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Chapter
Vestibular Schwannomas:
Diagnosis and Surgical Treatment
Gustavo Jung and Ricardo Ramina
Abstract
Over the last decades, significant advances in skull base surgery have enabled
many neurosurgical centers around the world to perform surgical resection of
vestibular schwannomas; otherwise, clinical observation and radiotherapy/radio-
surgery can be possible management options. Auditory pattern, the presence of
bilateral tumors, tumor size, and neurological symptoms are deeply considered in
the decision-making process. In this chapter, we expanded the general discussion of
vestibular schwannomas, discussing bases for an accurate diagnose and the techni-
cal aspects for the surgical approaches, drilling of internal auditory canal, and its
reconstruction as well as the technical nuances when handling very small and large/
residual tumors.
Keywords: vestibular schwannoma, acoustic neuroma, acoustic tumor,
cerebellopontine angle
1. Introduction
Vestibular schwannomas (VS) account for 6–8% of all intracranial neoplasms
and around 90% of cerebellopontine angle tumors (CPA) [1]. It is usually a sporadic
tumor but can be bilateral in cases of neurofibromatosis type 2, when larger tumors
are common.
Over the last decades, significant advances in skull base surgery have enabled
many neurosurgical centers around the world to perform surgical resection of VS
with good functional outcomes (facial nerve and hearing preservation). Deeper
observation about the natural history of these lesions and the development of
radiosurgery have increased the options to manage VS. The rates of surgical mor-
bidity and mortality have also declined dramatically, and functional preservation of
the facial nerve has been possible even in larger tumors.
2. Natural history
The natural history of VS is highly unpredictable. Some tumors exhibit continu-
ous growth, while others remain stable or even decrease in size, and its reason is not
known. In the literature, a mean growth rate of 2.9 mm per year is reported, and a
growth rate of 2.5 mm/year is associated with worse hearing function.
1
Primary Intracranial Tumors
3. Management options
Clinical observation, microsurgical removal, and radiotherapy/radiosurgery are
the management options. Different factors make treatment decision highly variable.
Small tumors may be followed with regular MRI examinations and audiograms.
Patients harboring small tumors and presenting progressive hearing loss, micro-
surgical removal, or radiosurgery should be considered. Preoperative hearing level
is a prognostic factor for postoperative hearing preservation. Tumors up to 3 cm
in diameter may be treated either by microsurgical removal or radiosurgery, and
larger tumors will require surgical resection. Cystic VS may present sudden growth
and surgical removal is the best option. The management of bilateral tumors in NF2
patients is complex, and the quality of hearing in both ears and size of the tumors
will be the main factors to decide how to treat these patients.
4. Diagnosis
VS commonly arise from the vestibular division of the eighth cranial nerve.
Dizziness, vertigo, and progressive hearing loss (earliest symptom) are the most
frequent complaints. Dizziness is routinely transient and episodic, and the patient
can neglect it for a variable period of time. Dizziness is a frequent complaint in daily
ENT practice, and patients complaining of unilateral hearing loss associated or
not to vestibular symptoms are frequently seen by ENT surgeons. Very often these
symptoms are not adequately investigated, and it is a common cause to miss the
diagnosis.
Facial nerve weakness is observed in only 6% of the patients [2]. In larger and
mainly cystic tumors that present fast growth, facial numbness (due to trigemi-
nal nerve compression) and gait ataxia (due to brain stem displacement) can
appear [3, 4].
Hydrocephalus is relatively common in VS patients. In larger tumors it is caused
by IV ventricle displacement, leading to obstructive hydrocephalus. In smaller
tumors, degenerative changes on the tumor content can increase the protein rates
on CSF, causing CSF malabsorption and consequent hydrocephalus [5]. When
hydrocephalus is present, a preoperative external ventricular CSF drainage is
required. Ventriculoperitoneal shunt is done especially in patients without major
CSF obstruction when the tumor removal is less probable to relieve.
5. Audiological evaluation
Hearing function is evaluated with audiograms with sound discrimination.
There are different classifications to preoperatively grade the hearing function.
Brain stem evoked response audiometry (BERA) provides reliable information on
the hearing function from the ear to the brain stem, and to determine the nerve of
origin in vestibular schwannomas, the video Head Impulse Test (vHIT) is usually
performed.
6. Radiological diagnosis
CT scan is useful to demonstrate the bony anatomy, the position of the jugular
bulb, and the semicircular canals. Vestibular schwannomas often expand the
internal auditory canal [10].
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Vestibular Schwannomas: Diagnosis and Surgical Treatment
DOI: http://dx.doi.org/10.5772/intechopen.81352
Magnetic resonance imaging (MRI) is the eligible test to diagnose and evaluate
patients with a vestibular schwannomas. T1, T2, FLAIR, and DWI images are usu-
ally sufficient for the diagnosis. Over 50% of vestibular schwannomas are isointense
in T1-weighted images; hypointensities usually represent a cystic component
(Figure 1). VS present usually intense homogeneous gadolinium enhancement on
T1-weighted images, but cystic lesions can present a heterogenous pattern. A hyper-
intense signal inside of the IAC in FLAIR images and nodular hyperintense signal
in the vestibular nuclei on the dorsal pons in T2-weighted images can additionally
differentiate vestibular schwannomas from other cerebellopontine angle tumors
(Figure 2). T2-weighted images and tractography may demonstrate the position of
the facial nerve and its relation to the tumor capsule [5, 6]. When a watchful wait-
ing is decided, MRI volumetric studies have an excellent accuracy to follow tumor
growth [7]. 3D T2 CISS or post-contrast 3D T1 MPRAGE MRI (evidence class II)
provides best images to monitor tumor growth [8, 9].
After surgical resection, a thin not-nodular enhancement is often visualized in
surgical resection field. It can persist for years but usually reduces over the time
[11]. Fat grafts, fibrin glue, and muscle grafts, used to reconstruct the IAC, can
generate a nodular enhancement which usually appear within the first 3 days after
surgery. New nodular enhancement appearing in the postoperative follow-up highly
suggests tumor recurrence [12].
Figure 1.
Large vestibular schwannoma. (A) T2-weighted image showing a solid (red arrow) and cystic tumor (black
arrow). (B) Post-contrast-weighted image exhibiting intense contrast enhancement in the solid portion
(red arrow) and heterogeneous pattern in the cystic component (black arrow).
Figure 2.
T2-weighted image with a small vestibular schwannoma (green arrow) with hyperintensity in the dorsal pons
(blue arrow).
3
Primary Intracranial Tumors
Figure 3.
(A) Expansion of IAC caused by vestibular schwannoma (green arrow). (B) Normal width of IAC in a
patient with CPA meningioma and IAC involvement (white arrow). Dural enhancement (blue arrow)
strongly suggests its diagnosis.
Meningiomas are the most frequent differential diagnosis between non-schwan-
nomatous lesions that arise or protrude into the IAC. Meningiomas usually present a
dural enhancement and display calcification, and hyperostosis in the adjacent bone
is usually seen (Figure 3).
7. Treatment
Vestibular schwannoma is a benign intracranial nerve sheath tumor, usually
sporadic but that may be bilateral in the context of neurofibromatosis type 2. Wait
and scan strategy, microsurgical resection, and radiotherapy/radiosurgery are the
options. Presenting symptoms, hearing status, growth rate, size and characteristic
of tumor, and surgeon preference will interfere in these treatment decisions [13].
8. Microsurgical resection
The goals of the treatment are radical resection with zero mortality and com-
plete anatomical and functional preservation of the involved cranial nerves. Some
authors propose partial resection followed, or not, by radiotherapy/radiosurgery to
preserve cranial nerve function [14]. However, the only treatment that offers cure is
radical microsurgical removal.
VS might be approached through translabyrinthine, retrosigmoid, or middle
fossa craniotomy. The main advantage of the translabyrinthine approach is to
minimize brain retraction. The difficulty to resect larger tumor damage to hearing
structures is the limitation of this surgical approach. Fat grafts are needed to close
the dura and avoid CSF fistula. In small tumors, best suited to tranlabyrinthine
approach, commonly serviceable hearing is present, which ultimately turns this
surgical option unfeasible.
The middle fossa approach (MFA) is a lateral access to the IAC and was popular-
ized by House in 1961. The exposition of the IAC through its superior wall makes it
a good option for small lateral tumors restricted to the internal auditory canal and
brings the lowest risk to the labyrinth structures.
The retrosigmoid (RS) approach is the most used access by the majority of
neurosurgeons offering an excellent exposition of all anatomical structures of
the posterior fossa. The IAC is opened through the RS, and injury to the inner ear
structures (labyrinth) and jugular bulb are avoidable complications [15]. This is
the approach of choice to VS, regardless of its size, in our department. The dorsal
4
Vestibular Schwannomas: Diagnosis and Surgical Treatment
DOI: http://dx.doi.org/10.5772/intechopen.81352
decubitus is preferred by the authors due to the lower risk of air embolism and to
provide a more comfortable work position for the surgeon.
A 4 cm diameter craniotomy is cut laterally bordering the sigmoid sinus to avoid
cerebellar retraction. In most cases, in the dorsal position, a cerebellar retractor is
only used to protect the cerebellum during the drilling of internal auditory canal.
After the dura opening, CSF is released from the cerebellomedullary cisterna to
relax the pressure in the posterior fossa. The inspection of the bridging veins over
the tentorial surface of the cerebellum is highly recommended as it can be a poten-
tial source of bleeding.
The IAC is drilled as the first step in most cases. It reduces the pressure over
the cochlear and the facial nerves. Piecemeal resection with ultrasound aspira-
tor is useful to debulk the cisternal component and reduces the traction over the
tumor capsule. The facial nerve is usually very attached to the tumor capsule at
its entrance in the IAC, and careful microsurgical dissection should be performed
under continuous electrophysiologic monitoring. Preservation of the cochlear
nerve is also attempted under continuous BERA monitoring. If an alteration of
the waves is observed, the surgical field is irrigated with papaverine solution
awaiting until recovery is recorded. Cystic and larger tumors usually present more
difficulty to dissect the facial nerve from tumor capsule. Hearing preservation is
a challenge in tumors larger than 3 cm in diameter. In NF2 patients with bilateral
tumor, preservation of hearing should be attempted even if residual tumor must
be left. Brain stem decompression with hearing preservation is the goal of treat-
ment in these cases.
Reconstruction of the IAC is extremely important to avoid CSF fistula and infec-
tion. To identify the mastoid air cells inside the IAC, the use of the endoscope can be
opportune, and small pieces of muscle or fat graft should be used to seal these cells.
9. Residual tumor
In our series of 541 VS surgically resected between 1987 and 2016, 31 patients
had residual/recurrent tumors. Twenty-seven patients had been operated elsewhere.
From the 4 patients in our own casuistry, two cases were recurrences and two were
residual lesions. One of the residual cases was a patient with neurofibromatosis type
2 and showing large bilateral VS who underwent radical resection in one side and
subtotal removal of the contralateral tumor for hearing preservation. The other case
was a 75-year-old patient with large cystic VS who underwent stereotactic aspira-
tion of the cyst to alleviate mass effect, since surgical resection was not advised for
medical reasons.
The causes for subtotal removal, as reported by the patient, were extensive
intraoperative bleeding, adherence to the brain stem or facial nerve, and intraop-
erative cerebellar edema. All patients were reoperated at our institution through
the retrosigmoid/transmeatal approach. The surgical procedure proved to be
significantly more difficult than in non-operated cases. Fibrosis from previous
procedure(s) altered the anatomical location of the transverse and sigmoid sinus, as
well as the dissection and opening of the dura mater. Intracranially, the arachnoid
plane usually found between the tumor and the brain stem and cranial nerves was
lost; thus, dissection of the tumor required more manipulation of those structures.
This was especially significant in irradiated patients. We observed that in cases in
which the IAC had not been previously opened, identification and dissection of the
facial nerve and subsequent dissection were less difficult. Postoperative anatomical
preservation of the facial nerve was possible in 13 (76%) of 17 patients with preop-
erative facial nerve function. There was no permanent morbidity or mortality. All
cases were histologically confirmed as WHO grade I schwannomas [16].
5
Primary Intracranial Tumors
10. Intralabyrinthine tumors
Intralabyrinthine VS are by definition tumors arising at the terminal end of
the eighth cranial nerve within the vestibule, cochlea, or semicircular canal [17].
According to its location, intralabyrinthine schwannomas may be anatomically
divided in six major types: intravestibular, vestibulocochlear, modiolar, transotic,
intracochlear, and transmacular schwannomas (Figures 4–7).
Figure 4.
(A) Intravestibular schwannoma. (B) Vestibulocochlear schwannoma. Intravestibular schwannomas are
located in the labyrinth. Vestibulocochlear schwannomas grow in the labyrinth and cochlea.
Figure 5.
(A) Modiolar schwannoma. (B) Transotic schwannoma.
Figure 6.
(A) Intracochlear schwannoma. (B) Transmacular schwannoma.
6
Vestibular Schwannomas: Diagnosis and Surgical Treatment
DOI: http://dx.doi.org/10.5772/intechopen.81352
Figure 7.
Post-contrast T1-weighted image demonstrating a transmacular schwannoma (white arrow).
Modiolar schwannomas arise at the cochlea and extend in the modiolus and the
IAC. Transotic schwannomas grow from the labyrinth into the IAC and middle ear.
Intracochlear schwannomas are located in the cochlea. Transmacular schwan-
nomas arise in the vestibule and extend into the internal auditory canal through the
macula cribrosa.
These tumors have been frequently observed on MRI examinations, but their
management was rarely reported in the literature [18].
Labyrinthitis and otitis may also cause gadolinium enhancement of the vestibu-
lar nerves and mimic intralabyrinthine tumors. However, in these pathologies the
enhancement is less sharp, and the cochlea, as well as the entire vestibular system,
may exhibit contrast enhancement [19].
Clinical observation is recommended in patients already deaf and if the ves-
tibular symptoms are slight and treatable. Microsurgical removal is curative, but
hearing preservation is very challenging since these tumors often affect the cochlea
and the semicircular canals. The retrosigmoid-transmeatal endoscopic-assisted
approach is very useful and provides an excellent view of the lateral portion of
IAC. A wide and deep opening of the IAC (about 1 cm in length) is required to
resect those lesions. Symptoms of intractable vertigo usually disappear after micro-
surgical removal of the lesion [20, 21].
Author details
Gustavo Jung* and Ricardo Ramina
Skull Base Division, Department of Neurosurgery, Neurological Institute of
Curitiba, Curitiba, Brazil
© 2019 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms
of the Creative Commons Attribution License (http://creativecommons.org/licenses/
by/3.0), which permits unrestricted use, distribution, and reproduction in any medium,
provided the original work is properly cited.
7
Primary Intracranial Tumors
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