Академический Документы
Профессиональный Документы
Культура Документы
Objective: Inner ear extension of vestibular involvement by vestibular schwannomas (VSs) because of
schwannomas (VSs) is a rare finding but has impor- the different clinical implications. The following study
tant clinical implications. This report reviews the reports 5 cases of VSs with inner ear invasion surgically
treatment options and presents the experience of the treated at the Gruppo Otologico, Piacenza, Italy. A sum-
Gruppo Otologico, Piacenza, Italy, in this field. Study mary of the main features of the five cases is reported in
Design: Case report and literature review. Methods:
Table I.
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 CASE REPORTS
4 cases, the inner ear extension was preoperatively
identified on magnetic resonance imaging, and the Case 1
A 24-year-old man reported a sudden right-sided hearing
surgical removal was planned through a transotic ap-
loss (HL) and tinnitus for 2 years, in the absence of any vestibular
proach. In the last case, the cochlear invasion was not
complaints. Audiometry revealed the presence of right-sided
detected preoperatively, and the lesion was removed
anacusis. MRI showed a right-sided internal auditory canal (IAC)
during a second surgery performed to seal a cerebro-
neoformation that invaded the basal turn of the cochlea. Cochlear
spinal fluid fistula. Conclusions: VSs with inner ear
extension was not mentioned on the radiologic report. Surgical
extension should be distinguished from pure intral-
removal was accomplished through a transotic (TO) approach.
abyrinthine schwannomas because of differences in
The patient was discharged after 5 days with normal facial func-
clinical significance. Cochlear involvement is more
tion. At follow-up after 1 year, the MRI did not show any tumor
frequent than vestibular involvement and is often ac-
recurrence.
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 Case 2
involvement requires the adoption of an approach A 49-year-old woman reported a progressive left-sided HL
that allows control of the cochlear turns, and we over 13 years, accompanied by occasional tinnitus and vertigo.
found the transotic approach to be the most suitable. Audiometry revealed the presence of left-sided dead ear. MRI
Undetected cochlear extensions that are left in place showed a cerebellopontine angle neoformation (size 1 cm) with
may grow with time. Key words: Vestibular schwan- invasion of the cochlea. Cochlear extension was not mentioned on
noma, vestibule, cochlea, inner ear, labyrinth. the radiologic report. The tumor was removed through a TO
Laryngoscope, 113:1605–1608, 2003 approach. The patient was hospitalized for 6 days and discharged
with a facial nerve (FN) dysfunction (grade III according to the
INTRODUCTION House-Brackmann scale). At the last follow-up, 1 year after sur-
Until the late 1980s, schwannomas confined to the gery, the FN function had recovered to grade II.
labyrinth were reported almost exclusively as intraopera-
tive or postmortem finding. The situation changed after Case 3
the introduction of magnetic resonance imaging (MRI), A 26-year-old woman reported a progressive right-sided HL
when the preoperative discovery of intralabyrinthine and tinnitus over 1 year. She did not experience any vestibular
schwannomas (ISs) became more frequent. In our opinion, disturbances. Audiometry revealed the presence of right-sided
dead ear. MRI revealed an intracanalicular neoformation. No
these lesions should be distinguished from labyrinthine
inner ear extension was mentioned on the radiologic reports of
the two MRI scans that had been done elsewhere. The tumor was
From the Gruppo Otologico, Piacenza, Italy. removed through a translabyrinthine approach (TLA). After 3
This work was supported by a grant from A.S.A.B. (Associazione days, the patient experienced a rhino-liquorrhea. Reevaluation of
Studio Aggiornamento Basicranio).
the preoperative MRI showed the presence of tumoral extension
Editor’s Note: This Manuscript was accepted for publication May 7,
into the basal turn of the cochlea (Fig. 1). During the revision
2003.
surgery for closure of the cerebrospinal fluid fistula, the cochlea
Send Correspondence to Maurizio Falcioni, MD, Gruppo Otologico,
Via Emmanueli 42, 29100 Piacenza, Italy. E-mail: maurizio.falcioni@ was drilled, and the residual tumor was removed. The patient
gruppootologico.it was hospitalized for 9 days and discharged with a FN palsy. One
1 HL, tinnitus Dead ear 2 years Basal turn IAC NO No IE involvement YES TO
2 HL, tinnitus, Dead ear 13 years Cochlea CPA (1 cm) NO No IE involvement YES TO
vertigo
3 HL, tinnitus Dead ear 1 year Basal turn IAC NO No IE involvement NO TLA and
(2 MRI) revision
4 HL, tinnitus, Dead ear 24 years All CPA (3 cm) YES No IE involvement YES TO
vertigo
5 HL, Dead ear 5 years Cochlea IAC NO No IE involvement YES TO
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 diologic diagnosis of an intracochlear schwannoma was
had recovered to a grade III. reported by Karlan et al.4 in 1972, through a polytomog-
raphy demonstrating nonvisualization of the basal turn of
Case 4 the cochlea. In 1994, Doyle and Brackmann5 reported the
A 48-year-old woman reported a left-sided tinnitus over 24 first series of preoperatively diagnosed ISs through MRI.
years accompanied by a left-sided progressive HL over 13 years.
Currently, refinements in the MRI technique allow the
She also experienced one episode of vertigo. Audiometry revealed
differentiation of lesions developing in the inner ear, in-
the presence of left-sided dead ear. The MRI showed a 3 cm
cerebellopontine angle neoformation extending to the IAC, all the cluding small tumors.6
inner ear, as well as the geniculate ganglion (Fig. 2). Both the The increased frequency of reporting ISs during the
inner ear and geniculate ganglion involvement were not men- last years has coincided with the development of the MRI.
tioned on the radiologic report. The computed tomography (CT) In some articles, pure ISs have been listed together with
scan showed an IAC enlargement and the presence of soft tissue labyrinthine involvement by VSs.5,7,8 In agreement with
in middle ear near the round window (Fig. 3). The geniculate Zbar et al.9, we think that the two lesions should be
fossa was enlarged as well. Tumor removal was planned through distinguished from each other because of the different
a TO approach. During the surgery, two different lesions were
clinical implications and, probably, the different site of
disclosed, originating respectively from the VIII and VII cranial
origin. In fact, although it is not possible to definitively
nerve. The tumor filled all the inner ear protruding into the
middle ear cavity through the round window. It was not possible identify the origin of a schwannoma with IAC and inner
to locate the central stump of the FN. The patient was hospital- ear involvement,10 the hypothesis of a progressive involve-
ized for 6 days and discharged with a total palsy of the VII cranial ment of the labyrinth during the growth of a VS seems to
nerve. Two months later, the patient underwent hypoglosso-facial be more reasonable. This is supported by the fact that
anastomosis. At the first follow-up, 3 months after the last sur- cochlear involvement is more frequent than vestibular
gery, the FN function was still grade VI. involvement. This difference can be explained by the pres-
ence of the weakest part of the fundus, the cribriform area,
Case 5 between the IAC and the cochlea.11
A 54-year-old man reported a right-sided HL and unsteadi- All our cases showed involvement of the cochlea. Two
ness over 5 years. Audiometry revealed the presence of right- of the smallest tumors (cases 1 and 3) had a minimal inner
sided dead ear. The MRI showed an IAC lesion involving all the
ear component, confined to the basal turn of the cochlea,
cochlea turns (Fig. 4). Surgical removal was planned through a
TO approach. The patient was hospitalized for 4 days and dis-
as if discovered at the beginning of the invasion. If the
charged with a normal FN function (grade I). Because he was tumor’s origin was in the cochlea, it is difficult to under-
operated on only few months ago, no follow-up is presently stand why such a tumor should first erode the cribriform
available. area before growing along the way of least resistance
offered by the cochlear turns.
DISCUSSION However, it seems reasonable to suppose the tumor
Histologically, VSs develops in the myelinated part of originated from the cochlear nerve.12 This is in agreement
the VIII cranial nerve, often in the IAC. However, with our surgical finding in which, in all three small
Schwann cells are also present in the modiolus, close to tumors, there was a clear involvement of the cochlear
the spiral ganglia1, so schwannomas theoretically may nerve. In the two remaining lesions, the IAC was com-
arise in this area. pletely filled by the lesion, and it was not possible to
The first report of an IS is attributed to Mayer, in establish the nerve of the origin.
1917.2 It was discovered at autopsy in a patient affected In rare cases, the tumor involving the inner ear can
by neurofibromatosis. Since then, a few cases have been have further growth into the middle ear, through the oval
reported as postmortem or intraoperative findings during window or the round window. Because of the natural
surgery for Ménière’s disease.3 The first preoperative ra- barrier represented by the footplate, the round window