Cholesteatoma etiology, theories, clinical features and management

1. CHOLESTEATOMA
DR. DIVYA RAANA
ENT PG
NMC

5.  Cholesteatoma is defined as a sac of keratinizing stratified squamous
epithelium in the middle ear cleft containing desquamated epithelium
as keratin debris.
 Has bone eroding property.
 Also described as ‘ Skin in the wrong place ’.
 Cholesteatomas are the end stage of (squamous epithelial) retractions
of the pars tensa or flaccida that are not self-cleansing, retain
epithelial debris and often elicit a secondary, inflammatory mucosal
reaction.

6. Cholesteatoma
Congenital Acquired
Primary Secondary

7.  Arises from embryonic
epidermal cell rests in middle
ear cleft or temporal bone.
 Occurs at : Middle ear, petrous
apex or cerebellopontine angle
 Presents as white mass behind
the intact TM

8. LEVENSON CRITERIA:
 White mass behind intact TM
 Normal pars flaccida and pars tensa
 No prior H/O otorrhea or any otological procedure

9. Primary:
 No previous H/O ear discharge or TM perforation
Secondary:
 Occurs in already diseased ear [ pre-existing TM perforation ]

10.  Retraction pocket theory
 Theory of migration
 Metaplasia
 Implantation theory
 Basal cell hyperplasia

11.  (A) Invagination theory
(retraction pocket theory).
 (B) Epithelial invasion or
migration theory
(immigration theory).
 (C) Squamous metaplasia
theory.
 (D) Basal cell hyperplasia
theory (papillary ingrowth
theory).

12. • Origin and Most vigorous : zone 1

13. GROWTH
PATTERN OF
CHOLESTEA
TOMA
Most common sites of origin:
Posterior epitympanum
Posterior mesotympanum
Anterior epitympanum

14.  Cholesteatomas invade
the middle ear and
mastoid along fairly
predictable pathways.

16.  Posterior epitympanic cholesteatoma
penetrates the epitympanum and
posterior mesotympanum lateral to
the ossicles.

17.  Posterior epitympanic
cholesteatoma
penetrating the aditus-
ad-antrum.

18.  Posterior epitympanic
cholesteatoma involving
the mastoid which
penetrated the
epitympanum and
posterior mesotympanum
lateral to the ossicles.

19.  Posterior mesotympanic
cholesteatoma illustrating the
pathways to the mastoid and
middle ear. Note that the
penetration to the mastoid is
medial to the ossicles.
 Posterior mesotympanic
cholesteatoma tends to
involve the posterior
tympanic spaces: the sinus
tympani and facial recess

20.  Anterior epitympanic
cholesteatoma penetrates the
supratubal recess and can
involve the geniculate
ganglion of the facial nerve

22. Ear discharge:
 Foul smelling – osteitis and saprophytic infection
 Continuous – osteitis
 Blood stained – granulations,osteitis
Hearing loss:
 Hearing is often preserved until a very late stage in ears containing
cholesteatoma in spite of the ossicular chain being disrupted. [keratoma
hearers]
 cholesteatoma sac bridges the gap between the functioning part of the
ossicular chain and the inner ear.
 The unfortunate consequence is that removal of the disease surgically may
reduce the hearing by increasing the air–bone gap.

23.  Ear ache : inflammation of meatal skin, intra cranial complication
 Dizziness : progression erosion of bony LSCC & fistula formation 
serous labyrinthitis
 Tinnitis
Signs :
 Foul smelling blood stained discharge, may contain whitish scales from
cholesteatoma
 Attic perforation or marginal perforation
 Retraction pocket in attic
 Granulation tissue in postero superior quadrant of TM
 Occassionally polyp/granulation tissue may be seen coming out of the
perforation
 Tuning fork tests: rinnes test negative, webers- lateralized to affected ear

26. INVESTIG
ATIONS

27.  Size and site of the defect
 State of the remaining TM
 Appearance of middle ear mucosa
 Presence of polyp & granulation
tissue- site of origin
 Retraction pocket- classification
 Also for suction clearance &
diagnosis

28. Culture & sensitivity from ear discharge
Rigid oto endoscopy
Audigram [PTA]:
Imaging

30.  Schuller’s view
 To see bony erosion
 Anatomy of mastoid
 Occasionally to diagnose cholesteatoma  cotton wool appearance

33. Imaging features of cholesteatoma:
 The hallmark of cholesteatoma is bony destruction.
 Presence of soft tissue density in the middle ear cavity coexistent
with ossicular and mastoid bony erosion is highly specific for
cholesteatoma.
 In attic cholesteatomas, the ingrowth of epithelium from the pars
flaccida into the epitympanum is evident by the erosion of the
scutum.

34. Axial CT image showing
non-dependent soft tissue
in the Prussack’s space,
lateral to ossicles in a
patient with pars flaccida
cholesteatoma (arrow)

35.  Coronal CT image showing normal scutum on the left side (arrow).
It is seen as a point of superior attachment of the tympanic
membrane.
 Scutum is eroded on the right side with soft tissue seen in the
middle ear and external auditory canal.

36.  Axial CT reveals widening
of aditus (denoted by A) and
formation of common cavity
between epitympanum
(denoted by E) and aditus
with soft tissue within

37.  Axial CT image showing
non-dependent soft
tissue medial to ossicles
in a patient with pars
tensa cholesteatoma
(arrow)

38.  Axial CT through the
mesotympanum shows the
sinus tympani (yellow arrow)
and facial recess (white
arrow) which are hidden
areas by otological
examination and should be
carefully inspected on
imaging for the presence of
soft tissue.


39.  Axial section through the
epitympanum shows the
normal anterior epitympanic
recess (arrow) which is the
medial extension of
epitympanum. Its importance
lies in its close proximity to the
tympanic segment of the facial
nerve canal (*)

40.  Axial CT shows normal “ice cream cone” configuration of ossicles in
epitympanum on the left side formed by the head of the malleus and the
body of the incus. The right ear shows soft tissue in the middle ear and
aditus with non-visualization of ice cream cone suggesting erosion. A
large defect is also seen in the sinodural plate on the right side (yellow
arrow). Left sinodural plate is intact (white arrow)

41.  Coronal (a) CT image reveals the “snail eye” view where the turns
of cochlea form the body of snail and labyrinthine and tympanic
segments of facial nerve being the eyes (arrow).
 Coronal(b) CT image shows the erosion of the floor of the horizontal
segment of the facial canal (arrow). Erosion of lateral mastoid cortex
is also seen (asterisk)

42.  Labyrinthine fistula is a
dreaded complication of
cholesteatoma. The most
commonly affected is the
lateral semi-circular canal
due to its close proximity to
the middle ear cavity.
 Extensive disease can affect
cochlea and other semi-
circular canals also.
 Axial CT image showing
erosion of the lateral wall of
the lateral semicircular canal
(arrow) with the formation of
labyrinthine fistula

43.  Extensive bony destruction of the mastoid and ossicles can occur which may give
the appearance similar to postsurgical cases termed as automastoidectomy.
Spontaneous evacuation of cholesteatoma may be seen with this, termed as mural
cholesteatoma leaving behind no residual mass
 Axial CT shows the right mastoid cavity filled with soft tissue and is seen to open
directly into the external auditory canal suggesting automastoidectomy (arrow)

44.  Coronal CT image
showing erosion with focal
defects in tegmen tympani
(arrow).

45. In addition to the evaluation of complications of cholesteatoma, imaging
also helps in warning the surgeon of any variant anatomy that may
predispose to life-threatening consequences during surgery or may affect
surgical access to disease such as the following:
 Dehiscent facial canal
 High and dehiscent jugular bulb
 Anteriorly lying sigmoid sinus
 Low lying tegmen and dura
 Specific patterns of pneumatization and aeration of mastoid

46.  Conventional MRI shows cholesteatoma
as a soft-tissue mass but does not
differentiate this from other soft tissues.
 It does not provide enough bony detail to
be useful on its own.
 More recently, non-echoplanar DW-MRI
has been shown to be very reliable at
detecting cholesteatoma and
differentiating it from other soft tissues.
 It has been shown to be of value in
detecting residual cholesteatoma and
reducing the necessity for second-look
surgery

48.  The aims of management of active squamous COM are to relieve the
patient’s symptoms and to minimize the risks of complications of the
disease.
 Surgical removal is the only effective treatment for cholesteatoma.
 When surgical treatment is considered appropriate, topical treatment for
granulations and associated mucosal disease while awaiting surgery may
lessen the otorrhoea and therefore be socially beneficial.
 Unfit ptsCareful aural toilet with removal of squamous debris from the
retraction pocket and topical treatment at regular intervals

49. SURGERY

50. The aims of surgery for active squamous COM are:
 eradication of disease
 An epithelialized, self-cleaning ear
 Hearing maintenance or improvement.
Categorized as :
 open cavity (canal wall-down) mastoidectomy
 closed cavity (canal wall-up) mastoidectomy

52. The method of choice for removal
of cholesteatoma is Modified
Radical Mastoidectomy 
anterior to posterior approach.
Also k/a Small cavity
mastoidectomy, or
atticoantrostomy
The cholesteatoma is identified in
the epitympanum or posterior
mesotympanum and followed
backwards.

53.  Conservative method
 Exenteration of disease process by removing posteriosuperior meatal
wall making middle ear, attic, aditus, antrum and cellular cavity
into a single chamber.
 Added various suitable tympanoplasty surgeries
 Accessory procedures like conchomeatoplasty of mastoid cavity

54.  Traditional method was posterior to anterior approach.
 The mastoid was opened behind the external auditory canal, the
cholesteatoma identified and followed forwards through the aditus into
the attic with removal of the posterior bony wall of the canal.
 This usually resulted in a large cavity, much larger than is required to
control the disease.
 Large cavities can be problematic : many continue to discharge and they
often do not self-clean.

55.  Beginning removal of
the posterior ear canal
wall.

57.  Lowering facial ridge
does not require exposure
of the facial nerve. It is
best to leave 2 to 3 mm of
bone over the nerve to
maintain a middle ear
space.

58.  Lowering the floor of
the middle ear to the
level of the facial ridge
(base of the ear canal).

59.  Cartilage and fascia
reconstruction in a canal
wall down mastoidectomy
 At last conchomeatoplasty
is done

60. In recurrent cases  Following significant differences are noted:
 High facial ridge
 Sump in cavity below floor of external auditory canal
 Perforation in tympanic membrane
 Small external auditory meatus.
 Can be reduced by good surgical technique and by partial obliteration of
cavities, either with cartilage or prosthetic materials.

61.  The cavity must be rounded and smoothly contoured with no over-
hanging ridges and no facial ridge in order to allow migration of
epithelium.
 The tympanic membrane should be repaired to close all
communication between the mastoid cavity and the mesotympanum
and Eustachian orifice.
 The meatus should be an adequate size relative to the size of the
cavity, so a meatoplasty is almost always required.

62.  The conchal cartilage removed from the meatus should be used to
reduce the size of the cavity.
 Lower rates of recurrence of cholesteatoma (5–15%)

63.  Combined approach tympanoplasty.
 Exploration of mastoid cavity by removing outer cortical wall of
mastoid antrum.
 Saucerization of medial wall of mastoid antrum with intact canal
wall.
 Middle ear structures are not disturbed.

66. Intact canal wall technique advantages :
 Middle ear air space is maintained
 Normal appearance of EAC
 For tympanoplasty procedure convenience

67.  Incidence of recurrent and residual cholesteatoma is high (20–50%),
therefore second-look operations after 12–18 months are necessary
in most cases, and some cases require further procedures
subsequently.
 Second look can often be avoided nowadays by the use of DW-MRI.

69.  Post-operative hearing results are better following canal wall-up
mastoidectomy.
 Many surgeons, by doing canal wall-down surgery but partially
obliterating the cavity, achieve an outcome that is similar to canal
wall-up surgery.