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Skull base surgery for removal of temporal bone tumors

Chong-Sun Kim a; Myung-Whan Suh a
a
Department of Otorhinolaryngology, Seoul National University College of Medicine, Yongon-Dong, Seoul,
Chongno-Gu, South Korea

Online Publication Date: 01 October 2007

To cite this Article Kim, Chong-Sun and Suh, Myung-Whan(2007)'Skull base surgery for removal of temporal bone tumors',Acta Oto-
Laryngologica,127:10,4 — 14
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 Acta Oto-Laryngologica, 2007; Suppl 558: 4
14

Skull base surgery for removal of temporal bone tumors

CHONG-SUN KIM & MYUNG-WHAN SUH

Department of Otorhinolaryngology, Seoul National University College of Medicine, Yongon-Dong, Chongno-Gu, Seoul
110-799, South Korea

Abstract
Conclusion. When selecting the appropriate surgical approach the pathological type of tumor, the physiological status as well
as the functional aspects should be considered. Understanding the strengths and weaknesses of each surgical technique and
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knowledge of the particular tumor biology facilitates selection of the most appropriate surgical approach and a successful
outcome. Objectives. The purpose of this study was to review cases that underwent skull base surgery for a variety of tumors
that involved the temporal bone. We reviewed a single center’s 25-year experience for epidemiologic characteristics,
symptoms, treatment type and outcomes. Patients and Methods. The medical records and radiological images of 91 patients,
who underwent skull base surgery, were retrospectively reviewed. Results. Among the 91 patients, 61 cases had benign
disease and 30 had malignancies. A facial nerve schwannoma was the most common benign intratemporal tumor and a
squamous cell carcinoma was the most common malignant tumor. With the facial nerve schwannoma, facial nerve paralysis
and hearing loss were the most common presenting complaints; otalgia was the most common presenting symptom for
temporal bone cancer. For patients with a glomus tumor, there was a characteristic pulsating tinnitus. A majority of the
facial nerve schwannomas were resectable through the transmastoid approach. The infratemporal fossa approach type A was
usually required for lower cranial nerve schwannomas and glomus jugulare tumors. However, the fallopian bridge technique
with hypotympanectomy was another surgical option. Partial temporal bone resection and subtotal temporal bone resections
were performed in cases with temporal bone cancer. The disease free 5-year survival of the temporal bone cancers was 42%
and for the squamous cell carcinomas, it was 44%.

Keywords: Skull base, temporal bone, schwannoma, glomus tumor, squamous cell carcinoma

Introduction well as benign and malignant tumors develop in

The modern era of skull base surgery started by
William House who first introduced the translabyr-
inthin approach in 1961 [1]. This new approach
enabled a wider view of the cerebellopontine angle
and a more precise removal of a large vestibular
schwannoma. After this surgical quantum leap by
House, Ugo Fisch opened another new field of
skull base surgery with the infratemporal fossa
approach in 1978 [2,3]. The previously inoperable
tumors near the jugular foramen could be success-
fully resected by this approach. These pioneers and
many others are responsible for the success of the
advanced skull base surgery currently used. Yet the
temporal bone presents a difficult surgical field; it
forms the auditory organ and the lateral part of the
skull base. Congenital and inflammatory lesions as
this area. However, the numerous vital structures
of the inner ear and the proximity of the brain
are significant obstacles to skull base surgery. The
surgeon should be able to utilize all available
surgical options in order to approach a given lesion
without damaging these vital structures. However,
extensive training is required to become familiar
with the various techniques currently available.
Subtotal petrosectomy, an operation that involves
removal of all of the pneumatic air cells of the
temporal bone, is the final step in middle ear
surgery and at the same time, it is the first step in
skull base surgery. Therefore, middle ear surgery
and skull base surgery overlap with temporal bone
surgery. The abundant accumulated experience
from middle ear surgery may provide a strong

Correspondence: Chong Sun Kim, Department of Otorhinolaryngology, Seoul National University College of Medicine, Yongon-dong, Jongno-gu, Seoul
110-744, Korea. Tel: 82-2-2072-2440. Fax: 82-2-745-2387. E-mail: chongkim@snu.ac.kr

ISSN 0365-5237 print/ISSN 1651-2551 online # 2007 Taylor & Francis

DOI: 10.1080/03655230701624806
 Skull base surgery 5
Table I. Pathology of the temporal bone tumors.
base of knowledge that can also be used for skull
base surgery.
Pathology No. %
The limited surgical literature in the field of skull
base surgery has provided little information for Benign tumors (N61)
otologists. There are a number of articles on skull Facial nerve schwannoma 25 41.0
base surgery for specific diseases but not many Lower cranial nerve schwannoma 9 14.8
Glomus tumor 10 16.4
articles on a large series of skull base surgery. The
Congenital epidermoid 5 8.2
purpose of this study was to review cases that Neurofibroma 2 3.3
underwent skull base surgery for a variety of tumors Petrous apex cyst 2 3.3
of the temporal bone. We reviewed a single center’s Fibrous dysplasia 2 3.3
25-year experience to determine epidemiologic Fibrous histiocytoma 1 1.6
Cementifying fibroma 1 1.6
characteristics, symptoms, treatment types, and
Ceruminous adenoma 1 1.6
outcomes. Chordoma 1 1.6
Chondrocalcinosis 1 1.6
Pigmented villonodular synovitis 1 1.6
Materials and Methods
Malignant tumors (N30)
From January 1982 through December 2006, 176 Squamous cell carcinoma 17 56.7
patients underwent skull base surgery at the depart- Adenocystic carcinoma 6 20.0
ment of Otorhinolaryngology, Seoul National Uni- Basal cell carcinoma 3 10.0
Chondrosarcoma 2 6.7
versity Hospital, for intratemporal or intracranial
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Sebaceous carcinoma 1 3.3

tumors. The senior author (C.S. Kim) performed
the majority of operations. Medical records were
retrospectively reviewed for age, gender, presenting
symptoms, operative procedures, location of the
tumor, and surgical outcomes. Radiological images
were also reviewed. In almost all cases temporal
bone CT and/or MRI were performed. In cases with
a glomus tumor, angiography was also carried out.
Among the 176 patients, a vestibular schwannoma
was the most common tumor; it was identified in 80
patients. However, in this article we focused on the
91 patients with intratemporal tumors excluding
intra cranial vestibular schwannoma, meningioma,
endolymphatic sac adenocarcinoma, and clivus
chondroblastoma. Pathological reports confirmed
that there were 61 cases with benign disease and
30 with a malignancy. Facial nerve schwannoma was
the most common benign intratemporal tumor and
squamous cell carcinoma was the most common
malignant tumor of the temporal bone (Table I).
The facial nerve function was graded according to
the House-Brackmann (HB) scale. The glomus
tumors were classified according to the Glasscock-
Jackson classification [4] and cancers of the temporal
bone were staged by the Arriaga staging system [5].
Apocrine carcinoma 1 3.3
Lower cranial nerve: cranial nerve IX, X, XI, XII.
to 61 years). Interestingly eight out of the nine
patients were female. Of the 10 patients with an
intratemporal glomus tumor, six were male and four
were female. The mean age of these patients was
42.0 years (range 20 to 60 years). Thirty patients
were diagnosed with temporal bone cancer. The
mean age of the patients was 53.4 years (range 29 to
79 years) and the sex ratio was 20:10.
Location and origin of the Tumor
The location of the facial nerve schwannoma was
analyzed according to the five segments and the two
genu of the facial nerve (Figure 1). In four (14%)
cases the tumor was located in the meatal portion,
10 (36%) in the labyrinthine portion, 15 (54%) in

Results
Patient Demographics
The mean age of the 25 patients with facial nerve
schwannomas was 35.8 years (range 1 to 59 years).
The sex ratio was 7:18 (male:female) with a slight
preponderance of female patients. Nine patients had
lower cranial nerve (cranial nerve IX, X, XI, XII)
schwannomas; their average age was 41.0 (range 26
Figure 1. Location of the facial nerve schwannoma. The location
of the 25 facial nerve schwannomas was analyzed according to the
five segments and the two genus of the facial nerve. The tympanic
portion and the second genu was the most common location of a
facial nerve schwannoma. A large tumor can involve more than
one portion of the facial nerve.
 6 C.-S. Kim & M.-W. Suh

the geniculate ganglion, 16 (57%) in the tympanic Clinical Manifestations

portion, 21 (75%) in the second genu, 19 (68%)
in the mastoid portion and seven (25%) in the
stylomastoid foramen. The number of cases is
not mutually exclusive because a large tumor may
involve more than one portion of the facial nerve.
The tympanic portion and the second genu were the
most common location of facial nerve schwannomas.
The lower cranial nerves, which the schwannomas
originated form, was assessed by the surgical find-
ings. Cranial nerve IX was the origin of the tumor in
three (33%) cases, Cranial nerve X in one (11%)
case, cranial nerve XII in three (33%) cases and the
origin was not identified in two cases (22%).
Among the 10 glomus tumor patients, seven
(70%) had a glomus jugulare and three (30%) had
a glomus tympanicum. The Glasscock-Jackson glo-
mus jugulare classification was type I in two patients,
type II in one patient, type III in one patient
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The signs and symptoms identified at the time of
presentation are summarized in Table II. For the
facial nerve schwannomas, facial nerve paralysis and
hearing loss were the most common presenting
complaints occurring in 24 (96%) and 12 (48%)
patients respectively. The clinical manifestations of a
lower cranial nerve schwannoma were classified into
otologic symptoms and non-otologic symptoms.
Hearing loss was the most common otologic symp-
tom; it was detected in five (55%) patients. On the
other hand, a hoareness (44%), shoulder weakness
(33%) and tongue deviation (33%) were the three
major non-otologic symptoms. The most common
symptoms associated with an intratemporal glomus
tumor were hearing loss and tinnitus, observed in
eight (80%) and seven (70%) patients respectively.
Tinnitus was a characteristically pulsating type in

and type IV in three patients. As for glomus
tympanicum, one patient had type I and two patients
had type II. Angiography was performed in nine of
the 10 patients. The ascending pharyngeal artery
was the main feeder artery in eight (80%) cases and
the middle meningeal artery in one (10%) case. The
occipital artery and posterior auricular artery also
served as subsidiary feeding arteries. The origin of
the cancer was suspected to be the external auditory
canal in 27 (90%) patients. The mastoid cavity was
suspected to be the origin in one (3%) patient who
had a chondrosarcoma. The origin was not clear in
the remaining two (7%) patients.
five (50%) patients. Facial nerve paralysis, intermit-
tent otorrhea, dizziness, and otalgia were also pre-
sent in a number of patients (Table II). The tumor
was visible through the tympanic membrane in eight
(80%) patients and two (20%) among them had a
tympanic membrane perforation. The mass was
noted to be pulsating when examined by an endo-
scope in four (40%) patients. In one patient with
hypertension, the tumor turned out to be a function-
ing glomus tumor that secreted vanillylmandelic acid
(VMA). In cases with temporal bone cancer, otalgia
was the chief complaint in 19 (63%) patients and
otorrhea in 17 (57%) patients. A mass in the external

Table II. Symptoms associated with temporal bone tumors.

Facial nerve Lower cranial nerve Glomus tumor Temporal bone

schwannoma (N25) schwannoma (N9) (N10) cancer (N30)
No. (%) No. (%) No. (%) No. (%)

Otologic symptoms
Hearing loss 12 (48.0) 5 (55.6) 8 (80.0) 14 (46.7)
Facial nerve paralysis 24 (96.0) 0 (0.0) 4 (40.0) 3 (10.0)
Tinnitus 6 (24.0) 3 (33.3) 7 (70.0) 5 (16.7)
Dizziness 8 (32.0) 2 (22.2) 3 (30.0) 17 (56.7)
Otorrhea 2 ( 8.0) 0 (0.0) 4 (40.0) 17 (56.7)
Otalgia 0 (0.0) 1 (11.1) 3 (30.0) 19 (63.3)
Nonotologic symptoms
Mass formation 9 (36.0) 0 (0.0) 2 (20.0) 16 (53.3)
Hoarse voice 0 (0.0) 4 (44.4) 0 (0.0) 0 (0.0)
Shoulder weakness 0 (0.0) 3 (33.3) 0 (0.0) 0 (0.0)
Tongue deviation 0 (0.0) 3 (33.3) 0 (0.0) 0 (0.0)
Ataxia 0 (0.0) 2 (22.2) 0 (0.0) 0 (0.0)
Swallowing difficulty 0 (0.0) 1 (11.1) 0 (0.0) 0 (0.0)
Aspiration 0 (0.0) 1 (11.1) 0 (0.0) 0 (0.0)
Dyspnea 0 (0.0) 1 (11.1) 0 (0.0) 0 (0.0)
Headache 0 (0.0) 1 (11.1) 0 (0.0) 0 (0.0)

The numbers are not mutually exclusive.

Lower cranial nerve: cranial nerve IX, X, XI, XII.

auditory canal, hearing loss, tinnitus, facial nerve
paralysis, and vertigo were also present (Table II).
Treatment
In 12 (48%) cases, the facial nerve schwannoma
was resected through a transmastoid approach.
An infratemporal fossa approach was performed
in seven (28%) patients, a middle fossa approach
in two (8%) patients, a middle fossa combined with
a transmastoid approach in two (8%) patients, a
translabyrinthin approach in one (4%) patient and a
transcochlear approach in one (4%) patient. The
location and size of the mass were the two major
factors considered in determining the surgical ap-
proach. No patient had a recurrence of the schwan-
noma. The follow up period was 6.3 years on average
(range from 8 months to 25 years).
For the lower cranial nerve schwannomas, an
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infratemporal fossa approach type A, with facial
nerve rerouting, was performed in all nine cases.
However, three cases where the temporal bone
schwannoma was combined with a large intracranial
mass had staged operations 1 to 2 weeks later in
order to remove the intracranial portion. Six patients
had no evidence of residual or recurrent disease
after surgery. The tumor recurred in two patients.
One of the two patients underwent radiation therapy
and the other underwent a second revision opera-
tion. These patients are still alive with recurrent
disease. Follow up was not possible in one patient.
The follow up period was 12 years on average (range
2.5 to 21 years).
For the three patients who had a glomus tympa-
nicum, the transmeatal approach was carried out
in one (33.3%) patient and an extended posterior
tympanotomy approach was performed in two
(66.7%) patients (Table III). In cases with glomus
jugulare, the feeding arteries were embolized 1 to
5 days prior to surgery with polyvinyl alcohol (PVA)
particles. Embolization was not possible in one
Table III. Surgical approaches and treatment outcome of glomus tumors.
Glasscock type Operation
Glomus tympanicum (N3)
Type I Transmeatal approach
Type II Transmastoid approach
Glomus jugulare (N7)
Type I Infratemporal fossa approach
Fallopian bridge technique
Type II Infratemporal fossa approach
Type III Infratemporal fossa approach
Type IV Infratemporal fossa approach
NED: no evidence of disease, DOC: dead of another cause, AWD: alive with disease, F/U: follow up.
The glomus tumors were classified according to Glasscock-Jackson classification.
Skull base surgery 7
(14%) case because multiple arteries in this patient
fed the glomus jugulare. Six (86%) patients, with
glomus jugulare, underwent a type A infratemporal
fossa approach. The remaining one (14%) patient,
with glomus jugulare, underwent a fallopian bridge
technique with hypotympanectomy. Currently eight
(80%) patients have no evidence of disease and
two (20%) patients are alive with residual disease.
Multiple metastases were detected in one of the
two patients. The follow up period was 26 months
on average (range 4 months to 10 years).
The T stage of the temporal bone cancer was T1
in eight patients, T2 in six patients, T3 in five
patients and T4 in 11 patients. The operation and
the treatment outcome for each patient are described
in Table IV. Local resections were performed in two
(7%) patients, partial temporal bone resections in
16 (53%) patients, subtotal temporal bone resec-
tions in seven (23%) patients and the infratemporal
fossa approach type A in five (17%) patients. A
modified radical neck dissection was also performed
together with partial temporal bone resection in two
(7%) patients who were thought to have metastatic
neck nodes. The Mean follow up duration was
65 months (range 3 months to 20 years). The five-
year disease free survival was 42% (Figure 2) and
that for squamous cell carcinoma was 44% (figure
not shown). The 5-year survival of early stage (T1,
T2) squamous cell carcinoma was 86% and that
of the advanced stage (T3, T4) squamous cell
carcinoma was 20% (Figure 3).
Management of the facial nerve
Because the removal of a facial nerve schwannoma
always requires complete resection of a segment of
the facial nerve, facial nerve repair was performed in
all the cases, except one. An interposition graft with
the sural nerve was carried out in 12 (48%) patients,
an interposition graft with the greater auricular nerve
in four (16%) patients, a XII-VII anastomosis in
No. Outcome
1 NED (N1)
2 NED (N2)
1 NED (N1)
1 NED (N1)
1 F/U loss (N1)
1 NED (N1)
3 AWD (N2), DOC (N1)
 8
C.-S. Kim & M.-W. Suh
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Table IV. Patient data with temporal bone cancer.

No. Sex Age Pathology T stage Operation Facial nerve Adjuvant treatment Recur F/U duration Outcome

1 M 29 ACC T1 PTBR None 128 NED

2 M 47 SqCC T1 PTBR None 70 NED
3 F 70 BCC T1 Excision None Distant 42 NED
4 F 34 BCC T1 PTBR RT 73 NED
5 M 40 SqCC T1 PTBR RT Local, Regional 142 NED
6 M 46 SqCC T1 PTBR RT 62 NED
7 F 48 ACC T1 Excision None Distant 39 NED
8 M 53 SqCC T1 PTBR RT Local 14 AWD
9 M 63 SqCC T2 IFTA B RT 51 NED
10 M 60 SqCC T2 PTBR, MRND IIb None 42 NED
11 F 79 SqCC T2 PTBR RT 90 NED
12 M 56 SqCC T2 PTBR, Supficial parotidectomy RT Regional 5 NED
13 F 56 SqCC T2 PTBR, Supficial parotidectomy None 161 NED
14 F 51 ACC T2 PTBR, Supficial parotidectomy None 120 NED
15 F 59 ACC T3 PTBR, MRND IIb, TRAM RT 36 NED
16 M 66 SqCC T3 ITFA A RT Regional 51 NED
17 F 79 SqCC T3 PTBR None 11 NED
18 M 48 Sebaceous carcinoma T3 STTBR Cable graft None 4 DOD
19 F 53 Apocrine carcinoma T3 PTBR Decompression RT 244 DOC

20 M 61 SqCC T4 STTBR, TRAM Hook up RT Local 63 NED

21 M 52 Chondrosarcoma T4 IFTA A Cable graft RT Distant 72 NED
22 M 58 SqCC T4 PTBR RT 53 NED
23 M 32 Chondrosarcoma T4 PTBR, ITFA A Cable graft RT 31 NED
24 M 46 SqCC T4 STTBR RT Local 129 NED
25 M 40 SqCC T4 STTBR Hook up RT 89 NED
26 F 66 BCC T4 ITFA A Hook up RT 63 NED
27 M 41 ACC T4 PTBR None 10 DOD
28 M 45 SqCC T4 STTBR Hook up RT Local 15 DOD
29 M 65 ACC T4 STTBR RT Local 26 DOC
30 M 60 SqCC T4 STTBR, Total parotidectomy, PMMC Hook up RT 3 DOC

SqCC: squamoud cell carcimoma, ACC adenoid cystic carcinoma, BCC: basal cell carcinoma, PTBR: partial temporal bone resection, STTBR: subtotal temporal bone resection, ITFA A:
infratemporal fossa approach type A, ITFA B: infratemporal fossa approach type B, TRAM: transverse rectus abdominis musculocutaneous free flap, PMMC: pectoralis major myocutaneous flap,
MRND IIb: modified radical neck dissection type IIb, RT: radiation therapy, NED: no evidence of disease, DOD: dead of disease, DOC: dead of another cause, AWD: alive with disease.

Figure 2. Cumulative survival of temporal bone cancer. The
disease free five-year survival was 42%.
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seven (28%) patients and a jump graft to the
hypoglossal nerve with the sural nerve in one (4%)
patient. In the one case where a facial nerve repair
was not performed, the tumor was peeled from the
main trunk of the facial nerve without severing the
nerve itself. In this case, the preoperative and post-
operative facial nerve functions were HB grade II.
The other 24 cases where the facial nerve was
repaired after segmental resection resulted in a HB
grade III in interposition graft cases and HB grade
IV in XII-VII hook up cases. Compared to the
preoperative facial nerve function, the facial nerve
impairment worsened after surgery in four (16%)
patients, and it improved in 14 (56%) patients. The
Figure 3. Cumulative survival of temporal bone cancer by stage.
The 5-year survival of early stage (T1, T2) squamous cell
carcinoma was 86% and that of the advanced stage (T3, T4)
was 20%.
Skull base surgery 9
remaining seven (28%) patients had no change in
the performance of the facial nerve. It was also noted
that a facial nerve schwannoma can develop without
any sign of facial nerve paralysis as in two cases in
this series.
For the lower cranial nerve schwannomas, facial
nerve palsy developed in eight (89%) cases after
undergoing an infratemporal fossa approach type A.
Four patients resulted in HB grade III, while the
remaining four resulted in HB grade II. After
removing the glomus tumor via an infratemporal
fossa type A, facial nerve paralysis developed in two
(33%) cases. One case recovered from HB grade II
to grade I, four months later. Among the 30 patients
who underwent surgery for temporal bone cancer,
nine (30%) required facial nerve repair. All cases had
a T stage 3 or 4 cancer with a variety of pathologies
(Table IV). Three (30%) of them underwent a cable
graft with the sural nerve or greater auricular nerve.
The distal facial nerve stump was connected to the
proximal portion of the hypoglossal nerve in five
(56%) patients. In one (11%) patient, only a facial
nerve decompression was performed. The best result
obtained after repairing the facial nerve was HB
grade III.
Lower Cranial Nerve Function
Additional cranial nerve palsy that was not present
preoperatively developed after removing a lower
cranial nerve schwannoma via the infratemporal
fossa approach type A. Vocal cord palsy developed
in three (33%) patients and abducens nerve palsy
developed in one (11%) patient.
Case review
A 53-year-old male dentist visited the otology clinic
with a chief complaint of pulsating tinnitus. Five
years previously, the tinnitus had started intermit-
tently; however, recently the nature of the tinnitus
had changed and became continuous. A reddish
mass was visible through the tympanic membrane,
but there was no perforation noted (Figure 4). The
patient had a history of left side facial nerve paralysis
diagnosed 20 years ago, but he had fully recovered.
MRI findings were consistent with glomus jugulare
Glasscock-Jackson classification type III (Figure 5).
The preoperative left side pure tone audiogram
showed air conduction of 70 dB and the bone
conduction of 30 dB. The hearing on the right side
was normal.
Angiography and embolization were performed
five days before surgery (Figure 6). The tumor was
suspected to have originated from the hypotympa-
num based on the intraoperative findings. The mass
 10 C.-S. Kim & M.-W. Suh
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Figure 4. Tympanic membrane of the patient with glomus
jugulare tumor. A reddish pulsating mass was visible through
the tympanic membrane.
was removed by an infratemporal fossa approach
type A. After separating the incudostapedial joint,
the incus was removed. The long process of the incus
was eroded by the tumor. The retrofacial air cells
and infralabyrinthin air cells were occupied by the
tumor. The facial nerve was skeletonized and
rerouted anteriorly from the geniculate ganglion to
the main trunk of the extratemporal portion. Re-
routing of the facial nerve enabled a much wider
Figure 5. MRI findings of the patient with glomus jugulare tumor.
A glomus jugulare tumor is occupying the left middle ear, mastoid
cavity and petrous apex. MRI findings were consistent with glomus
jugulare tumor of Glasscock-Jackson classification type III.
view of the tumor. The glomus tumor was removed
very carefully. There was no invasion noted into the
otic capsule. The tumor extended into the petrous
apex, pericarotid region and above the internal
jugular vein. The distal portion of the sigmoid sinus
and the jugular bulb was removed because the tumor
had infiltrated into these vascular structures. The
carotid artery was preserved. Cranial nerves IX, X,
XII were identified and they were all preserved. After
completely removing the tumor (Figure 7), the
Eustachian tube, middle ear and mastoid were
obliterated with an abdominal fat and temporalis
muscle flap. The immediate postoperative facial
nerve function was HB grade II, but it was recovered
to HB grade I after four months. There was no
evidence of any damage to the other lower cranial
nerves. The patient was followed for three years with
no evidence of recurrence.
Discussion
Schwannoma
Surgical resection is the primary approach to the
treatment of a facial nerve schwannoma. However,
this surgery is almost always associated with a
significant degree of facial nerve paralysis [6,7].
Therefore, some clinicians prefer to wait or to only
decompress the tumor in order to preserve facial
nerve function for as long as possible [8,9]. Although
in this review, we focused only on surgical cases we
agree in part with this cautious approach. Normal or
good facial nerve function better than an HB grade
III, cannot be expected once the continuity of the
nerve is severed; as supported by the results pre-
sented in this paper. The only patient with a HB
grade II result, the best outcome in our series,
resulted from dissecting off the tumor from the
main trunk without cutting it. Preoperative counsel-
ing of the outcome of facial nerve function after
surgery is an important part of management. This is
especially important for those patients whose facial
nerve function is near normal preoperatively. We had
two patients with good facial nerve function before
surgery; however, it was not possible to remove
the tumor and preserve facial nerve function,
because the facial nerve fanned out and the full
extent of the tumor could not be completely traced.
Angeli and Brackmann [8] reported that wide
decompression is an alternative to surgical excision,
especially in cases where facial neuromas are not
suspected and in patients with normal facial func-
tion. The management of facial nerve schwannomas,
in our department, has become slightly more con-
servative during the last two decades.
 Skull base surgery 11

A B

Figure 6. Pre-embolization and post-embolization angiography findings of the patient with glomus jugulare tumor. Angiography and
embolization were performed 5 days before surgery. The ascending pharyngeal artery, occipital artery and caroticotympanic artery were
suspected to be the feeding vessels. The visible mass (A) disappeared in the post-embolization angiography (B).

There were two children with facial nerve schwan-
nomas in our series. The age at first visit to the
hospital was 13 and 17 months respectively. How-
ever, all the photos and video films taken by their
Downloaded By: [TÜBTAK EKUAL] At: 21:56 1 November 2009
this approach. A transmastoid approach was used
when a small tumor was located distal to the
geniculate ganglion. For tumors, involving the labyr-
inthine segment, a middle fossa approach or com-
bined middle fossa and transmastoid approach was

parents showed that these babies have had facial

paralysis from birth, indicating the possibility of
prenatal presence of the facial nerve schwannoma.
Although many cases have been reported in the
world literature, facial nerve tumors in such young
patients are very rare. A detailed history of these two
patients has been reported elsewhere [10]. A facial
nerve schwannoma should also be considered in the
differential diagnosis when a young child presents
with unilateral facial nerve paralysis.
The preoperative hearing level, location and size
of the tumor were important factors to consider
when selecting an operative technique. When the
hearing level was poor and the tumor was big, the
infratemporal fossa approach was frequently used.
However, in those patients who had serviceable
hearing it was difficult to preserve the hearing with
used. The postoperative facial nerve function was
HB grade II in the case of combined middle fossa
and transmastoid approach.
Glomus Tumor
According to Sanna et al [11] the most common
symptoms caused by a glomus tumor were hearing
loss and pulsatile tinnitus, consistent with our
results. Among these symptoms, unilateral pulsating
tinnitus was a unique characteristic associated
with these tumors. We treated five patients with
this symptom. Other causes that can also produce
pulsation tinnitus should be considered such as an
aberrant carotid artery, high jugular bulb and
arteriovenous malformation. O’Leary et al [12]
reported that an aberrant carotid artery is more

Figure 7. Operative findings after removing the whole mass (left eat). The glomus tumor was completely removed together with the jugular
bulb. The sigmoid sinus and carotid artery are exposed and the facial nerve rerouted anteriorly. VII: facial nerve, ICA: internal carotid
artery, SS: sigmoid sinus, C: cochlea, L: labyrinth, Et: Eustachian tube, Ant: anterior, Post: posterior, Sup: superior, Inf: inferior.
 12 C.-S. Kim & M.-W. Suh
likely when the mass is located at the anterior
portion of the tympanic membrane and a glomus
tumor or a high jugular bulb are more likely
when the mass is located at the posterior portion.
Although this point may be straightforward, it
cannot be applied to all cases. There were seven
patients in our series who were operated on due to a
glomus jugulare but only two patients had the typical
postero-inferior tympanic mass. However, it seems
to follow that the mass is located at the inferior
portion of the tympanic membrane. The location of
the tympanic mass of the glomus tumor is rather
dependent on the stage of the disease process.
Although the majority of glomus tumors are thought
to secrete catecholamine, only 2% of glomus tumors
are reported to produce clinical symptoms [13].
Typical symptoms may be severe headache, pallor,
palpitation, nausea, and vomiting. When the tumor
is functioning, it can abruptly release catecholamine
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into the bloodstream during surgery and cause
disastrous cardiovascular events [14]. Twenty-four
hour urine VMA and metanephrine should be
evaluated before surgery in order to prevent such
emergencies. We also had one patient with a
functioning tumor in our series of patients.
Recent advances in stereotactic radiosurgery sug-
gest that radiation therapy has similar or better
results than surgery for management of glomus
tumors. Although these tumors are typically radio-
sensitive, radiation therapy can eliminate the entire
tumor in only 30% of cases [15]. The goal of
radiation is growth inhibition rather than elimination
of the tumor [16]. Therefore, surgery is superior to
radiation for immediate and complete tumor elim-
ination [17]. Moreover, residual tumor may remain
dormant for the rest of the patient’s life in some
cases, but in others, it may become a malignant
tumor. It is reported that 1 to 5% of glomus jugulare
tumors are malignant and exhibit metastatic spread
[18,19]. We treated one such patient out of 10 in
our series that had distant metastasis after malignant
transformation. Complete resection of the tumor
can be safer than leaving residual tumor after
radiation therapy. Radiation may have a more
important role in salvage treatment for patients
who cannot undergo surgery because of unresectable
tumors or residual disease.
In our experience and from other reports, glomus
tympanicum was usually accessible via a transmeatal
approach or an extended posterior tympanotomy
approach [12,20]. However, glomus jugulare re-
quired greater consideration of residual functions
of hearing and the lower cranial nerves. Six of the
seven patients with glomus jugulare were treated
with the infratemporal approach type A. Most of
them did not have serviceable hearing before sur-
gery. Postoperatively, one patient suffered from
multiple lower cranial nerve palsies including cranial
nerves IX, X, XII; the rest of the patients were
successfully managed without damaging other lower
cranial nerves. However, as demonstrated by our
series of lower cranial nerve schwannomas, the
infratemporal fossa approach type A has the poten-
tial risk of facial nerve impairment. During the
standard infratemporal fossa approach type A, the
facial nerve was removed from the fallopian canal
and rerouted anteriorly. Devascularization of the
vasa nervorum is the suspected cause of facial nerve
palsy during this procedure. Although the facial
nerve impairment may be mild and/or transient,
it should be considered as a specific risk associated
with this type of surgery. We currently use the
fallopian bridge technique with hypotympanectomy
in selected cases. This is an approach that removes
the temporal bone inferior to the vestibule and
cochlea leaving the fallopian canal in situ [21].
There is a better chance of preserving the facial
nerve function and hearing compared to the infra-
temporal fossa approach type A. A patient who
underwent this procedure currently has a facial nerve
function of HB grade I and her postoperative hearing
is excellent with no air bone gap.
Temporal bone cancer
Squamous cell carcinoma and adenocarcinoma were
the two most common histological types of temporal
bone carcinomas, consistent with other studies [22].
Of the various symptoms of temporal bone cancer,
otalgia is among the most common. In addition,
investigators have suggested that the character of
pain becomes more deep seated and unremitting
as the disease advances [23,24]. Many patients
visit the otology clinic with nonspecific ear pain.
Although the majority of clinic patients do not have
any specific abnormality, clinicians must be able to
distinguish those among them that may have a
temporal bone malignancy. Deep seated and unre-
mitting pain may be a sign that alerts the clinician to
a more serious problem.
Various staging systems for temporal bone cancer
have been proposed. However, there is no consensus
on any of the staging systems proposed to date.
Some investigators have suggested the need for a
multi-institutional effort to develop a staging system
and prospective treatment protocols [25]. The lack
of a unified staging system makes it difficult to
compare treatment outcomes between studies. In
this article, we used the Pittsburgh staging system
proposed by Arriaga in 1990. Originally, this staging
system was proposed to classify cancer of the
external auditory canal. It may not be appropriate

for classifying temporal bone cancers of other
origins. Although there are limitations using this
staging system, the Pittsburgh staging system seem
to be the most widely used for similar cases
currently.
The 5-year survival rate for patients with temporal
bone cancer in other studies has been reported to be
91% for localized disease and 45% for extensive
disease [26
28]. In another study it was reported to
be 50% overall and 27% for advanced cases [29].
In our series, it was 86% for early stage squamous cell
carcinoma and 20% for advanced stage squamous
cell carcinoma. The treatment results of advanced
stage cancer at our center appear to be worse than
other centers. However, as is shown in Table IV there
were many more T4 stage patients than T3 stage,
which may resulted in a poorer treatment outcome.
In addition, direct comparison of survival among
studies may be difficult, because the pathologies of
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the cancer are not identical in the studies compared
and there is no unified staging system.
Although a few patients with a very small lesion had
a simple excision, partial temporal bone resection is
recommended as the basic treatment for external
auditory canal malignancy. This procedure allows for
an en block removal of the soft tissues and bony
structures, lateral to the otic capsule. In advanced
cases, a wider resection may be needed such as a
subtotal temporal bone resection. In addition, other
procedures such as superficial parotidectomy, mod-
ified radical neck dissection and facial nerve repair
are required depending on the location and extent of
the cancer. The skull base surgeon should be
prepared to provide all of the listed procedures to
be able to manage life-threatening diseases.
Conclusion
The temporal bone location is one of the most
difficult anatomic sites to perform surgery. A hard
bony wall covers the lesions and the many vital
structures such as the facial nerve, cochlea, labyrinth,
jugular vein and carotid artery may be nearby or even
overriding the lesion. As has been shown in this
report many skull base operations may be required to
approach the lesion so that vital structures are not
damaged. The pathological type and the physiologi-
cal status of the tumor were important factors in
determining the specific transtemporal surgery ap-
propriate for a given patient. In addition, the patient’s
clinical functioning should be considered when
selecting the appropriate surgical approach. In
some cases, we had to sacrifice residual hearing so
that the tumor could be completely removed, and in
others, we had to reroute the facial nerve despite the
risk of facial nerve impairment. Understanding the
Skull base surgery 13
strength and weakness of each surgical technique
and knowledge of the particular tumor biology
may facilitate selection of the appropriate surgical
approach and result in a successful outcome.
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