Beruflich Dokumente
Kultur Dokumente
DOI 10.1007/s13244-011-0126-z
PICTORIAL REVIEW
Received: 10 May 2010 / Revised: 4 September 2011 / Accepted: 9 September 2011 / Published online: 15 October 2011
# The Author(s) 2011. This article is published with open access at Springerlink.com
External ear
Clinically relevant imaging findings Radiological findings Treatment options if the other ear is functioning well,
are critical for accurate classification of the severity of unilateral atresia is not treated. Bilateral atresia is treated
disease and surgical decision-making. For selection of best surgically at the age of 56 years. Usually middle ear
surgical candidates, images should be evaluated according surgery is preceded by auricle reconstruction. It is unnec-
to the grading system proposed by Jahrsdorfer et al. [9], essary to perform CT examination in early childhood,
who evaluated eight critical areas of temporal bone before the middle ear grows to almost-adult size. Unilateral
anatomy, each area receiving 1 rating scale point, with the atresia is also effectively managed with a Bahaa
exception of the presence of a stapes, which received 2 surgically implantable system which works through direct
points (Table 1). Patients with a pre-surgical rating of 5 bone conduction.
points should not be considered surgical candidates. A pre-
surgical rating of 8 points translates into an 80% chance of
resorting hearing to normal or near-normal levels.
Often the malformation involves ossicles and middle ear
space to varying degrees. The inner ear is most often
normal [8]. Among frequently observed middle ear devel-
Structure Points
Stapes present 2
Oval window open 1
Middle ear space 1
Facial nerve 1
Malleus/incus complex 1
Mastoid pneumatised 1
Fig. 2 Congenital external ear malformation. Axial CT scan, bone
Incus-stapes connection 1 window. Deformed ossicular chain is visible within hypoplastic
Round window 1 tympanic cavity. Proper malleus and incus together with malleo-
Appearance external ear 1 incudal joint can not be identified, instead, there is a V-shaped bony
structure, representing the fused malleo-incudal complex
Insights Imaging (2012) 3:3348 35
Fig. 4 Malignant external otitis. a Axial contrast enhanced CT scan window, subtle bone erosion is visible (arrow) and mastoid air cells
shows thickening and heterogenous contrast enhancement of the soft are filled with fluid
tissue around auricle and in masticator space (arrow). b In the bone
36 Insights Imaging (2012) 3:3348
EAC Exostoses
Clinically relevant imaging findings exostoses are typically Definition post-inflammatory, acquired atresia of EAC. The
seen as benign bony overgrowth with normal overlying soft disease is characterised by formation of fibrous tissue,
tissue, always bilateral, and located close to the tympanic overlying lateral surface of tympanic membrane. In many
annulus at the tympanomastoid and tympanosquamous cases (60%) it occurs bilaterally [4, 6].
sutures (Fig. 6) [13, 14]. The lumen of the EAC may be
significantly narrowed (Fig. 7). Symptoms Clinically the disease may be discrete, with
conductive hearing loss, otorrhea and history of chronic otitis.
Differential diagnosis Includes: radiologically, EAC osteo-
ma; clinically, medial canal fibrosis, necrotizing external Preferable imaging method Temporal bone HRCT is the
otitis and EAC cholesteatoma. On imaging, the last three examination of choice.
entities are visible as soft tissue lesions with or without
bone destruction. A history of cold water and wind Clinically relevant imaging findings medial canal fibrosis
exposure facilitates the diagnosis [13]. typically is seen as a homogenous soft tissue against the
tympanic membrane (Fig. 8a, b), there are no EAC bone
changes.
Symptoms These include otorrhea, otalgia, hearing loss and Differential diagnosis This includes medial canal fibrosis,
bleeding. Diagnosis is usually delayed because symptoms are keratosis obturans, necrotizing external otitis and EAC
similar to other benign otological conditions such as chronic cholesteatoma.
suppurative otitis media or EAC cholesteatoma [15, 16] .
Treatment options These depend on the tumour stage
Preferable imaging methods Temporal bone HRCT best (see Table 2) and are generally based on the combination
predicts tumour extent in terms of osseous destruction; in of surgery and radiotherapy. The extent of disease,
larger lesions, enhanced MRI images are necessary to assess positive margins, dural involvement, facial nerve paraly-
soft tissue and intracranial involvement, also perineural sis, cranial nerve involvement, or moderate to severe pain
spread. Imaging should always include parotid gland, since on presentation have been associated with poorer out-
parotid nodes are a first-order drainage nodal group. comes [16]. When resected with free margins, 5-year
survival reaches 100%. With incomplete resection, recur-
Clinically relevant imaging findings A mass in the EAC, rence rate depends on tumour stage and is reported
with underlying bony destructive changes (Fig. 9). Very between 25 to 100% [8].
small tumours may not infiltrate bone; they present only as Note: secondary EAC involvement from regional prima-
lobular, soft tissue mass. Larger lesions spread around the ry SCC is much more common than primary EAC
auricle, infiltrate parotid gland and enhance heteroge- squamous cell cancer.
neously after contrast (Fig. 10). It should be remembered
that bone invasion predicts treatment outcome.
Fig. 9 Squamous cell cancer of EAC. Axial bone-window CT shows Fig. 10 Squamous cell cancer of EAC. Axial contrast-enhanced CT
mass, invading mastoid air cells with signs of profound bone demonstrates heterogenously enhancing soft tissue mass infiltrating
destruction of mastoid and temporo-mandibular joint auricle, parotid gland and EAC
38 Insights Imaging (2012) 3:3348
T1 Tumour limited to EAC without bone erosion and soft tissue involvement
T2 Tumour with limited EAC osseous erosion or soft tissue involvement
T3 Tumour eroding osseous EAC with limited soft tissue/middle ear/mastoid involvement
T4 Tumour eroding deeper inner ear structures/temporomandibular joint/deep soft tissue extension/facial nerve paresis/distant metastases
Symptoms Fluid filling the middle ear cavity causes Non-traumatic opacified middle ear: acute inflammation/
tympanic membrane bulging with no signs of acute infection
infection (redness, pain, oedema). Over time, middle
ear fluid can become very thick and glue-like (glue Definition Acute middle ear infection (acute otitis media,
ear), which increases the likelihood of conductive acute otomastoiditis) usually presenting with typical clinical
hearing loss. image and in most cases not requiring imaging.
Preferable imaging methods Temporal bone HRCT is the Symptoms These include earache, fever, pain, otorrhea,
examination of choice; however, when nasopharyngeal conductive hearing loss. On otoscopy tympanic membrane
pathology is suspected, contrast-enhanced MRI study is red and bulging [1, 2].
should be considered. In children, diagnosis is usually Both from clinical and radiological points of view, it is
made based on tympanometry, therefore CT should not important to differentiate between acute otitis media and
be ordered. secretory otitis [21].
Secretory otitis means fluid in the middle ear cavity
Clinically relevant imaging findings Middle ear cavity and without signs or symptoms of infection; this is usually
mastoid air cells are completely, homogeneously opacified. caused when the Eustachian tube patency is compromised
There is no ossicular chain erosion (Fig. 16). and fluid is trapped in the middle ear. Signs and symptoms
Fig. 12 Temporal bone trauma. Axial bone CT demonstrates Fig. 13 Temporal bone trauma. Sagittal CT scan illustrates commu-
opacified middle ear (arrow), clinically visible as haemotympanum. nication of fracture line with facial nerve canal in mastoid segment
Detachment of malleus from incus is also visible (arrow)
40 Insights Imaging (2012) 3:3348
Clinically relevant imaging findings Since in severe cases Definition When the inflammation persists at least 6 weeks
ACOM may lead to severe complications, like abscess and is associated with otorrhea through a perforated
being the stapes headbetween them the incudostape- In the setting of chronic middle ear inflammation,
dial joint is well visible (Fig. 23) cholesteatomas arise as the result of tympanic membrane
retraction or perforation [24, 25].
In cases of post-inflammatory ossicular chain erosion, any
The classic acquired cholesteatoma develops from medial
or all of three signs will not be visualised on axial CT scans.
retraction of the pars flaccida into the epitympanum (pars
Differential diagnosis includes congenital ossicular de-
flaccida cholesteatoma). As this process continues, the scutum
formity, cholesteatoma with ossicular erosion, post-
becomes eroded; ossicular destruction is common. If the
operative ossicular loss.
cholesteatoma invades posteriorly into the aditus ad antrum
Post-inflammatory erosion sometimes involves walls of
and the mastoid itself, erosion of the tegmen tympani, with
tympanic cavity. Based on CT multiplanar images the
exposure of the dura and/or erosion of the lateral semicircular
following structures should first of all be evaluated:
canal may appear [17] (Fig. 24).
Bony cover of lateral semicircular canal A second, less frequent type of an acquired cholestea-
Bony cover of the second (tympanic) segment of the toma arises when the posterior quadrant of the tympanic
facial nerve membrane is retracted into the posterior middle ear (pars
Roof of the middle ear cavity (tegmen tympani) tensa cholesteatoma). As retraction continues, squamous
epithelium envelops the suprastructure of the stapes and
fills in sinus tympani. Cholesteatomas arising from the
Chronic otitis media with acquired cholesteatoma posterior tympanic membrane are likely to produce facial
nerve exposure, eroding bony cover of facial nerve canal,
Definition Cholesteatoma means skin in the wrong place. and destruction of the stapedial suprastructure [26, 27].
It consists of squamous epithelium that is trapped within the
middle ear and mastoid [23].
Fig. 19 Chronic middle ear inflammation. Axial CT scan demon- Fig. 20 Chronic middle ear inflammation. Axial CT in bone window
strates focal calcifications in thickened tympanic membrane (arrow). demonstrates focal calcifications (arrow) in the tympanic cavity, close
This is called myringosclerosis to ossicular chain. This is called tympanosclerosis
Insights Imaging (2012) 3:3348 43
Fig. 23 Normal anatomy of the middle ear. Axial CT, bone window.
Fig. 21 Normal anatomy of middle ear. Axial CT, bone window. Two dots, visible posteriorly to malleus neck (arrowhead) represent
Malleus head (long arrow) and incus body (short arrow) with short incus lenticular process (long arrow) and stapes head (short arrow)
process are visible in epitympanum, forming a so-called ice cream
cone
should be made between patient evaluation prior to first
stage surgery and patient evaluation prior to second-stage
Symptoms Those symptoms typical for cholesteatoma
surgery looking for residual cholesteatoma. Both imaging
include painless otorrhea, persistent or frequently recurrent
techniquesCT and MRIare valuable but the choice
in spite of aggressive antibiotic treatment, conductive
depends on the clinical situation.
hearing loss, rarely vertigo.
Clinically relevant imaging findings Typical cholesteatoma
Treatment Solely surgical. Surgery is usually performed
features, visible on CT images, include:
with canal wall up tympanoplasty, where the wall of
external auditory canal is preserved, and sometimes canal Pars flaccida cholesteatoma:
wall down procedure, when the wall of external auditory soft tissue mass in Prussaks space, medial to attic
canal is removed and large mastoid cavity is left [24, 26 wall, lateral to head of malleus and body of incus,
28]. Detailed description of these two methods, their often accompanied by scutum and ossicle erosion
advantages and disadvantages are beyond the scope of this most commonly long process of incus (Fig. 25a, b).
review. Ossicles are very often displaced medially.
Fig. 25 Chronic middle ear inflammation with cholesteatoma. a Axial well visible (arrow). The second arrow points at cholesteatoma,
CT scan, bone window. Lobulated soft tissue mass is visible laterally growing typically in Prussaks space
to ossicular chain (arrow). b Coronal CT scan: erosion of scutum is
Pars tensa cholesteatoma: Bony walls of tympanic segment and anterior genu of
erosive mass of soft tissue density in posterior the facial nerve
tympanum, medial to ossicles, may involve sinus Possible extension of cholesteatoma towards sinus
tympani, facial recess, mastoid (Fig. 26). Early ossicle tympani (since it correlates with high post-surgical
erosion is usually present (incus long process, stapedial recurrence rate)
superstructure, manubrium of malleus). Ossicles can be Possible involvement of oval window niche
displaced laterally.
Typical cholesteatoma features, visible on MR images,
include:
When evaluating CT images for the presence of choles-
Bright, hyper-intense signal on B-1,000 images, on
teatoma, the following features should be addressed:
diffusion-weighted MRI images (Fig. 27)
Ossicle erosion and displacement Hypo-intense signal on ADC map
Bony cover of tegmen tympani and lateral semicircular Lack of enhancement on post-gadolinium T1-weighted
canal sequences
Fig. 28 Postoperative middle ear suspected for cholesteatoma under the tegmen tympani (B 1,000 image), which represents residual
recurrence. a Axian CT scan, bone window, demonstrates diffuse cholesteatoma. Case courtesy of Dr Bert De Foer, Department of
opacification of middle ear cavity. b MRI coronal image. On this non- Radiology, AZ Sint-Augustinus, Wilrijk, Belgium
EPI diffusion-weighted sequence a small hyperintense point is visible
Moderate intensity on T2-weighted images, clearly the resection cavity (granulation tissue, effusion, scar tissue
lower than the hyperintensity of inflammation. or cholesteatoma). CT has a high negative predictive value
In case of a clear-cut clinical and otoscopical image of a only in a well aerated middle ear and post-operative cavity.
cholesteatoma, CT will do as primary and sole imaging However, this is seldom seen. In the literature, two MRI
technique prior to first-stage surgery. It will be able to techniques have been described for the evaluation of pre
demonstrate ossicular and epitympanic erosion and will second look patients: delayed post-gadolinium T1-weighted
nicely show tegmen delineation. Facial nerve canal and sequences and diffusion-weighted MRI (EPI as well as non-
lateral semicircular canal integrity can be evaluated as well. EPI) or a combination of both techniques. NonEPI is
However, in case of suspected infection/inflammation regarded as far superior to EPI.
and/or complications such as fistulisation to the lateral Using the combination of non-EP DW sequences and
semicircular canal, MRI including delayed post-gadolinium delayed post-gadolinium sequences, a high sensitivity
T1-weighted sequences and non-echo planar diffusion- (90%) and specificity (100%) was achieved in detection
weighted (non-EP DWI) sequences is required. It will be of residual and recurrent cholesteatoma. Recent literature
able to differentiate the infection/inflammation from the has also demonstrated that non-EP DWI sequences alone
cholesteatoma and it will show the status of the membra- have the same sensitivity, specificity, positive predictive
nous labyrinth. value and negative predictive value that the combination of
In case of an unequivocal clinical suspicion of choles- non-EP DWI plus delayed post-gadolinium T1-weighted
teatoma, non-EP DWI sequence alone can be used as a sequences together (Fig. 28a, b) [28].
screenings tool to exclude the presence of cholesteatoma. So, non-EPI sequences can be used alone in pre-second-
The approach, however, becomes completely different in look patients as a screening tool avoiding the need of
patient evaluation prior to second look. In these cases, CT delayed post-gadolinium T1-weighted sequences. In this
cannot distinguish between different types of soft tissues in setting, CT scan is preserved for the immediate pre-
operative setting in order to see local temporal bone contrast medium administration (Fig. 29a, b). Large GTP
anatomy can fill whole middle ear cavity; this could result in fluid
collection in mastoid. The floor of the middle ear cavity
Non-traumatic opacified middle ear: glomus tympanicum should be intactif dehiscent or permeative, glomus
paraganglioma (GTP) jugulotympanicum should be suspected (Fig. 30). Very
large GTP may show so-called aggressive bone changes
Definition Paraganglioma that arises in glomus bodies eroding the medial wall of the middle ear cavity and
situated in the cochlear promontory. It is the most common ossicles.
primary neoplasm of the middle ear [29]. For the most efficient communication with ENT
surgeons, GTP should be evaluated according to
Symptoms When growing typically lateral to the cochlear Glasscock-Jackson classification [31] (Table 4).
promontory, may abut the inner margin of the tympanic Differential diagnosis includes aberrant internal carotid
membrane, and present with conductive hearing loss and a artery, dehiscent jugular bulb, glomus jugulotympanicum
red, pulsatile, vascular retrotympanic mass. Other clinical paraganglioma, facial nerve schwannoma from tympanic
symptoms include pulsatile tinnitus and, rarely, facial nerve segment.
paralysis [29, 30]. Treatment is solely surgical, but in elderly patients may
not be necessary, since GTP grows very slowly.
Preferable imaging method HRCT should be the examina-
tion of choice, if GTP is clinically suspected. In other
settings a thin-slice CT before and after contrast provides Non-traumatic opacified middle ear: middle ear
the diagnosis. MRI is used, if based on CT findings glomus schwannoma
jugulotympanicum is suspected.
Clinically relevant imaging findings: on CT images GTP Definition tumour arising within middle ear cavity, origi-
presents typically as a soft-tissue mass, with a flat base on nating from the facial nerve, chorda tympani nerve (7th
cochlear promontory, vividly and quickly enhancing after branch), Jacobson nerve (9th branch), or Arnold nerve
Table 4 Glasscock-Jackson
classification of GTP Type Description
Type I Small mass limited to cochlear promontory
Type II Tumour completely filling middle ear space
Type III Tumour filling middle ear and extending into mastoid air cells
Type IV Tumour filling middle ear, extending into mastoid air cells or through
Tympanic membrane to fill external auditory canal; may extend anteriorly to the carotid artery
Insights Imaging (2012) 3:3348 47
(10th branch), with the facial nerve being the most common Acknowledgements The authors are very grateful to Dr Bert De
Foer, from Department of Radiology, GZA Hospitals Sint-Augustinus,
nerve of origin.
Antwerp, Belgium, for expert advice concerning cholesteatoma
Schwannomas have been identified along the entire imaging and permission to use examples of non-EPI DW images.
course of the facial nerve, although intratemporal tumours
appear to be much more common than intracranial tumours. Open Access This article is distributed under the terms of the
Creative Commons Attribution Noncommercial License which per-
Within the temporal bone, the most common sites of
mits any noncommercial use, distribution, and reproduction in any
involvement, in descending order, are the geniculate medium, provided the original author(s) and source are credited.
ganglion, labyrinthine segment, tympanic and vertical
segments, and the internal auditory canal [32].
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