delayed hear-
tored in real time, thus giving the surgeon intraoperative ing declines.6,41 We reconstructed the internal auditory
feedback.8,51 This feedback enables the surgeon to refine canal after tumor removal by using the petrous dura flap
the surgical technique in an attempt to preserve hearing to restore the CSF space of the internal auditory canal.50
and facial function intraoperatively, thereby improving Recovery of the CSF space prevents scarring of the pre-
functional outcome. Several points with regard to surgi- served nerve, and this method of internal auditory canal
cal technique for preservation of hearing and facial func- reconstruction can contribute to long-term hearing pres-
tion are detailed above. However, the most critical point ervation after tumor removal. Mazzoni et al. recently re-
for preservation of hearing and facial function is sharp ported that after retrosigmoid AN removal, 87% of pre-
dissection with microscissors and microknives. Blunt dis- served useful hearing was maintained at the same level
section not only damages nerves by indirect compression over a long-term follow-up period of 6–21 years.26 Re-
but also damages small vessels that have mild adhesions. cent studies of the middle fossa approach tumor removal
Although removal of medium or large ANs carries a showed no or minimal hearing decline in the postopera-
considerable risk for facial palsy,38 a series of recent stud- tive follow-up period.16,49
ies of removal of small ANs reported high rates of facial
function preservation (88%–100%)9,13,16,18,21,23,24,27,36,37,42 Learning Curve for Microsurgery and Radiosurgery
(Table 3). In our study, the rate of normal facial function
preservation (House-Brackmann Grade I) was 100%. In Surgical removal of ANs requires a profound knowl-
reports published during the 1980s–1990s, the rates of edge of anatomy and long-standing experience with so-
good facial function preservation after small AN removal phisticated microsurgical technique. The expertise of the
were unsatisfactory (72%–94%).3,17,22,38 The increased surgeon and/or surgical teams affects the outcome of this
success can be attributed to the development of surgical technically demanding procedure, and the existence of a
and monitoring techniques. With regard to surgical ap- learning curve has been reported.14,46 To attain surgical
proaches, we and others have found that the retrosigmoid results similar to those reported by expert surgeons, expe-
approach can offer better postoperative facial function rience with 20–50 cases is necessary.5,28,47 Before starting
than the middle fossa approach.32,36 This is so because for the series of cases reported here, the senior author had re-
the access route of the middle fossa approach, the facial moved more than 60 ANs, most of which were large/giant
nerve is located in front of the tumor, but for the retro- tumors.53 Despite the relatively small number of patients
sigmoid approach, the nerve is located behind the tumor; in this series, we removed 240 previously untreated ANs
thus, during tumor removal, the middle fossa approach during the same period. Our study shows excellent results
requires greater manipulation of the facial nerve.32 Pres- regarding preservation of hearing and facial function as
ervation of facial function is also excellent (95%–100%) well as total tumor removal; therefore, no learning curve
after radiosurgery.7,30,35 was observed. Radiosurgical treatment of ANs requires
precise anatomical definition and optimal dose distribu-
Progressive Hearing Decline After AN Treatment tion. Because of continuous advances in imaging technol-
ogy and the refinement of radiosurgical technology, ra-
The highest rates of hearing preservation after treat- diosurgery requires improvement of skill and knowledge.
ment of small ANs are achieved with radiosurgery; use- A learning curve exists for radiosurgical dose planning of
ful hearing is preserved for 61%–78% of patients15,31,45 AN treatment, although the effect on the clinical outcome
However, preserved hearing after radiosurgery has been has not yet been elucidated.56
shown to progressively decline. This progressive decline
is typical and definite; hearing preservation rates are Management Paradigm for Small ANs
43%–57% at 5 years after radiosurgery and decrease to
34%–45% at 10 years.7,20,34 In this study, we analyzed the long-term outcome of
In our study, after retrosigmoid tumor removal, the 44 patients with preserved hearing who had undergone re-
occurrence of postoperative decline of preserved hearing moval of small ANs; we showed that retrosigmoid tumor
was rare and the level of the postoperative decline was removal with auditory monitoring can accomplish curative
minimal; that is, over the long term, useful hearing and tumor removal and excellent functional outcome without
serviceable hearing were preserved for 80% and 100% of deaths or other postoperative complications. During wait-
patients, respectively. Yomo et al. reported that the rate and-scan of small ANs in patients with preserved hearing,
of annual hearing decrease among AN patients was 5.39 progressive hearing decline occurs frequently, irrespective
dB per year and 3.77 dB per year before and after ra- of tumor growth. After radiosurgery, progressive hearing
diosurgery, respectively.55 In our study, the rate of annual decline is inevitable. Therefore, we propose that tumor
hearing decrease after retrosigmoid small AN removal removal, with preservation of hearing and facial func-
was 1.2 dB per year, which is much lower than that after tion, should be the first-line management for small ANs
radiosurgery. In addition, it is possible that the postopera- in younger patients with preserved hearing. Figure 4 out-
tive hearing decline found in this study might represent lines this management algorithm. Briefly, patients younger
natural hearing decline from aging (presbycusis). Postop- than 70 years of age with preserved hearing and a small
erative declines in hearing after tumor removal through AN should be offered tumor removal with preservation of
the middle fossa approach have been documented.41 After hearing and facial function. For patients 70 years of age or
tumor removal, scarring of the preserved cochlear nerve older, the wait-and-scan approach should be offered; there-
occurs as a consequence of fat or muscle graft packing of after, patients with tumor growth during the wait-and-scan

560 J Neurosurg / Volume 121 / September 2014


Retrosigmoid removal of small acoustic neuroma

Fig. 4.  Management algorithm for a patient with small AN. RS = radiosurgery; S = surgery (tumor removal); S/RS = surgery or
radiosurgery; W&S = wait-and-scan. Age is represented in years.

period should be offered either tumor removal or radiosur- nature of ANs makes a well-controlled, randomized tri-
gery. al unpractical because attaining statistical significance
would require sample sizes of more than 6700 patients
Study Limitations and Strengths in each study group.48 Despite the limited number of pa-
This study has some limitations and several strengths. tients in our study, the auditory monitoring using CNAPs
The limitations include the retrospective nature of the and ABRs might have contributed to the excellent func-
study, limited number of patients, potential biases of pa- tional results achieved in this study. A major strength of
tient selection, and unknown natural history of AN. At this study is the long-term postoperative follow-up result
the start of this study, we determined the inclusion crite- obtained by Gd-enhanced MRI and audiological exami-
ria for patients who would undergo curative tumor remov- nations.
al with preservation of hearing and facial function (small
AN, serviceable hearing, and age younger than 70 years). Conclusions
However, not all patients conforming to the inclusion
criteria underwent this surgery because each patient se- Using the surgical technique, including sharp dissec-
lected his or her own treatment plan after being informed tion and internal auditory canal reconstruction, in concert
of the 3 treatment options (wait-and-scan, radiosurgery, with intraoperative auditory monitoring of CNAPs and
or tumor removal). Not being a prospectively controlled ABRs, we found that removal of small ANs via a retro-
trial, this study could not evade patient selection bias. Al- sigmoid approach accomplished curative removal and ex-
though the natural history of hearing and tumor growth cellent functional outcomes. No tumor recurred and the
of small ANs is discussed above in relation to the results preserved hearing demonstrated no decline or minimal
of wait-and-scan study series, the real natural history of decline during the long-term follow-up period. Hear-
small ANs is unknown. The long-term outcomes after ing decline during wait-and-scan and after radiosurgery
tumor removal and radiosurgery should be determined is common and inevitable for patients with small ANs;
with regard to the real natural history of small ANs by therefore, we propose that curative tumor removal with
using a well-controlled, randomized trial. However, the preservation of hearing and facial function should be the

J Neurosurg / Volume 121 / September 2014 561


I. Yamakami, S. Ito, and Y. Higuchi

first-line management strategy recommended for younger Knife surgery of vestibular schwannomas. Neurosurg Focus
patients with a small AN and preserved hearing. 27(6):E3, 2009
16.  Friedman RA, Kesser B, Brackmann DE, Fisher LM, Slattery
WH, Hitselberger WE: Long-term hearing preservation after
Disclosure middle fossa removal of vestibular schwannoma. Otolaryn-
The authors report no conflict of interest concerning the mate- gol Head Neck Surg 129:660–665, 2003
rials or methods used in this study or the findings specified in this 17.  Gantz BJ, Parnes LS, Harker LA, McCabe BF: Middle cranial
paper. fossa acoustic neuroma excision: results and complications.
Author contributions to the study and manuscript preparation Ann Otol Rhinol Laryngol 95:454–459, 1986
include the following. Conception and design: Yamakami, Ito. 18.  Gjurić M, Wigand ME, Wolf SR: Enlarged middle fossa ves-
Acquisition of data: all authors. Analysis and interpretation of data: tibular schwannoma surgery: experience with 735 cases. Otol
all authors. Drafting the article: Yamakami, Ito. Critically revising Neurotol 22:223–231, 2001
the article: all authors. Reviewed submitted version of manuscript: 19.  Hasegawa T, Kida Y, Kato T, Iizuka H, Kuramitsu S, Yama-
all authors. Approved the final version of the manuscript on behalf moto T: Long-term safety and efficacy of stereotactic radio-
of all authors: Yamakami. Statistical analysis: Yamakami, Higuchi. surgery for vestibular schwannomas: evaluation of 440 pa-
Administrative/technical/material support: Yamakami. Study super- tients more than 10 years after treatment with Gamma Knife
vision: Yamakami. surgery. Clinical article. J Neurosurg 118:557–565, 2013
20.  Hasegawa T, Kida Y, Kato T, Iizuka H, Yamamoto T: Fac-
tors associated with hearing preservation after Gamma Knife
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diosurgery for vestibular schwannomas presenting with high- Please include this information when citing this paper: pub-
level hearing. Neurosurgery 64:289–296, 2009 lished online July 4, 2014; DOI: 10.3171/2014.6.JNS132471.
46.  Wang AY, Wang JT, Dexter M, Da Cruz M: The vestibular Address correspondence to: Iwao Yamakami, M.D., Department
schwannoma surgery learning curve mapped by the cumula- of Neurosurgery, Chiba Central Medical Center, 1835-1 Kasori-
tive summation test for learning curve. Otol Neurotol 34: cho, Wakaba-ku, Chiba 264-0017, Japan. email: yamakami@ccmc.
1469–1475, 2013 seikei-kai.or.jp.

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