Otolaryngol Clin N Am

40 (2007) 479–519

Lesions of the Petrous Apex: Diagnosis

and Management
Brandon Isaacson, MD*, J. Walter Kutz, MD,
Peter S. Roland, MD
Department of Otolaryngology–Head and Neck Surgery,
The University of Texas–Southwestern Medical Center,
5323 Harry Hines Blvd., Dallas, TX 75390-9035, USA

Anatomy
The petrous apex is a pyramid-shaped structure that is the most medial
aspect of the temporal bone. The base of the pyramid is the otic capsule,
semi-canal of the tensor tympani and the petrous carotid artery. The superior
surfacedor meatal planedextends from the arcuate eminence to the pre-
cavernous carotid artery and Meckel’s cave. The posterior surface faces the
cerebellopontine angle and begins laterally at the common crus/vestibular
aqueduct and ends medially at Dorello’s canal and petroclinoid ligament.
The jugular fossa and inferior petrosal sinus are located at the inferior aspect
of the petrous apex. The internal carotid artery enters the petrous apex along
the inferior surface via the carotid canal. The internal auditory canal begins at
the porus acousticus on the posterior face of the petrous apex and ends at the
fundus. When viewed from above, the petrous apex may be divided into
anterior and posterior segments by drawing a parallel line through the in-
ternal auditory canal. The cochlear aqueduct enters the petrous apex along
its inferior surface just medial to the jugular fossa. The petrous apex is
primarily composed of bone marrow or dense bone, with only 9% to 30%
of subjects showing some pneumatization. Pneumatization of the petrous
apex is variable and often differs among sides in the same subject [1].

Presentation
Petrous apex lesions can present with various symptoms and signs, de-
pending on the size, location, and nature of the pathologic process. Muckle

* Corresponding author.
E-mail address: brandon.isaacson@utsouthwestern.edu (B. Isaacson).

0030-6665/07/$ - see front matter Ó 2007 Elsevier Inc. All rights reserved.
doi:10.1016/j.otc.2007.03.003 oto.theclinics.com
480 ISAACSON et al

and colleagues [2] reported hearing loss as the most common symptom
followed by vestibular dysfunction, headache, tinnitus, facial spasm, diplo-
pia, facial paralysis, and otorrhea. Symptoms often present months or years
before diagnosis, and incidental discovery is not uncommon.

Imaging
CT
CT of the temporal bone enables a detailed evaluation of the osseous
architecture of the petrous apex. Lesions within or involving the petrous
apex may be further characterized based with CT by evaluating for different
patterns of bone erosion or invasion. A distinct advantage of CT is the abil-
ity to determine the proximity of a petrous apex lesiondwith submillimeter
accuracydto critical structures, such as the internal auditory canal, otic
capsule, and carotid artery. CT is often complementary to MRI in evaluat-
ing petrous apex pathology [4,5].

MRI
Before the introduction of MRI, evaluation of petrous apex pathology
was limited to evaluating changes in the temporal bone with plain films,
tomography, and early CT. In most cases, MRI with the history and phys-
ical examination allows the clinician to make a diagnosis without obtaining
tissue for pathologic examination. Most of the reported petrous apex lesions
have unique MRI characteristics that allow for definitive diagnosis (Table 1)
[4,5].

Angiography: conventional, magnetic resonance angiography,

magnetic resonance venography, computed tomography
angiography/venography
Petrous apex lesions may compress, invade, or abut the carotid artery,
jugular bulb, and inferior and superior petrosal sinuses. Angiographic studies
provide detailed information about these vascular structures and may help
the surgeon to select an appropriate surgical approach. Conventional angiog-
raphy via femoral artery or vein catheterization provides an opportunity to
assess a lesion’s vascularity and its relationship to other vascular structures
[6]. This technique also provides an opportunity to devascularize lesions
and extensive feeding vessels to decrease intraoperative blood loss [7–9].
The primary disadvantage of conventional angiography is its risk of hema-
toma, stroke, pseudoaneurysm formation, and risk of cranial nerve palsies
[6]. The introduction of magnetic resonance angiography and venography
has provided a low-risk technique of assessing the skull base vasculature.
Although the resolution of these studies is good, the primary disadvantage
of magnetic resonance angiography/venography is the inability to perform
 LESIONS OF THE PETROUS APEX 481

interventional measures [10]. CT angiography and venography allow for an-

other detailed means of assessing petrous apex lesions and vessels. This tech-
nique uses ultra-thin slices with timed administration of contrast and
provides incredible detailed information. An additional advantage of tempo-
ral bone CT angiography compared with its counterparts is the ability to
define the vascular structures and their relationship with the lesion and other
structures in or adjacent to the petrous apex [11].

Management
Surgical approaches
Nonhearing preservation
Infracochlear. Traditionally, the most common approach to cystic lesions of
the petrous apex in patients with serviceable hearing was the infralabyrin-
thine approach. This approach is limited in patients with a high jugular
bulb, however. The alternative in these patients is the infracochlear ap-
proach to the petrous apex [12,13]. There are several advantages to the in-
fracochlear approach, including dependent drainage in a well-aerated
middle space adjacent to the eustachian tube, adequate access to the petrous
apex despite a high jugular bulb, simple revision if required, and preserva-
tion of the normal middle ear mechanisms [14].
Continuous intraoperative facial nerve monitoring is recommended. A
standard postauricular incision is made. An incision is made in the mastoid
periosteum along the linea temporalis and is extended inferiorly to the
mastoid tip. The periosteum is elevated anteriorly until the osseous external
auditory canal is exposed. The external auditory canal is transected through
the cartilaginous portion. Canal incisions are made at 2 and 10 o’clock, and
a tympanomeatal flap is elevated. The tympanic membrane remains atta-
ched to the umbo and superior canal wall. Removing an adequate amount
of anterior and inferior tympanic bone within the external auditory canal is
a critical step to expose the hypotympanum. Once exposure is adequate, the
chorda tympani nerve can be traced posteriorly and inferiorly to identify the
facial nerve. Next the jugular bulb and petrous carotid artery are identified.
Once these structures have been identified, medial dissection continues with
diamond burs and curettes. The boundaries of the dissection include the
carotid artery anteriorly, the jugular bulb inferiorly, the facial nerve poste-
riorly, and the basal turn of the cochlea superiorly. Jacobson’s nerve can be
followed inferiorly and leads to the bone between the carotid artery and jug-
ular bulb. Confining the exposure inferior to the round window prevents in-
advertent injury to the basal turn of the cochlea during dissection. The cyst
is entered and the loose contents are removed using suction and irrigation. A
piece of Silastic tubing is placed in the surgical defect. The bony defect of the
inferior bony annulus is repaired with bone pate. The tympanomeatal flap is
 482
Table 1
Radiology
MRI
T1 fat saturated
Lesion T1 pre T1 post gadolinium T2 CT Other
Cholesterol Hyperintense No enhancement Hyperintense Smooth erosion
granuloma
Petrous apicitis Hypointense Rim enhancement Rim enhancement Hyperintense Destroyed septae
CSF cyst/ Hypointense No enhancement No enhancement Hyperintense Smooth erosion, May connect to
cephalocele FLAIR, Meckel’s cave
hypointense,
diffusion-

ISAACSON
weighted,
hypointense
Epidermoid Hypointense No enhancement Hyperintense FLAIR -

et al
hyperintesne,
diffusion-weighted
imaging,
hyperintense
Effusion Isointense or Enhancement Hyperintense Intact septation
hypointense
Mucocele Isointense No enhancement No enhancement Hyperintense Destroyed septae
Asymmetric Hyperintense Some enhancement Hypointense, no Hypointense Marrow on lesion
pneumatization enhancement side, air cells on
contralateral side
Carotid New thrombus, Hyperintense Smooth expansion MRI, central
aneurysm hypointense; of carotid canal, flow void,
older thrombus, heterogeneous onion skin
hyperintense contrast appearance
enhancement
Chordoma Hypointense/ Enhancement less Enhancement Hyperintense Lobulated, bone Centrally located
isointense intense than destruction with in clivus with
chondrosarcoma residual bone lateral spread
fragments to petrous apex
Chondrosarcoma Hypointense/ Enhance Enhancement Hyperintense, Infiltrative, Centered in petrous
isointense, heterogeneous remnants of apex in region
homogeneous eroded bone of foramen
lacerum,
calcified areas
may show as
signal voids

LESIONS OF THE PETROUS APEX

Metastasis Depends on Enhancement Enhancement Depends Bone erosion Depends on
primary on primary primary tumor
Paraganglioma Isointense Enhancement Enhancement Hyperintense Opacified and Flow voids, blush
destroyed on angio, salt
air cells and pepper
Meningioma Isointense/ Enhancement Enhancement Isointense/ Hyperostosis, Dural tails, sessile,
hyperintense hyperintense isointense or eccentric
hyperdense,
calcification
Schwannoma Isointense Enhancement Enhancement Hyperintense or May show dilation Centered in
hypointense of internal internal
auditory canal auditory canal
Data from References [3,4,69,75,84,89].

483
484 ISAACSON et al

returned to its normal position. The external auditory canal is packed with
Gelfoam, and the postauricular incision is closed [12,13].
Brackmann and Toh [14] reported a recurrence rate of 16.7% in choles-
terol granulomas treated with the infracochlear approach. Fibrous occlusion
of the drainage tract was responsible for all failures. The use of Silastic tub-
ing decreases the rate of recurrence in infralabyrinthine and infracochlear
approaches [14,15]. Because recurrence can occur years after the initial sur-
gery, long-term radiographic follow-up is necessary. Preoperative symptoms
of hearing loss, vertigo, and cranial nerve deficits often improve or resolve
after treatment [2]. Complications are rare; however, inadvertent injury to
the carotid artery is a potentially devastating injury. The infracochlear ap-
proach to the petrous apex should be performed by a surgeon with an inti-
mate knowledge of the anatomy.

Subtemporal approaches
The subtemporal approaches provide varying degrees of exposure to the
petrous apex, clivus, ventral brainstem, and anterior cerebellopontine angle
[3]. The standard and extended middle fossa approaches provide excellent
exposure of the internal auditory canal from the porus acousticus to the
fundus [16]. The extended approach requires more extensive bone removal
anterior and posterior to the internal auditory canal. This additional bone
removal, which exenterates a significant portion of the anterior and poste-
rior petrous apex, provides additional exposure to the cerebellopontine
angle. The anterior limit of dissection is the lateral wall of Meckel’s cave,
whereas the posterior limit is the blue-lined superior semicircular canal
[17]. The Kawase approach is a further elaboration of the extended middle
fossa approach that requires downfracturing of the zygoma [18,19]. This
approach provides access to the ventral pons by removing the anterior
petrous apex down to the level of the horizontal petrous carotid artery.
The primary disadvantage of the subtemporal approaches is an increased
risk of facial nerve injury because of its anterior superior location in the
internal auditory canal; however, several studies have demonstrated no
long-term difference [20,21]. Temporal lobe injury from prolonged retrac-
tion or injury to the vein of Labbé is also an important consideration
when using these approaches [22]. The subtemporal approaches do not
permit visualization of the inferior cerebellopontine angle. The subtemporal
approaches may be combined with the pre- and postsigmoid approaches to
address lesions that extend into multiple intracranial compartments [23].
Various skin incisions may be used for the subtemporal approaches. Once
the skin and subcutaneous tissues are elevated, the temporalis muscle is cut
just proximal to its origin from the temporal line to provide a site of attach-
ment during closure. The temporalis muscle is elevated anteriorly and
inferiorly and is secured with retractors or hooks. Identification of the
zygoma and the osseous external auditory canal provides landmarks for
 LESIONS OF THE PETROUS APEX 485

the craniotomy bone flap. A 4 cm  5 cm craniotomy bone flap that is

centered 1.5 cm anteriorly to the root of zygoma is elevated. Occasionally,
additional squamous temporal bone cortex must be removed so that the
craniotomy is flush with the middle fossa floor. Once the craniotomy
bone flap has been elevated, the dura is elevated off the floor of the middle
fossa from posterior to anterior to prevent avulsion of the greater superficial
petrosal nerve. The dura is elevated from the petrous ridge posteriorly to the
dura of Meckel’s cave anteriorly. Cauterization or clipping of the middle
meningeal is often necessary. The greater superficial petrosal nerve, arcuate
eminence, petrous ridge, middle meningeal artery, and foramen ovale all
serve as landmarks to guide the surgeon. House [24], Fisch [25], and Jackler
[26] each described three separate techniques to identify the internal audi-
tory canal. A lateral to medial technique, described by Fisch, begins by
blue-lining the superior semicircular canal and then identifying the internal
auditory canal at a 60 degree angle. William House [27] identified the fun-
dus of the internal auditory canal by tracing the greater superficial petrosal
nerve to the geniculate ganglion. Jackler and Gladstone [26] described a me-
dial to lateral technique that begins with drilling at the petrous ridge and
identifying the internal auditory canal at the porous acousticus and follow-
ing it laterally to the fundus. Once tumor removal is complete, air cells are
waxed and muscle or fat is used to obliterate the defect.

Infralabyrinthine approach
During the preantibiotic era, the infralabyrinthine approach was most
commonly used to drain petrous apex abscesses [28,29]. With the advent
of antibiotics, petrous apex infections are rare; however, the same
approaches can be used to drain cystic lesions of the petrous apex. The
advantages of the infralabyrinthine approach to the petrous apex include
an anatomy familiar to most otologists, direct route to most cysts of the
petrous apex, and avoidance of entering the middle ear. A high-riding jug-
ular bulb may necessitate the use of the infracochlear approach as opposed
to the infralabyrinthine approach.
The surgical procedure begins with a simple mastoidectomy. The middle
fossa plate, sigmoid sinus, and facial nerve are identified. The posterior
portion of the lateral semicircular canal and the posterior semicircular canal
are skeletonized, with care taken not to create a fistula in the semicircular
canals. Next, the sigmoid sinus is followed inferiorly until the jugular bulb
is identified. The boundaries of the approach include the posterior semicir-
cular canal, facial nerve, jugular bulb, and the posterior fossa dura. Medial
dissection continues with diamond burs and curettes until the cyst is entered.
The loose contents of the cyst are removed with suction and irrigation.
A piece of Silastic tubing is placed through the surgical tract to prevent
restenosis of the surgical drainage tract.
Complications reported as a result of the infralabyrinthine approach
include sensorineural hearing loss and facial nerve paresis [15,30].
486 ISAACSON et al

Preoperative deficits, including hearing loss, imbalance, and cranial nerve

neuropathies, may resolve after surgery, depending on the lesion. Long-
term radiographic follow-up is necessary because recurrences can occur
years after drainage. Importantly, the lack of aeration alone postoperatively
should not be considered a failure; however, increased size of the lesion or
return of symptoms is a sign of recurrence and may require re-exploration.

Subarcuate and sinodural angle approaches

The subarcuate and sinodural angle approaches are mainly used in
suppurative processes of the petrous apex. These approaches are rarely
used because of the limited access provided. A standard mastoidectomy is
used in both approaches. In the subarcuate approach all three semicircular
canals are skeletonized. A small diamond bur is used to open the area infe-
rior to the superior semicircular canal and superior to the vestibule that
follows the path of the subarcuate artery. In cases of suppuration of the pos-
terior petrous apex, fistulous tracts may be found in this pathway and can be
followed to the petrous apex. The sinodural angle is another avenue that can
be followed to the posterior petrous apex, where air cells of the sinodural
angle are followed to the posterior petrous apex [1].

Supracochlear approach
The supracochlear approach allows for drainage or biopsy of lesions
located on the anterior superior aspect of the petrous apex. This approach
begins with a standard mastoidectomy followed by an extended antrotomy
that exposes the zygomatic root cells. In some cases the incus body and
occasionally the malleus head must be removed. Drilling with a 2- to 3-mm
diamond bur begins anterior to the superior semicircular canal, between the
tegmen tympani and the tympanic facial nerve. In the series by Telischi and
colleagues [31] of 20 temporal bones, removal of the incus was required in
many of the specimens to access the supracochlear air cell tract. Telischi
found that the mean dimensions of the supracochlear triangle are 7.0 
5.3  4.8 mm. The advantage of this approach, which provides access to
the anterior superior petrous apex, is preservation of the external auditory
canal and labyrinth. Unfavorable aspects of this approach include a signifi-
cant risk to the labyrinthine facial nerve and the potential need for removal
of the malleus head and incus. Intraoperative facial nerve monitoring is rec-
ommended with this approach because of the proximity of the tympanic and
labyrinthine facial nerve.

Retrosigmoid approach
The retrosigmoid approach gives excellent access for tumors that arise in
the cerebellopontine angle and involve the posterior cranial fossa. This
approach is limited when tumors extend into the middle fossa and involve
Meckel’s cave, however. In 1995, Cheung and colleagues [32] described an
approach combining a retrosigmoid craniotomy with opening of Meckel’s
 LESIONS OF THE PETROUS APEX 487

cave by removing bone anterior to the internal auditory canal. Samii and
colleagues [33] later reported a series of 12 petroclival meningiomas ap-
proached through a similar approach called the retrosigmoid intradural
suprameatal approach. The suprameatal extension of the retrosigmoid
approach allows improved access to the petrous bone anterior to the inter-
nal auditory canal and allows better visualization of the prepontine cistern,
clivus, oculomotor nerve, and the posterior cerebral arteries.
Seoane and Rhoton [34] studied the anatomic relationships concerning the
suprameatal approach to Meckel’s cave. The suprameatal tubercle is a bony
prominence superior to the internal auditory canal and blocks access to
Meckel’s cave, the prepontine cistern, and clivus from a posterior approach.
Removal of this bone allows approximately 1 cm additional exposure of the
posterior root of the trigeminal nerve and an additional 1.3 cm of anterior ex-
tension to the retrosigmoid approach. This approach is most ideal for petro-
clival meningiomas or posterior fossa trigeminal schwannomas with a limited
amount of middle fossa involvement. Disadvantages include drilling in a tight
space with adjacent critical neurovascular structures, poor access to the
cavernous sinus, and further working distance to the petrous apex when
compared with other approaches [35].

Combined approaches
The combined approaches provide exposure for lesions that extend in the
middle and posterior fossa. These approaches typically use a transtemporal
approach (retrolabyrinthine, translabyrinthine, and transcochlear) in addi-
tion to a middle fossa craniotomy. The technical details of this approach
already have been described except for several critical steps. The superior pe-
trosal sinus must be divided after cauterization or placement of clips. After the
transtemporal and middle fossa craniotomies are completed, the tentorium is
divided being careful not to injure the trochlear nerve, Dandy vein, superior
cerebellar artery, and vein of Labbé [23]. Prolonged retraction of the temporal
lobe dura poses a risk of injury or thrombosis of the vein of Labbé. Intermit-
tent release of the retractor or division of the sigmoid sinus may prevent injury
to the vein of Labbé [22]. The primary advantages of these combined ap-
proaches are decreased brain retraction, which eliminates the need for a staged
approach, improved exposure, which allows for a more complete excision,
and the possibility of hearing preservation (retrolabyrinthine). The primary
disadvantage to the combined craniotomy is sacrifice of hearing if either the
translabyrinthine or the transcochlear approach is used [3].

Infratemporal approach
The preauricular infratemporal approach and its modifications provide
generous access to the petrous apex and clivus. Various incisions have
been used and described for the infratemporal approach, including a Y
incision, a large postauricular C-shaped incision, and a pretragal incision
488 ISAACSON et al

[36–38]. The skin flaps are elevated over the temporalis fascia until the tem-
poral fat pad is identified. An incision is made in the superficial layer of the
deep temporal fascia and dissection proceeds just lateral to the temporal fat
down to the level of the zygoma. The periosteum of the zygoma is transected
along its superior-medial surface and elevated to preserve the frontal branch
of the facial nerve. Identifying the main trunk of the extratemporal facial
nerve at the stylomastoid foramen allows for more generous inferior expo-
sure of the infratemporal fossa. The zygoma is exposed from the root to the
orbital rim. Miniplate drill holes are placed before performing the zygomatic
osteotomy for later reconstruction. The zygoma is then transposed inferiorly
with the attached masseter muscle.
The temporalis muscle is cut along its origin, taking care to preserve
a cuff of muscle for later reattachment if necessary. The temporalis is care-
fully reflected inferiorly toward its insertion on the coronoid process so as
not to interrupt the vascular supply. The mandibular condyle is displaced
out of the glenoid fossa with a retractor or can be resected to provide
more generous exposure. A high-speed otologic drill is then used to remove
the glenoid fossa and expose the petrous carotid artery. The eustachian tube
serves as a useful landmark for the carotid artery and must be obliterated.
Division and cauterization of the mandibular division of the trigeminal
nerve and middle meningeal artery permits exposure of the precavernous
petrous carotid artery and the anterior clivus. Once the petrous carotid
artery has been skeletonized, generous exposure of the anterior petrous
apex and clivus is afforded. Inferior mobilization of the carotid artery pro-
vides improved visualization of the petrous apex and clivus but is associated
with an additional risk of inadvertent injury or vasospasm. If a large defect
is present, the temporalis muscle may be rotated into the defect. Lesions that
extend into the middle ear may require additional exposure [36,37].

Transnasal endoscopic approach

Endoscopic-assisted pituitary surgery has become the favored technique
in many centers, with resulting decreased hospital stay, decreased recovery
times, and better visualization for tumor excision [39,40]. More recently,
several centers have pushed the envelope by performing transnasal endo-
scopic approaches to posterior, middle, and anterior cranial base lesions.
Kassam and Jho [41,42] recently reported on a modular approach to access-
ing posterior fossa lesions, including the petrous apex and jugular foramen,
via a transnasal endoscopic approach. These techniques require intimate
knowledge of the complex skull base anatomy. Petrous apex cholesterol
granulomas and epidermoids occasionally can be drained into the sphenoid
sinus if the carotid artery is not in the drainage pathway [43,44]. Kassam
and colleagues [41] also described an endoscopic technique that mobilizes
the petrous carotid artery to access the petrous apex. These endoscopic skull
base techniques hold the same potential promise that endoscopic pituitary
approaches provide but require advanced knowledge and training.
 LESIONS OF THE PETROUS APEX 489

Translabyrinthine approach
The translabyrinthine approach was reintroduced by Drs. Hitselberger
and House [45], and with the introduction of the operating microscope, ini-
tiated the era of modern skull base surgery. This approach is often used in
the surgical management of petrous apex lesions when hearing is poor or the
tumor is large [3].
A postauricular incision is made to expose the mastoid periosteum. The
mastoid periosteum is divided carefully so as not to overlap the cutaneous
incision from the periosteal incision. Once the periosteum is dissected
from the cortex, an extensive mastoidectomy is performed carefully so as
to identify the mastoid tegmen, posterior fossa plate, sigmoid sinus, and
external auditory canal. The antrum is opened and the lateral semicircular
canal is identified along with the previously mentioned structures. A labyrin-
thectomy is performed, leaving the ampullated end of the superior semicir-
cular canal as a landmark. The internal auditory canal is then identified, and
a thin layer of bone is left over its surface. Removing bone inferior and
superior to the internal auditory canal often exposes air cells or pathology
involving the anterior petrous apex. This approach gives wide exposure to
the posterior petrous apex and adequate exposure to the anterior petrous
apex. The primary disadvantages of this approach are the loss of any resid-
ual hearing, worsening balance function, and vertigo that occurs immedi-
ately after the surgery. There is also a significant risk of a cerebrospinal
fluid (CSF) leak if the dura is violated. An abdominal fat graft is often
harvested and later used to obliterate the cavity once the resection has
been completed [3].

Transotic and transcochlear approaches

The transotic and transcochlear approaches, described by Fisch and
House [46,47], respectively, were introduced to provide more anterior cere-
bellopontine angle exposure. These approaches and their variations allow
for substantial exposure of the prepontine cistern and its contents, including
the pons, medulla, basilar artery, vertebrobasilar junction, cranial nerves V
through XI, and the clivus [48]. The transcochlear approach is an extension
of the translabyrinthine approach but includes removal of the entire otic
capsule and posterior rerouting of the facial nerve from the porous acousti-
cus to the stylomastoid foramen. The originally described transcochlear
approach preserves the middle ear and external auditory canal, whereas
the extended version requires removal of these structures with obliteration
of the eustachian tube and over closure of the external auditory meatus
[46,48]. The transotic approach was introduced to remove small cerebello-
pontine angle tumors and decrease the risk of CSF leak. This approach pre-
serves the bone overlying the sigmoid sinus and requires removal of the
external auditory canal with obliteration of the eustachian tube. The facial
nerve is left in situ, and the external auditory meatus is overclosured to pro-
vide a water-tight seal [47]. The primary disadvantages of these approaches
490 ISAACSON et al

are hearing sacrifice and the potential for permanent facial nerve weakness
in cases in which the nerve is mobilized. For complete facial nerve mobiliza-
tion, additional morbidity includes sectioning of chorda tympani and
greater superficial petrosal nerves [3].

Lesions of the petrous apex

Inflammatory/cystic
Cholesterol granuloma
Cholesterol granuloma is the most common abnormality found within the
petrous apex. It is a cystic lesion that was first recognized as a distinct clinical
entity in the early 1980s [49]. Together with mucocele and cholesteatoma,
cholesterol granuloma accounts for more than 90% of the lesions of the
petrous apex. Cholesterol granuloma is an intraosseous cyst filled with
dark, viscous, chocolate brown fluid and granulation tissue. Birefringent
cholesterol crystals can be seen on microscopy. The cyst is often contained
within a fairly thick fibrous capsule but has no true epithelial lining. (Conse-
quently, complete surgical excision is not required.) Lo and colleagues [50]
estimated that cholesterol granulomas of petrous apex occur in approxi-
mately 0.6 per 1 million individuals. Cholesterol granulomas have been
recognized in the middle ear and mastoid for many decades and have
traditionally been described as ‘‘blue dome eardrum’’ or ‘‘blue dome cyst.’’
They are seen occasionally in individuals with chronic middle ear or mastoid
disease. They can occur anywhere within the pneumatized temporal bone
[51,52].
Traditional pathophysiology proposes that the cysts form in response to
anaerobic catabolism of blood and blood products. Specifically, these cysts
are believed to arise as an inflammatory reaction to cholesterol crystals
released from blood breakdown products. Several histopathologic studies
have verified that blood breakdown products can incite an inflammatory
response [53–57].
The traditionally accepted hypothesis is that blood enters the mucosa-
lined pneumatic air cell tracts of the temporal bone as a result of negative
pressure, which arises either as a consequence of eustachian tube dysfunc-
tion or because mucosal edema has obstructed the passage of air through
the air cell system. Negative pressure within these mucosa-lined air cells
then results in transudative hemorrhage. Anaerobic breakdown of the tran-
sudated red blood cells frees cholesterol crystals. A foreign body giant cell
reaction and cyst formation then develops in response to irritation produced
by these cholesterol crystals.
Jackler and Cho [58] raised cogent objections to this conventional hy-
pothesis and noted the following points:
1. Transudative hemorrhage rarely follows as a result of long-term eusta-
chian tube dysfunction. Negative pressure generated as a consequence
 LESIONS OF THE PETROUS APEX 491

of obstruction of air flow through the mastoid air cell tract system does
not seem to be sufficient to produce hemorrhage and bleeding, even
when it is sufficient (over time) to produce significant tympanic membrane
retraction.
2. Cholesterol granuloma occurs only in well-pneumatized temporal
bones. Extensive pneumatization is usually associated with a patent eu-
stachian tube and unrestricted movement of air through the air cells
tracts. Extensively pneumatized temporal bones are only uncommonly
associated with chronic middle ear or mastoid disease.
3. Once air cells have been filled with effusion (bloody or otherwise), the
pressure should be equalized. The process should then stop. No addi-
tional bleeding should occur. Jackler and Cho [55] stated several argu-
ments that a single episode of bleeding would be insufficient to produce
an expanding cholesterol granuloma.

Jackler and Cho [58] proposed an alternative mechanism. They suggested

that cholesterol granuloma results as a consequence of dehiscence of the
bony partition between the petrous apex air cell system and bone marrow
in the anterior temporal bone or clivus. Exposed marrow is a potential
source for repeated hemorrhage. Their argument is supported by a radio-
logic investigation that demonstrates dehiscence between bone marrow in
the petrous air cell system contralateral to a cholesterol granuloma in 6 of
13 patients. No control patients with highly pneumatized petrous apicesd
but without cholesterol granuloma formationdhad such dehiscences.
Extensive pneumatization of the petrous apex is variously reported as
occurring in somewhere between 10% and 30% of patients; thus only a limited
segment of the population is potentially vulnerable to development of
a petrous apex cholesterol granuloma [59,60].
Brackmann and Toh [14] listed the signs and symptoms present in individ-
uals at the time they seek help for cholesterol granulomas: 65% of patients
had hearing loss or one of its associated symptoms (tinnitus, aural fullness).
More than 50% of individuals reported dizziness. Headache occurred in one
third of patients. One of five patients had either facial twitching or facial
numbness. Facial weakness and diplopia were uncommon.
Unfortunately, Brackmann and Toh [14] do not indicate the reason
for which individuals sought care or accepted surgery for the removal of
their cholesterol granulomas. Given the frequency of hearing loss in the
population, it is likely that hearing loss was present incidentally and not
as a consequence of the petrous apex abnormality in at least some
subjects.
Hughes and colleagues [49] indicated that most patients seeking inter-
vention for petrous apex cholesterol granuloma do so because of headache.
The pathognomonic headache for petrous apex cholesterol granuloma is
retro-orbital, although generalized temporoparietal headache may occur as
a consequence of irritation of the petrous apex.
492 ISAACSON et al

Care should be taken to establish the petrous apex lesion as the cause of
the headache. Headaches are common, and an effort should be made to de-
termine whether the headache is related to the petrous apex cholesterol
granuloma. A neurology consultation or migraine management is often
helpful in determining the relationship of the lesion to the symptom.
Facial twitching is much more common than facial weakness and occurs
in one fifth of patients with cholesterol granuloma of the petrous apex.
Facial numbness and paresthesia (usually in the distribution of V3) also
occurs in approximately one out of five patients and is easier to link to
the pathologic process [14]. It is often difficult to determine whether the cys-
tic petrous apex lesion is causing the patient’s symptoms. If the symptoms
are consistent with the anatomic location of the lesion, however (ie, neuro-
sensory hearing loss is present and the lesion clearly encroaches on the otic
capsule or internal auditory canal), then treatment should be initiated.
Conversely, if the patient complains of facial paresthesia but the cholesterol
granuloma is restricted to the interior/posterior petrous air cells, it is prob-
ably prudent to observe the lesion.
Diagnosis depends on imaging. Cholesterol granulomas are relatively
unique in that they show a hyperintense signal on T1 and T2 images on
MRI (Figs. 1 and 2). Most of the other lesions confused with cholesterol
granuloma (eg, cholesteatoma, fluid, mucocele, neoplasia) demonstrate
only low or medium signal intensity on T1 images. The exception is
asymmetric pneumatization of the petrous apex [14,49]. Bone marrow in
the nonpneumatized apex may suggest the presence of a lesion. Bone mar-
row can have a high signal intensity on T1- and T2-weighted images, but

Fig. 1. Cholesterol granuloma. Axial T1-weighted magnetic resonance fat-saturated image with
gadolinium demonstrates a heterogeneous, hyperintense lesion in the right petrous apex.
 LESIONS OF THE PETROUS APEX 493

Fig. 2. Cholesterol granuloma. Axial T2-weighted MRI demonstrates a heterogeneous, hyper-

intense lesion in the right petrous apex.

it is usually lower on T2-weighted images, which is sufficient to distinguish it

from a granuloma on MRI [61]. Cholesterol granulomas do not enhance
with contrast, and contrast enhancement suggests a neoplasm.
The decision to operate on cholesterol granulomas of the petrous apex is
often made only after determining that the lesion shows evidence of bone
erosion or expansion or both. Consequently, fine-cut CT of the temporal
bone is usually more useful in assessing and following these lesions than
is MRI.
The first thing one should look for on a CT is the presence of normal
septation of the air cell system in the petrous apex. If the lesion is not erod-
ing and destroying these thin delicate septae, it is unlikely that it is growing
or expanding. The presence of intact septation suggests that the lesion is not
causing symptoms and can be ‘‘left alone.’’ Lesions that show normal
septation rarely require intervention. There are some exceptions, however,
as discussed in the section on petrous effusion.
Signs of erosion and expansion include scalloping and notching of the
lesion into the clivus and posterior expansion into the labyrinth or internal
auditory canal. Large cholesterol granulomas frequently bulge medially into
the posterior fossa. They usually carry with them a rim of sclerotic bone that
is often thin but detectable on CT. Cholesterol granulomas do not generally
enhance with intravenous contrast, although some rim enhancement is
consistent with a cholesterol granuloma.
If it is decided that intervention is necessary, several surgical approaches
are available to remove lesions of the petrous apex (Box 1). If serviceable
hearing is present, every attempt should be made to conserve it; several
surgical procedures can do so. The infralabyrinthine approach has been
494 ISAACSON et al

Box 1. Surgical procedures

1. Infracochlear
2. Infralabyrinthine
3. Middle fossa
4. Transsphenoidal
5. Transtemporal, preauricular
6. Translabyrinthine
7. Transcochlear

widely used as a hearing conservation technique. It is relatively quick and

easy to perform and can provide dependent drainage to the cyst. Anatomic
studies have shown that as much as 11 mm of space may be available using
this approach, although on average approximately 5 mm is available [62,63].
If the jugular bulb is high, however, access through this approach is not
possible, which seems to be the case in approximately 40% to 50% of
patients [62].
Surgical approaches to the petrous apex. First five approaches can all
preserve hearing, the last two cannot.
The infracochlear approach has been championed by Brackmann and
Toh. An infracochlear channel into the petrous apex cyst seems to have
the highest likelihood of staying open. The approach is nondestructive
and relatively straightforward. As noted by Brackmann and Toh [14], the
infracochlear approach permits a fairly straightforward revision surgery.
The middle fossa approach permits wider exposure than the infracochlear
and infralabyrinthine approach, except for the most inferior/posterior air
cells. Cholesterol granulomas are often fairly extensively loculated, and it
can be difficult or even impossible to break up these loculations and drain
the entire cyst through the small openings afforded by the infracochlear
or infralabyrinthine approaches.
The biggest disadvantage to the middle fossa approach is the lack of de-
pendent drainage. At UT Southwestern we have used a small piece of shunt
tubing placed from the petrous apex over the internal auditory canal into
the mastoid air cell system to encourage aeration. Long-term postoperative
aeration of the involved petrous apex has been the exception rather than the
rule, however.
The endoscopic transsphenoidal approach is appealing for lesions that
expand anteriorly into the sphenoid sinus itself. In such circumstances, the
endoscopic transsphenoidal approach can be used successfully to drain
the cyst. Brackmann and Presutti [14,64], however, indicated that long-
term aeration is uncommon after endoscopic transsphenoidal procedures.
If there is significant bone between the anterior margins of the petrous
 LESIONS OF THE PETROUS APEX 495

apex cyst and the sphenoid sinus itself, it is a difficult and potentially dan-
gerous operation.
Neurosurgeons sometimes use the transtemporal, preauricular approach
so as to completely remove cholesterol granulomas of the petrous apex but
save hearing. Although such approaches seem to be successful, they seem
unnecessarily invasive compared with the infracochlear and infralabyrin-
thine approaches [65].
In individuals who have lost all hearing (usually as a result of disease),
translabyrinthine and transcochlear approaches provide generous access
to the petrous apex and a high likelihood of persistent postoperative aera-
tion. An experienced otologic surgeon can perform these procedures safely
and relatively rapidly. Complete loss of residual hearing is inherent in these
approaches. Most patients recover well from the associated loss of balance
function, but these operations should be used with caution in individuals
who have contralateral labyrinthine hypofunction.
Classical literature suggests that pneumatization is required to prevent
recurrence. The extent to which this is true is unclear. Simple opacification
of the cyst with an effusion does not necessarily indicate recurrence of the
lesion; that is, nonopacified petrous apex cholesterol granulomas may
remain asymptomatic indefinitely. The fact that the cystic area has filled
with fluid does not necessarily mean it has become expansive or erosive.
In Brackmann and Toh’s [14] series of 21 patients who received postopera-
tive imaging, only lesions were aerated. All lesions that were aerated had
undergone infracochlear approaches. Of the 15 nonaerated lesions, only 1
had increased in size and only 5 have required revision surgery.

Epidermoid/cholesteatoma
Petrous apex epidermoid or cholesteatoma is a rare entity that comprises
4% to 9% of all petrous apex lesions [66,67]. These lesions are classified as
acquired or congenital, with the former being referred to as cholesteatoma
and the latter referred to as epidermoid [68]. Cholesteatoma that involves
the petrous apex originates from disease in the middle ear or mastoid that
gains access to the petrous apex via several preformed pathways. Epithelial
cell remnants from an anomalous developing first branchial cleft or from
multipotential embryonic cells or displaced otic capsule cells are thought
to be the mechanism for epidermoid formation. Despite their different ori-
gins, cholesteatoma and epidermoid have an identical appearance on path-
ologic examination [68]. An anatomic classification system reported by
Sanna [69] includes five categories: supralabyrinthine, infralabyrinthine,
massive labyrinthine, infralabyrinthine-apical, and apical. Presenting symp-
toms include hearing loss, facial paresis/paralysis, dizziness, otorrhea,
trigeminal paresthesia, and diplopia. In Kaylie’s [70] series, cranial nerves
V, VII, and VIII were most commonly involved. Cranial nerve deficits are
thought to arise from encasement with resultant ischemia. CT often demon-
strates a low-density mass that does not enhance but demonstrates smooth
496 ISAACSON et al

bone erosion. The T1- and T2-weighted MRI characteristics of petrous apex
cholesteatomas are the same as CSF and arachnoid cysts (Figs. 3 and 4).
Unlike CSF and arachnoid cysts, fast fluid-attenuated inversion-recovery
(FLAIR) and diffusion-weighted imaging reveal a hyperintense lesion with
petrous cholesteatomas (Figs. 5 and 6) [71,72]. The presence of gadolinium
enhancement should raise the suspicion of carcinoma within the lesion. The
presence of a focus of squamous cell carcinoma despite a complete excision
portends a poor prognosis [3].
Surgical excision or exteriorization is the treatment of choice depending
on the location of the lesion and the presenting symptoms. Various surgical
approaches have been described, including the middle fossa, translaby-
rinthine, transotic, transcochlear, and infratemporal approaches. The appr-
oach selected depends on the extent and location of the disease and the
hearing status [68,70,73]. The geniculate facial nerve is the most common
site of involvement in patients who present with facial weakness from petro-
sal cholesteatomas. The nerve may have undergone extensive fibrosis, and as
a result, decompression and rerouting have little chance of improving func-
tion. Axon [68,69] recommended excision of the ischemic, fibrotic segment
with the adherent matrix and primary anastomosis to maximize chances
of recovery in patients with complete paralysis. Chemical meningitis as a re-
sult of spillage of keratin debris in the subarachnoid space is another possi-
ble complication. Yasargil and colleagues [74] and Yamakawa and
colleagues [75] demonstrated a reduction in the rate of chemical meningitis
with the administration of perioperative steroids. Recurrence is another

Fig. 3. Epidermoid. Axial T1-weighted MRI with fat saturation and gadolinium demonstrates
a hypointense signal in the clivus and right petrous apex. Notice there is no enhancement.
 LESIONS OF THE PETROUS APEX 497

Fig. 4. Epidermoid. Axial T2-weighted MRI demonstrates a hyperintense signal in the clivus
and right petrous apex.

Fig. 5. Epidermoid. Axial FLAIR MRI demonstrates an isointense signal in the clivus and
right petrous apex and does not have the same hypointense signal of CSF.
498 ISAACSON et al

Fig. 6. Epidermoid. Axial diffusion-weighted MRI demonstrates an isointense signal in the

clivus and right petrous apex and does not have the same hyperintense signal of CSF.

significant consideration when managing petrosal cholesteatomas, with the

most common location for recurrence being the petrous carotid artery.
Adherence of matrix to critical and delicate structures, such as the carotid
artery, jugular bulb, facial nerve, and dura, makes complete excision chal-
lenging even in the most experienced hands. Generous exposure with various
approaches decreases recurrence by allowing direct visualization of the dis-
ease. Visualization also may be improved with the use of rigid straight and
angled endoscopes [68]. Exteriorization of the disease may result in a large
cavity that may be difficult to maintain. Obliteration with blind-sac closure
of the external auditory canal to decrease problematic cavity maintenance
was described recently. MRI provides an excellent means to follow subjects
with obliterated cavities because different imaging techniques allow for differ-
entiation of recurrence from obliteration materials (ie, fat and muscle) [3].

Effusion
Petrous apex effusion, also known as ‘‘retained’’ or ‘‘trapped’’ fluid, is most
commonly discovered as an incidental finding on routine imaging studies. The
incidence of this lesion is 1% of all cranial MRI studies and is thought to be
one of the most common petrous apex imaging findings, according to
 LESIONS OF THE PETROUS APEX 499

Harnsberger [76]. Effusions can be found only in a pneumatized petrous apex,

which occurs in approximately one third of patients. Effusions are thought to
arise as a result of infection in the middle ear or mastoid with subsequent trap-
ping of residual fluid in the petrous apex from mucosal edema [77].
Presenting symptoms may include hearing loss, positional vertigo, head-
ache, aural pressure, pain, and facial spasm. Petrous apex effusions appear
hyperintense on T2-weighted images and hypo- or isointense on T1-weighted
images, with no enhancement with gadolinium. CT demonstrates opacifica-
tion of the petrous apex air cells with no evidence of coalescence [77].
In Arriaga’s [77] recent series of 31 patients, 4 of 18 symptomatic patients
were treated successfully with a 20-day course of antibiotics and a 14-day
prednisone taper. The 14 patients who failed medical management underwent
surgical drainage via various approaches (eg, infracochlear, retrolabyrin-
thine, middle fossa, infratemporal) depending on the location of the effusion.
Eleven of 14 patients who underwent surgical management showed impr-
ovement, resolution, or stabilization of their symptoms, with the middle fossa
approach being the most successful. At surgery, minimal fluid was seen
but thick, granular mucosa, which was culture negative in all cases, was
encountered.
Arriaga [77] concluded that asymptomatic patients should be followed
with imaging, whereas symptomatic patients should be treated initially
with oral antibiotics and steroids. If symptoms persist after medical therapy,
surgical drainage may be offered, with the location of the effusion dictating
the most appropriate operative approach.

Mucocele
Mucoceles are more commonly identified in the paranasal sinuses but
occasionally may be seen in the petrous apex. These lesions form in pneuma-
tized regions of the skull base when a mucosalized air space with mucous
secreting cells becomes obstructed [78]. Symptoms and imaging characteris-
tics are similar to that of effusions, with several exceptions. CT shows
smooth bone erosion with loss of septae and a CSF isodense signal. MRI
shows an isointense signal on T1-weighted images and a hyperintense signal
on T2-weighted images with no evidence of gadolinium enhancement. Loss
or erosion of osseous septae is what differentiates this entity from effusion or
trapped fluid [79]. Symptomatic lesions often can be treated with a drainage
procedure, with the approach being dictated by the location of the muco-
cele. Patients without symptoms may be simply observed with serial imag-
ing. Intradural resection of a mucocele may increase the risk of meningitis
from contamination of the CSF with cyst contents [3,80].

Cerebrospinal fluid cysts and cephaloceles

CSF cysts, also known as arachnoid cysts, are most commonly located in
the middle fossa and arise from splitting of the arachnoid membrane [72].
500 ISAACSON et al

CSF cysts located in the cerebellopontine angle may erode the posterior aspect
of the petrous apex [71]. These lesions are often incidentally discovered but oc-
casionally produce symptoms of hearing loss, vertigo, tinnitus, headaches,
and dysequilibrium [71]. Symptoms are thought to arise from several possible
mechanisms, including direct compression of cranial nerves, brainstem, or cer-
ebellum and obstruction of the normal flow of CSF [81,82]. Incidentally dis-
covered lesions that are asymptomatic may be followed with serial imaging
[71]. Not surprisingly, these lesions have the exact MRI characteristics as
CSF. Diffusion-weighted imaging and FLAIR sequences allow differentiation
from epidermoids [71]. There have been occasional reports of cyst wall en-
hancement, but this is an exception. In most cases, CT shows smooth or
slightly scalloped bone erosion involving the posterior petrous apex [83]. Pa-
tients whose symptoms can be attributed to the cyst because of neural or brain-
stem compression may warrant surgical intervention via shunting procedures
or retrosigmoid craniotomy with wide marsupialization [71].
CSF cephaloceles, also known as Meckel’s cave diverticulum, are thought
to arise from the protrusion of arachnoid or dura from Meckel’s cave [84].
Increased intracranial pressure may be an initiating factor that causes pro-
gressive cephalocele enlargement. These lesions are typically located in the
anterior petrous apex and may erode into the otic capsule or internal audi-
tory canal. Patients may present with cranial nerve findings, headaches, and
CSF otorhinorrhea [71]. These lesions have the same MRI characteristics as
CSF cysts except they are centered in the anterior petrous apex and are often
continuous with Meckel’s cave (Fig. 7). CT may show extensive erosion of
the petrous apex with either a smooth or scalloped border (Fig. 8) [84]. An
extradural middle fossa approach with obliteration of the cyst cavity with
fat or muscle is the recommended approach for symptomatic lesions. Serial
imaging is used for patients with asymptomatic lesions or patients who
symptoms cannot be attributed to the location of the lesion. An infraco-
chlear or infralabyrinthine approach is not recommended because these
cysts often communicate with the subarachnoid space [71].

Petrous apicitis/abscess
Petrous apicitis is a rarely encountered entity since the introduction of an-
tibiotics, with an incidence of 2 in 100,000 patients with acute otitis media [85].
These infections were often associated with high morbidity and mortality rates
in the preantibiotic era, when surgical management was the only treatment op-
tion available [86]. The pathophysiology of petrous apicitis results from
spread of infection to the petrous apex through preformed air-cell tracts in
the temporal bone [59]. The most common offending organisms are the
same as those that cause acute otitis media: Haemophilus influenzae, Strepto-
coccus pneumoniae, and B-hemolytic streptococcus species. Staphylococcus
and Pseudomonas may be identified in individuals with skull base osteomyeli-
tis or chronic otitis media [3,59,85,86].
 LESIONS OF THE PETROUS APEX 501

Fig. 7. MRI of a petrous apex cephalocele. (A) Axial T2-weighted image shows a hyperintense
lesion (arrow). (B) Axial fluid inversion recovery image (FLAIR) shows the same lesion (arrow),
which is hypointense. (C) Axial T1-weighted image without gadolinium or fat saturation shows
a hypointense lesion (arrow) abutting the petrous carotid artery. (D) Coronal T1-weighted
image without fat saturation with contrast demonstrates a hypointense lesion (arrow) centered
in the right petrous apex.

It is the exception rather than the norm that a patient present with the
complete picture of Gradenigo’s syndrome, which includes retro-orbital
pain, otorrhea, and lateral gaze palsy from abducens nerve inflammation.
Intracranial extension of the infection may result in meningitis, epidural,
subdural, or intraparenchymal abscess, and dural venous sinus thrombosis
[87].
502 ISAACSON et al

Fig. 8. Axial CT scan of a petrous apex cephalocele using a bone window algorithm. (A) An
expansile lesion (large arrow) with smooth margins is noted in the right petrous apex at the level
of the cochlear aqueduct (small arrow). (B) At the level of the internal auditory canal (small
arrow), the lesion (large arrow) demonstrates significant erosion of the otic capsule bone and
encroaches on the internal auditory canal.

CT scans often demonstrate opacification and destruction of the petrous

apex air cell system (Fig. 9). MRI may show a hypointense lesion with rim
enhancement on T1-weighted images and a hyperintense signal on T2-
weighted images (Figs. 10 and 11) [86]. Fortunately, petrous apicitis is often

Fig. 9. Petrous apicits. Axial temporal bone CT scan demonstrates destruction of the left
petrous apex air cells septae and adjacent cortex. Note the opacification of the middle ear
and mastoid air cells.
 LESIONS OF THE PETROUS APEX 503

Fig. 10. Petrous apicitis. Axial T1-weighted MRI with fat saturation and gadolinium demon-
strates an enhancing lesion centered in the left petrous apex abutting the precavernous carotid
artery.

treated successfully with intravenous antibiotics and myringotomy with

aspiration and culture of the middle ear [87]. Patients who fail to improve
or develop progressive symptoms despite medical management may require
surgical treatment. Approaches that can be used to treat petrous apicitis
include subarcuate, supralabyrinthine, infralabyrinthine, infracochlear, mid-
dle fossa, and circumferential petrousectomy in patients with intact hearing.
The translabyrinthine approach offers wide exposure of the petrous apex in
patients with no functional hearing [86].

Petrous carotid aneurysm

Petrous carotid artery aneurysm is a rare entity that is often discovered
as an incidental finding on imaging studies [87–90]. The petrous carotid
artery begins at the skull base at the carotid canal and consists of a 1-cm
vertical segment, the genu, and a 2-cm horizontal segment. The petrous
carotid artery is in close proximity to multiple structures, including the
cochlea (posterior-superior), eustachian tube (lateral), tensor tympani
(lateral), and greater and lesser superficial petrosal nerves (superior-lateral)
[91,92].
Petrous carotid aneurysms are thought to originate from weak areas in the
arterial wall at the origin of several embryonic vessels, including the vidian,
caroticotympanic, pterygoid, and stapedial arteries. Three main mechanisms
for aneurysm formation include traumatic, congenital, and mycotic. True an-
eurysms of the petrous carotid artery are most commonly fusiform and have
walls that are continuous with the normal vessel. Pseudoaneurysms lack a true
wall, may expand rapidly if they communicate with the vessel lumen, and
504 ISAACSON et al

Fig. 11. Petrous apicitis. Axial T2-weighted MRI demonstrates a hyperintense lesion centered
in the left petrous apex. Notice a similar signal intensity in the mastoid and middle ear.

originate from injuries of all layers of the arterial wall. These lesions may be
asymptomatic or can present with various symptoms, including headache,
hearing loss, dizziness, pulsatile tinnitus, and cranial nerve palsies. Aneurysm
rupture results in massive hemorrhage either from the nose or ear with no re-
ports of intracranial bleeding because of the vessels’ extradural location [91].
Imaging characteristics of petrous carotid aneurysms include smooth
bone erosion of the petrous apex that is centered in the carotid canal.
MRI often shows a central flow void if the vessel is patent and a character-
istic onionskin appearance. Once a petrous carotid aneurysm is suspected,
conventional angiography with possible balloon test occlusion is needed if
treatment becomes necessary [3].
A detailed discussion of management options for petrous carotid aneu-
rysms is beyond the scope of this article, but options include observation
with serial imaging, balloon occlusion, endovascular coil or stent placement,
and aneurysm exclusion with bypass revascularization [91].

Asymmetric pneumatization
The petrous apex contains air cells in 10% to 30% of temporal bones [1].
Asymmetric pneumatization is defined as the presence of unilateral petrous
apex air cells occurring concurrently with a marrow-filled petrous apex in
 LESIONS OF THE PETROUS APEX 505

the contralateral temporal bone in one individual. Asymmetric pneumatiza-

tion of the petrous apex occurs in 5% to 10% of individuals. Bone marrow,
depending on its fat content, can produce a hyperintense signal on T1- and
T2-weighted MRI. Although it is reasonable to assume that bone marrow in
the petrous apex should have the same signal intensity as bone marrow in
the clivus, this is not always the case. Consequently, it is possible for
a hyperintense signal to be generated on T1-weighted MRI in one petrous
apex that is meaningfully different than the image generated in either
the contralateral pneumatized petrous apex or the clivus. The nonpneuma-
tized petrous apex can be confused with a cholesterol granuloma, although
signal intensity typically is iso- or hypointense on T2-weighted images,
especially with longer echo time. This slight dimunition of signal intensity
on T2-weighted images compared with T1-weighted images can distinguish
normal bone marrow from cholesterol granulomas and mucoceles [61].
In cases in which the significance of such asymmetric signal intensity is
unclear, an unenhanced fine-cut CT of the temporal bone is almost always
diagnostic. Asymmetric petrous apex pneumatization is clearly an unusual
variant of normal anatomy that requires no treatment [4].

Neoplasms
Chondrosarcoma
Chondrosarcomas are rare malignancies that arise from embryologic
cartilage rests along the sphenopetroclival fissure. They account for 0.15%
of all intracranial tumors and 6% of skull base neoplasms [93]. Chondrosar-
comas are more common in patients with Ollier’s disease, Maffucci syndrome
(enchondroma with multiple angiomas), Paget’s disease, and osteochon-
droma, although most chondrosarcomas arise de novo.
Because of the slow growth and insidious nature of chondrosarcomas,
diagnosis is not uncommonly delayed. Diplopia, headache, and hoarseness
are the most common presenting symptoms; however, a wide variety of
presenting signs and symptoms can be found, including hearing loss, dizzi-
ness, aural fullness, dysphagia, facial paresthesias or paralysis, and visual
impairment [94,95].
The differential diagnosis for solid lesions of the petrous apex is broad
but can be narrowed with radiographic evaluation. CT provides excellent
bony detail and is useful for determining the location of origin of skull
base neoplasms. Chondrosarcomas generally arise from the sphenopetro-
clival synchondrosis, although 28% may originate from the clivus [96].
CT often demonstrates areas of calcification, giving a characteristic ‘‘pop-
corn’’ pattern [97]. Chondrosarcomas are generally hypo- to isointense
with T1-weighted MRI, hyperintense on T2-weighted MRI, and demon-
strate heterogeneous enhancement with gadolinium enhancement [98].
506 ISAACSON et al

Chondrosarcomas are classified into five different histologic subtypes:

conventional, clear cell, myxoid, mesenchymal, and dedifferentiated. Prog-
nosis depends on the histologic grade, which is divided into grades I (well
differentiated) to grade III (poorly differentiated). Evans and colleagues
[99] reported 5-year survival rates for grade I, II, and III to be 90%,
81%, and 43%, respectively. Immunohistochemical staining is important
to differentiate chondrosarcoma from chondroid chondroma; however,
unlike chordomas, chondrosarcomas do not stain for epithelial membrane
antigen or cytokeratin [96].
The mainstay of treatment for chondrosarcoma of the skull base is surgery.
Gross tumor removal often can be achieved with contemporary skull base
techniques [100,101]. Surgical approach should be selected based on tumor
location and surgeon’s preference and experience. Multiple surgical ap-
proaches have been described, including translabyrinthine, transcochlear,
type B infratemporal fossa, extended middle fossa, subtemporal-infratempo-
ral, fronto-orbitozygomatic, transmaxillary, and endoscopic transnasal
transsphenoidal approaches. The petrous carotid artery and cavernous sinus
are often involved with tumor, and there is considerable debate as to how
aggressive the surgeon should be in attempting gross tumor removal. Because
they are generally slow-growing tumors with a low incidence of metastasis,
preservation of vital structures despite less than total tumor removal is a viable
option. Some authors recommend resection and grafting of the petrous
carotid artery when involved and collateral arterial flow is confirmed with
angiography [102].
Tzortzidis and colleagues [102] also demonstrated decreased disease-free
survival in patients who require multiple operations. Radiation therapy is
an important adjunctive therapy in the management of chondrosarcomas. Be-
cause most series include chordomas and chondrosarcomas together, results
of radiotherapy for chondrosarcomas are difficult to interpret. Radiosurgery
has shown better control rates for chondrosarcoma than chordoma and is
useful to treat small residual tumors after incomplete resection [103,104].
Fractionated proton beam therapy also has shown promise in the treatment
of residual chondrosarcoma and achieves better control rates than chordo-
mas [105,106]. Oghalai and colleagues [100] demonstrated a decreased inci-
dence of recurrence in patients who underwent postoperative radiotherapy.
Chondrosarcomas of the skull base are rare neoplasms that present a for-
midable challenge to the skull base surgeon. Surgical management is the
mainstay of therapy; however, adjuvant radiation therapy is often needed
to control residual tumor. Stereotactic radiosurgery and fractionated proton
beam therapy seem to offer the best control for residual disease.

Chordoma
Chordomas are rare tumors that originate from embryologic remnants of
the notochord that forms the nucleus pulposus of the intervertebral disks in
 LESIONS OF THE PETROUS APEX 507

normal patients. They are midline lesions that occur anywhere from the
clivus to the sacrum, with approximately one third involving the clivus
[107]. Chordomas are rare, with an incidence of 0.08 cases per 100,000
[108]. Chordomas are more common in male patients and rarely present
in patients older than age 40. Female patients and younger patients are
more likely to present with a chordoma located in the skull base [108].
Because chordomas are slow growing and have an insidious course, most
tumors are large at the time diagnosis. In a large series by Tzortzidis and
colleagues [109], 81% of tumors were larger than 2 cm at presentation,
with 37.8% larger than 4 cm. The most common presenting symptoms are
diplopia, headache, and lower cranial nerve deficits [110,111]. Skull base
chordomas are typically more aggressive in the pediatric population, with
a wider range of presentation, atypical morphology, and greater incidence
of metastasis [112].
Radiographic evaluation is essential not only for diagnosis but also for
treatment planning. CT demonstrates a locally destructive lesion centered
at the clivus (Fig. 12). Bony trabeculae may be seen and true calcifications
may occur in the chondroid chordoma variant. There is moderate to marked
enhancement with contrast, and low signal areas may be present and repre-
sent areas of gelatinous material. MRI allows determination of the extent of
disease and reveals the presence of intracranial or cavernous sinus involve-
ment. With T1-weighted MRI, chordomas are hypointense and contrast
well against the hyperintense fat in the clival bone marrow. Chordomas
appear hyperintense on T2-weighted MRI and contrast well against adja-
cent neural structures (Fig. 13). With T1-weighted MRI enhanced with

Fig. 12. Chordoma. Axial temporal bone CT scan demonstrates a lytic lesion centered in the
clivus. This lesion extends into the right petrous apex posterior to the vertical petrous carotid
artery.
508 ISAACSON et al

Fig. 13. Chordoma. Axial T2-weighted MRI demonstrates a hyperintense lesion centered in the
right petrous apex and clivus.

gadolinium, chordomas have variable enhancement and may have a charac-

teristic ‘‘honeycomb’’ pattern (Fig. 14) [113].
Grossly, chordomas are grayish, semitransparent, and multiloculated. His-
tology reveals the characteristic physaliphorous cell, which has a vacuolated

Fig. 14. Chordoma. Axial T1-weighted MRI with fat saturation and gadolinium demonstrates
an enhancing lesion centered in the right petrous apex and clivus.
 LESIONS OF THE PETROUS APEX 509

cytoplasm and a ‘‘soap bubble’’ appearance. Immunohistochemical staining

is important to differentiate chordoma from chondrosarcoma because the his-
tologic appearance may be similar and prognosis much different. Chordomas
stain positive for S-100, epithelial membrane antigen, and cytokeratin. Chon-
drosarcomas do not stain positive for cytokeratin or epithelial membrane an-
tigen [114]. Chondroid chordoma is a variant with cartilaginous components
found in the matrix [115].
Because of the extensive nature of chordomas and the surrounding vital
structures, a combination of surgery and radiation therapy is generally
required. The location of the lesion and surgeon’s preferences dictates the
surgical approach, with combined approaches sometimes necessary. Tumors
located on the upper clivus and limited to the midline may be treated with
a transbasal craniotomy. Tumors that involve the middle and upper clivus
with more lateral extension beyond the cavernous sinus are better approached
with a fronto-orbitozygomatic craniotomy. Tumors that are centered at the
midclivus or are paramedian may be approached with a subtemporal-
infratemporal approach with transpetrosal extension if the posterior petrous
apex is involved. Tumors that involve the nasopharynx or craniocervical
junction can be managed with a transmaxillary approach or extreme lateral
transcondylar approaches. Endoscopic transnasal transsphenoidal approa-
ches to the skull base recently were described for resection of clival chordomas
[42,116,117].
Despite advances and decreased morbidity with contemporary skull base
techniques, gross total resection is achieved less than half the time
[110,118]. Surgical seeding also has been reported [119,120]. As a result
of these various factors, recurrences are common and long-term radio-
graphic surveillance is necessary. Options for recurrence include reopera-
tion, external beam radiation therapy, radiosurgery, and proton beam
therapy. Patients who require reoperation have a lower recurrence-free sur-
vival rate compared with patients who require only primary treatment
[109]. Chordomas are relatively radioresistant. Treatment with external
beam radiotherapy has produced poor tumor control rates [121–123]. Ste-
reotactic radiosurgery has produced superior results to external beam radi-
ation therapy. Krishnan and colleagues [103] reported a 72% control rate
for chordomas treated with a median tumor margin dose of 15 Gy. Debus
and colleagues [104] also had good tumor control rates of 82% at 2-year
follow-up using fractionated radiosurgery; however, the control rate fell
to 50% at 5 years. Better control rates have been achieved with a combina-
tion of photon and proton beam therapy. In a large series of 132 patients
that combined photon and proton beam radiation therapy, Terahara and
colleagues [124] achieved actuarial local control rates of 59% and 44%
at 5 and 10 years, respectively. For unclear reasons, female patients treated
with proton therapy for skull base chordomas have an increased incidence
of recurrence [105,124]. Carbon ion radiotherapy has been used for treat-
ment of skull base chordomas with encouraging results; however, longer
510 ISAACSON et al

follow-up is needed to demonstrate superior efficacy to already established

therapies [125].
Chordomas present a unique challenge to the skull base surgeon because of
their location, surrounding vital structures, and propensity for recurrence.
Surgical therapy is the mainstay of treatment; however, recurrences are com-
mon and adjunctive therapies are often required. There is no consensus for the
optimal treatment for skull base chordomas; however, fractionated proton
beam radiosurgery likely will continue to evolve as an important component
of treatment.

Meningioma
In 1922, Cushing [126] introduced the term ‘‘meningioma’’ to describe
a tumor that was initially described by Felix Plater in 1614. Meningiomas
comprise 13% to 26% of all intracranial tumors and are more commonly
discovered in middle-aged and elderly women. It seems that the incidence
of meningiomas increases with age and has increased with time. This tumor
is rarely found in children and is typically more aggressive or even malignant
in this population. Aside from female gender, radiation exposure is the only
known factor that increases the risk of developing a meningioma. Most
meningiomas are sporadic, with only 2% being related to syndromes,
including neurofibromatosis type 2 [3,127]. Arachnoidal cap cells from the
external layer of the arachnoid membrane are the cells of origin for menin-
giomas. These tumors are typically have a broad-based attachment to the
dura and may be associated with hyperostosis of the underlying calvarium
or skull base [3]. Meningiomas are classified into three separate World
Health Organization grades according histologic subtype and likelihood
of recurrence. The grade I lesions are the most common and include the
meningothelial, fibrous, transitional, microcystic, secretory, lymphoplasma-
cyte-rich, metaplastic, psammomatous, and angiomatous histologic types.
These grade I lesions are associated with a low risk of recurrence with
complete excision. Grade II lesions consist of the atypical, chordoid, and
clear cell histologic subtypes. Grade II lesions are typically more aggressive
and have a higher incidence of recurrence when compared with grade I
tumors. Grade III tumors have the highest rates of recurrence and consist
of the anaplastic, papillary, and rhabdoid histologic subtypes. In addition
to these subtypes, any meningioma with a high proliferation index (O20
mitoses per high powered field) with or without brain invasion is classified
as grade III [3,127,128]. A 2.4-mm per year mean growth rate was reported
in 60 patients with asymptomatic meningiomas [129].
MRI with gadolinium is the procedure of choice when evaluating menin-
giomas, and it may detect lesions as small as 3 mm. Meningiomas appear as
sessile, extra-axial mass with accompanying dural tails. These lesions are
most commonly isointense or have a slight hypointensity on T1-weighted
images and enhance with gadolinium (Fig. 15). On T2-weighted images,
 LESIONS OF THE PETROUS APEX 511

Fig. 15. Meningioma. An axial T1-weighted MRI with fat saturation and gadolinium demon-
strates an enhancing sessile, eccentric left petrous lesion.

meningiomas most commonly have an isointense to hyperintense signal

[130]. Petroclival meningiomas may present with myriad symptoms, includ-
ing headache, facial pain, decreased hearing, balance disturbance, and vom-
iting, with gait disturbance being the most common [131–133]. Cranial nerve
deficits are present in a large majority of patients, with nerves V, VII, and
VIII being the most commonly affected [131].
The management of a petroclival meningioma is predicated on multiple
factors, including a patient’s age, medical comorbidities, presenting symp-
toms, and the location and size of the lesion. Treatment options include ob-
servation, microsurgical excision, external beam radiation, and stereotactic
radiosurgery alone or in combination. Resection of these lesions is compli-
cated because of their location and frequent involvement of the dural venous
sinuses, brainstem, cranial nerves, petrous carotid artery, and vertobrobasi-
lar arterial system [134,135]. Extension to the internal auditory canal, Meck-
el’s cave, foramen magnum, and middle cranial fossa is not unusual. The
retrosigmoid, combined, frontotemporal approaches, and many other ap-
proaches have been reported for excision of petroclival meningiomas [3].
These tumors were previously considered inoperable because of the high
morbidity and mortality (50%) associated with attempted excision [136].
More recent series have demonstrated a significant decline in morbidity
and mortality (!10%). A recent meta-analysis by Selesnick and colleagues
[137] demonstrated that complete excision of these tumors is achieved in
68% of cases. Reasons for incomplete excision include brainstem adherence,
adhesions to cranial nerves, encasement of vessels, and hypervascularity.
512 ISAACSON et al

Incomplete excision with observation or adjunctive radiation is a reasonable

management strategy in patients with large tumors or significant neurovas-
cular involvement [138].
Conventional fractionated radiation and stereotactic radiosurgery are
acceptable and sometimes preferred treatment options in patients who
have petrous apex meningiomas. Conventional external beam radiation
has not traditionally been used as a primary treatment modality and
typically has been used as adjuvant therapy for incompletely excised tumors.
A combination of surgery and radiation has favorable tumor control results
in comparison to incomplete excision alone. Several centers using stereo-
tactic radiosurgery have demonstrated favorable tumor control rates
(86.7%–100%) with limited follow-up. Radiosurgery is not an option in
patients with larger tumors (O3 cm). Complications of radiosurgery include
cranial nerve deficits, hydrocephalus, radiation necrosis, and rarely, malig-
nant transformation [138,139]. In certain circumstances, observation may
be prudent, especially in elderly patients, patients with multiple medical
comorbidities, and patients with small asymptomatic tumors [131].

Metastasis
Most metastatic neoplasms are identified in patients between the ages of
50 and 70 years old. Metastatic petrous apex lesions are most commonly
identified in the same age group [140,141]. The petrous apex is the most
commonly involved site in the temporal bone (82.9%), with it being the
sole site of involvement in 31.6% of cases in a series of 212 patients. The
most commonly reported symptom is hearing loss; in one study, however,
36.2% of patients had no neurotologic symptoms [141]. Other studies
have demonstrated that most temporal bone metastatic disease is asymp-
tomatic [142,143]. Vertigo, dizziness, facial weakness, tinnitus, otalgia,
otorrhea, external auditory canal mass, and nystagmus have been reported
in patients with metastasis. In the series by Gloria-Cruz and colleagues
[141], 14 of 212 patients had facial canal involvement, with only 6 showing
signs of facial nerve weakness. All 6 patients with facial weakness had tumor
invasion of the facial nerve epineurium.
The mechanisms for metastatic tumor involvement include hematogenous
spread from distant tumors or leukemia/lymphoma, direct extension via an
extra or intracranial neoplasm, and leptomeningeal extension from a distant
or intracranial primary neoplasm. Hematogenous spread is thought to occur
because of slow blood flow through the petrous apex marrow, which allows
filtering and deposition of tumor cells [141,144]. In the series by Gloria-
Cruz and colleagues [141] that discussed 415 temporal bones from 212
patients with nondisseminated malignant tumors, 76 (18.3%) temporal bones
and 47 (22.2%) patients were found to have temporal bone involvement.
Twenty different tumor cell types were identified, with breast carcinoma
being the most commonly encountered metastatic temporal bone neoplasm,
 LESIONS OF THE PETROUS APEX 513

followed by lung, prostate, melanoma, and kidney. Adenocarcinoma was the

most common cell type identified in the petrous apex. Seventy-five percent of
tumors were found to spread by a hematogenous route. Otic capsule involve-
ment was rare, and when invasion was identified, no patient had vestibular
manifestations. No cases were reported in which the temporal bone was the
only site of hematogenous spread, with the lung and gastrointestinal tract
being the most common concurrent sites. Twelve patients were found to
have an occult neoplasm discovered at autopsy; only 1 was found to have tem-
poral bone involvement in the petrous apex. The imaging characteristics of
petrous apex metastasis depend largely on the primary tumor but often
show significant bone destruction and enhance with the administration of
contrast. Surgical management of petrous apex metastasis is unwarranted,
and treatment often consists of palliative therapy.

Uncommon lesions
Schwannomas, paragangliomas, and endolymphatic sac tumors occasion-
ally involve the petrous apex. Petrous apex schwannomas may originate from
cranial nerves IV, V, VI, VII, or VIII. Petrous apex involvement usually
results from direct extension and typically does not arise within the petrous
apex. Paragangliomas also may invade the petrous apex via preformed air
cell tracts from their site of origin in the jugular foramen or middle ear.
Endolymphatic sac tumors arise from the proximal rugose portion of the
endolymphatic sac located halfway between the internal auditory canal and
jugular foramen along the posterior aspect of the temporal bone [145]. These
tumors typically extend into the mastoid from the inner ear and may invade
the petrous apex [146].

Summary
Advances in diagnostic imaging, microsurgical techniques, and the intro-
duction of stereotactic radiosurgery have made the management of petrous
apex pathology less daunting for modern skull base surgeons. Decreasing
patient morbidity and mortality and improving outcomes are the ultimate
goal for surgeons treating petrous apex pathology. Unfortunately, meaning-
ful prospective outcome studies are lacking and not likely to occur because
of the rarity of these lesions.

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