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CT Imaging of the Temporal Bone: an anatomical review with illustrative cases of Cholesteatomas Dunia Abdul-Aziz, HMS III Dr. Gillian Lieberman Beth Israel-Deaconess Radiology May 19, 2008

CT Imaging of the Temporal Bone: an anatomical review with

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Page 1: CT Imaging of the Temporal Bone: an anatomical review with

CT Imaging of the Temporal Bone: an anatomical review with illustrative cases of Cholesteatomas

Dunia Abdul-Aziz, HMS IIIDr. Gillian LiebermanBeth Israel-Deaconess RadiologyMay 19, 2008

Page 2: CT Imaging of the Temporal Bone: an anatomical review with

Outline

Review of the Normal Ear Anatomy

Tympanic Membrane

Middle Ear Cavity

Ossicular Chain

Spaces

Walls

Characteristic CT of the Normal Temporal Bone

Axial

Coronal

Cholesteatoma: Illustrative Pathology on CT

Patient presentation

Overview of cholesteatoma

Diagnosis, Pathogenesis, Differential, Management

Selected cases from companion patients

Page 3: CT Imaging of the Temporal Bone: an anatomical review with

Human Ear

Netter FH, 2003

Page 4: CT Imaging of the Temporal Bone: an anatomical review with

Human EarExternal Ear

Netter FH, 2003

Page 5: CT Imaging of the Temporal Bone: an anatomical review with

Human EarMiddle Ear

Netter FH, 2003

Page 6: CT Imaging of the Temporal Bone: an anatomical review with

Human EarInner Ear

Netter FH, 2003

Page 7: CT Imaging of the Temporal Bone: an anatomical review with

Middle Ear Anatomy

Air-containing space

communicates with the nasopharynx via the eustachian tube.

normally sealed laterally by TM.

Function:

transmission and amplification of sound from TM to stapes footplate.

Anderson JF, 1983

Page 8: CT Imaging of the Temporal Bone: an anatomical review with

Spaces of the Middle Ear

Middle Ear divided into five spaces based on relationship to tympanic annulus.

Epitympanum (Attic) - superior to annulus

Contains: body of incus and head of malleus.

communicates with the mastoid via aditus.

Mesotympanum - on level with the TM

Contains:

oval and round windows

long process of incus, articulation with stapes.

facial nerve in bony canal

Hypotympanum – below annulus

Contains: jugular bulb

Protympanum - in anterior recess of the middle ear

Contains: eustachian tube

Retrotympanum – posterior to annulus

Contains: sinus tympani, facial recess

Anderson JF, 1983

Page 9: CT Imaging of the Temporal Bone: an anatomical review with

Inside the Middle Ear: Ossicular

Chain

Lalwani AK, 2004

Malleus: long process (manubrium), short process, head

Tensor tympani m.

Incus: short process, long process

Lenticular process: distal portion of long process

Most tentative blood supply susceptible to resorption in otitis media

Stapes: Superstructure and footplate

Stapedius m.

Page 10: CT Imaging of the Temporal Bone: an anatomical review with

Walls of Middle Ear (Tympanic) Cavity

Agur AM, 1999

Page 11: CT Imaging of the Temporal Bone: an anatomical review with

Walls of Tympanic Cavity: Roof and Floor

Roof: tegemen tympani

Floor: Superior bulb of internal jugular v.

Agur AM, 1999

Page 12: CT Imaging of the Temporal Bone: an anatomical review with

Walls of Tympanic Cavity: Lateral

Lateral:•TM, malleus handle

Roof: tegemen tympani

Floor: Superior bulb of internal jugular v.

Grant’s Atlas, 1999

Page 13: CT Imaging of the Temporal Bone: an anatomical review with

Walls of Tympanic Cavity: Medial

Lateral:•TM, malleus handle

Roof: tegemen tympani

Floor: Superior bulb of internal jugular v.

Medial:•Promontory•Oval window--stapes•Round window (inferiorly)

Agur AM, 1999

Page 14: CT Imaging of the Temporal Bone: an anatomical review with

Walls of Tympanic Cavity: Posterior

Lateral:•TM, malleus handle

Roof: tegemen tympani

Floor: Superior bulb of internal jugular v.

Medial:•Promontory•Oval window--stapes•Round window (inferiorly)

Posterior:•aditus ad antrum: epitympanic recess

mastoid cells. •pyramidal eminencestapedius m.•Chorda tympani n. aperture

Agur AM, 1999

Page 15: CT Imaging of the Temporal Bone: an anatomical review with

Walls of Tympanic Cavity: Anterior

Lateral:•TM, malleus handle

Roof: tegemen tympani

Floor: Superior bulb of internal jugular v.

Medial:•Promontory•Oval window--stapes•Round window (inferiorly)

Posterior:•aditus ad antrum: epitympanic recess

mastoid cells. •pyramidal eminencestapedius m.•Chorda tympani n. aperture

Anterior: tensor tympani m.eustachian tube.

Agur AM, 1999

Page 16: CT Imaging of the Temporal Bone: an anatomical review with

Walls of Tympanic Cavity

Lateral:•TM, malleus handle

Roof: tegemen tympani

Floor: Superior bulb of internal jugular v.

Medial:•Promontory•Oval window--stapes•Round window (inferiorly)

Posterior:•aditus ad antrum: epitympanic recess

mastoid cells. •pyramidal eminencestapedius m.•Chorda tympani n. aperture

Anterior: tensor tympani m.eustachian tube.

Agur AM, 1999

Let’s take another look inside the Tympanic cavity by cutting

through the cavity in this plane…

Page 17: CT Imaging of the Temporal Bone: an anatomical review with

Middle Ear Anatomy: An additional view

Netter FH, 2003

Page 18: CT Imaging of the Temporal Bone: an anatomical review with

CT imaging of the temporal bone

Axial View

Page 19: CT Imaging of the Temporal Bone: an anatomical review with

Axial Slice (Superior to Inferior)

• The following slides are representative axial slices through the normal temporal bone, proceeding inferiorly from the most superior cut (through the superior semicircular canal).

Axial View Coronal ViewP I

SA

Silver JA et al. 1987

Page 20: CT Imaging of the Temporal Bone: an anatomical review with

Axial: Superior slice

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 21: CT Imaging of the Temporal Bone: an anatomical review with

Axial: Superior SCC

Superior SCC:

perpendicular to long axis of temporal bone

Superior petrosal sinus:

receives blood from cavernous sinus, drains to transverse sinus

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 22: CT Imaging of the Temporal Bone: an anatomical review with

Axial: Epitympanum

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 23: CT Imaging of the Temporal Bone: an anatomical review with

Epitympanum: space above TM annulus (ring)

What do the structures in the box resemble?

Ice cream cone!

Correspond to ossicles: malleus head, incus body

Axial: Epitympanum

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 24: CT Imaging of the Temporal Bone: an anatomical review with

Epitympanum: space above TM annulus (ring)

What do the structures in the box resemble?

Ice cream cone!

Correspond to ossicles: malleus head, incus body

Axial: Epitympanic

Structures

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 25: CT Imaging of the Temporal Bone: an anatomical review with

Epitympanum: space above TM annulus (ring)

What do the structures in the box resemble?

Ice cream cone!

Correspond to ossicles: malleus head, incus body

Axial: Epitympanum

“ice cream”

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 26: CT Imaging of the Temporal Bone: an anatomical review with

Epitympanum: space above TM annulus (ring)

What do the structures in the box resemble?

Ice cream cone!

Correspond to ossicles: malleus head, incus body

Axial: Ossicles

in the Epitympanum

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 27: CT Imaging of the Temporal Bone: an anatomical review with

Anterior genu (ag):

first turn of the facial nerve

labyrinthine segment in fallopian canal (fc) turns to tympanic segment.

Axial: Anterior Genu

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 28: CT Imaging of the Temporal Bone: an anatomical review with

Anterior genu (ag):

first turn of the facial nerve

labyrinthine segment in fallopian canal (fc) turns to tympanic segment.

Axial: Course of the Anterior Genu

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 29: CT Imaging of the Temporal Bone: an anatomical review with

Facial nerve: Anterior genu

Anterior geniculate/First genu

Labyrinthine segment

curls anteriorly over cochlear terminates at geniculate

Tympanic segment

courses from 1st (anterior) to 2nd (posterior) genu.

underneath horizontal SCC and superior to oval window

can be identified using short process of the incus as landmark. (box)

1st genu

2nd genu

Gaillard F. http://www.radiopaedia.org/index.php?title=Facial_nerve_(CN_VII)

Page 30: CT Imaging of the Temporal Bone: an anatomical review with

Axial: moving inferiorly…

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 31: CT Imaging of the Temporal Bone: an anatomical review with

Axial: IAC outlined

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 32: CT Imaging of the Temporal Bone: an anatomical review with

Internal auditory canal (IAC) PFN SV

C IVA

Axial: Internal auditory canal

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 33: CT Imaging of the Temporal Bone: an anatomical review with

Internal auditory canal (IAC)

Note:

Structures anterior to IAC are cochlear

PFN SV

C IVA

Axial: relationship of IAC to cochlea

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 34: CT Imaging of the Temporal Bone: an anatomical review with

Internal auditory canal (IAC)

Note:

Structures anterior to IAC are cochlear

Structures posterior to IAC are vestibular

PFN SV

C IVA

Axial: relationship of IAC to vestibular system

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 35: CT Imaging of the Temporal Bone: an anatomical review with

Axial: Characteristic landmark

What do the structures in the box resemble?

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 36: CT Imaging of the Temporal Bone: an anatomical review with

Axial: “Signet Ring”

What do the structures in the box resemble?

Signet ring!

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 37: CT Imaging of the Temporal Bone: an anatomical review with

Axial: Vestibule and LSCC

What do the structures in the box resemble?

Signet ring!

Corresponds to Vestibule and LSCC

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 38: CT Imaging of the Temporal Bone: an anatomical review with

Vestibular aqueduct:

bony canal that passes to posterior fossa dura

Opens lateral to IAM

transmits endolymphatic duct and 2 blood vessels into blind sac (reservoir for excess endolymph)

Common crus: union of superior and posterior SC ducts.

Axial: Vestibular Aqueduct

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 39: CT Imaging of the Temporal Bone: an anatomical review with

Note:

The relationship of the incus (short process) to help identify facial n. (tympanic segment)

Axial: Relationship of Incus

(short process) to the Facial n.

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 40: CT Imaging of the Temporal Bone: an anatomical review with

Axial: moving inferiorly…

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 41: CT Imaging of the Temporal Bone: an anatomical review with

Review: Middle Ear Cavity Superiorly

Netter FH, 2003.

Medial wall of Tympanic cavity

Round Window Promontory

Eustachian tube

Tensor tympani m.

Prominence of Lateral SCC

A section through this plane will thus reveal the tensor tympani m., lateral SCC and stapes.

Stapes

Page 42: CT Imaging of the Temporal Bone: an anatomical review with

Tensor tympani m.:

arises from cartilaginous and bony margins of eustachian tube

inserts on handle of malleus

Dampens ossicular chain

Lateral SCC

Stapes

Axial: Tensor tympani canal

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 43: CT Imaging of the Temporal Bone: an anatomical review with

Review: Middle Ear Cavity Illustration

Netter FH, 2003.

Medial wall of Tympanic cavity

Round Window Promontory

Eustachian tube

Tensor tympani m.

Prominence of Lateral SCC

A section through this plane will thus reveal the round window, promotory and eustachian tube

Page 44: CT Imaging of the Temporal Bone: an anatomical review with

Axial: Middle Ear Cavity

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 45: CT Imaging of the Temporal Bone: an anatomical review with

Round window:

opening between middle and inner ear, associated with scala tympani

below oval window

Promontory:

medial wall of tympanic cavity formed by basal turn of cochlea

Axial: Round Window

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Promontory Promontory

Page 46: CT Imaging of the Temporal Bone: an anatomical review with

Axial: continuing to move inferiorly…

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 47: CT Imaging of the Temporal Bone: an anatomical review with

Facial nerve (mastoid segment, ms), here it gives off:

Chorda tympani (ct):

taste fibers to anterior 2/3 tongue

preganglionic parasympathetic to submandibular & sublingual glands

Courses between incus and handle of malleus

Agur AM, 1999.Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Axial

Page 48: CT Imaging of the Temporal Bone: an anatomical review with

Axial: Inferior view, facial n.

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 49: CT Imaging of the Temporal Bone: an anatomical review with

Facial nerve (mastoid segment, ms)

Courses from second genu to the stylomastoid foramen

Gives off 3 branches:

Nerve to stapedius (ns)

Chorda tympani (ct)

nerve from the auricular branch of vagus (pain fibers to posterior auditory canal)

Axial: Facial nerve branches

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 50: CT Imaging of the Temporal Bone: an anatomical review with

CT imaging of the temporal bone

Coronal View

Page 51: CT Imaging of the Temporal Bone: an anatomical review with

Coronal Slice (Posterior to Anterior)

Axial View Coronal ViewP I

SA

Silver JA et al. 1987

• The following slides are representative coronal slices through the normal temporal bone, proceeding anteriorly from the most posterior cut (through the semicircular canals).

Page 52: CT Imaging of the Temporal Bone: an anatomical review with

Coronal: Semicircular Canals (posteriorly)

Posterior, Lateral and Superior SCC

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 53: CT Imaging of the Temporal Bone: an anatomical review with

90° angle between Lateral and Superior SCC

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Coronal: Semicircular Canal Relationship

Page 54: CT Imaging of the Temporal Bone: an anatomical review with

Coronal: more anteriorly, the vestibule is visualized:

Vestibule: union of Superior and Posterior SC ducts.

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 55: CT Imaging of the Temporal Bone: an anatomical review with

Coronal: continuing anteriorly

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 56: CT Imaging of the Temporal Bone: an anatomical review with

Coronal: External auditory canal

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

External Auditory Canal External Auditory Canal

External Auditory Canal

Page 57: CT Imaging of the Temporal Bone: an anatomical review with

Coronal: Scutum

Scutum: spur-shaped bony projection top of tympanic membrane

Often eroded by cholesteatoma

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 58: CT Imaging of the Temporal Bone: an anatomical review with

Coronal: Tympanic Membrane

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Outline of Tympanic Membrane

Page 59: CT Imaging of the Temporal Bone: an anatomical review with

Coronal: Prussak’s space

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 60: CT Imaging of the Temporal Bone: an anatomical review with

Coronal: Prussak’s space

Prussak’s space: anterior recess of TM, between neck of malleus and pars flaccida of tympanic membrane

*most common site of acquired attic cholesteatoma. Prussak’s space (pars flaccida (lateral border) removed)

Gulya JA, 1995.

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 61: CT Imaging of the Temporal Bone: an anatomical review with

Coronal

Observe:

Cochlea “snail”

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.comAnderson JF, 1983

Page 62: CT Imaging of the Temporal Bone: an anatomical review with

Coronal

Observe:

Cochlea “snail”

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.comAnderson JF, 1983

Page 63: CT Imaging of the Temporal Bone: an anatomical review with

Coronal

Observe:

Cochlea “snail”

Two low attenuating areas above

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.comAnderson JF, 1983

Page 64: CT Imaging of the Temporal Bone: an anatomical review with

Coronal

Observe:

Cochlea “snail”

Two low attenuating areas above

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.comAnderson JF, 1983

Page 65: CT Imaging of the Temporal Bone: an anatomical review with

Coronal

Snake’s eyes: Snake’s eyes:

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

“snake eyes”: distal labyrinthine and proximal tympanic segments of facial nerve.

Locate anterior to the “snail” (cochlea)

Page 66: CT Imaging of the Temporal Bone: an anatomical review with

Coronal

Question: How can the facial nerve appear twice in a single coronal slice?

Snake’s eyes: Snake’s eyes:

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 67: CT Imaging of the Temporal Bone: an anatomical review with

Coronal

Question: How can the facial nerve appear twice in a single coronal slice?

Answer: facial nerve turns at the anterior genu (turn)!

Gaillard F. http://www.radiopaedia.org/index.php?title=Facial_nerve_(CN_VII)Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 68: CT Imaging of the Temporal Bone: an anatomical review with

Coronal: Anterior genu

Anterior genu

Fatterpekar et al., 3D CT of the temporal bone. www.terarecon.com

Page 69: CT Imaging of the Temporal Bone: an anatomical review with

Patient Presentation

Page 70: CT Imaging of the Temporal Bone: an anatomical review with

Patient 1: HPI

S. A. is a 32 year old male with left-sided hearing loss and foul smelling otorrhea for 2 months.

No otalgia, dizziness or tinnitus.

PMH: Frequent ear infections as a child.

Medications/Allergies: None

FH: negative for hearing loss or ear abnormalities.

Physical Exam:

Weber localizes to left.

Rinne:

Right: AC>BC

Left: BC>AC (abnormal)

Facial nerve intact

Otoscopic Exam:

Audiometry: Left conductive hearing deficit.

Page 71: CT Imaging of the Temporal Bone: an anatomical review with

Patient 1: PMH/Meds/Allergies/FH

S. A. is a 32 year old male with left-sided hearing loss and foul smelling otorrhea for 2 months.

No otalgia, dizziness or tinnitus.

PMH: Frequent ear infections as a child.

Medications/Allergies: None

FH: negative for hearing loss or ear abnormalities.

Physical Exam:

Weber localizes to left.

Rinne:

Right: AC>BC

Left: BC>AC (abnormal)

Facial nerve intact

Otoscopic Exam:

Audiometry: Left conductive hearing deficit.

Page 72: CT Imaging of the Temporal Bone: an anatomical review with

Patient 1: Physical Exam

S. A. is a 32 year old male with left-sided hearing loss and foul smelling otorrhea for 2 months.

No otalgia, dizziness or tinnitus.

PMH: Frequent ear infections as a child.

Medications/Allergies: None

FH: negative for hearing loss or ear abnormalities.

Physical Exam:

Weber Test localizes to left.

Rinne Test:

Right: AC>BC

Left: BC>AC (abnormal)

Facial nerve intact.

Audiometry: Left conductive hearing deficit.

Otoscopic Exam:

Page 73: CT Imaging of the Temporal Bone: an anatomical review with

Patient 1: Otoscopic

Exam

S. A. is a 32 year old male with left-sided hearing loss and foul smelling otorrhea for 2 months.

No otalgia, dizziness or tinnitus.

PMH: Frequent ear infections as a child.

Medications/Allergies: None

FH: negative for hearing loss or ear abnormalities.

Physical Exam:

Weber localizes to left.

Rinne:

Right: AC>BC

Left: BC>AC (abnormal)

Facial nerve intact

Audiometry: Left conductive hearing deficit.

Otoscopic Exam:

Roland PS. http://www.emedicine.com/ent/topic220.htm

Page 74: CT Imaging of the Temporal Bone: an anatomical review with

Patient 1: CT of Left Temporal Bone (coronal view)

Coronal C- CT, Left ear

PACS, MEEI. Courtesy of Dr. Hines-Parelta

Page 75: CT Imaging of the Temporal Bone: an anatomical review with

Patient 1: Describe the lesion

Coronal C- CT, Left ear

PACS, MEEI. Courtesy of Dr. Hines-Parelta

Page 76: CT Imaging of the Temporal Bone: an anatomical review with

Patient 1 CT Evaluation: Start with a Comparison

Compare to Right Ear (normal)!

PACS, MEEI. Courtesy of Dr. Hines-Parelta

Coronal C- CT, Right ear (normal) Coronal C- CT, Left ear

Page 77: CT Imaging of the Temporal Bone: an anatomical review with

Patient 1 CT Evaluation: Familiar findings

Compare to Right Ear (normal)!

Observe:

Cochlea “snail”

Snake’s eyes: labyrinthine and tympanic segments of facial nerve

PACS, MEEI. Courtesy of Dr. Hines-Parelta

Coronal C- CT, Right ear Coronal C- CT, Left ear

Page 78: CT Imaging of the Temporal Bone: an anatomical review with

Right ear:

normal scutum

Prussak’s space

ossicles in epitympanum.

PACS, MEEI. Courtesy of Dr. Hines-Parelta

Coronal C- CT, Right ear Coronal C- CT, Left ear

Patient 1 CT Evaluation: Right ear

Page 79: CT Imaging of the Temporal Bone: an anatomical review with

PACS, MEEI. Courtesy of Dr. Hines-Parelta

Patient 1 CT Evaluation: Left ear

Page 80: CT Imaging of the Temporal Bone: an anatomical review with

Patient 1: Temporal Bone CT: Axial Views

PACS, MEEI. Courtesy of Dr. Hines-Parelta

Page 81: CT Imaging of the Temporal Bone: an anatomical review with

Patient 1: Axial CT evaluation: Right ear

PACS, MEEI. Courtesy of Dr. Hines-Parelta

Page 82: CT Imaging of the Temporal Bone: an anatomical review with

Axial View

PACS, MEEI. Courtesy of Dr. Hines-Parelta

Page 83: CT Imaging of the Temporal Bone: an anatomical review with

Patient 1: Diagnosis and Management

Diagnosis: Acquired Cholesteatoma

Patient’s presentation, physical exam/otoscopic findings consistent with Acquired Cholesteatoma.

CT findings of soft tissue mass with bony erosion support diagnosis of Cholesteatoma and illustrate the extent of disease.

Management:

S.A. underwent tympanomastoidectomy for removal of the cholesteatoma

no evidence of recurrence to date.

Page 84: CT Imaging of the Temporal Bone: an anatomical review with

Cholesteatoma: Overview

Benign epidermoid cyst of the middle ear or mastoid

“Skin in the wrong place”

Prone to recurrent infection

Grows progressively as it accumulates epithelial debris

Progressive erosion of bone of ossicles and otic capsule

Incidence:

estimated 3 in 100,000 (children) and 9 in 100,000 (adults)

http://www.marshfieldclinic.org/patients/Default.aspx?page=ent_ear_cholesteatoma

Page 85: CT Imaging of the Temporal Bone: an anatomical review with

Clinical Presentation

Hallmark: chronic otitis, painless otorrhea, conductive hearing loss.

Persistent/recurrent despite antibiotics

difficult to treat (lack of blood supply)

Hearing loss

Middle ear space filled with desquamated epithelium +/- mucopurulent discharge

ossicular damage.

Occasionally present with complications of cholesteatoma:

Dizziness and hearing loss (labyrinthine fistula)

Facial nerve paralysis

Intracranial infection

Sigmoid sinus thrombosis

Epidural abscess

Meningitis

Page 86: CT Imaging of the Temporal Bone: an anatomical review with

Pathology

Histology:

Perimatrix: granulation tissue

secrete proteolytic enzymes

Matrix: keratinizing squamous cell epithelium

Cystic: keratin filled center

Mechanism of bone erosion:

Mechanical:

pressure effects of growing mass induce bony remodeling

Biochemical:

bacterial elements (endotoxin)

host inflammatory mediators and enzymes

Dornelles, C. http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0034-72992005000400023&lng=en&nrm=iso

Page 87: CT Imaging of the Temporal Bone: an anatomical review with

Etiology

Three subtypes of Cholesteatomas reported

Congenital

Primary acquired

Secondary acquired

Differ in presentation, pathogenesis and natural history.

At least four theories have been proposed to explain the pathogenesis of acquired cholesteatomas, with different theories predominating in primary vs. secondary acquired cholesteatomas.

Page 88: CT Imaging of the Temporal Bone: an anatomical review with

Congenital Cholesteatoma

Rare: 2% of all cases of Cholesteatomas

Clinical presentation:

Mass behind intact/normal TM

Young child (6mo-5 years)

no history of recurrent infections/perforation.

M:F ratio= 3:1

Etiology: squamous epithelium rests trapped within temporal bone during embryogenesis.

Location: anterior mesotympanum, perieustachian tube area, petrous apex.

Page 89: CT Imaging of the Temporal Bone: an anatomical review with

Primary Acquired Cholesteatoma

Etiology: Eustachian tube dysfunction, negative middle ear pressure

Most common type: 82% of acquired cases

Location/Extension:

pars flaccida retraction into the epitympanum, erosion of scutum (lateral wall of epitympanum).

Posteriorly (most common): Prussak’s space epitympanum, lateral to incus body

Inferiorly: via pouch of von Tröltsch into the middle ear

Anteriorly: into the protympanum

Swartz JD, 1984

Page 90: CT Imaging of the Temporal Bone: an anatomical review with

Secondary Acquired Cholesteatoma

Etiology: secondary to TM injury

18% of acquired cases

posterior marginal perforation from otitis media, trauma, surgery

Extension route: pars tensa retracts into mesotympanum to form cholesteatoma

Ossicles displaced laterally

Can invade sinus tympani and facial recess

difficult to remove

Bailey BJ et al. 2006

Swartz JD, 1984

Page 91: CT Imaging of the Temporal Bone: an anatomical review with

Pathogenesis of Acquired Cholesteatomas

Four theories:

Imagination of the tympanic membrane (retraction pocket)

Epithelial ingrowths through a perforation (migration)

Basal cell hyperplasia

Squamous metaplasia of middle ear epithelium

Page 92: CT Imaging of the Temporal Bone: an anatomical review with

Negative pressure theory (primary acquired)

Eustacian tube dysfunction resulting in poor aeration and relative negative pressure in the middle ear

medial retraction of pars flaccida

progressive deeping of the retracted areas

pit formation and collection of epithelial debris

poor clearance of debris

encystment and cholesteatoma formation.

Cummings CW et al., 2005

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Migration theory (secondary acquired)

Chronic infection/ recurrent otitis media

Destruction of the marginal portion of pars tensa

in growth of epithelium from external auditory canal into middle ear through perforation

progressive deeping of the retracted areas

subsequent encystement and cholesteatoma formation.

Cummings CW et al., 2005

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Basal cell hyperplasia theory

Breaks in the basal lamina

invasion of epithelial cones into subepithelial connective tissue

Microcholesteatomas form and enlarge

perforate secondarily through the TM (resulting in classic appearing attic cholesteatoma)

Cummings CW et al., 2005

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Metaplasia theory

Middle ear infection/chronic inflammation

metaplasia of middle ear mucosa to stratified squamous epithelium secondary to middle ear infection

secondary TM perforation

subsequent encystement and cholesteatoma formation.

Cummings CW et al., 2005

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Cholesteatoma Subtypes: Summary

Primary Acquired Secondary Acquired Congenital

Frequency 98% 2%

82% 18%

Clinical Presentation Eustachian tube dysfunction

TM perforationHistory of infection, trauma or surgery

Child with mass behind intact TMNo history of chronic infection

Pathogenesis (theories) Negative middle ear pressure

Inward migration of squamous epithelium

Embryonic epithelial rests

Inflammation Noninflammed ear Inflammed ear Noninflammed ear

Tympanic membrane perforation

None, retraction pocket in pars flaccida

Marginal perforation in pars tensa

None

Location and direction of growth

Prussak’s spaceSuperiorly into antrumDisplaces ossicles medially

Inferior tympanumDisplaces ossicles laterally

Petrous apex, mastoid, middle ear.

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Diagnosis of Cholesteatoma

Roland PS. http://www.emedicine.com/ent/topic220.htm

Attic Cholesteatoma (early)

Middle Ear Cholesteatoma (advanced, destruction of landmarks)

Otoscopic Exam:

Canal filled with mucous and granulation tissue.

Yellowish mass eroding through TM

TM perforation > 90% cases

Exception: congenital cholesteatomas.

Audiometry:

Conductive hearing loss

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Role of Imaging in Diagnosis of Cholesteatoma

Diagnosis often made otoscopically

Radiologic imaging serves as adjunct:

Assess extent of disease

Evaluate complications:

chronic supportive otitis media (CSOM).

Suspected congenital abnormalities.

Loss of landmarks due to previous surgery.

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Characteristic Findings of Cholesteatoma on CT Imaging

CT without contrast:

Modality of choice:

identifies soft tissue masses and bony erosions

Findings:

nondependent soft tissue mass and bony erosion (80% specificity)

Can be difficult to distinguish cholesteatoma from granulation tissue, pus or fluid.

Often different pathologies related to chronic ear infection coexist

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Characteristic Findings of Cholesteatoma on MRI Imaging

MRI:

Advantage: differentiate cholesteatoma from surrounding fluid/inflammation

New reports suggesting role in post-surgical surveillance of recurrence

Limitation: poor visualization of bony landmark

Findings:

Medium signal intensity on T1-weighted images

High intensity on T2-weighted images

Nonenhancing (unless infiltrated with granulation tissue)

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Differential Diagnosis: Soft Tissue Mass in Middle Ear

Cholesteatoma*

Cholesterol Granuloma*

Chronic otitis media*

Globus tympanicum tumor

Granulation tissue

Malignant otitis externa

Neoplasm

Squamous cell carcinoma (adult)

Rhabdomyosarcoma (children)

* Can have accompanying bone erosion

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Complications: bone erosion and recurrent infection

Hearing loss:

conductive: erosion of ossicles (esp incus)

sensorineural: suppurative labyrinthitis or from the cochlear hair cell loss adjacent to cholesteatoma

Labyrinthine fistula: 10%

horizontal SCC most common

sensorineural hearing loss and vertigo

Facial nerve paralysis:

acute (infection) or chronic (compression from expanding cholesteatoma)

anterior geniculate most common

Dural involvement:

Intracranial infections, subdural/epidural abscess, sigmoid sinus thrombosis, brain hernia or cerebrospinal fluid leakage (erosion of tegmen tympani or tegmen mastoideum)

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Complications identified by CT imaging

The following slides are illustrative CT images from patients with Cholesteatomas that help to illustrates additional pathologies.

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Companion Patient 1: Tegemen

erosion

Extensive cholesteatoma

tegemen erosion; dura exposed

bony sclerosis obliterating cochlea

Yates PD et al., 2002.

Coronal C- CT, Right ear

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Companion Patient 2: Labyrinth Fistula

Extensive Cholesteatoma

Ossicular erosion

Labyrinth fistula (white arrow)

Erosion of bone covering lateral SCC.

Silver JA et al. 1987

Round window (black arrow)

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Companion Patient 3: Erosion of Facial n. canalObserve:

Familiar Landmarks:

Cochlea “snail”

“Snake’s eyes”: tympanic and labyrinth segments of facial n.

Pathology:

1.epitympanic cholesteatoma

Erosion of tegmen tympani, scutum, ossicles

2. erosion of bony canal of facial nerve

Waizel, S. http://www.emedicine.com/RADIO/topic676.htm

Erosion of facial n. canal

Coronal C- CT, Right ear

Comparison: Coronal C- CT, Left ear, Normal

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Companion Patient 4: Identify the Structure

PACS, MEEI. Courtesy of Dr. Hines-Parelta

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Companion Patient 4: TORP

Total ossicular replacement prosthesis (TORP)

http://www.kbbmnt.com/torpporp.html

PACS, MEEI. Courtesy of Dr. Hines-Parelta

TORP:

positioned between stapes footplate and tympanic membrane.

prosthesis is placed perpendicularly to footplate to ensure optimum sound transmission.

Axial C- CT, Left ear

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Companion Patient 4: Hearing Loss s/p TOPR

10-year-old male with a congenital cholesteatoma (left) s/p removal with TORP.

Now with decreased hearing for 2 years.

Evaluate for recurrence.

PACS, MEEI. Courtesy of Dr. Hines-Parelta

Axial C- CT, Left ear

Axial C- CT, Left ear

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Companion Patient 4: Malpositioned Prosthesis

10-year-old with a congenital cholesteatoma (left) s/p removal with TORP.

Now with decreased hearing for 2 years.

Evaluate for recurrence.

Observe:

No soft tissue mass/recurrence.

Thickened TM secondary to scarring.

Prosthesis displaced anteriorly and articulates mostly with bone adjacent to oval window.

PACS, MEEI. Courtesy of Dr. Hines-Parelta

Axial C- CT, Left ear

Axial C- CT, Left ear

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Management: Surgical Options

Surgery to remove Cholesteatoma is the mainstay of treatment.

Indication: eradication of disease, complications

Options:

Atticotomy: transcanal

Simple mastoidectomy

Tympanomastoidectomy:

Canal-wall-down procedure:

Bony partition between external ear canal and mastoid removed.

Outcome: enlarged meatus, difficult to fit hearing aids; low rate of persistant/recurrent dz, canal cleaning needed, can have problem with water exposure. single procedure.

Canal-wall-up procedure:

Bony parition intact.

Outcome: normal appearance, relatively high rate of recurrent/persistent dz, high tolerance for water exposure. Staged procedure to evaluate recurrence.

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Management: Conservative therapy

Conservative Therapy

Reserved for patients who are poor surgical candidates or refuse surgery

Regular ear cleaning

Topical microbial therapy +/- systemic therapy as adjunct

Helps control infection and may slow growth

Does not stop expansion

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Prognosis

Recurrent/residual cholesteatoma: 15 to 40% in 5 years

Canal wall up tympanomastoidectomies usually return to the OR for a “second look” in one year.

Regular follow up required.

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Netter FH. Atlas of Human Anatomy. 3rd Edition. Icon Learning Systems. 2003.

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Reeder MM. Reeder and Felson's Gamuts in Radiology. 4th Edition. Springer 2003. (Gamuts B-31 and B-32)

Roland PS. Middle Ear, Cholesteatoma. eMedicine. 2006 http://www.emedicine.com/ent/topic220.htm Swartz JD. Cholesteatomas of the Middle Ear: Diagnosis, Etiology and Complications. Symposium on CT of the Ear, Nose and Throat. Radiologic Clinics of North America. Mar 1984. 22:1. p 15-35.

Silver JA, Janecka I, Wazen J, Hilal S, Rutledge JN. Complicated Cholesteatomas: CT Findings in Inner Ear Complications of Middle Ear Cholesteatomas. Radiology 1987; 164:47-51

Swartz JD et al. The temporal bone. Head and Neck imaging. Radiologic Clinics of North America. Sept 1998. 36:5.

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Watts S, Flood LM, Clifford K. A systematic approach to interpretation of computed tomography scans prior to surgery of middle ear cholesteatoma. The Journal of Laryngology and Otology, 114 (200), 248– 253.

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