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REVIEWS AND COMMENTARY 


Imaging Review of the Temporal
Bone: Part II. Traumatic, Postoperative,
and Noninflammatory Nonneoplastic

n  STATE OF THE ART


Conditions1
Amy F. Juliano, MD
The first part of this review of the temporal bone dis­
Daniel T. Ginat, MD
cussed anatomy of the temporal bone as well as inflamma­
Gul Moonis, MD
tory and neoplastic processes in the temporal bone region
(1). This second part will first discuss trauma to the tem­
poral bone and posttraumatic complications. The indica­
tions for common surgical procedures performed in the
temporal bone and their postoperative imaging appear­
ance are then presented. Finally, a few noninflammatory
nonneoplastic entities involving the temporal bone are re­
Online SA-CME
See www.rsna.org/education/search/ry viewed. They are relatively uncommon diagnoses com­
pared with infectious or inflammatory diseases. However,
because patients present with symptoms that are either
Learning Objectives: common (hearing loss) or distinctive (sensorineural hear­
After reading the article and taking the test, the reader will ing loss in a child), they are important for the radiologist
be able to:
to be aware of and recognize.
n Explain the basic clinical features and imaging
appearances of the various sequelae of temporal bone
trauma © RSNA, 2015
n Describe imaging appearances of common surgical
procedures performed in the temporal bone
n Discuss the disease process and progression of
otospongiosis and the corresponding imaging features
n Describe the clinical features, imaging technique, and
imaging appearance of semicircular canal dehiscence
and large vestibular aqueduct syndrome
Accreditation and Designation Statement
The RSNA is accredited by the Accreditation Council for
Continuing Medical Education (ACCME) to provide continuing
medical education for physicians. The RSNA designates this
journal-based SA-CME activity for a maximum of 1.0 AMA
PRA Category 1 Credit™. Physicians should claim only the
credit commensurate with the extent of their participation in
the activity.
Disclosure Statement
The ACCME requires that the RSNA, as an accredited
provider of CME, obtain signed disclosure statements from
the authors, editors, and reviewers for this activity. For this
journal-based CME activity, author disclosures are listed at
the end of this article.

1
  From the Department of Radiology, Massachusetts Eye
and Ear Infirmary, 243 Charles St, Boston, MA 02114
(A.F.J.); Department of Radiology, The University of Chicago
Medicine, Chicago, Ill (D.T.G.); and Department of
Radiology, Beth Israel Deaconess Medical Center, Boston,
Mass (G.M.). Received April 3, 2014; revision requested
May 19; revision received August 13; accepted September
22; final version accepted October 3. Address
correspondence to A.F.J. (e-mail: amy_juliano@meei.
harvard.edu).

q
 RSNA, 2015

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STATE OF THE ART: Imaging Review of the Temporal Bone Juliano et al

I
n part 2 of this review, we will first jury (eg, stick, gunshot wound), blunt is perpendicular to the petrous pyr­
discuss trauma to the temporal bone trauma (eg, motor vehicle accident), amid. The fracture extends from the
and posttraumatic complications. barotrauma (eg, sudden change in foramen magnum, extends through the
pressure), thermal injury, and avulsion petrous pyramid involving the labyrinth,
injury. Fractures of the temporal bone and exits into the middle cranial fossa.
Imaging of Temporal Bone Trauma are common in patients with major A transverse fracture is associated with
There are various types of trauma mech­ head trauma, with a reported incidence a higher risk of facial nerve injury.
anisms that can result in temporal bone of 18%–22% of skull fractures involving A limitation of the traditional classifi­
injury. These include penetrating in­ the temporal bone (2). It requires sub­ cation system is that it does not address
stantial force (1875 lb) to fracture the oblique or mixed fractures, which, ac­
temporal bone; therefore, initially, the cording to many studies, represent the
Essentials
temporal bone injury is often overshad­ majority of temporal bone fractures (7).
nn Ossicular injury is usually seen owed or masked by other associated In addition, this classification system
with longitudinal fractures, and clinically important injuries (3). The does not correlate well with clinical out­
dislocations are more common clinical signs and symptoms of temporal come and potential complications. To
than fractures; the incus is the bone fracture include hemorrhagic otor­ address the limitations of the traditional
most commonly injured ossicle rhea, hemotympanum, tympanic mem­ system, newer classification systems have
owing to its larger size and lack brane perforation, vertigo, hearing been proposed. Ishman and Friedland (8)
of ligamentous and muscular sup­ loss, facial nerve palsy, and nystagmus. classified fractures into petrous and
port and incudostapedial joint The Battle sign refers to postauricular nonpetrous types. Petrous fractures in­
separation is the most common ecchymosis from traumatic rupture of a volve the petrous apex and otic capsule;
injury of the ossicular chain (may mastoid emissary vein (4). It has been the nonpetrous fractures involve the
be missed due to subtlety of its noted that temporal bone fractures do middle ear and mastoid. While most
imaging appearance). not demonstrate normal bone healing, complications—facial nerve injury,
nn Compared with the traditional possibly related to low metabolic activ­ cerebrospinal fluid (CSF) leak, senso­
scheme for temporal bone ity of the bone of the otic capsule (5). rineural hearing loss—occurred in the
fracture classification (longitudi­ While high-spatial-resolution tem­ petrous group, there was increased in­
nal vs transverse), newer poral bone computed tomography (CT) cidence of conductive hearing loss in
classification schemes (otic is the modality of choice for evaluat­ the nonpetrous group owing to involve­
capsule violating vs sparing) have ing temporal bone trauma, particularly ment of the ossicular chain.
better correlation with clinical fractures, CT arteriogram/venogram or There is a newer system proposed by
outcome and complications. magnetic resonance (MR) arteriogram/ Kelly and Tami (9) that classifies frac­
nn Non–echo-planar diffusion-weighted venogram and high-spatial-resolution MR tures depending on whether the otic
imaging can potentially serve as imaging may be helpful for the assess­ capsule is spared (otic capsule–sparing
an alternative to second-look sur­ ment of complications. fracture) or violated (otic capsule–
gery for the detection of recurrent The traditional system for classi­ violating fracture). The otic capsule–
cholesteatoma, which typically fying temporal bone fractures does so sparing fracture is much more common
demonstrates reduced diffusion. according to their orientation relative (94%–97%), results from a temporopa­
to the long axis of the petrous pyramid rietal blow, and has an increased inci­
nn In a patient presenting with con­
(Fig 1) (6). The longitudinal fracture dence of conductive hearing loss due
ductive hearing loss without tym­
type is more common (80%–90% of to ossicular injury. The otic capsule–-vi­
panic membrane abnormality and
temporal bone fractures) and results olating fracture (3%–6%) results from
with no history of middle ear in­
from temporoparietal impact. The frac­ an occipital blow and has a higher inci­
flammation, there should be a
ture line is parallel to the longitudinal dence of facial nerve paralysis (30%–
high level of suspicion for fenes­
axis of the petrous portion of the 50%), sensorineural hearing loss,
tral otosclerosis, evidenced by
temporal bone. The fracture begins in
demineralization in the region of
the squamosal portion of the temporal
the fissula ante fenestram at CT.
bone, extends along the posterior supe­ Published online
nn In the late phase of otosclerosis, rior bony margin of the external auditory 10.1148/radiol.2015140800  Content codes:
demineralization may not be seen, canal, crosses the tegmen, and exits in Radiology 2015; 276:655–672
and instead there may be sclerotic the middle cranial fossa anterior to the
thickening around the oval window, labyrinth. This type of fracture results Abbreviations:
at the stapes footplate, and around in ossicular and tympanic membrane CSF = cerebrospinal fluid
the cochlear capsule; obliterative DWI = diffusion-weighted imaging
injury. The less common transverse
LVA = large vestibular aqueduct
disease (at the oval and round win­ fracture (10%–20%) results from fron­
dows) should be reported. tooccipital trauma, and the fracture line Conflicts of interest are listed at the end of this article.

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STATE OF THE ART: Imaging Review of the Temporal Bone Juliano et al

Figure 1  between two axes of rotation. Simulta­


neous tetanic contractions of the tensor
tympani tendon and stapedius tendon
have also been implicated. The tensor
tympani muscle resides in a semi canal
in the anterior wall of the middle ear
cavity and passes posteriorly, forming a
right angle at the cochleariform process
to insert into the neck of the malleus.
The stapedius muscle arises from the
posterior wall of the middle ear cavity;
its tendon emerges through the tip of
the pyramidal eminence to insert into
the head of the stapes. Temporal bone
trauma of sufficient force can result in
sudden retraction of the incus medially
and the stapes posteriorly with separa­
tion of the incudostapedial joint (12).
Incudostapedial joint separation may be
missed at CT owing to subtle imaging
Figure 1:  Traditional classification of temporal bone fractures. Axial CT images demonstrate (a) a longitudi- findings (11). On serial axial CT sec­
nal fracture and (b) a transverse fracture in two different patients. The longitudinal fracture is parallel to the tions, it is manifested by a gap between
long axis of the petrous pyramid (arrow, a) and traverses the mastoid portion of the temporal bone. The the lenticular process of the incus and
transverse fracture is oriented perpendicular to the petrous pyramid (arrow, b) and traverses the basal turn of the head of the stapes (Fig 2). Incudom­
the cochlea (arrowhead, b). alleal joint separation is easier to visual­
ize and manifests as a gap between the
“ice cream” (head of the malleus) and
and CSF fistula. This new classification membrane rupture, but if conductive the “ice cream cone” (body and short
system shows better correlation with hearing loss persists beyond the first process of the incus) in the epitympa­
clinical outcome and complications (10). month or so after injury (by which time num (Fig 3). Total incus dislocation is
On CT images, the optic capsule–violat­ hemotympanum should have resolved), also possible when incudomalleal and
ing frac­ture line can involve the vestibule, ossicular injury should be suspected. incudostapedial joint separation occur
semicircular canals, cochlea, and/or fa­ The incus is the most commonly in­ concomitantly. The dislocated incus
cial nerve. Although classifying temporal jured ossicle since it is the heaviest may remain in the epitympanic recess,
bone frac­tures is useful in understand­ ossicle, has no muscular attachments, prolapse into the hypotympanum, or ex­
ing complications and for treatment and has the least amount of ligamen­ trude out through the external auditory
planning, on an individual case basis, tous support (11). Ossicular dislocation canal and may not be visualized in situ.
it may be more important to describe is more common than ossicular fracture If the incudomalleal joint resists separa­
the vital structures involved (ossicles, (11). CT findings of ossicular injury may tion as a result of the trauma, the incu­
otic capsule, facial nerve, tegmen, ex­ be difficult to appreciate acutely due domalleal complex may dislocate as one
ternal auditory canal, internal audi­ to hemotympanum. Comparison with unit into the meso- or hypotympanum.
tory canal, carotid canal) rather than the normal side and supplementary There can be concomitant incudosta­
trying to make the findings “fit” into a reformatted images in planes parallel pedial joint separation with this type
classification scheme. (Pöschl) and perpendicular (Stenvers) of injury. Stapediovestibular dislocation
to the plane of the superior semicir­ is very rare and occurs when there is
cular canal may be useful for problem disruption of the annular ligament at­
Complications of Temporal Bone solving. taching the stapes footplate to the oval
Trauma There are five types of dislocations: window, which causes the stapes to be
incudostapedial joint separation, incu­ dislocated into the vestibule (internal
Ossicular Injury domalleal joint separation, incus dislo­ dislocation) or into the tympanic cavity
Ossicular chain disruption is usually cation, incudomalleal complex disloca­ (external dislocation). Stapediovestibu­
seen with longitudinal fractures or otic tion, and stapediovestibular dislocation lar dislocation creates a perilymphatic
capsule–sparing fractures of the tem­ (11). Incudostapedial joint separation is fistula, which is an abnormal communi­
poral bone. Conductive hearing loss the most common injury of the ossicular cation between the middle ear and inner
after temporal bone trauma is usually chain. The incudostapedial articulation ear (13). Perilymphatic fistula manifests
related to hemotympanum or tympanic is a fragile diarthrodial joint that lies clinically as vertigo and fluctuating sen­

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STATE OF THE ART: Imaging Review of the Temporal Bone Juliano et al

Figure 2  of the external auditory canal, presum­


ably related to a suction-type mecha­
nism (15). This finding may be better
appreciated on a coronal or Pöschl view
of the temporal bone CT study.
Fracture of the stapes crura typically
results from torsional injury, with the
posterior crus involved more frequently
than the anterior crus (16). The sta­
pes footplate can also implode into the
vestibule from penetrating injury and
result in a perilymphatic fistula (17).
On CT scans, the stapes crura are not
visualized in their expected location at
the oval window and can be noted in
the meso- or hypotympanum. Isolated
fracture of the incus is very rare (11).
Cochlear concussion results from in­
jury to the membranous labyrinth with
posttraumatic sensorineural hearing loss
Figure 2:  Incudostapedial separation. (a) Axial CT image demonstrates a gap (black arrow) between the and is occult on CT scans since no bone
lenticular process of the incus (white arrow) and the head of the stapes (arrowhead) in the setting of a longi- injury is present (18). MR imaging may
tudinal fracture. (b) Axial CT image of a normal incudostapedial articulation (arrow). demonstrate hemorrhage in the laby­
rinth manifesting as high signal inten­
sity on unenhanced T1-weighted images
Figure 3  Figure 4  (19).

CSF Leak
CSF leak is seen in 11%–45% of tem­
poral bone fractures, particularly those
involving the tegmen (8,10,20). Otic
capsule–violating fractures have a two
to four times increased risk of CSF
leak. Clinically, patients present with
CSF otorrhea and CSF rhinorrhea (leak
of CSF through the Eustachian tube
into the nasal cavity in the setting of
an intact tympanic membrane). Some
patients may experience a sense of full­
ness in the ear and conductive hearing
loss. Beta-2 transferrin is found almost
exclusively in the CSF, and testing of
the leaking fluid for beta-2 transferrin
Figure 3:  Incudomalleal joint separation. Axial CT Figure 4:  Stapediovestibular dislocation with is very helpful. However, it is important
image demonstrates a gap (arrow) between the ice perilymphatic fistula. Axial CT image demonstrates
to note that beta-2 transferrin is also
cream (head of malleus) and the cone (body and pneumolabyrinth (arrowhead) and dislocation of the
present in the perilymph, and testing
short process of incus). This occurred in the setting footplate of the stapes into the vestibule (black
for beta-2 transferrin may be positive in
of a longitudinal fracture. Compare with the normal arrow). A normal incudostapedial joint can be appre-
cases of perilymphatic leaks. Most cases
incudomalleal joint in Figure 4. ciated on this image, with no gap between the head
of the malleus and the incus body and short process of CSF leak occur in the first week after
(white arrow). trauma and close spontaneously with
conservative medical management (strict
sorineural hearing loss. Pneumolaby­ bed rest, elevation of bed by 30°, and
rinth (air in the inner ear structures) Isolated fracture of the manubrium avoidance of straining). If the CSF leak
and opacification of the round window (or handle) of the malleus is a rare con­ does not respond to conservative mea­
are important CT findings of perilym­ dition that presents as sudden-onset sures, CSF diversion by means of a lum­
phatic fistula (Fig 4) (14). hearing loss after digital manipulation bar drain is performed. The risk of men­

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Figure 5  Figure 6 

Figure 5:  Posttraumatic CSF leak. Axial CT image Figure 6:  Posttraumatic encephelocele. Coronal T2-weighted MR image in
demonstrates a defect in the anterior tegmen (arrow) the same patient as in Figure 5 obtained 1 year later demonstrates an en-
in this patient who presented withCSF otorrhea after cephelocele protruding into the attic via the tegmen defect (arrows). There con-
trauma. Also note opacification of the mastoid air tinues to be fluid in the mastoid air cells.
cells (∗) by CSF.

ingitis increases if the CSF leak lasts for provides good gray matter–white mat­ Figure 7 
more than 1 week. Multidetector CT of ter contrast; the brain parenchyma in
the temporal bone should be performed turn is easily distinguishable from the
for localization of the leak site (Fig 5). dark signal intensity of bone and air in
Stone et al (21) found that multidetec­ the mastoid and middle ear region.
tor CT depicted a bone defect in 70% of
cases with clinically active leaks. They Facial Nerve Injury
suggested that contrast-enhanced CT If the facial nerve is transected, the
cisternography or radionuclide cister­ patient experiences immediate facial
nography (each of which involves a lum­ paralysis. Delayed facial nerve injury
bar puncture) should be reserved for (1 day to 16 days after injury) is related
patients with multiple bone defects or to contusion, edema, or hematoma.
patients in whom initial high-spatial- The severity and timing of onset of fa­
resolution CT did not identify a bone cial paralysis are important prognostic
defect. Cisternography should be per­ factors. Incomplete facial paralysis can
formed during clinically active leaks be managed by observation and high-
to improve sensitivity (21). Shetty et al dose steroids, with surgical exploration
(22) found multidetector CT to be 93% considered only if an obvious bone frag­
accurate and MR cisternography to be ment is seen impinging the facial nerve
Figure 7:  Facial nerve injury. Axial CT image dem-
89% accurate in diagnosing CSF leaks. canal (23). Early complete paralysis
onstrates subtle enlargement of the geniculate fossa
Surgical repair should be performed may predicate the need for urgent sur­
(arrows), which is the most common site for facial
if the leak persists for more than gical exploration (23–26). The perigenic­ nerve injury. There is also a complex right temporal
7–10 days despite CSF diversion, be­ ulate area is more susceptible to injury bone fracture.
cause of the risk of meningitis. Menin­ due to traction from the greater super­
gocele and encephelocele are late com­ ficial petrosal nerve (24,25). On high-
plications of temporal bone fractures, spatial-resolution CT images, one should Labyrinthitis Ossificans
and MR imaging is invaluable for de­ search for a fracture line coursing When a temporal bone fracture involves
tection utilizing a coronal true inversion through the facial nerve, and for bone the inner ear structures (usually an otic
recovery pulse sequence (Fig 6). This is fragments, ossicles, or hematoma im­ capsule–violating fracture), labyrinthi­
a high-spatial-resolution sequence that pinging on the facial canal (Fig 7). tis ossificans can result, in which the

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STATE OF THE ART: Imaging Review of the Temporal Bone Juliano et al

Figure 8 

Figure 8:  Labyrinthitis ossificans. (a) Axial CT image demonstrates an otic capsule-violating fracture (arrow). There is mineralization
of the lateral semicircular canal (arrowhead). (b) Axial three-dimensional heavily T2-weighted MR image shows lack of fluid signal inten-
sity in the expected location of the right lateral semicircular canal (white arrow) and in the left vestibule (black arrow) and lateral semi-
circular canal (arrowhead) compatible with labyrinthitis ossificans. The patient also had an otic capsule-violating fracture on the left (not
shown). Incidentally noted is a mildly dysmorphic right vestibule.

fluid-filled lumen of the otic capsule is in 30% of patients with basilar skull tivity for adequately excluding blunt ce­
replaced by bone (or fibrous tissue if in fractures (28). Resnick et al (29) found rebrovascular injury, it may still prove
the early stages) (1,27). Clinically, this that 24% of patients with basilar skull useful in the setting of trauma when
is associated with profound sensorineu­ fractures had fractures involving the ca­ there is a high pretest probability of in­
ral hearing loss and loss of vestibular rotid canal, 11% of whom had vascular jury.
function. On high-spatial-resolution CT complications (laceration, cavernous ca­ CT angiography findings indicating
images, osseous attenuation is noted rotid fistula, pseudoaneurysm, or dissec­ arterial injury include irregularity of the
within the inner ear. The correspond­ tion) directly related to the intracranial lumen, intimal flap, out-pouching of the
ing MR images show loss of fluid signal carotid injury. The presence of a carotid lumen, lumen occlusion, or extravasa­
intensity within the membranous laby­ canal fracture is highly suggestive of in­ tion of contrast material. In the setting
rinth and enhancement on gadolinium- jury to the internal carotid artery pass­ of a high risk of vascular injury and a
enhanced images. Heavily T2-weighted ing through that canal, with a sensitiv­ negative CT angiography study, cathe­
high-spatial-resolution sequences with ity of 60% and specificity of 67% (30). ter angiogram remains the reference
high contrast-to-noise ratio, such as According to McKinney et al (31), there standard for diagnosis.
true fast imaging with steady state was a high rate of blunt vascular injury Venous injury can manifest as tran­
precession (known as FISP), are most in the presence of a carotid canal or ver­ section or thrombosis of the venous si­
sensitive for detection at its earliest (fi­ tebral transverse foramen fracture seen nuses (Fig 9). CT venogram should be
brous) stage. MR findings precede CT at multidetector CT, and they recom­ performed in suspected cases where
changes by many months, as the earlier mended using foraminal involvement as the fracture line traverses the venous
fibrous stage prior to ossification may a screening criterion for further imaging sinuses. A filling defect in the sinuses
not be detectable on CT images but may of these patients using CT angiography from a thrombus is highly suggestive of
be seen on MR images as loss of fluid or catheter angiography. venous sinus thrombosis. An MR imag­
signal intensity in the membranous lab­ According to the meta-analysis by ing or CT study will also depict venous
yrinth (Fig 8) (1). Roberts et al (32), the diagnostic per­ infarcts or hematomas.
formance of CT angiography varied
Vascular Injury considerably across studies, which was
Arterial injury can occur in the form of believed to be due to variation in diag­ Imaging of the Postoperative Temporal
dissection, pseudoaneurysm, transec­ nostic threshold across different trauma Bone
tion, occlusion, or arteriovenous fistulas. centers. They suggested that although Temporal bone imaging may be per­
Carotid injury is reported to be present CT angiography appears to lack sensi­ formed in patients with a history of

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Figure 9  Figure 10  Figure 11 

Figure 10:  Tympanostomy tube. Axial CT image


shows a plastic grommet (arrow) that traverses the
Figure 11:  Canal-wall-up mastoidectomy. The
tympanic membrane.
Figure 9:  Venous sinus injury. Axial contrast- patient had a history of mastoiditis. Axial CT image
enhanced CT image demonstrates a filling defect shows an intact posterior wall of the external audi-
(black arrow) in the right sigmoid sinus compatible tory canal (arrow) and a clear mastoid bowl (∗).
with posttraumatic thrombosis. Also note some air tympanostomy tubes into the middle
bubbles (white arrow) at the jugular foramen from ear occasionally occurs and can result
adjacent temporal bone fracture (not shown). in conductive hearing loss (35). Figure 12 

Mastoidectomy
otologic or neurotologic surgery, specif­ The different types of mastoidectomy
ically for evaluating the results of the essentially consist of resecting variable
surgery or for unrelated reasons. In portions of the mastoid air cells and
either case, it is important to be famil­ adjacent structures, which may be per­
iar with some of the more commonly formed for treatment of mastoiditis,
performed procedures and their corre­ cholesteatoma resection, cochlear im­
sponding imaging findings. plantation, or endolymphatic surgery,
among other indications (33,36). Canal-
Myringotomy and Tympanostomy Tube wall-up mastoidectomy involves exen­
Tympanostomy (ventilation) tubes are teration of the mastoid air cells with
commonly inserted into the tympanic preservation of the posterior wall of
membrane via an incision in the tym­ the external auditory canal, creating a
panic membrane (myringotomy) for mastoid bowl or cavity (Fig 11). Canal-
Figure 12:  Radical mastoidectomy. The patient
treating chronic or recurrent otitis me­ wall-down mastoidectomy consists of
had a history of extensive middle ear cholesteatoma
dia recalcitrant to medical management performing the same steps as in canal-
with ossicular erosion. Axial CT image shows a large
(33,34). Tubes are available in different wall-up mastoidectomy, but in addi­
mastoid bowl (∗) with absence of the posterior wall
shapes, sizes, and materials, most com­ tion the posterior wall of the external of the external auditory canal and ossicular chain.
monly Teflon or silicone, which have auditory canal is resected to increase
soft-tissue attenuation on CT images exposure to middle ear contents. A rad­
(Fig 10). Although CT studies may not ical mastoidectomy can be performed
be obtained specifically to evaluate tym­ for extensive disease of the middle ear casionally, the mastoid bowl may be
panostomy tubes, it is helpful to avoid cavity with ossicular involvement. This obliterated using bone, cartilage, fat,
misidentifying these as unintended for­ procedure includes removal of the tym­ or hydroxyapatite to minimize the size
eign bodies or dislocated ossicles and panic membrane, malleus, and incus, of the mastoidectomy cavity following
to confirm the presence of a tube not with attempted preservation of the canal-wall-down procedures (37). Oth­
apparent on otoscopic evaluation (34). stapes, in addition to canal-wall-down erwise, the mastoid bowl should remain
The presence of middle ear fluid can mastoidectomy (Fig 12). Tympanomas­ clear and the presence of soft-tissue at­
obscure tympanostomy tubes, espe­ toidectomy refers to mastoidectomy tenuation material on CT images may
cially those composed of plastic. The performed in conjunction with a middle represent granulation tissue or, in the
tubes usually fall out on their own after ear procedure, such as tympanoplasty appropriate scenario, recurrent choles­
a few months, but medial migration of and/or ossicular reconstruction. Oc­ teatoma.

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STATE OF THE ART: Imaging Review of the Temporal Bone Juliano et al

Figure 13  Figure 14  Figure 15 

Figure 13:  Recurrent cholesteatoma. Postopera- Figure 14:  Stapes prosthesis. Pöschl reformatted Figure 15:  Total ossicular replacement prosthesis.
tive coronal DWI shows a focus of reduced diffusion CT image of the right temporal bone shows a me- Axial CT image shows a hydroxyapatite prosthesis
in the region of the mastoidectomy bowl (arrow) tallic stapes prosthesis (arrow) that extends from the that extends from the thick reconstructed tympanic
compatible with a cholesteatoma. incus to the stapedotomy site. membrane (arrow) toward the region of the stapes
footplate (arrowhead).

Evaluation of recurrent cholestea­ in patients with conductive hearing loss Figure 16 
toma is an important indication for im­ due to otosclerosis or congenital anom­
aging after mastoidectomy (Fig 13). On alies and typically consist of two compo­
CT images, recurrent cholesteatoma is nents—a loop and a piston. The loop of
suspected when there is new bone ero­ the stapes prosthesis usually attaches to
sion associated with a soft-tissue focus, the lenticular process of the incus, while
although the attenuation characteristics the piston sits in a groove formed in the
are nonspecific and it can be difficult stapes footplate (stapedotomy) by means
to differentiate these lesions from gran­ of laser or drill (Fig 14). A partial ossic­
ulation tissue. On MR images, choles­ ular replacement prosthesis substitutes
teatoma demonstrates high T2 and low the malleus and incus, and thus extends
T1 signal, without central enhance­ from the tympanic membrane to the
ment, while granulation tissue demon­ head of the stapes, while a total ossicular
strates internal enhancement. Further­ replacement prosthesis extends from the
more, cholesteatoma characteristically tympanic membrane to the stapes foot­
shows reduced diffusion, and MR im­ plate or oval window and is utilized if the
aging with non–echo-planar imaging stapes is also diseased (Fig 15). Ossicu­ Figure 16:  Dislocated partial ossicular replace-
diffusion-weighted imaging (DWI) (1) lar prostheses can be composed of var­ ment prosthesis. Coronal CT image of the left tem-
poral bone shows that the prosthesis (straight arrow)
can potentially serve as an alternative to ious materials including hydroxyapatite,
is detached from the tympanic membrane (curved
second-look surgery for the detection of metal, and plastic. Ossicular prosthesis
arrow) and the head of the stapes (arrowhead).
recur­rent cholesteatoma. Indeed, non– failure most commonly results from mi­
echo-planar imaging DWI confers a gration or dislocation (Fig 16) but can
positive predictive value for cholestea­ also result from prosthesis fracture, re­ cus interposition can be performed for
toma of 93%–100% (38,39). It is im­ current cholesteatoma, pressure erosion reconstruction of the ossicular chain af­
portant to interpret the DWI studies in of the ossicles, and perilymphatic fistula. fected by cholesteatoma or chronic otitis
conjunction with the conventional pulse Many of these complications can be iden­ media and consists of resecting, sculpt­
sequence images to avoid false-positive tified at high-spatial-resolution CT. For ing, and repositioning the incus, such
interpretations, which can result from the example, pressure erosion appears as that it extends from the malleus to either
presence of fat grafts and hemorrhage. hypoattenuation or truncation of the os­ the stapes or oval window (Fig 17). In­
sicles, often with associated dislocation cus interposition bone grafts are viable,
Ossicular Reconstruction of the prosthesis; the diagnosis of peri­ as opposed to artificial prostheses (41).
A variety of techniques can be per­ lymphatic fistula can be suggested by the However, interposition grafts may be
formed for ossicular reconstruction presence of pneumolabyrinth or a new, compromised by dislocation, necrosis,
(33,36,40). Stapes prostheses are used unexplained middle ear effusion (40). In­ and recurrent cholesteatoma.

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Figure 17  Figure 18 

Figure 17:  Incus interposition. Axial CT image Figure 18:  Cochlear implant. Stenvers view radiograph obtained at the con-
shows a sculpted incus (white arrow) that extends clusion of cochlear implantation surgery shows expected positioning of the
from the manubrium of the malleus (black arrow) to cochlear implant electrodes (white arrow) in relation to the internal auditory
the stapes (arrowhead). canal (black arrow), superior semicircular canal (white arrowhead), lateral semi-
circular canal (black arrowhead), and mastoidectomy cavity (∗).

individual electrodes after implantation ing structures, with a mean maximal


Cochlear Implantation in greater detail, and three-dimensional anterior-posterior dimension of 6.6 cm
Cochlear implants are electronic de­ stereoscopic CT images with pseudo­ and a lateral dimension of 4.8 cm (45).
vices that can restore hearing in pa­ color technique can be helpful for pro­
tients with severe sensorineural hearing viding an overall view of the hardware Vestibular Schwannoma Surgery
loss by direct stimulation of the cochlear (Fig 19) (43). Complications related to Three main surgical approaches can be
nerve. The basic components of a co­ cochlear implantation occur in less than implemented for resection of vestibular
chlear implant include a microphone, 1% of cases and include device malposi­ schwannomas, including translabyrin­
speech processor, and transmitter coil tion or migration, breakage, facial nerve thine, retrosigmoid (suboccipital), and
externally, and a receiver-stimulator canal dehiscence and stimulation or middle cranial fossa approaches. The
internally, which leads to a cable end­ injury, pneumolabyrinth, and bone ero­ translabyrinthine and the retrosigmoid
ing in an electrode array within the co­ sion, which are best evaluated with CT, approaches can be used for all tumor
chlea. The electrode array usually con­ and abscess, meningitis, and labyrinthi­ sizes, while the middle cranial fossa
tains eight to 24 stimulators that are tis, which are best evaluated with MR approach is most useful for removal of
tuned to particular acoustic frequencies imaging (33,42). Ultimately, high-spa­ intracanalicular tumors (46). However,
(33,36,42). Typical surgery for cochlear tial-resolution temporal bone CT and hearing preservation can be achieved
implantation consists of performing MR imaging are useful for proper pre­ only by means of the retrosigmoid and
canal-wall-up mastoidectomy and intro­ operative planning and avoiding compli­ middle cranial fossa approaches (47).
ducing the electrode through the mid­ cations and failure of cochlear implan­ MR imaging is routinely performed
dle ear cavity through the facial recess tation. In particular, CT is useful for following surgical resection, mainly to
and into a widened round window delineating bony anatomy and identify­ assess for residual or recurrent tumor,
niche. The electrode enters the cochlea ing potential hazards and contraindica­ but also to evaluate for suspected com­
through the round window and into the tions to surgery, such as labyrinthitis plications (33,46,48). A typical postop­
scala tympani for approximately 1.5 ossificans and facial nerve canal dehis­ erative MR imaging examination consists
turns. The round window niche is then cence, while MR imaging is useful for of an internal auditory canal protocol
plugged with fascia or muscle graft af­ delineating the integrity of the cochlear that includes axial thin-section pre­
ter insertion of the electrodes. Intraop­ nerve and inner ear structures (44). Pa­ contrast T1-weighted, axial and coronal
erative and postoperative radiographs, tients can safely undergo 1.5-T MR im­ thin-section postcontrast fat-suppressed
including the Stenver view, are often aging after cochlear implantation, even T1-weighted, T2-weighted fast-spin-
obtained to confirm proper position­ with devices that contain magnetic echo, T2-weighted fluid-attenuated in­
ing of the cochlear implant electrodes components, if such devices are tightly version recovery (FLAIR), DWI, and
(Fig 18) (42). Multiplanar high-spatial- secured prior to imaging. However, the high-spatial-resolution cisternography
resolution CT images can depict the ex­ cochlear implants can produce artifacts (heavily T2-weighted) sequences, such
pected intracochlear positioning of the on MR images that obscure surround­ as constructive interference in steady

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Figure 19 

Figure 19:  Cochlear implant. (a) Stenvers reformatted CT image shows the electrodes (arrow), which enter the cochlea in the region of the round window (arrow-
head). Soft tissue graft has been used to obliterate the mastoid bowl (∗), which was created during the cochlear implantation surgery. (b) Stereoscopic three-dimen-
sional color CT image provides an overview of the cochlear implant components.

Figure 20  bilateral (85%), predominantly affects


women (65%–72%), and is seen much
more commonly in Caucasian than in
African-American, Asian, and Native
American subjects (49). Patients pre­
sent with hearing loss, typically in the
2nd to 4th decades of life. Early on, the
type of hearing loss is almost always
conductive in nature. Later on, mixed
hearing loss or sensorineural hearing
loss sets in as the disease progresses in
extent (50).
The otic capsule is normally com­
posed of an inner layer of endosteum,
an outer layer of periosteum, and a
middle layer of persistent primary en­
dochondral bone. In otosclerosis, the
Figure 20:  Residual/recurrent vestibular schwannoma. (a) Axial T1-weighted MR image shows a fat graft dense middle layer of endochondral bone
within the decompressed right internal auditory canal (arrow) following vestibular schwannoma surgery by is resorbed and replaced by spongy vas­
means of a retrosigmoid approach. (b) Axial fat-suppressed contrast-enhanced T1-weighted MR image cular bone. Thus otosclerosis is also re­
shows enhancing tumor within the fundus of the internal auditory canal and in the cerebellopontine angle ferred to as “otospongiosis,” and this
(arrows). Signal in the fat graft is suppressed and it appears hypointense (arrowhead). phase is referred to as the vascular
phase of otosclerosis. As the disease
progresses or becomes inactive, the
state (CISS) or fast imaging employ­ the altered bony anatomy and identify­ “spongy” areas recalcify, becoming less
ing steady-state acquisition (FIESTA) ing hemorrhage. vascular and more sclerotic (50). The
(46). The use of fat suppression is use­ most common location to be involved in
ful for distinguishing enhancing tumor otosclerosis early on is around the em­
from the intrinsic high signal intensity Noninflammatory Nonneoplastic bryologic fissula ante fenestram, a thin
of the fat grafts, which are commonly Entities fold of embryonic cartilage and con­
implanted in the surgical beds during nective tissue in a small cleft just ante­
tumor resection to minimize CSF leak­ Otosclerosis rior to the oval window (51,52). When
age (Fig 20). CT can also play a role Otosclerosis is an osteodystrophy of disease is limited to this area near the
in postoperative imaging for vestibular the otic capsule affecting approximately oval window, it is referred to as fenes­
schwannoma, particularly for defining 1% of the population. It is commonly tral otosclerosis. The abnormal bone

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Figure 21  Figure 22 

Figure 21:  Thickened stapes footplate in exten-


sive fenestral and retrofenestral otosclerosis. Axial
CT image shows extensive demineralization anterior
to the oval window in the region of the fissula ante Figure 22:  Obliterative fenestral otosclerosis. (a) Coronal CT image shows loss of the normal contour of
fenestram (arrowhead), extending over the lateral the left oval window (arrow) compatible with obliteration. (b) Compare with the normal contour of the right
aspect of the cochlea with involvement of the co- oval window (arrow).
chlear promontory (white arrow). The stapes foot-
plate is thickened (black arrow).
membrane, and absence of middle the oval window. The oval window is
ear inflammation (50). Imaging is narrowed and the stapes footplate is
impinges on the nearby stapes footplate, helpful for confirming the diagnosis, thickened (Fig 21). The formation of
resulting in mechanical fixation of the determining the extent of oval window thickened bony plaques may lead to
stapes footplate within the oval win­ involvement, assessing for a retrofenes­ complete obliteration of the oval window,
dow, thereby causing conductive hear­ tral component, and excluding clinical seen in 2% of cases (50), referred to as
ing loss. The round window is another mimickers such as superior semicircu­ obliterative fenestral otosclerosis (Fig 22).
favored site and may be involved as well lar canal dehiscence. Retrofenestral locations (beyond/behind
(52,53). As the disease progresses, High-spatial-resolution CT of the the region of the oval window) should
the remainder of the bony labyrinth temporal bone with a multidetector be examined for signs of involvement
becomes involved more extensively, unit with submillimeter sections and as well, as this may alter clinical man­
resulting in retrofenestral otosclerosis, multiplanar reformats (axial, coronal, agement. The round window is a par­
including around the cochlea. In addi­ Stenvers, Pöschl) is the examination ticularly important area to scrutinize,
tion to conductive hearing loss, these of choice for evaluation of otosclerosis. as the round window area is small and
patients have sensorineural hearing loss, In fenestral otosclerosis, there is focal even a positive finding there can be sub­
thought to result from damaging meta­ demineralization in the region of fissula tle (Fig 23a). On MR images, enhance­
bolic substances, toxins, or proteolytic ante fenestram, which can range from ment in the region of the oval window or
enzymes diffusing into the cochlear tiny, subtle, and hazy to prominent and round window (Fig 23b) should raise the
fluid causing hyalinization of the spiral well defined. This reflects replacement suspicion for otosclerosis.
ligament (54,55). Some patients have of the dense otic capsule by vascular When otosclerosis is suspected on
vestibular symptoms such as unstead­ spongiotic bone. As the disease pro­ one side, it is important to scrutinize
iness and vertigo (56,57). Retrofenes­ gresses, the area of demineralization the contralateral temporal bone for
tral otosclerosis rarely occurs without expands and involves the oval window signs of disease, since otosclerosis is
fenestral involvement, and patients al­ (Fig 21). In the later inactive, sclerotic frequently bilateral. Aside from exam­
most never have isolated sensorineural phase, the abnormal spongiotic bone ining the region at and around the oval
hearing loss. becomes denser, and may in fact be­ window for disease, one must also as­
Fenestral otosclerosis.—The char­ come isodense with adjacent normal sess for signs of retrofenestral involve­
acteristic clinical findings of fenestral areas of otic capsule, rendering detec­ ment, report findings that may lead to
otosclerosis are progressive conductive tion at CT challenging. In this situation, intraoperative complications or poor
hearing loss up to 50–60 dB, absent diseased regions are identified as areas surgical outcomes (eg, obliterated oval/
stapedial reflexes, a normal tympanic of abnormal sclerotic thickening around round windows, anomalous course of

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STATE OF THE ART: Imaging Review of the Temporal Bone Juliano et al

Figure 23 

Figure 23:  Round window involvement by otosclerosis. (a) Axial CT image demonstrates demineralization in the region of the round
window (arrow). (b) Axial contrast-enhanced T1-weighted MR image in the same patient shows enhancement in the region of the right
round window (arrow). There is subtle enhancement in the left round window (arrowhead) as well, where demineralization reflecting
otosclerosis can be seen at CT (not shown).

facial nerve, inner ear anomaly, inflam­ transmitting constant increased stress
Figure 24 
matory process in the middle ear and on the long process of the incus (58).
mastoid, dehiscence of bony covering CT can be used to evaluate for labyrin­
over the tympanic facial nerve or jugu­ thine fistula and labyrinthitis ossificans,
lar bulb), and evaluate for an alternative although the early fibrotic stage of laby­
process that may be a cause or the cause rinthitis ossificans may not be apparent
of conductive hearing loss (eg, ossicular at CT and may only be detected on MR
chain anomaly or abnormality, middle images. MR imaging is also useful for
ear mass, semicircular canal dehiscence). detection of other processes within the
Treatment of fenestral otosclerosis membranous labyrinth and vestibule,
is surgical, consisting of stapedotomy such as infectious labyrinthitis, repar­
(creation of a tiny hole in the stapes ative granuloma, and intravestibular
footplate), complete or partial stape­ foreign body, using a combination of
dectomy, and placement of a stapes heavily T2-weighted sequences and
prosthesis (Fig 14) (58). CT is useful contrast-enhanced high-spatial-resolution
for identifying causes of post-stapedec­ T1-weighted sequences through the
tomy failure (59). The most common temporal bone.
reason is migration or dislocation of the Retrofenestral otosclerosis.—Retro­
prosthesis. On CT images, the stapes fenestral otosclerosis rarely occurs in
implant may be abnormally rotated or isolation and is almost always seen in
displaced, the piston may protrude ex­ association with fenestral otosclerosis.
cessively into the vestibule (Fig 24), or Demineralization occurs extensively in
a gap may be seen between the native the otic capsule beyond the region of
incus and the stapes implant or between the fissula ante fenestram, typically in­ Figure 24:  Migrated stapes prosthesis. Sagittal
the stapes implant and the vestibule. volving the pericochlear region (cochlear oblique reformatted CT image demonstrates a sta-
The “lateralized piston syndrome” re­ capsule), but may extend to surround pes prosthesis abnormally protruding into the vesti-
fers to lateral migration of the implant the vestibule, semicircular canals, and bule (arrow).
piston out of the oval window related to internal auditory canal. Demineraliza­
necrosis of the incus. Incus erosion may tion may also be seen at the round win­
also occur if the implant is too long, dow niche, cochlear promontory, and

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Figure 25  Figure 26  Figure 27 

Figure 25:  Cochlear otosclerosis in a 44-year-old


man. Axial CT image demonstrates the characteris-
tic hypoattenuating “halo” around the cochlea (ar-
rows), representing the abnormal spongiotic bone in Figure 26:  Cochlear cleft in an 11-year-old boy. Figure 27:  Late phase of cochlear otosclerosis in
the middle layer of the otic capsule. Axial CT image shows a thin curvilinear lucency a 54-year-old man. Axial CT image shows thickened
around the cochlea (arrow), representing a non- sclerotic bone in the basal turn of the cochlea (black
pathologic entity referred to as the cochlear cleft, arrows), representing the sclerotic phase of otoscle-
and not cochlear otosclerosis. rosis. In this patient, there are still demineralized
tympanic facial nerve canal (60). Oc­ areas in the otic capsule visible (white arrow).
casionally, there may be isolated round
window involvement without oval win­
dow or pericochlear involvement (61). prevalent among infants and children Treatment for retrofenestral oto­
If there is involvement of the cochlear younger than 3 years. Among the el­ sclerosis is usually medical, with the
promontory (Fig 21), the promontory derly, Paget disease is an imaging mim­ use of fluorides or chelating agents to
may have a pink- or red-tinged appear­ icker of cochlear otosclerosis (50). In limit the growth of active otosclerotic
ance at otoscopic examination, termed the later phase of cochlear otosclerosis, foci and promote remineralization of
the Schwartze sign, reflecting vascular when the diseased middle layer of the the otosclerotic plaques (50,56). Pa­
proliferation and dilatation with increased otic capsule becomes sclerotic, the only tients with bilateral profound sensori­
blood flow to the promontory (62). finding on CT images may be mild ir­ neural hearing loss may be considered
On CT images, retrofenestral oto­ regularity in the outline of the cochlear for cochlear implantation, provided
sclerosis involving the cochlear capsule capsule without abnormal hypoattenu­ that there is no substantial ossification
manifests as a characteristic “halo” ation or other findings discernible at within the cochlea that may lead to sur­
around the cochlea, referred to as the CT (Fig 27) (64). Knowledge of the gical challenges and complications such
“double ring,” “fourth ring,” or “fourth patient’s clinical history is thus crucial as partial insertion and misplacement
turn” sign, representing the abnormal to the radiologist to help avoid a false- of electrode arrays (50).
hypoattenuating spongiotic bone in the negative interpretation.
endochondral layer of the otic capsule On MR images, retrofenestral oto­ Superior Semicircular Canal Dehiscence
(Fig 25). The finding is often bilater­ sclerosis can be seen as a ring of abnor­ The dense otic capsule surrounding the
ally symmetric (51). This should not be mal signal intensity in the pericochle­ membranous labyrinth normally has
mistaken for normal pericochlear hy­ ar and perilabyrinthine otic capsule, two mobile windows, the oval window
poattenuating foci, or “cochlear clefts” with intermediate signal intensity on and the round window. Abnormal de­
(Fig 26), seen in children with no ev­ T1-weighted images, and mild-to- hiscence of the bony covering of the
idence of otosclerosis and no history moderate enhancement on postcontrast membranous labyrinth at a third loca­
of osteogenesis imperfecta (63,64). T1-weighted images, seen especially in tion creates a “third window,” resulting
There are various theories as to what the active phase. Enhancement in the in abnormal movement of the endo­
the cochlear cleft represents (63–65), lateral wall of the inner ear in the re­ lymph that can lead to a variety of ves­
but it is widely believed to be related gion of the oval window and/or round tibular and cochlear symptoms induced
to normal variant development of the window indicates concurrent fenestral by sound and pressure stimuli, known
otic capsule, since the finding is highly otosclerosis (53). as the “third window phenomenon.”

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STATE OF THE ART: Imaging Review of the Temporal Bone Juliano et al

Figure 28  and abnormal communication of the


CSF space with the dehiscent supe­
rior semicircular canal by others (66).
When tested, there is a lower threshold
for elicitation of the vestibular-evoked
myogenic potential response in the af­
fected ear.
The vertical torsional nystagmus as­
sociated with the Tullio phenomenon,
the Hennebert sign elicited by the fistula
test, and vestibular-evoked myogenic
potential testing are highly sensitive and
specific for superior semicircular canal
dehiscence, but high-spatial-resolution
CT imaging of the temporal bone is
important for confirmation. On CT im­
ages, if an intact bony covering cannot
be visualized, superior semicircular
canal dehiscence can be suspected. It
is important to assess the bony covering
over all three semicircular canals as de­
hiscence over the lateral and posterior
semicircular canals is possible, although
Figure 28:  Superior semicircular canal dehiscence. (a) Pöschl and (b) Stenvers views from a CT study rare (70). Alternative pathologic condi­
reveal wide dehiscence of the bony covering over the superior semicircular canal (arrows). This 52-year-old tions that may account for conductive
patient presented with sound- and straining-induced vertigo. hearing loss should be sought, such as
middle ear mass, ossicular abnormality,
and fenestral otosclerosis. Studies have
This third window phenomenon is char­ inner ear malformations (68). Potential shown that the prevalence of superior
acterized by loss of acoustic energy and acquired causes include trauma, chronic semicircular canal dehiscence and the
abnormal stimulation of the vestibular otitis media with cholesteatoma, and in­ size of the defect are overestimated
system (66). Dehiscence of the bony creasing age and osteopenia, especially at CT, owing to difficulty detecting ex­
covering over the superior semicircular if the bony covering over the superior tremely thin but intact bone over the
canal is more common than that over semicircular canal is inherently thin or canal related to limitations in scanner
the lateral and posterior semicircular otherwise susceptible to external pres­ resolution and partial volume effect, re­
canals and is referred to as superior sure or other factors a priori (66,68). spectively (66,70–73). Submillimeter
semicircular canal dehiscence (Fig 28). Patients with superior semicircular (0.5 mm) section thickness with images
This was first described in 1998 by Mi­ canal dehiscence typically present in reformatted in planes parallel (Pöschl)
nor et al (67). The prevalence of su­ the 4th to 5th decade of life with ver­ and perpendicular (Stenvers) to the
perior semicircular canal dehiscence tigo and oscillopsia induced by loud plane of the superior semicircular canal
is not definitely known, as it varies sounds (Tullio phenomenon), eye move­ help to reduce false-positive rates and
depending on the method of diagnosis. ments induced by pressure in the exter­ improve specificity.
Based on cadaver examination, the prev­ nal auditory canal (Hennebert sign), or Surgery is generally reserved for
alence of superior semicircular canal vestibular signs and symptoms elicited patients with clinically important ves­
dehiscence is reported to be 0.5%; by Valsalva maneuvers (69). The eye tibular or auditory symptoms. The
based on high-spatial-resolution CT ex­ movements are typically in the same bony dehiscence can be surgically re­
amination, the prevalence is generally plane as that of the dehiscent semicircu­ surfaced, plugged, or capped by means
higher, ranging from 3% to 12% (68). lar canal. Patients may also experience of a transmastoid approach or middle
The cause of superior semicircular auditory symptoms such as conductive cranial fossa approach (66).
canal dehiscence is not well understood, hearing loss, autophony, and pulsatile
and in a majority of cases no clear un­ tinnitus. Another common clinical Large Vestibular Aqueduct Syndrome
derlying etiology is discovered. Congen­ symptom is abnormal amplification of Imaging is an important component in
ital anomalies are thought to be possible internal sounds of the body such as the the evaluation of pediatric sensorineu­
culprits, as superior semicircular canal heartbeat and eye movement, thought ral hearing loss for excluding structural
dehiscence has been reported in children to be related to increased sensitivity to abnormalities. At high-spatial-resolu­
and in patients with anomalies such as bone-conducted sounds by some (69) tion CT and MR imaging, abnormalities

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STATE OF THE ART: Imaging Review of the Temporal Bone Juliano et al

Figure 29  Figure 30  measured greater than 1.5 mm at the


midpoint at tomography. In 2007, Bos­
ton et al (77) and Vijayasekaran et al
(78) proposed that the vestibular aq­
ueduct should be considered enlarged
on CT images when it measures greater
than 1.9 mm at the operculum and/or
greater than 0.9 mm at the midpoint in
children (ages ranging from 3 years to
14 years in their study). These are now
the most commonly used measurement
criteria and are referred to as the Cin­
cinnati criteria. It is important to take
into account both the operculum and
midpoint measurements, as otherwise
the shape of the vestibular aqueduct
(eg, flared versus bulbous) may lead to
a falsely normal measurement at one
Figure 30:  LVA with modiolar deficiency and in- of the two locations when in fact it is
Figure 29:  LVA. Axial CT image shows enlarge- complete partition type II. Axial CT image depicts an clearly abnormally enlarged as a whole.
ment of the vestibular aqueduct (arrow). This enlarged vestibular aqueduct (black arrows), with CT offers excellent visualization of
11-month-old boy presented with sensorineural associated deficiency of the cochlear modiolus (ar- bone detail and hence configuration
hearing loss and has bilateral LVA. rowhead) and incomplete partition of the cochlea of the vestibular aqueduct, while MR
(IP-II) (white arrow). Similar findings are seen on the imaging using a heavily T2-weighted
contralateral side. This 9-year-old girl has bilateral sequence allows visualization of its con­
sensorineural hearing loss. tents, namely the endolymphatic duct
are identified in up to 37% of children and sac. High-spatial-resolution CT is
with previously unexplained sensori­ the traditional workhorse, but each
neural hearing loss (74). When CT im­ modality has relative strengths (76).
aging findings for pediatric sensorineu­ activities that may lead to increased MR imaging has the advantage of delin­
ral hearing loss are positive, the most intracranial pressure and encouraging eating cranial nerves, including the co­
common finding is a large vestibular the use of protective head gear when chlear nerve, and can offer some infor­
aqueduct (LVA) (Fig 29) (74). participating in moderate-risk activities mation on bony structures such as the
In 1978, Valvassori and Clemis (75) among this patient population (77,78). otic capsule and vestibular aqueduct
were the first to describe an associa­ It is unclear what the exact relationship based on the contour of the fluid within
tion between sensorineural hearing loss is between LVA and sensorineural hear­ them. The absence of ionizing radiation
and LVA, diagnosed at polytomography, ing loss; LVA may be a cause of sen­ in MR imaging is an important consid­
and coined the term “large vestibular sorineural hearing loss, or it may be a eration among the pediatric population.
aqueduct syndrome” to describe chil­ marker for an underlying process that On CT images, LVA typically has a
dren with LVA and sensorineural hear­ causes sensorineural hearing loss. It has flared morphology. Heavily T2-weight­
ing loss that is frequently progressive. been noted that LVA often occurs along­ ed MR imaging shows the enlarged
Symptoms of vestibular disturbance side other inner ear abnormalities, and endolymphatic sac and duct within
may also occur as part of this syndrome it is thought that most, if not all, LVAs the vestibular aqueduct (Fig 31). The
(76). Multiple studies since then using are associated with abnormalities of fluid in the enlarged endolymphatic sac
CT as the imaging modality have esti­ the membranous labyrinth (74). There is often hypointense to CSF owing to
mated the prevalence of LVA among pa­ is an association of LVA with modiolar its very high protein content (79). It
tients with sensorineural hearing loss to deficiency (79), an anomaly where the is important to examine the cochlear
be anywhere from 0.64% to 32% (77). modiolus (cone-shaped bone in the cen­ modiolus for abnormally decreased
The probability of progressive hearing ter of the cochlea) is abnormally low volume to exclude an associated mo­
loss increases linearly with increasing in volume (Fig 30). Some cases of LVA diolar deficiency. Funnel-shaped LVA
width of the vestibular aqueduct. In have a genetic cause, with one study re­ with incomplete partition type II (IP-
children with LVA, sudden sensorineural porting 27% of LVA patients having the II) or modiolar deficiency is associated
hearing loss has been reported to occur SLC26A4 gene mutation (80). with mutations in the SLC26A4 gene
following minor head trauma, leading In the initial description by Valvas­ that encodes for the pendrin protein
to the clinical practice of recommend­ sori and Clemis, the vestibular aque­ (Fig 30). SLC26A4 gene mutations are
ing against contact sports or other duct was considered enlarged when it seen in more than 50% of patients with

Radiology: Volume 276: Number 3—September 2015  n  radiology.rsna.org 669


STATE OF THE ART: Imaging Review of the Temporal Bone Juliano et al

Figure 31  findings and that radiologists may eas­ 12. Swartz JD, Zwillenberg S, Berger AS. Ac­
ily miss or underdiagnose if they are quired disruptions of the incudostapedial
articulation: diagnosis with CT. Radiology
unaware of the precise locations to in­
1989;171(3):779–781.
spect on imaging studies. Although not
as common as infection or inflamma­ 13. Ederies A, Yuen HW, Chen JM, Aviv RI,

Symons SP. Traumatic stapes fracture with
tion, these entities are nevertheless im­
rotation and subluxation into the vestibule
portant for the radiologist to recognize, and pneumolabyrinth. Laryngoscope 2009;
especially since they have very charac­ 119(6):1195–1197.
teristic imaging appearances that can
14. Malis DJ, Magit AE, Pransky SM, Kearns
point at the specific diagnoses, confer­
DB, Seid AB. Air in the vestibule: computed
ring on radiologists an important role in tomography scan finding in traumatic peri­
the clinical evaluation of these patients. lymph fistula. Otolaryngol Head Neck Surg
1998;119(6):689–690.
Disclosures of Conflicts of Interest: A.F.J.
disclosed no relevant relationships. D.T.G. dis­ 15.
Chien W, McKenna MJ, Rosowski JJ,
closed no relevant relationships. G.M. disclosed Merchant SN. Isolated fracture of the ma­
no relevant relationships. nubrium of the malleus. J Laryngol Otol
2008;122(9):898–904.

16. Swartz J, Kang M. Trauma to the temporal


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