Imaging of

Hearing Loss
Lubdha M. Shah, MDa,*, Richard H.Wiggins III, MDb,c,d

KEYWORDS
 Hearing loss  Conductive  Sensorineural
 Mixed  Temporal bone

Physical and radiologic examinations are crucial
and complementary in the evaluation of hearing
loss. In addition to imaging evaluation of patients
with hearing loss, a complete physical examina-
tion should be performed, including an accurate
history of not only the hearing loss itself (unilateral
or bilateral, slow onset or fast onset) but also the
patient’s history (possible trauma, ototoxic drugs,
associated facial nerve palsies). Hearing involves
three main components: the external ear, the
middle ear, and the inner ear. The external ear
collects and directs sound. The middle ear
converts the sound waves into mechanical motion,
and the inner ear transforms this vibratory motion
into electrical energy (ie, nerve impulses). A variety
of pathologies can affect one or more of these
parts, leading to hearing loss. Hearing loss can
be classified as conductive, sensorineural, or
mixed, and these categories can be congenital or
acquired in etiology. Modern imaging of these
structures with CT and MR imaging is fundamental
in the diagnosis and treatment of these cases. As
with many skull base pathologies, these modalities
are complementary, with specific strengths
contributing to the evaluation of hearing loss.
In this article, we review the common entities
causing these clinical presentations and divide
these pathologies into conductive hearing loss
(CHL), sensorineural hearing loss (SNHL), and
mixed hearing loss (MHL). With this format, congen-
ital pathologies are addressed first followed by the
acquired entities. We review the common patholo-
gies and their imaging findings of each section
affecting the hearing pathways from outside to
inside, starting at the external auditory canal (EAC)
and moving inward toward the midbrain.
IMPORTANT ANATOMY
Knowledge of the anatomy of the temporal bone
and all the components of hearing (ie, the external,
middle, and inner ear) is fundamental to diagnosing
pathology. The EAC has a fibrocartilaginous and an
osseous component through which sound waves
are funneled. Sound vibrates the tympanic
membrane, which separates the EAC from the
middle ear. The malleus, which is attached to the
tympanic membrane, also vibrates. The mechanical
energy of the vibrations is transmitted through the
malleoincudal and incudostapedial articulations to
the oval window. The oval window is the interface
between the air-filled middle ear cavity and the
fluid-filled inner ear. The otic capsule surrounds
the membranous labyrinth of the inner ear. The latter
is composed of neural elements and the fluid
compartments of the cochlea, vestibule, and the
semicircular canals. The vestibule and semicircular
canals convey information about movement.
Mechanical energy is transduced into electric
impulses within the cochlea, specifically within
the organ of Corti, scala tympani, scala vestibuli,
and scala media. When the stapes footplate

a
Department of Radiology, University of Utah Health Care, 30 North, 1900 East, #1A71, Salt Lake City, UT
neuroimaging.theclinics.com

84132-2140, USA
b
Department of Radiology, University of Utah Health Care, 30 North, 1900 East, #1A71, Salt Lake City, UT
84132-2140, USA
c
Department of Otolaryngology, Head and Neck Surgery, University of Utah Health Care, 30 North, 1900 East,
#1A71, Salt Lake City, UT 84132-2140, USA
d
Department of Biomedical Informatics, University of Utah Health Care, 30 North, 1900 East, #1A71, Salt Lake
City, UT 84132-2140, USA
* Corresponding author.
E-mail address: lubdha.shah@hsc.utah.edu (L.M. Shah).

Neuroimag Clin N Am 19 (2009) 287–306

doi:10.1016/j.nic.2009.06.010
1052-5149/09/$ – see front matter ª 2009 Elsevier Inc. All rights reserved.
288 Shah & Wiggins
vibrations compress the oval window, the fluid
within the cochlea moves and bends the neuroepi-
thelial hair cells of the organ of Corti. This move-
ment leads to depolarization of the hair cells and
electrical impulses are sent to the afferent neurons
of the spiral ganglion of the cochlea.1 The cochlea
is organized by pitch such that high pitch is de-
tected in the basal turn and low pitch is detected
in the apical turn. The cochlear division of cranial
nerve VIII is formed from the neurons of the spiral
ganglion and maintains its ‘‘cochleotopic’’
arrangement of nerve fibers by pitch.2 When eval-
uating a patient who has hearing loss, similar to
any cranial nerve evaluation, the entire course of
the cranial nerve VIII, including the restiform
body of the medulla (area of cochlear nuclei),
cisternal segment in the cerebellopontine angle–
internal auditory canal (CPA-IAC) cistern and the
membranous labyrinth should be included. From
the brainstem nuclei, the nerve impulses travel to
the ipsilateral and contralateral superior olivary
complexes, lateral lemniscus, inferior colliculus,
medial geniculate body, and auditory cortex in
the superior temporal gyri.
IMAGING TECHNIQUES
CT and MR imaging are complementary modalities
in the evaluation of hearing loss. CT of the
temporal bones demonstrates the cochlea, vesti-
bule, ossicles, oval and round window niches, fis-
sula ante fenestram, and vestibular aqueduct. MR
imaging is superb for evaluating the membranous
labyrinth, the cranial nerves, and the adjacent
brain parenchyma. Modern CT is performed with
the patient in the supine position in the anatomic
axial plane. Recent advances with multidetector
CT scanners include the ability to acquire submil-
limeter slices with low radiation dose and reduced
scan time. Submillimeter (0.4–0.6 mm) multiplanar
reconstructions can be performed with minimal
overlap (0.5 mm). In the setting of trauma, osteo-
sclerosis, and Paget’s disease, CT is of particular
importance in evaluating osseous integrity (Fig. 1).
In addition to the standard axial views with
coronal reformations, short axis and long axis
(Poschl and Stenver projections) can be included
easily with diagnostic CT temporal bone evalua-
tion (Figs. 2 and 3). These reformations can be
completed independent of the radiologist by the
CT technologist with minimal training. Bone algo-
rithm CT is the modality of choice in demonstrating
inner ear dysplasias in children who have with
SNHL. MR imaging is complementary to CT in
the evaluation of hearing loss. Dedicated head
and temporomandibular joint coils are important
for achieving optimal signal. Screening high-
Fig. 1. Axial CT demonstrates longitudinal fracture
through the right temporal bone. The fracture line
extends through the vestibule and the oval window.
There is fluid in the middle ear cavity and the mastoid
air cells.
resolution thin-section T2 MR through the CPA-
IAC is useful for uncomplicated SNHL (Fig. 4).
For complicated SNHL cases that include other
symptoms such as cranial neuropathy, long tract
signs, and headache, imaging should include
whole brain and posterior fossa sequences (axial
T2 and/or FLAIR). Additional thin-section coronal
and axial T1 fat saturated, contrast-enhanced
MR imaging through the posterior fossa and
CPA-IAC is helpful. The CPA-IAC protocol
includes thin section heavily T2-weighted
sequences (eg, constructive interference steady
state and three-dimensional FIESTA).
It is important to be aware of common imaging
pitfalls. Enhancement of a prominent anterior infe-
rior cerebellar artery loop may be mistaken for
a small vestibular schwannoma.3 Choroid plexi
protruding through the lateral recesses of the
fourth ventricle bilaterally can appear as tear-
shaped enhancing masses in the CPA cisterns.
The cerebellar flocculus normally projects into
the posterolateral aspect of the CPA cistern.4
With fat saturated postcontrast T1-weighted
images, the marrow can enhance and be errone-
ously interpreted as an IAC tumor, and asymmetric
aeration of the petrous apices with unilateral
benign trapped fluid can be mistaken for
pathology (Fig. 5).
The stellate ganglion in the IAC may be mistaken
for a small vestibular schwannoma, so it is important
to be wary of reporting ‘‘very small IAC schwanno-
mas.’’ Most of these masses can be safely followed
over time without sending a neuro-otologist to
 Imaging of Hearing Loss 289

Fig. 2. Axial CT (left) shows orientation of the reformations for the short axis (Poschl) projection (right). Training
of a technologist to simply ‘‘connect the dots’’ of the superior semicircular canals can produce these projections.

chase a 2-mm possible schwannoma only to find
normal vasculature.
CONDUCTIVE HEARING LOSS
CHL may be caused by transmission fixation or trans-
mission discontinuity. Important anatomy to assess
includes the EAC, tympanic membrane, ossicular
chain, and oval window. In CHL, there is likely to be
a CT abnormality. If upon initial review there is no
abnormality, the radiologist should look again. MR
imaging findings depend on the pathology, such as
inflammatory disorders or tumors.
Congenital Conductive Hearing
Loss Pathologies
Congenital CHL pathologies include external audi-
tory atresia, oval window atresia, ossicular anom-
alies (eg, malleoincudal fixation, stapes fixation,
and incudostapedial dislocation), congenital cho-
lesteatoma, and fenestral otosclerosis (rare in
childhood).5 EAC atresia can be partial or
complete with associated auricle deformity. The
severity of the auricular dysplasia parallels the
degree of deformity of the middle ear and ossi-
cles.6 The spectrum of this congenital malforma-
tion ranges from the mildest form with a
narrowed EAC to no identifiable EAC because of
bone, soft tissue, or mixed components. Hypo-
plasia of the middle ear and mastoid complex
may be present. The ossicular chain may be dys-
morphic; for example, the malleus and incus may
be fused or rotated (see Fig. 5A).
Congenital cholesteatoma can be seen in up to
10% of these cases.6 The facial nerve has an aber-
rant course and may exit the skull base into the gle-
noid fossa or lateral to the styloid process. The
tympanic segment may be dehiscent, bowing into

Fig. 3. Axial CT (left) shows orientation of the reformations for the long axis (Stenvers) projection (right). The
technologist can simply create a small stack perpendicular to the short axis series to create these projections.
290 Shah & Wiggins
Fig. 4. Axial (left) and coronal (right) high-resolution thin-section T2 MR through the CPA-IAC shows the seventh
and eighth nerve complexes and the normal bright signal in the IACs and membranous labyrinth. (Courtesy of
Amirsys, Inc., Salt Lake City, UT; with permission.)
the middle ear cavity, or it may overlie the oval
window, especially in cases of oval window atresia.
The descending (or mastoid) segment of the facial
nerve is often anteriorly displaced with EAC atresia
cases. The inner ear and IAC are usually normal. In
unilateral cases, the affected side shows CHL and
the other ear has normal hearing. In every EAC
atresia case, a thorough evaluation needs to
address the type and thickness of the atretic plate,
the size of the mastoid complex and middle ear
cavity, status of the ossicular chain, status of the
oval and round windows, the course of the facial
nerve, possible presence of a cholesteatoma, and
the status of the inner ear.7
In oval window atresia, there is an absent
cleavage plane between lateral semicircular canal
above and cochlear promontory below with an
associated anomalous stapes and malpositioned
facial nerve.8 The oval window is covered by
a thin bony plate in these cases (Fig. 6). The stapes
superstructure and distal incus are usually mal-
formed with oval window atresia. There is absence
of the normal paired crura of the stapes. The
tympanic segment of the facial nerve (cranial nerve
VII) is inferomedially positioned overlying the
expected position of the oval window, which
complicates surgical repair.1 Embryologically, it
is hypothesized that the primitive stapes fails to
fuse with the primitive vestibule during the seventh
week of gestation.9 Congenital stapes fixation
results if the stapes forms but the annular ligament
does not. In malleoincudal fixation, the malleus
head and incus body are fused or fixed to the epi-
tympanic wall (Fig. 7).
Congenital cholesteatoma is suggested by the
presence of a homogenous mass of soft tissue
attenuation within the middle ear on CT scans
with osseous destruction (Fig. 8). They occur in
the mesotympanum around the incudostapedial
joint manifesting as unilateral CHL.10 Small choles-
teatomas are encapsulated masses in the anterior
tympanic cavity. Larger congenital cholesteato-
mas may erode the ossicles, the middle ear wall,
the lateral semicircular canal, and the tegmen
tympani. Bone erosion is less common than with
acquired cholesteatomas and occurs late in the
disease course.11 The long process of the incus
and the stapes superstructure are the most
commonly destroyed. The lesion is iso- to hypoin-
tense on T1-weighted imaging and intermediate
on T2-weighted imaging and may show peripheral
enhancement.
A persistent stapedial artery can cause CHL and
may be found in association with an aberrant
internal carotid artery. In the embryo, the stapedial
artery runs through the stapes and regresses by
the third month of gestation.12 A persistent stape-
dial artery courses across the floor of the middle
ear and cochlear promontory and passes between
the crura of the stapes, where it can present with
CHL (Fig. 9). The lack of regression of this anoma-
lous vessel may replace the normal blood supply
of the middle meningeal artery, leading to an ipsi-
lateral absence of the foramen spinosum postero-
lateral to foramen ovale on CT of the skull base.13
Fibrous dysplasia is a rare cause of congenital
CHL.14 Bone CT shows increased bone volume
with a ‘‘ground glass’’ appearance (see Fig. 10).
 Imaging of Hearing Loss 291

Fig. 5. Axial T2 and axial CT demonstrate asymmetric aeration of the petrous apices. (A) Fluid in the right petrous
apex is hyperintense on the T2 MR image and can be mistaken for pathology. (B) Axial CT image of EAC atresia
illustrates a hypoplastic middle ear cavity with dysmorphic ossicles (yellow arrow). The facial nerve has an aber-
rant course (red arrow). The auricle is deformed. (Courtesy of Amirsys, Inc., Salt Lake City, UT; with permission.)

There is osseous expansion of the temporal bone
with relative sparing of the otic capsule. In this
slowly progressing, inherited bony disease, fibro-
osseous replacement of bone occurs. Although
there is usually CHL or EAC bony stenosis, hearing
loss also may be sensorineural or mixed. Cystic,
pagetoid, and sclerotic appearances represent
the phases of the disease in descending order of
activity.15
Acquired Conductive Hearing
Loss Pathologies
Causes of acquired CHL include EAC pathologies,
neoplasm, trauma, tympanosclerosis, effusion,
malleus fixation, acquired cholesteatoma, and
fenestral otosclerosis. EAC exostoses are broad-
based osseous masses associated with chronic
irritation and classically described in cases of
repeated cold water exposure, such as in surfers
(see Fig. 10A). They cause bony stenosis of EAC
with increased wall thickness and bone density.16
Cholesteatoma of the EAC caused by accumula-
tion of squamous epithelium can rarely present
with CHL. This EAC lesion is composed of exfoli-
ated keratin within stratified squamous epithelium
and is usually unilateral. It usually occurs in individ-
uals aged 40 years and older and tends to be an
isolated disease process. On imaging, this EAC
mass shows bony erosion with adjacent scallop-
ing. Bony flecks appear in approximately 50% of
cases (Fig. 11).17
In keratosis obturans, desquamated keratin
debris fills the EAC causing CHL. This inflamma-
tory process usually occurs in individuals younger
than age 40 years and is usually bilateral. It is asso-
ciated with bronchiectasis and chronic sinusitis.
Imaging shows a benign keratin plug occluding
292 Shah & Wiggins
Fig. 6. Coronal reformatted image through the IAC
shows a thin bony plate (yellow arrow) covering the
oval window. (Courtesy of Amirsys, Inc., Salt Lake
City, UT; with permission.)
the EAC without bony erosion (Fig. 12).18 Medial
canal fibrosis is the formation of fibrous tissue in
medial EAC. The classic imaging appearance is
of a fibrous crescent in medial EAC covering the
external surface of the tympanic membrane (see
Fig. 12A). Approximately 60% of cases have
bilateral involvement.19 Rarely, squamous cell
carcinoma and ceruminous adenomas can involve
the EAC, possibly leading to CHL on presentation.
Fractures through the temporal bones are classi-
cally described as longitudinal, transverse, and
Fig. 7. Axial CT shows ossified fixation of the malleus
to the epitympanum (yellow arrow). (Courtesy of
Amirsys, Inc., Salt Lake City, UT; with permission.)
Fig. 8. Axial CT demonstrates a homogenous mass of
soft tissue attenuation in the middle ear adjacent to
the ossicles (yellow arrow). There is erosion of the
medial margin of the incus. (Courtesy of Amirsys,
Inc., Salt Lake City, UT; with permission.)
oblique, depending on their relationship to the
petrous apex. Longitudinal fractures are along the
long axis of the petrous portion of the temporal
bone and comprise approximately 80% of
temporal bone fractures.14 These are more likely
to involve the ossicles and result in CHL (Fig. 13).
Fig. 9. Coronal CT of the temporal bone illustrates an
aberrant internal carotid artery (white arrow) and
an associated persistent stapedial artery (black arrow).
The persistent stapedial artery passes through the
stapes crura and travels along the tympanic segment
of the facial nerve (Courtesy of Amirsys, Inc., Salt
Lake City, UT; with permission.).

Fig.10. (A) Axial CT of the right temporal bone shows bony exostoses narrowing the EAC. (B) Axial bone algorithm
CT illustrates expansion and ground glass appearance of the right temporal bone. There is relative sparing of otic
capsule. (Courtesy of Amirsys, Inc., Salt Lake City, UT; with permission.)
Approximately 20% of longitudinal temporal bone
fractures are associated with facial nerve injury.20
Tympanic membrane rupture and hemotympanum
also may occur. Longitudinal fractures of the
temporal bone that disrupt the tympanic
membrane may allow an ingrowth of squamous
epithelium that also can lead to an acquired choles-
teatoma.21 Transverse fractures are perpendicular
to long axis of petrous portion and are more likely
to involve the bony labyrinth to result in SNHL.
Approximately 50% of transverse fractures are
associated with facial nerve injury.20
Tympanosclerosis is a healing variant of chronic
otitis media characterized by deposition of hyali-
nized collagen in the tympanic cavity. The suspen-
sory ligaments and tendons may ossify or calcify.
The tympanosclerosis surrounding the ossicles
can make it appear as if there are extra ossicles
or ‘‘hairy ossicles’’ (Fig. 14). This postinflammatory
new bone deposition is seen in the tympanic
membrane, middle ear, ossicles, mastoids, and
oval and round windows. In contradistinction to
fenestral otospongiosis, new bone deposition
within the oval window is irregular. A calcified
degenerative stapes footplate arthritis may mimic
this disorder.22 Recurrent inflammation and heal-
ing lead to calcification of the tympanic membrane
(myringosclerosis), which can cause CHL.23
Middle ear effusions may impede the movement
of ossicles and decrease tympanic membrane
compliance.24 Fluid in the middle ear and mastoid
air cells is easily demonstrated on CT and MR
examinations; however, infected effusions are
a clinical distinction. Myringotomy or tympanos-
tomy tubes allow middle fluid to drain into the
Imaging of Hearing Loss 293
EAC. Pathology of the ossicles also can result in
hearing loss. Malleus fixation can be idiopathic,
posttraumatic, developmental, or postinfectious
secondary to chronic otitis media (see Fig. 14A).
Syphilis can cause adhesions between the foot-
plate and oval window, which can be difficult to
detect on CT.25
Chronic otitis media can lead to middle ear atel-
ectasis with retraction of the tympanic membrane
onto the ossicles or cochlear promontory. In cases
that are longstanding, there may be erosion of the
incus and stapes with pockets of desquamated
keratin and epithelium in which acquired choles-
teatomas can develop.21 Acquired cholesteato-
mas also can occur when there is invasion of
squamous epithelium of the EAC through a perfo-
ration in the tympanic membrane and when there
is squamous metaplasia of inflamed middle ear
epithelium.26 The pars flaccida is the anterior and
superior aspect of the tympanic membrane. A
Fig. 11. Coronal CT illustrates a soft tissue mass with
bony erosion (yellow arrows) along the inferior EAC,
with bony flecks, consistent with EAC cholesteatoma.
(Courtesy of Amirsys, Inc., Salt Lake City, UT; with
permission.)
294 Shah & Wiggins

Fig. 12. (A) Coronal CT through the right temporal bone shows soft tissue within the EAC covering the tympanic
membrane, with a lateral cresent, consistent with medial canal fibrosis. (B) Coronal CT reformations of the
temporal bones show benign soft tissue masses in the bilateral EACs without bony erosions. These are keratin
plugs of keratosis obturans. (Courtesy of Amirsys, Inc., Salt Lake City, UT; with permission.)

perforation in the pars flaccida allows squamous
epithelium access to Prussak’s space, which is
the recess between the ossicles and the lateral
wall of the epitympanum (or attic). Careful atten-
tion should be paid to the scutum along the roof
of the EAC, because erosion of this bony ‘‘spur’’
is one of the earliest abnormalities of a cholesteato-
ma to be detected on CT, particularly on coronal
images (Fig. 15A).
From Prussak’s space, the cholesteatoma may
erode the ossicles or medially displace them. It
can extend through the middle ear into the aditus
ad antrum and into the mastoid antrum. In addition
to ossicular destruction, other complications of
cholesteatomas include facial nerve paralysis
and labyrinthine fistula. Erosion of the tegmen
tympani can occur; evaluation of temporal lobe
for possible herniation is important. This superior
bony erosion also can result in intracranial compli-
cations such as meningitis, thrombosis, or
abscess. CT with soft tissue algorithm may be
helpful; however, coronal MR, especially thin
section T2-weighted images, can best delineate
herniated brain parenchyma. SNHL may result if
there is erosion into the labyrinth that causes
a labyrinthine fistula. The cholesteatoma may drain
spontaneously or erode into the EAC, possibly
leading to an automastoidectomy. The radiologic
sine qua non of a cholesteatoma is bone erosion
(see Fig. 15). Acquired cholesteatoma of the pars
tensa can extend into the sinus tympani and is
usually medial to the ossicles (Fig. 16).
Fenestral otosclerosis (or otospongiosis) is
a pathologic condition of the perifenestral bony
labyrinth of unknown cause. Spongy bone foci
appear as abnormal bony density localized
anterior to the oval window, at the fissula ante fen-
estram,27 early in the disease (Fig. 17). The oval
window may appear too wide (osteoclastic resorp-
tion). Late in the course of the disease, there is
heaped up new bone along the oval and round
window margins on CT and plugging of oval window
(obliterative otosclerosis). On enhanced contrast-
enhanced MR imaging, punctate enhancing foci
may be present in the medial wall of the middle
ear cavity in the active phase. Fenestral otospon-
giosis begins at the fissula ante fenestram and
spreads posteriorly along the oval window
margins toward the round window. Involvement
of the otic capsule indicates cochlear otospongio-
sis. Stapes footplate fixation to the oval window
results in progressive CHL, usually bilaterally.
Seventy percent of cases are inherited (autosomal
dominant with incomplete penetrance); the
remaining are sporadic.23 Fenestral otospongiosis
(85%) is more common than cochlear otospongio-
sis (15%).28 In fenestral otospongiosis there is
progressive CHL. In 70% of cases, patients have
early tinnitus, and most cases are bilateral (85%).
Fenestral otospongiosis is always present in cases
of cochlear otosclerosis, but the cochlear type is
present in less than 15% of cases of fenestral oto-
spongiosis.29 Cochlear otospongiosis (retrofenes-
tral) presents clinically with progressive SNHL.
Fig.13. Axial CT shows a longitudinal fracture through
the left temporal bone (black arrowhead) with hemo-
tympanum in a patient with trauma and hearing loss.
 Imaging of Hearing Loss 295

Fig.14. (A) CT of the right temporal bone in an obliqued coronal plane shows an angulated incus, which is abnor-
mally attached to the lateral tympanic wall (white arrow). (B) Coronal CT depicts tympanosclerosis surrounding
the ossicles, which appear indistinct. There is soft tissue filling the middle ear cavity. (Courtesy of Amirsys, Inc.,
Salt Lake City, UT; with permission.)

SENSORINEURAL HEARING LOSS In addition to CT of the temporal bones, thin section

SNHL indicates dysfunction of the cochlea,
cochlear nerve, and brain and can be measured
with a tuning fork touched to the top of the head,
by conduction through bone, so that the sound
localizes to the normal side in these patients. The
structures of note include the cochlea and the
cochlear nerve from the origin nucleus through
the CPA-IAC cistern into the membranous labyrinth.
MR imaging with T1-weighted pre- and postcon-
trast with fat saturation sequences, T2-weighted
sequences, and three-dimensional constructive
interference steady state/three-dimensional T2
fast spin echo/three-dimensional fourier transform
(FT) constructive interference steady state
sequences are essential in the thorough evaluation
of SNHL.

Fig.15. (A) Coronal CT shows a soft tissue mass in Prussak’s space eroding the scutum (yellow arrow). (B) Axial CT
of an acquired cholesteatoma shows bony erosive changes along the anterior margin of the epitympanum (red
arrow) and of the ossicles (yellow arrow). Cholesteatoma of the pars flaccida is the most common type and is
usually lateral to ossicles. (Courtesy of Amirsys, Inc., Salt Lake City, UT; with permission.)
296 Shah & Wiggins
Fig. 16. Coronal CT illustrates a homogenous soft
tissue mass with the medial aspect of the left middle
ear cavity in keeping with a cholesteatoma of the
pars tensa. (Courtesy of Amirsys, Inc., Salt Lake City,
UT; with permission.) There is sparing of Prussak’s
space.
Congenital Sensorineural Hearing
Loss Pathologies
Congenital SNHL etiologies include membranous
labyrinthine dysplasias, cochlear/vestibular apla-
sia/dysplasia, enlarged vestibular aqueduct, IAC
atresia/stenosis, and perilymphatic fistula. Radio-
graphic abnormality is seen in 20% of cases.
Genetic anomalies cause approximately half of
Fig. 17. Axial CT shows radiolucent bone anterior oval
window at the fissula ante fenestram (yellow arrow)
in this case of early fenestral otospongiosis.
the cases of congenital SNHL.1 Although the
cause is unknown in 25% to 40% of cases,30 intra-
uterine toxin exposure, infections, and perinatal
insults account for many cases pediatric SNHL.1
The spectrum of inner ear anomalies ranges
from complete aplasia to subtle dysplasia. Laby-
rinthine aplasia (also known as Michel deformity)
is the complete absence of the cochlea, vestibule,
and semicircular canals (Fig. 18). In cases of laby-
rinthine aplasia, there is SNHL from birth, and
cochlear implantation in the affected ear is not
an option. The bony labyrinth is featureless, and
the petrous apex may be hypoplastic or absent.
The IAC may be small or absent. The middle ear
may be normal in the mild form to abnormal with
fused ossicles in the more severe form. The lateral
wall of the inner ear is flat. The geniculate ganglion
is posterior to its normal expected location.31
There is no normal high signal intensity fluid in
the membranous labyrinth on high-resolution T2
MR imaging. Labyrinthine aplasia occurs when
there is arrest of the otic placode development at
the third gestational week. It may be associated
with Klippel-Feil syndrome or thalidomide
exposure.
Cochlear aplasia occurs in late third week arrest
with absent cochlea, dysmorphic vestibule, and
semicircular canals (Fig. 19).32 The vestibule and
IAC may be dilated. The cochlear promontory is
flat. The labyrinthine, geniculate ganglion, and
anterior tympanic portions of the facial nerve are
located where the cochlea should be. The EAC,
middle ear, ossicular chain, bony vestibular
Fig. 18. Axial CT depicts bilateral labyrinthine aplasia
in this patient with associated skull base dysplasia.
(Courtesy of Amirsys, Inc., Salt Lake City, UT; with
permission.)
 Imaging of Hearing Loss 297

Fig. 19. Axial CT image (left) show a dysplastic vestibule-semicircular canal and a small IAC. (Courtesy of Amirsys,
Inc., Salt Lake City, UT; with permission.) The cochlea is absent. Fluid signal is seen on the axial T2 MR (right) in the
dysplastic vestibule-semicircular canal.

aqueduct, and endolymphatic duct are normal in anomalies. The embryologic insult occurs at 6 to
size. If there is an arrest in the fourth week after 8 weeks’ gestation. In addition to SNHL, there
differentiation of the otic placode into the may be CHL caused by oval window atresia and
otocyst, a common cavity deformity occurs and ossicular chain anomalies.35 It can be sporadic
an ovoid single inner ear cavity is all that is formed. or associated with congenital syndromes (eg,
The semicircular canals are usually absent but CHARGE, Alagille, Waardenburg, Crouzon, Apert
may be present and dysplastic.32 The IAC size is syndromes). In CHARGE syndrome for instance,
concordant with the size of the common cavity there is absence of the semicircular canal bilater-
and enters the cavity at the center portion. The ally, dysmorphic vestibule, and cochlear anoma-
modiolus is absent on high resolution T2 MR lies with absence of the cochlear nerve canal.36
imaging (Fig. 20).33
Oblique sagittal T2 images through the IAC are
important to assess the presence of the cochlear
nerve if cochlear implantation is planned
(Fig. 21).33 A cystic cochleovestibular anomaly
results if there is arrest in the fifth gestational
week. A ‘‘figure-of-eight’’ cystic cochlea and vesti-
bule with semicircular canal dysplasia is seen
(Fig. 22). There are no internal features and no
modiolus is identified in these cases.34 The vesti-
bule is cystic and dilated.35 The semicircular
canals are variable in shape and degree of dilata-
tion.35 The IAC is normal in size with a normal facial
nerve; however, the cochleovestibular nerves are
deficient or absent. The EAC, middle ear, ossicular
chain, bony vestibular aqueduct, and endolym-
phatic duct are normal in size.
Semicircular canal dysplasia is a spectrum of
congenital deformities (Fig. 23). The most
common and least severe dysplasia in this spec-
trum is a common cavity formed by the lateral
semicircular canal and the vestibule. There is Fig. 20. Axial thin section T2 image illustrates a case of
associated oval window atresia. The cochlea cochlear dysplasia with absence of the central modio-
may be affected with incomplete partition of the lus. (Courtesy of Amirsys, Inc., Salt Lake City, UT; with
apical and middle turns. There may be ossicular permission.)
298 Shah & Wiggins

Fig. 21. Sagittal oblique T2-weighted MR image

demonstrates the normal structures with the IAC.
(F, facial nerve; C, cochlear nerve; S, superior vestib-
ular nerve; I, inferior vestibular nerve; AICA, anterior
inferior cerebellar artery).

Large endolymphatic sac anomaly with cochlear Fig. 23. Top coronal CT shows normal lateral semicir-
dysplasia is seen when arrest occurs in the seventh cular (yellow arrow), and bottom coronal CT shows
gestational week. This is the most common a dysplastic lateral semicircular canal (red arrow), in
congenital inner ear anomaly found by imaging a case with associated cohlear dysplasia. (Courtesy of
and is bilateral in 90% of cases.37 It is a familial Amirsys, Inc., Salt Lake City, UT; with permission.)
lesion with autosomal recessive inheritance. There
is an association with Pendred syndrome, which is
semicircular canal, so it is easy to simply compare
severe SNHL with thyroid pathology. Patients are
the diameter of the sac to the lateral and posterior
able to hear at birth, but their hearing deteriorates
semicircular canals, which should be larger than
over the early years of life. Typical clinical presenta-
the aqueduct.37 There is no relationship between
tion involves a child or teenager with progressive
the size of the endolymphatic sac and the severity
SNHL. There may be posttraumatic potentiation
of the SNHL. Although the aqueduct itself is best
of SNHL. It is the most commonly missed cause
evaluated with CT, the bright signal intensity on
of congenital deafness. Rarely, patients have distal
T2 within the sac is best seen on thin T2-weighted
renal tubular acidosis that results in varying
degrees of metabolic acidosis.
The criteria for determining an enlarged vestib-
ular aqueduct (in which the endolymphatic sac
lies) are vague. Bony vestibular aqueduct diameter
measures of more than 1.5 mm in the transverse
dimension at the midpoint or an opercular
measurement of more than 2 mm is generally
considered to be the defining characteristics
(Fig. 24).38,39 The vestibular aqueduct is usually
found at the level of the vestibule and lateral

Fig. 24. Axial CT demonstrates an enlarged vestibular

Fig. 22. Coronal CT shows dysmorphic cochlea bilater- aqueduct. It is larger in size than the ipsilateral semi-
ally. The IACs are diminutive in size. circular canal.

MR imaging. In more than 75% of cases, there is
an associated cochlear dysplasia.37 The apical
turn of the cochlea in these cases is dysmorphic
with modiolar deficiency.40 High-resolution T2
MR imaging may be able to distinguish more
subtle abnormalities of scalar chamber asymmetry
with the more anterior scala vestibuli larger than
the more posterior scala tympani.41 Approximately
50% of cases have associated vestibular or semi-
circular canal anomalies (Fig. 25).
In patients with sudden hearing loss, studies
have shown wider vestibular aqueducts in the
affected ear as compared with controls.42 The
endolymphatic sac can show enhancement,42,43
which may be caused by inflammation of the
endolymphatic tissue or venous engorgement. It
is hypothesized that a wide vestibular aqueduct
may be associated with insufficient maturation of
the inner ear.44 The congenital ‘‘fragile’’ inner ear
may receive abnormal pressure transmission
through the vestibular aqueduct.45
Acquired Sensorineural Hearing
Loss Pathologies
Acquired SNHL etiologies include tumors, infec-
tious/inflammatory processes, trauma, autoim-
mune/immune disorders, and degenerative/
idiopathic processes. Tumors can cause
SNHL, including vestibular (acoustic) schwanno-
mas (60%–90%),46 meningiomas (3%–6%),47
epidermoids (3%–6%),48 schwannomas of cranial
nerves V, VII, IX, X, XI, and XII, lymphoma,
Fig. 25. Axial T2 MR depicts an enlarged endolym-
phatic sac. (Courtesy of Amirsys, Inc., Salt Lake City,
UT; with permission.) There is also subtle abnormality
of the scalar chambers of the cochlea.
Imaging of Hearing Loss 299
leukemia, metastases,49 and meningeal carcino-
matosis. A vestibular schwannoma is a benign
tumor arising from Schwann cells that wrap the
vestibulocochlear nerve in the CPA-IAC, more
often from the vestibular portion of cranial nerve
VIII at the glial-Schwann cell junction. Adults
present with unilateral SNHL in their 40s to 60s.46
Vestibular schwannomas are avidly enhancing
lesions that have a cylindrical morphology if they
involve only the IAC or an ‘‘ice cream cone’’
appearance if there is involvement of both the
IAC and CPA (Fig. 26).50
Intralabyrinthine schwannoma is a benign tumor
that arises from Schwann cells with the membra-
nous labyrinth, which results in progressive
hearing loss.51 This lesion appears as a tissue
density lesion within the high signal inner ear fluid
and shows focal enhancement. It appears as
a focal enhancing mass in the membranous laby-
rinth on T1 contrast-enhanced MR imaging and
as a filling defect within the perilymph on high-
resolution T2 MR imaging. These lesions can be
subdivided according to location: intracochlear,
vestibulocochlear, transmodiolar, transmacular,
and transtotic (Fig. 27).52
Endolymphatic sac tumors (ELSTs) are adeno-
matous tumors of the endolymphatic sac in
Fig. 26. Axial enhanced T1-weighted image of MR
image demonstrates bilateral avidly enhancing masses
in the CPAs. The lesions widening the porus acousticus
and extend into the IAC. Bilateral vestibular schwan-
nomas is highly suggestive of neurofibromatosis 2.
(Courtesy of Amirsys, Inc., Salt Lake City, UT; with
permission.)
300 Shah & Wiggins
Fig. 27. Enhanced axial T1-weighted MR image with
fat saturation shows an enhancing mass slightly ex-
panding the vestibule. (Courtesy of Amirsys, Inc.,
Salt Lake City, UT; with permission.)
a retrolabyrinthine location. They are centered in
the fovea of the endolymphatic sac in the posterior
surface of the petrous temporal bone. Larger
lesions (> 3 cm) can spread to involve the middle
ear, CPA cistern, and/or jugular foramen. On CT,
the lesion shows intratumoral bone spicules. The
margins may appear destructive and aggressive
(Fig. 28).53 A thin rim of calcification along the
posterior margin of the tumor is common. CT
angiogram shows enlarged distal vessels from
Fig. 28. Axial CT shows speculated calcifications
throughout the tumor matrix. There is dehiscence of
the posterior wall of the IAC by the ELST. (Courtesy
of Amirsys, Inc., Salt Lake City, UT; with permission.)
the ascending pharyngeal and occipital arteries
feeding the vascular tumor.54 There are high signal
foci within the tumor matrix on T1 MR imaging
when the lesion is larger than 3 cm and along the
margin when the lesion is less than 3 cm. Most
tumors (80%) have these foci of increased signal
intensity.53 Flow voids can be seen when the
tumor is larger than 2 cm. These lesions show
heterogeneous enhancement on contrast-
enhanced MR imaging (Fig. 29).
SNHL is seen in virtually all patients. Other
symptoms include facial nerve palsy, pulsatile
tinnitus, and vertigo. If ELST is bilateral, then von
Hippel-Lindau syndrome is likely present. Hearing
loss and ELST are frequently associated with von
Hippel-Lindau syndrome and should be consid-
ered when screening individuals at risk for von
Hippel-Lindau syndrome and when monitoring
patients with an established diagnosis of von Hip-
pel-Lindau syndrome. Audiologic evaluation and
MR imaging should enable early detection and
enhance management of hearing loss in these
patients. Many patients with von Hippel-Lindau
syndrome have hearing loss without radiographic
evidence of an ELST. Whether it is caused by an
ELST that is too small to be detected by MR
imaging or is produced by some other cause is still
unknown.55
Vestibulocochlear neuritis, labyrinthitis, and lab-
yrinthitis ossificans can lead to SNHL. In subacute
labyrinthitis, the fluid spaces of the inner ear—
especially the membranous labyrinth—are in-
flamed or infected. In the early stage, CT findings
may be normal. Contrast-enhanced T1 MR
Fig. 29. Unenhanced axial T1 image illustrates T1
hyperintense signal with the ELST on the left.
 Imaging of Hearing Loss 301

imaging shows faint to moderate enhancement
within normally fluid-filled spaces of the inner ear
(Fig. 30A). T2 MR imaging shows diminished
signal; however, there is higher signal than seen
with an intralabyrinthine schwannoma.56,57 These
fluid-filled spaces may become ossified in the
late chronic phase (labyrinthitis ossificans).
Patients present with SNHL, vertigo, and tinnitus
acutely. In the tympanogenic membranous type,
there is a history of otomastoiditis, meningitis of
the meningogenic membranous type, and viral
infection of the hematogenous membranous
type.57 Labyrinthitis also can be posttraumatic as
the result of a unilateral fracture with
a perilymphatic fistula or postsurgical after stape-
dectomy or other inner ear surgery.
Labyrinthitis ossificans is fibrous and includes
eventual bony proliferation in the endolymphatic
structures. It may arise from purulent material
reaching endolymph via cerebrospinal fluid and
from adjacent inflammatory disease. It begins
with fibrosis and progresses to ossification as
early as 2 months.58 In cochlear labyrinthitis ossi-
ficans, the fluid spaces of the cochlea are affected.
In noncochlear labyrinthitis ossificans, the fluid
spaces of the semicircular canal or vestibule are
affected. Labyrinthitis ossificans can contraindi-
cate or complicate cochlear implantation;

Fig. 30. (A) Enhanced axial T1-weighted MR illustrates pathologic enhancement of the cochlear apex and vesti-
bule. There is also linear enhancement within the IAC, which is compatible with associated facial neuritis. (B)
Axial CT (left) shows ossification of the membranous labyrinth involving the cochlea, vestibule and semicircular
canal. Axial T2 (right) demonstrates correlative loss of the normal fluid signal within the membranous labyrinth.
(Courtesy of Amirsys, Inc., Salt Lake City, UT; with permission.)
302 Shah & Wiggins
therefore, precochlear implant evaluation of the
temporal bone with CT is essential.59 Hazy
increased density within the normally fluid-filled
spaces of the membranous labyrinth is seen in
mild cases, whereas severe cases show complete
obliteration by bone replacement. On three-
dimensional FT- constructive interference steady
state sequences, there is loss of normal fluid signal
intensity within the cochlea because of fibrous
proliferation of the labyrinth. The modiolus can
appear ‘‘enlarged.’’58 The enhancement of
membranous labyrinthitis can persist in the ossi-
fying stages of labyrinthitis ossificans (see Fig. 30).
Otosyphilis caused by the bacterium spirochete
Treponema pallidum can cause acute and fluctu-
ating hearing loss and vertigo. CT shows perme-
ative demineralization of the otic capsule.39
Contrast-enhanced MR imaging demonstrates
enhancement of cranial nerves VII and VIII. There
may be syphilitic labyrinthitis with enhancement
of the membranous labyrinth.60
Fractures through the temporal bones can be
described as longitudinal, transverse, and oblique.
Transverse fractures through the temporal bones
often involve the inner ear, with SNHL and facial
nerve canal involvement being common.14 In the
medial subtype, the fracture extends along the
posterior or petrous surface through the fundus
of the IAC to the first genu of the facial nerve,
with permanent hearing loss being common.61 In
the lateral subtype, the fracture extends along
the posterior petrous surface through the laby-
rinth, with perilymphatic fistula being common
(Fig. 31).61
Intralabyrinthine hemorrhage can occur in
patients with history of anticoagulation, sickle
cell disease, trauma, leukemia, or other hypervis-
cosity syndromes. Patients experience acute
onset of unilateral SNHL. There is high signal
within normally fluid-filled spaces of the labyrinth
on T1 MR imaging. Demyelinating processes,
such as multiple sclerosis, can cause SNHL if the
plaque involves the cochlear nucleus. MR imaging
of the brain reveals additional white matter pla-
ques in a characteristic periventricular and perical-
losal distribution (Fig. 32). Autoimmune SNHL has
been described62–64 in studies in which the clinical
pattern did not fit with known entities. All patients
responded to treatment for an autoimmune
disease, namely, chronic cortisone and cyclo-
phosphamide therapy. Clinical results in the study
by Kataoka and colleagues63 suggested that
endolymphatic hydrops may contribute to autoim-
mune SNHL. Immune-mediated audiovestibular
dysfunction is either a separate disease entity or
part of a more generalized (auto-)immune
process.64
Fig. 31. Axial CT reveals an oblique fracture through
the temporal bone. The fracture extends through
the oval window, which results in a perilymphatic
fistula. The diffuse middle ear and mastoid opacifica-
tion is likely a combination of blood and cerebro-
spinal fluid. (Courtesy of Amirsys, Inc., Salt Lake City,
UT; with permission.)
Osteopetrosis is also associated with SNHL,
with generalized osteosclerosis.65 Bones may be
uniformly sclerotic, but alternating sclerotic and
lucent bands may be noted in iliac wings and
near ends of long bones. The bones might be
club-like or appear like a bone within bone (endo-
bone). The entire skull is thickened and dense,
especially at the base, in type 1 autosomal reces-
sive osteopetrosis.65 Type 2 spares the calvarium.
Contrast-enhanced MR imaging may show extra-
medullary hematopoiesis with enhancing extrac-
erebral spaces.66 Flared IACs and large
subarcuate fossae are classically seen.65 Sinuses
are small and underpneumatized. Vertebrae are
radiodense and may show alternating bands,
known as the rugger-jersey sign. In autosomal
recessive osteopetrosis, MR angiography can
demonstrate compromise of the petrous segment
of the internal carotid artery.67 MR venography
may show stenosis of the dural venous sinuses.
Presbycusis can occur as a sequela of aging,
infectious processes, inflammatory processes,
trauma, toxicity of certain medications, and long-
term exposure to loud noise.
MIXED HEARING LOSS
Mixed hearing loss (MHL) occurs from a combina-
tion of CHL and SNHL. There may be damage in
the outer or middle ear and in the inner (cochlea)
or auditory nerve.
 Imaging of Hearing Loss 303

Fig. 32. Axial T2-weighted image (left) shows a demyelinating plaque in the region of the left cochlear nucleus.
Axial fluid attenuated inversion recovery image (FLAIR) (right) of the brain reveals characteristic periventricular
white matter lesions.

Mixed Hearing Loss Pathologies site affected.28 The cochlea is involved in 35% of
cases.29 The fenestral form demonstrates a large
The classic cause of MHL is otospongiosis. Oto-
plaque of otospongiosis narrowing the oval
spongiosis (also known as otosclerosis) is
window. The retrofenestral or cochlear form shows
a primary bone dysplasia of the otic capsule in
focal lytic plaques in pericochlear bony labyrinth
which normal endochondral bone is replaced by
(Fig. 33).29
spongy irregular new bone.28 It occurs more often
Otosclerosis presents bilaterally and symmetri-
in white women between 11 and 30 years of age
cally in 85% of cases.28,29 There is no bony
who present with CHL or MHL. The fissula ante
encroachment of the membranous labyrinth,
fenestram is the cleft of fibrocartilaginous tissue
which would indicate labyrinthitis ossificans.
just anterior to the oval window and often the initial

Fig. 33. Axial CT images show lucency around the cochlea bilaterally (yellow arrows).
304 Shah & Wiggins
Cochlear otospongiosis is usually associated with
fenestral otospongiosis.29 The pathology involves
cytotoxic enzyme diffusion into the fluid of the
membranous labyrinth. On CT, focal lucencies
are apparent within the otic capsule, especially
late in the course of the disease. MR imaging
shows punctate enhancement in the otic capsule.
Osseous fixation of the malleus can be a cause
of a combined conductive and SNHL. Katzke and
colleagues68 described the characteristic clinical,
audiologic, and histologic findings and the etiology
of 66 patients with an idiopathic fixation of the
head of the malleus. Ninety-four percent of these
patients had a sensorineural component to their
hearing loss—far more than the loss that could
be attributed to presbycusis.
SUMMARY
Hearing loss may be sensorineural, conductive, or
mixed with myriad etiologies. Knowledge of the
important temporal bone anatomic landscape is
fundamental in understanding the pathophysi-
ology of hearing loss. Dedicated imaging of the
temporal bones, IACs, and brain with CT and MR
imaging is fundamental to the complete evaluation
of hearing loss.
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