The Laryngoscope
Lippincott Williams & Wilkins, Inc.
© 2003 The American Laryngological,
Rhinological and Otological Society, Inc.
Inner Ear Extension of Vestibular
Schwannomas
Maurizio Falcioni, MD; Abdelkader Taibah, MD; Di Giuseppe Trapani, MD; Tarek Khrais, FRCS, HSD, MD;
Mario Sanna, MD
Objective: Inner ear extension of vestibular
schwannomas (VSs) is a rare finding but has impor-
tant clinical implications. This report reviews the
treatment options and presents the experience of the
Gruppo Otologico, Piacenza, Italy, in this field. Study
Design: Case report and literature review. Methods:
Five cases of VSs with inner ear extension were sur-
gically removed. In all of them, the cochlea was par-
tially or completely invaded by the lesion. Results: In
4 cases, the inner ear extension was preoperatively
identified on magnetic resonance imaging, and the
surgical removal was planned through a transotic ap-
proach. In the last case, the cochlear invasion was not
detected preoperatively, and the lesion was removed
during a second surgery performed to seal a cerebro-
spinal fluid fistula. Conclusions: VSs with inner ear
extension should be distinguished from pure intral-
abyrinthine schwannomas because of differences in
clinical significance. Cochlear involvement is more
frequent than vestibular involvement and is often ac-
companied by a dead ear. Dead ear caused by small
VSs should alert the surgeon to the possibility of a
cochlear extension. The presence of an intracochlear
involvement requires the adoption of an approach
that allows control of the cochlear turns, and we
found the transotic approach to be the most suitable.
Undetected cochlear extensions that are left in place
may grow with time. Key words: Vestibular schwan-
noma, vestibule, cochlea, inner ear, labyrinth.
Laryngoscope, 113:1605–1608, 2003
INTRODUCTION
Until the late 1980s, schwannomas confined to the
labyrinth were reported almost exclusively as intraopera-
tive or postmortem finding. The situation changed after
the introduction of magnetic resonance imaging (MRI),
when the preoperative discovery of intralabyrinthine
schwannomas (ISs) became more frequent. In our opinion,
these lesions should be distinguished from labyrinthine
From the Gruppo Otologico, Piacenza, Italy.
This work was supported by a grant from A.S.A.B. (Associazione
Studio Aggiornamento Basicranio).
Editor’s Note: This Manuscript was accepted for publication May 7,
2003.
Send Correspondence to Maurizio Falcioni, MD, Gruppo Otologico,
Via Emmanueli 42, 29100 Piacenza, Italy. E-mail: maurizio.falcioni@
gruppootologico.it
Laryngoscope 113: September 2003
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involvement by vestibular schwannomas (VSs) because of
the different clinical implications. The following study
reports 5 cases of VSs with inner ear invasion surgically
treated at the Gruppo Otologico, Piacenza, Italy. A sum-
mary of the main features of the five cases is reported in
Table I.
CASE REPORTS
Case 1
A 24-year-old man reported a sudden right-sided hearing
loss (HL) and tinnitus for 2 years, in the absence of any vestibular
complaints. Audiometry revealed the presence of right-sided
anacusis. MRI showed a right-sided internal auditory canal (IAC)
neoformation that invaded the basal turn of the cochlea. Cochlear
extension was not mentioned on the radiologic report. Surgical
removal was accomplished through a transotic (TO) approach.
The patient was discharged after 5 days with normal facial func-
tion. At follow-up after 1 year, the MRI did not show any tumor
recurrence.
Case 2
A 49-year-old woman reported a progressive left-sided HL
over 13 years, accompanied by occasional tinnitus and vertigo.
Audiometry revealed the presence of left-sided dead ear. MRI
showed a cerebellopontine angle neoformation (size 1 cm) with
invasion of the cochlea. Cochlear extension was not mentioned on
the radiologic report. The tumor was removed through a TO
approach. The patient was hospitalized for 6 days and discharged
with a facial nerve (FN) dysfunction (grade III according to the
House-Brackmann scale). At the last follow-up, 1 year after sur-
gery, the FN function had recovered to grade II.
Case 3
A 26-year-old woman reported a progressive right-sided HL
and tinnitus over 1 year. She did not experience any vestibular
disturbances. Audiometry revealed the presence of right-sided
dead ear. MRI revealed an intracanalicular neoformation. No
inner ear extension was mentioned on the radiologic reports of
the two MRI scans that had been done elsewhere. The tumor was
removed through a translabyrinthine approach (TLA). After 3
days, the patient experienced a rhino-liquorrhea. Reevaluation of
the preoperative MRI showed the presence of tumoral extension
into the basal turn of the cochlea (Fig. 1). During the revision
surgery for closure of the cerebrospinal fluid fistula, the cochlea
was drilled, and the residual tumor was removed. The patient
was hospitalized for 9 days and discharged with a FN palsy. One
Falcioni et al: Extension of Vestibular Schwannomas
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Patient Features.
Symptoms Inner ear
Patient Symptoms Hearing Duration Involvement
1 HL, tinnitus Dead ear 2 years Basal turn
2 HL, tinnitus, Dead ear 13 years Cochlea
vertigo
3 HL, tinnitus Dead ear 1 year Basal turn
4 HL, tinnitus, Dead ear 24 years All
vertigo
5 HL, Dead ear 5 years Cochlea
unsteadiness
IAC ⫽ internal auditory canal; CPA ⫽ cerebellopontine angle; HL ⫽ hearing loss; IE ⫽ inner ear; TO ⫽ transotic approach; TLA ⫽ translabyrinthine
approach;MRI ⫽ magnetic resonance imaging.
year later, the MRI showed no recurrence, and the FN function
had recovered to a grade III.
Case 4
A 48-year-old woman reported a left-sided tinnitus over 24
years accompanied by a left-sided progressive HL over 13 years.
She also experienced one episode of vertigo. Audiometry revealed
the presence of left-sided dead ear. The MRI showed a 3 cm
cerebellopontine angle neoformation extending to the IAC, all the
inner ear, as well as the geniculate ganglion (Fig. 2). Both the
inner ear and geniculate ganglion involvement were not men-
tioned on the radiologic report. The computed tomography (CT)
scan showed an IAC enlargement and the presence of soft tissue
in middle ear near the round window (Fig. 3). The geniculate
fossa was enlarged as well. Tumor removal was planned through
a TO approach. During the surgery, two different lesions were
disclosed, originating respectively from the VIII and VII cranial
nerve. The tumor filled all the inner ear protruding into the
middle ear cavity through the round window. It was not possible
to locate the central stump of the FN. The patient was hospital-
ized for 6 days and discharged with a total palsy of the VII cranial
nerve. Two months later, the patient underwent hypoglosso-facial
anastomosis. At the first follow-up, 3 months after the last sur-
gery, the FN function was still grade VI.
Case 5
A 54-year-old man reported a right-sided HL and unsteadi-
ness over 5 years. Audiometry revealed the presence of right-
sided dead ear. The MRI showed an IAC lesion involving all the
cochlea turns (Fig. 4). Surgical removal was planned through a
TO approach. The patient was hospitalized for 4 days and dis-
charged with a normal FN function (grade I). Because he was
operated on only few months ago, no follow-up is presently
available.
DISCUSSION
Histologically, VSs develops in the myelinated part of
the VIII cranial nerve, often in the IAC. However,
Schwann cells are also present in the modiolus, close to
the spiral ganglia1, so schwannomas theoretically may
arise in this area.
The first report of an IS is attributed to Mayer, in
1917.2 It was discovered at autopsy in a patient affected
by neurofibromatosis. Since then, a few cases have been
reported as postmortem or intraoperative findings during
surgery for Ménière’s disease.3 The first preoperative ra-
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TABLE I.
IAC/CPA Middle Ear Preoperative
Involvement Involvement Radiological Report Detection Approach
IAC NO No IE involvement YES TO
CPA (1 cm) NO No IE involvement YES TO
IAC NO No IE involvement NO TLA and
(2 MRI) revision
CPA (3 cm) YES No IE involvement YES TO
IAC NO No IE involvement YES TO
diologic diagnosis of an intracochlear schwannoma was
reported by Karlan et al.4 in 1972, through a polytomog-
raphy demonstrating nonvisualization of the basal turn of
the cochlea. In 1994, Doyle and Brackmann5 reported the
first series of preoperatively diagnosed ISs through MRI.
Currently, refinements in the MRI technique allow the
differentiation of lesions developing in the inner ear, in-
cluding small tumors.6
The increased frequency of reporting ISs during the
last years has coincided with the development of the MRI.
In some articles, pure ISs have been listed together with
labyrinthine involvement by VSs.5,7,8 In agreement with
Zbar et al.9, we think that the two lesions should be
distinguished from each other because of the different
clinical implications and, probably, the different site of
origin. In fact, although it is not possible to definitively
identify the origin of a schwannoma with IAC and inner
ear involvement,10 the hypothesis of a progressive involve-
ment of the labyrinth during the growth of a VS seems to
be more reasonable. This is supported by the fact that
cochlear involvement is more frequent than vestibular
involvement. This difference can be explained by the pres-
ence of the weakest part of the fundus, the cribriform area,
between the IAC and the cochlea.11
All our cases showed involvement of the cochlea. Two
of the smallest tumors (cases 1 and 3) had a minimal inner
ear component, confined to the basal turn of the cochlea,
as if discovered at the beginning of the invasion. If the
tumor’s origin was in the cochlea, it is difficult to under-
stand why such a tumor should first erode the cribriform
area before growing along the way of least resistance
offered by the cochlear turns.
However, it seems reasonable to suppose the tumor
originated from the cochlear nerve.12 This is in agreement
with our surgical finding in which, in all three small
tumors, there was a clear involvement of the cochlear
nerve. In the two remaining lesions, the IAC was com-
pletely filled by the lesion, and it was not possible to
establish the nerve of the origin.
In rare cases, the tumor involving the inner ear can
have further growth into the middle ear, through the oval
window or the round window. Because of the natural
barrier represented by the footplate, the round window
Falcioni et al: Extension of Vestibular Schwannomas
route is more probable and has been reported more fre-
quently in literature.11,13,14 In one of our patients, the
tumor completely filled the inner ear, with a small com-
ponent protruding into the middle ear cleft through the
round window. This extension was visible on MRI and was
further confirmed by a CT scan. As already reported, CT
scan is useful as a complementary evaluation for schwan-
nomas involving the middle ear, allowing a clear defini-
tion of the lesion’s extent and its relationship with the
surrounding structures.11
HL, tinnitus, and vertigo are the symptoms usually
reported in presence of an IS. It seems that vertigo is
related only to the involvement of the posterior labyrinth.5
This may not be the case in VSs with inner ear extension
because the symptomatology is also related to the retro-
cochlear component of the tumor. All of our five cases
presented with HL that progressed to anacusis at the time
Fig. 1. Magnetic resonance imaging, axial view, patient 3. The scan
of diagnosis. Vertigo was reported at the beginning of the shows an internal auditory canal lesion with an extension to the
symptoms by two patients. Dead ear seems to be particu- basal turn of the cochlea.
larly common in cases of VSs with cochlear involvement.5
As a consequence, the contemporary presence of a dead
ear and a VS should prompt the surgeon to carefully sure of the cerebrospinal fluid fistula. Green and McKen-
evaluate the MRI to detect any lateral extension into the zie7 reported two cases in which a VS with undetected
cochlea. Although toxic and metabolic theories have also cochlear extension was treated with a TLA, leaving the
been proposed, HL in these patients seems to be related to inner ear component of the tumor. One patient required a
the direct cochlear invasion.15 revision surgery, whereas the other did not show any
Labyrinthine invasion is clearly visible on MRI as an tumor growth on serial MRI. The classic TLA allows open-
enhanced mass lateral to the IAC fundus after gadolinium ing and control of the vestibule and semicircular canal
infusion or as a lack of inner ear fluid in the fast spin echo while the cochlea remains inaccessible. In contrast, in the
sequences. However, particularly in cases with limited TO approach, the external and middle ear are removed,
inner ear involvement, this component of the lesion can be the external auditory canal is closed as a blind sac, and
overlooked with a nonaccurate scan evaluation. In all our the cochlea is drilled out. Although this approach is more
cases, the inner ear extension was not pointed out in the time consuming, it allows a direct control of any tumor
radiologic report. All the scans had been carried out in extension inside the cochlea. As a consequence, in the
other centers, probably without adequate experience in presence of an intralabyrinthine extension of a VS, the
petrous bone and cerebellopontine angle (CPA) lesions, TLA can be adopted only when the intralabyrinthine com-
and those incomplete reports could have been of particular ponent is limited to the vestibule and the semicircular
significance when planning the surgical removal. In four canals, whereas involvement of the cochlea requires man-
cases, the labyrinthine component was identified by the agement through a TO approach. The retrosigmoid ap-
physicians of Gruppo Otologico during office scan evalua- proach, not allowing access to the inner ear, is contrain-
tion, but in one patient, the cochlear extension escaped
this reassessment (case 3).
From a clinical point of view, management of pure IS
depends only on symptomatology, in particular the pres-
ence of vertigo. If no disabling vertigo is present, the
tumor can be radiologically monitored.8 On the contrary,
VSs involving the inner ear should be treated according to
the IAC/CPA extension, and the patient’s age and wishes.
In both instances, no hearing preservation procedure is
feasible. The only pure IS patient that presented to our
center has been conservatively treated and is being radio-
logically monitored. All five cases of VSs with inner ear
extension presented in this series have been surgically
treated. In the four cases in which the inner ear involve-
ment has been preoperatively detected, the removal was
planned through a TO approach.16,17 The last case was
originally treated by means of a TLA, and the tumor
remnants inside the cochlea were discovered during re-
Fig. 2. Magnetic resonance imaging, axial view, patient 4. A large
evaluation of the preoperative scan because of the devel- lesion involving the cerebellopontine angle and internal auditory
opment of a rhinoliquorrea. A revision surgery was then canal. Enhancement of the cochlea, vestibule, and lateral semicir-
planned, allowing removal of the residual lesion and clo- cular canal is also clearly visible.

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Fig. 3. Computed tomograph, coronal view, patient 4. The tumor
protrudes from the round window, partially occupying the middle
ear cleft.
dicated in the presence of intralabyrinthine tumoral
extension. Takizawa et al.18 reported two cases of recur-
rence after a retrosigmoid approach in patients affected by
VSs with intralabyrinthine extensions. Both recurrences
occurred mainly in the extradural space. The middle cra-
nial fossa approach theoretically can offer a good exposure
of all the vestibule and the turns of the cochlea. However,
because complete tumor removal in this pathology re-
quires the sacrifice of hearing, use of such a procedure,
which was originally meant to preserve hearing, does not
make sense.
CONCLUSIONS
Currently, inner ear involvement by VSs is more
frequently reported because of the use of high-resolution
MRI. In our opinion, VSs with labyrinthine extension
should be distinguished from pure ISs because of the
different clinical significance and, probably, the different
Fig. 4. Magnetic resonance imaging, axial view, patient 5. The
fundus of the internal auditory canal is filled by a lesion extending to
the basal turn of the cochlea.
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1608
origin. According to our data, involvement of the cochlea is
more frequent than vestibular involvement and is often
accompanied by a dead ear. Cochlear invasion by a VS
should always be looked for on the MRI, especially in
presence of a dead ear, and, because such extensions
could be missed by radiologists, the surgeon should
personally evaluate the MRI for their presence. The
presence of an intracochlear tumoral extension requires
the adoption of an approach allowing direct control of
the cochlear turns. Because it is impossible to preserve
any hearing, the TO approach seems to be the most
appropriate procedure. Undetected cochlear extensions
left in place during surgery may grow with time and
require a revision surgery. Theoretically, because of the
frequent anacusis present in these patients, this growth
may go on asymptomatically until the tumor reaches
considerable size.
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