Chronic Otitis Media - Squamous

(Theories and spread of cholesteatoma, clinical features, excluding

treatment)

DR. Bikash Gurung

Second year resident
GMSMA ENT-HNS
MMC,TUTH,IOM
 Content
1. History
2. Definition
3. Classification
4. Theories
5. Pathways of spread
6. Prevalence
7. Clinical features
8. Diagnosis
9. Recent advances
 History
 1838 – Johannes Muller described a “layered pearly tumor of fat”
 1850- Toynbee: Molluscous tumor – arise in hair follicles/glands.
 Cruveilhier – Tumeur perlee (Pearly Tumor)
 Tumarkin, 1961- Epidermosis
 McGuckin,1963 – Keratosis
 Schuknecht,1974 – Keratoma

 Cholesteatoma: Chol (Bile) + steatos (Fat) + -oma (Tumor)

 INTRODUCTION
 Chronic otitis media (COM): An inflammatory process middle-ear space - long-
term, permanent changes in TM including atelectasis, dimer formation,
perforation, tympanosclerosis, retraction pocket development, or
cholesteatoma
(Glasscock-Shambaugh 6th Ed.)
 COM squamous – retraction of pars flaccida or tensa (PS) - retained squamous
debris and can be associated with inflammation and production of pus
(Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery 8th Ed.)
 COM Squamous - types
Inactive /
Retraction
• Retraction of pars flaccida or
tensa (PS) which has
potential to become active
with retained debris
• Epidermization
Active /
Cholesteatoma
• Retraction of pars flaccida or
tensa (PS) that has retained
squamous debris and
associated with inflammation
and production of pus, from
adjacent mucosa.
5
 Cholesteatoma - Definition

 Cyst-like, expansile lesion of the temporal bone lined by stratified squamous

epithelium that contain desquamated keratin
(Cummings, 6th
th Ed.)

 Cholesteatoma is an erosive process defined by trapped squamous epithelium that

produces and accumulates desquamated keratin debris.
(Shambaugh, 6th Ed.)
Most often middle ear and mastoid
Anywhere in pneumatized temporal bone
Classification
Congenital
Acquired

• Primary
• Secondary
• Tertiary (Iatrogenic)

7
Congenital Cholesteatoma
Teed’s Epithelial cell rest theory

Friedberg’s implantation theory (amniotic fluid)

“epidermal inclusion cyst”
Ruedi’s invagination behind
theory intactinfection)
(inutero TM

Michael’s epidermoid formation theory

Aimi’s epithelial migration theory (overcome annulus)

8
Congenital Cholesteatoma

“epidermal inclusion cyst” behind intact TM

Theories
1. Epithelial cell rests in anterior epitympanum
2. Invagination of squamous epithelium from developing
ear canal
3. Ingestion of squamous elements in amniotic fluid

9
Criteria of Derlaki and Clemis :

1. White mass medial to intact TM

2. Normal pars tensa and flaccida
3. No previous history of ear
discharge, perforation or otological
procedures
4. Prior bouts of AOM not excluded

10
 Congenital Cholesteatoma
 Commonly occur in :
1. Anterior epitympanum
2. Petrous apex
3. Cerebellopontine angle

11
Staging of Congenital Cholesteatoma

 Derlacki and Clemis (1965) first to stage congenital

cholesteatoma:
1. Petrous pyramid cholesteatoma
2. Cholesteatoma involving the
mastoid cavity
3. Cholesteatoma involving the middle
ear cavity.

12
Potsic’s staging:

Stage I : Single quadrant involvement with no

ossicular / mastoid involvement.
Stage II : Multiple quadrant involvement with no
ossicular / mastoid involvement
Stage III : Ossicular involvement without
mastoid involvement
Stage IV : Mastoid extension

13
Nelson's staging:
Type I : Involvement of mesotympanum
without involvement of incus / stapes
Type II : Involvement of mesotympanum / attic
along with erosion of ossicles without
extension into the mastoid cavity
Type III : Involvement of mesotympanum with
mastoid extension

14
 C/F
 Mean age of presentation is 4-5 yrs (Bennett et al,
2006)
 M:F = 3:1 (Freiderg et al 1988)

 Incidence is 0.12 per 100,000 people ( Mirco et al, 2000)

 Asymptomatic
 Hearing loss (CHL/SNHL)
 No prior history of otitis media
 Usually painless condition
 Vertigo/facial palsy
 Loss of Caloric Response Source:
 White mass visible through intact TM Sataloff’s1
15
st
Ed
 Investigations

 PTA: Usually normal

 CT scan: low attenuated area
 MRI: investigation of choice

16
Acquired Cholesteatoma

Theories for Acquired Cholesteatoma

1. Invagination theory
2. Basal cell hyperplasia theory
3. Epithelial invasion (Migration) theory
4. Metaplasia theory
5. New theories

17
 18
Cumming’s 6th Edition
 Theories contd…
1. Invagination –
Whitmaack, 1933

Chronic ETD - retraction pocket in the weakest portion of the

TM

Continued negative pressure deepens retraction pocket

keratin debris accumulates

19
Supported by experimental creation of retraction pockets by obstructing
ET and EAC ligation
Pars flaccida retraction Pars tensa retraction
 Theories contd…
2. Basal cell hyperplasia theory
Lange -1920s, supported by Ruedi

Epithelial cells (prickle cells) of pars flaccida invade sub

epithelial tissue

Basal lamina breaks  invasion of epithelial cones into sub

epithelial connective tissue

Microcholesteatoma formation

Microcholesteatomas enlarge perforating

21 TM
 Theories contd…
3. Epithelial invasion theory - Habermann,1889

After perforation

Keratinizing epithelium introduced or migration

into ME

22
 Contd…
 Epithelial
cells will migrate along a surface by process called Contact guidance
& when they meet another epithelium –stop migrating ie Contact Inhibition
(Weiss et al;1958 )

 Inner mucosal layer –damaged –inflammation-allowing outer keratinizing

epithelium to migrate inwards cholesteatoma formation
(Jackson et al;1978)

 Cytokeratin,CK -10 seen in meatal epidermis & migrating epitheliumlocated

in cholesteatoma matrix

(Grote et al, 1988)

 Theories contd…
4. Metaplasia – Sade’s

Transformation of chronically inflamed ME mucosa into

keratinizing epithelium e.g Barrett’s Esophagus

Keratinizing area enlarge-accumulate debris & contact TM

intercurrent infection & inflammation –cholesteatoma 

lysis of TM & perforation
24
Combination of Retraction and Proliferation
Theory
 IHC of attic cholesteatomas showed
proliferation of keratinocytes within epithelial
cones growing toward underlying stroma.
 Cones with focal discontinuities of basement
membrane.

4-step Theory
1. Retraction pocket stage
2. Proliferation stage of into
cone formation and fusion
3. Expansion stage
4. Bone resorption Sudoff and Toss,2000
25
 Theory:
 origin of a retraction pocket—natural attempt by the body to cure an
underlying inflammation. Analogous phenomena ;the migration of the
omentum.
• mucosa of the undersurface of the TM +
mucosa of the lateral surface of ossicles
• become coupled

• Mucosal blanket migration or mucosal

migration or sequential adhesion
• Mucosal bilayer becomes reabsorbed
Fig. Three potential types of mucosal interaction that drive cholesteatoma formation.
 Tertiary Cholesteatoma:

 Implantation of Squamous epithelium into middle ear

 Accounts for small proportion of cholesteatoma
 Trauma to TM
 Squamous epithelium left behind tympanomeatal flap or TM
graft
 Cytokeratin protein subunit : ME cholesteatoma and skin of EAC
and TM similar
Spread - Pathways

32
Most frequent origins:

 Posterior epitympanum (most common)

 Posterior mesotympanum

 Anterior epitympanum

33
Epitympanum Cholesteatoma

Originates in Prussak’s Pouch

◦ Between pars flaccida and neck of
malleus
◦ Floor is lateral process of malleus
and ass. folds

Fig. Posterior epitympanic cholesteatoma

passing through the superior incudal
space and the aditus and antrum

34
Posterior Epitympanum Spread
 Through superior incudal space lateral to body of
incus
 Then through aditus and antrum to mastoid

35
 Posterior Mesotympanum Spread

 Reaches ME descending through floor of

Prussak’s space into posterior space of von
Troeltsch
◦ Von Troeltsch’s space
 between TM and posterior mallear fold
 Inferior edge is chorda tympani nerve
◦ Open to mesotympanum
◦ May involve stapes, round window, sinus
tympani, facial recess
36
 Anterior Epitympanum Origin
Retraction anterior to head of malleus Bordered
by:
◦ Anteriorly is middle cranial fossa, petrous tip,
zygoma root
◦ Posteriorly is cog extending to cochleariform
process
◦ Superiorly by middle cranial fossa
◦ Laterally by tympanic bone and chorda tympani
nerve
◦ Floor associated with facial nerve -> may affect
CN7

Extend to supratubal recess via anterior pouch of 37

von Troeltsch’s space.
 Posterior Mesotympanum Origin

 Pars tensa retraction

Passes medial to malleus and incus
Invade:
◦ Sinus tympani
◦ Facial recess

38
Mucosal changes:
Chronic inflammation + Edema +
Submucosal Fibrosis + Hypervascularity

Area of mucosa ulcerate—

proliferation of blood vessels,
fibroblast and inflammatory cells

Granulation
tissue

39
 Ossicular chain:
Resorptive osteitis
Long and Lenticular process (I) > Suprastrucure (S) > Crurae (S) > Body (I) >
Head (M) > Handle of Malleus

Infection/Inflammation/Pressure/Keratin –Cytokines
Protein mediators: IL1,6,TNF, Growth Factors
Non Protein: PG, Neurotransmitter, NO

Recruit and Activate: Osteoclasts

40
 Contd…
Cholesterol Granuloma

 Found in active COM

 Area of giant cell reaction around

cholesterol crystal

 Breakdown of hemorrhage, Middle ear

effusion
Bone erosion in cholesteatoma:

1. Pressure theory 2. Pyogenic osteitis

3. Enzymatic theory
theory

42
Natural history of Inactive COM Squamous

Progression:
 Active COM squamous
 Loss of elasticity and mobility of TM
 Erosion of Ossicles

Resolve : Pars tensa retraction in children with OME resolves

spontaneously in 70% of cases

Static : Some
43
Natural history of Active COM Squamous
Progression toward healing
Automastoidectomy Cavity
 Disease process selectively erode outer attic
wall +/- posterior canal wall
Progression of active disease
 Involvement of ossicular chain
 Bony erosion - IC and extracranial complication

44
Childhood cholesteatoma Adult cholesteatoma
Cont…
Recidivistic disease is higher Lower

Pneumatic mastoids Sclerotic mastoids

Pars tensa cholesteatoma higher Attic cholesteatoma higher

Involves extremes of middle ear More frequently –ant

space epitympanum
Cholesteatoma spread –post post epitympanum
mesotympanum
45
 Epidemiology
 Incidence :
9.2/1,00,000 per year in adults and 3/1,00,000 per year in children
Danish and Finnish individuals

 4.4% Bilateral & M:F = 1.4

(Kemppainen et al,1999)

 Prevalence of ear drum pathology in Nepal – 7.4 %

COM squamous (AA) – 9.7%,
(Little et al,1993) 46
Clinical History
Detailed otologic history
Otorrhea - scant but persistent, mucopurulent, foul-smelling &
blood stained
Hearing loss
Otalgia – associated AOE, IC complications
Tinnitus
Vertigo
Symptoms s/o complications
Prior surgery
Previous h/o middle ear diseases

47
SYMPTOMS Adult (%) Children (%)
Otorrhoea 85.1 88.9
Heaing Loss 68.8 66.3
Pain 14.4 12.6
Dizziness 8.2 0
Facial Palsy 1.2 0.5
Tinnitus 6.5 2.0
Abscess 1.3 0
Neither deaf nor 1.7 2.1
discharge Scott-Brown’s, 7th Ed.

Scott’s Brown 7th edition 48

Pars Flaccida Retraction Pocket
Grading Tos’s grading system
(1982):

49
Grade I Grade II

Grade III Grade IV

Sade’s atelectasis staging system
(1976):

51
Grade I Grade II

Grade III Grade IV

Posterior Superior Retraction
Staging (Sade)

53
OTOMICROSCOPY:- Gold
standard
• Discharge, TM & polyp removal.

• x6 – Cleaning meatus.

• x10 – TM examination.

• Great value in children-inspect

TM closely

54
 Assess in OPD
• Essential to record : RP
• Occurs in pars tensa or flaccida
• Totally in view or part out of view
• Self cleansing or not
• Part out of view may not be self cleansing ,
potential to become active with cholesteatoma

55
 Assess in OPD
• Foul smelling white or yellowish colored cheesy
material +/-
• Discharge +/- bony erosion

• Attic or posterior TM
• In majority , cholesteatoma extent can’t be
determined otoscopically
• Aural polyp obscuring attic or in PSQ

56
 Assess in OPD
• Clean ear thoroughly of discharge & debris with cotton-tipped applicators
/suction
• Culture wet, infected ears and treat with topical and/or oral antibiotics
• Mobility assessment: pneumatic otoscopy/tympanometry
• Hearing evaluation : tuning fork : 512 HZ , PTA: degree and type of
hearing loss

• Fistula test
• Status of facial nerve
57
58
 Radiology
X-ray MASTOID :
Towne’s view/ Lateral oblique view
- Position of dural and sinus plate
- Degree of Pneumatization
- Cavity
- Approach to surgery

Key areas : attic, aditus, antrum 59

Lateral oblique views

60
Towne’s View

61
HRCT Temporal bone

0.6 mm section coronal and axial plane of temporal bone

Role of CT scan :
 Revision cases due to altered landmarks from previous surgery
 Diagnosis of COM ,TM not be visualized (narrowing or stenosis of
EAC)
 Suspected congenital abnormalities
 In cases with complication

62
 HRCT - Cholesteatoma
HALLMARK
1. Erosion & destruction of outer attic wall (Scutum)
2. Widened/Destructed aditus extends into antrum (Coalescent)
3. Displacement/Destruction of ossicles
4. Fistula Formation with LSCC/PSCC
5. Erosion into facial canal
6. Dehiscence of Tegmen Tympani/ Sigmoid Plate with/without thrombosis
7. Automastoidectomy
8. Erosion and sagging of external canal roof/PS part.
63
Blunt scutum, ossicles- Displaced ossicles, widened aditus ad antrum
Labyrinthine fistula Tegmen tympani defect
Sinus plate defect
 Drawbacks of HRCT
• Granulation tissue vs. cholesteatoma

• Specific soft tissue problems

– Dural involvement
– Abscess
– Brain herniation
– Labyrinth involvement
MRI needed
– Sigmoid sinus thrombosis

67
CT scan is generally considered to be
superior to MRI for the detection of 68

choesteatoma-induced bone erosion

Fig. HRCT Temporal bone Fig. Destruction of petrous apex
axial view showing erosion 69

of sinus plate and LSCC

MRI:
Delineates intracranial pathology that complicates COM

 Cholesteatoma differentiated from other soft tissue

 Assessment of lesion near the petrous apex

 Diagnosing residual cholesteatoma

 Limitation: Poor visualization of bony landmark

Diffusion weighted MRI allow detection of smaller lesions and may be sufficient
to replace second-look surgery in patients with prior cholesteatoma
resection 70
(Schwartz et al, 2011)
Vercruysse et al, 2009
 Magnetic Resonance

Dubrelle F et. al. Diffusion weighted MR imaging sequence in the detection of

postoperative recurrent cholesteatoma. Radiology Feb 2006; 238 (2): 604-610.
Others:
ENDOSCOPY:
 In teaching
 Photograph: Comparison of retraction
pocket over time

BACTERIOLOGY:
 Valuable: Ear is active or in infective
complication
73
(Cummings, 6th Ed.)
I. Congenital cholesteatoma
II. Acquired cholesteatoma
1) Retraction pocket cholesteatoma
a) Pars flaccida cholesteatoma (Attic cholesteatoma)
b) Pars tensa cholesteatoma (Sinus cholesteatoma)
c) Combination of pars flaccida and pars tensa cholesteatoma

2) Nonretraction pocket cholesteatoma

a) Cholesteatoma secondary to a chronic tensa perforation Cholesteatoma
following trauma and/or otologic procedures

3) Post surgery cholesteatoma( residual, recurrent)

III. Unclassifiable cholesteatoma

76
Divisions of the middle ear space using
the STAM system

Difficult access sites (S), tympanic

cavity (T), attic (A), and mastoid (M).
S1; supratubal recess (also called the
anterior epitympanum
or protympanum) and S2; sinus
tympani
Staging systems for respective cholesteatoma types

1) Pars flaccida cholesteatoma (attic cholesteatoma)

Stage I: Cholesteatoma localized in the attic
Stage II: Cholesteatoma involving two or more sites
Stage III: Cholesteatoma with extracranial complications
Stage IV: Cholesteatoma with intracranial complications
2) Pars tensa cholesteatoma, cholesteatoma secondary to a tensa
perforation, and congenital cholesteatoma
Stage I: Cholesteatoma localized in the tympanic cavity
Stage II: Cholesteatoma involving two or more sites
Stage III: Cholesteatoma with extracranial complications
Stage IV: Cholesteatoma with intracranial complications

 The staging system does not apply to petrous bone cholesteatoma

Cholesteatoma with extracranial complications or pathologic
conditions includes
 Facial palsy,
 Labyrinthine fistula: with conditions at risk of membranous
labyrinth,
 Labyrinthitis,
 Postauricular abscess or fistula,
 Zygomatic abscess,
 Neck abscess,
Cholesteatoma with intracranial complications includes
 Purulent meningitis,
 Epidural abscess,
 Subdural abscess,
 Brain abscess,
 Sinus thrombosis, and
 Brain herniation into the mastoid cavity.
L - Life threatening complications
LX – Not identifiable (not specified)
L2 – Mastoiditis, Mastoid fistula, Bezold’s or Luc’s abscess,
Facial palsy, Labyrinthine fistula, Labyrinthitis or Tegmen
defect requiring surgical repair
L4 – Meningitis, Brain abscess, Seizures or Sigmoid sinus
thrombosis
E - Eustachian tube ventilation and mastiod
pneumatisation
EX – Not identifiable (not specified)
E0 – > 50 % of cells aereated
E1 – < 50 % of cells aereated
E2 – Poor pneumatisation and ventilation
Staging classification

•Stage I – sum of classification values between 1 and 3 points

•Stage II – sum of classification values between 4 and 8 points

•Stage III – sum of classification values over 8 points

If at least one of the classifications (Ch, O, L, E) is not identifiable /

not specified “x” postfix (Ix, IIx, IIIx) is given
 References:
1. Scott-Brown, 7th & 8th Edition
2. Cumming’s otorhinolaryngology, 6th Edition
3. Shambough’s Surgery of Ear, 6th Edition
4. Images from Textbooks and google images
5. Pubmed for articles

89
THANK YOU