Cholesteatoma is an abnormal skin growth in the middle ear that can cause hearing loss and other complications if left untreated. It arises from either congenital or acquired causes. Acquired cholesteatomas are more common and develop due to retraction pockets or epithelial migration through the eardrum. Diagnosis involves examination, audiometry and CT/MRI imaging. Treatment is surgical to fully remove the growth and restore hearing, with the goal of preventing recurrence. Complications can include bone erosion, infection, and damage to nearby structures like the facial nerve if not addressed. Close follow up is important after surgery to monitor for regrowth.

1. CHOLESTEATOMA
Dr Safika Zaman
Post-GraduateTrainee
Dept of ENT & Head Neck surgery
VIMS,RKMSP

2. Introduction
■ Abramson et al defined cholesteatoma as
–
“A three dimensional epidermal and
connective tissue structure, usually in the
form of a sac, and frequently conforming to
the architecture of various spaces of the
middle ear, attic and mastoid. This
structure has the capacity for progressive
and independent growth at the expense of
underlying bone and has the tendency to
recur after removal. .
Gray’s definition : cholesteatoma is “skin in
the wrong place

3. History of cholesteatoma
■ The French anatomist Du Verney first
reported a case of cholesteatoma-like
symptoms in 1683 .
■ In 1829, Cruveilhier described the
pathologic features of what he referred
to as pearly tumor.
■ In 1838 , Johannes Muller, a German
anatomopathologist,coined the term to
describe a tumor that appeared “greasy
in nature”.
■ Nonetheless, this is something of a
misnomer.

4. Epidemiology
■ Annual incidence of acquired cholesteatoma ranges from
approximately 9 to 12.6 cases per 100,000 adults and from 3 to 15
cases per 100,000 children.
■ Male predominance of - 1.4 : 1
■ In the paediatric population, cholesteatomas account for10% of
chronic otitis media cases.
■ There is a high prevalence in Caucasian populations.
■ Middle ear cholesteatoma peaks in the second and third decade
of life.

5. Classification of COM
Based on the disease pathogenesis:
1.Congenital
2.Acquired

6. Congenital cholesteatoma
Derlacki and Levenson’s critria
Approximately 2% of the entire
cholesteatoma cases .
Mean age of presentation is 4.5 yrs

7. Theories of Congenital
Cholesteatoma
■ Epidermal rest theory: This theory is
based on a finding of cell rests of
non-keratinizing squamous epithelial
cells, localized in the lateral wall of
the Eustachian tube, close to the
tympanic ring.
■ Inclusion Theory: Other authors favor
even a way of migration from cells
coming initially from the external ear
through non evident injuries of the
tympanic membrane

8. Acquired cholesteatoma
Most common type of
cholesteatoma.
Divided into –
1.Primary acquired.
2.Secondary acquired

9. Primary acquired
.1.pars flaccida
pocket,
2 pars tensa
pocket,
3. combined forms

10. Secondary cholesteatoma
■ Secondary to infection, trauma
or perforation

11. Prominent theories of etiopathogenesis of
acquired cholesteatoma
1.Invagination theory
(retraction pocket
theory)
2.Epithelial invasion or
migration (immigration
theory)
3.Squamous metaplasia
theory.
4. basal cell hyperplasia
theory (papillary
ingrowth theory)
Etiopathogenesis of Acquired Cholesteatoma:
Prominent Theories and Recent Advances in
Biomolecular Research Chin-Lung Kuo, MD

12. Invagination Theory (Retraction
Pocket Theory) of Toss & Wittmaack
Two things are
present in
cholesteatoma
and not present in
safe retraction
pocket: epithelial
hyperproliferation
and abnormal
Skin migration

13. Theory of Epithelial Migration (Immigration
Theory) of Habermann & Bezold
Perforation of the eardrum, traumatic or iatrogenic, gives
access to the squamous epithelium of the eardrum or of the
outer ear canal skin, to invade or migrate into the middle ear
leading to the formation of a Secondary acquired
cholesteatoma excessive production of
keratin lead to a cholesteatoma formation.

14. The squamous metaplasia theory of Wendt
■ The metaplasia theory stipulated that the epithelium of the
middle ear changes into squamous epithelium under the effect
of a persistent chronic inflammation.

15. Basal Cell Hyperplasia Theory (Papillary
Ingrowth Theory) of Lange
Basal cell hyperplasia theory postulates that keratin-filled
microcysts, buds, or pseudopods formed in the basal layer of the
pars flaccida epithelium, invade the sub-epithelial tissue, fuse
together, resulting in the formation of cholesteatoma of Prussak’s
space.

16. • Arise from postero superior quadrent
• Nonaerated spaces growth
• Stable atelectasis and cholesteatoma
• Normal Eustachian tube and tube
• Tympanostomy does not prevent
• Associated with mastoid hypo-
pneumatization

17. Cont…

18. CHOLE CLASSIFICATION

19. Paediatric vs Adult cholesteatoma
■ Paediatric cholesteatomas present a more exacerbated
inflammatory degree
.
■ In children recurrence rates were higher than that in adults.
■ 80% retraction pockets of the pars tensa whereas in adults,
■ Postoperative hearing levels were better in children,

20. Histopathology of cholesteatoma

21. Electron microscopy of
cholesteatoma
■ matrix has the same
histological and cellular
structure as the
epidermis of EAC.
■ Inflammatory cells,
Langerhans’ cells, and
Merkel cells are
identified in the stratum
spinosum layer of the
cholesteatoma matrix in
a higher amount
compared to the normal
epidermis

22. Genomic alteration
■ the cholesteatoma epithelium exhibits a significantly higher
percentage of proliferation marker-labelled cells.
■ Aneusomy of chromosomes 7 and 17 has also been suggested
to play a crucial role in cholesteatoma growth and bone
destruction.
■ Microarray analysis by. revealed that the expression of GJB2
gene is higher in cholesteatoma tissue than in the skin of the
external auditory canal(codes connecxin 26).
■ James et al. found that only 14% of children with
cholesteatoma present variants of the gene GJB2.

23. Moleculer pathogenesis
■ Langerhans’ cells require the
cooperation of activated T-
lymphocytes to become functional.
These activated T-lymphocytes
represent the “vital union” .
■ Langerhans cells have tropism
towards the keratinized squamous
epithelium.

24. Cont…
The fundamental difference between the healing process in normal
skin and in cholesteatoma, is that in cholesteatoma there is a loss of
the growth inhibition by “cell to cell contact”. Two factors are
involved in this loss of growth control in cholesteatoma:
1.The cholesteatoma develops beyond its normal anatomical site for
a “skin”. The middle ear environment is not adequate to induce the
habitual cell contact inhibition.
2. The inflammatory process produces a self-maintained
immunological cycle

25. Moleculer
pathogenesis

26. Role of angiogenesis
Release a variety of angiogenic factorsvascular( VEGF, EGF,
TGF-a, PDGF, IL8),
Angiogenesis within the perimatrix
Migration of keratinocytes into the middle ear cavity
Progression of disease

27. Bone absorption
■ the labyrinth- most rigid bone of the body.
Factors are-
inflammation
local pressure,
specific enzymes –MMPs, collagenase
prostaglandin E
Acidic pH , of keratin debris is a critical factor in bone destruction

28. Role of infection
■ Pseudomonas lipopolysaccharide has
been shown to activate keratinocyte
hyperproliferation
■ Bacteria also prevent the
cholesteatoma epithelium from
activating terminal differentiation
and returning to a quiescent state.

29. Biofilm formation
The keratin layer of cholesteatoma is an
ideal environment for biofilm
development.
The presence of antibiotic-resistant
bacterial biofilms in cholesteatomas may
also explain their aggressiveness
Evidence for microbial biofilms in cholesteatomas.
Richard A. Chole, Brian T. Faddis
Published 2002
Biology, Medicine
Archives of otolaryngology--head & neck surgery

30. Pathogenesis

31. Pathway
■ Growth pattern of the acquired cholesteatoma is oriented by
two main factors:
1. site of origin of the cholesteatoma.
2.the anatomical compartments in the middle ear cleft.
■ The ligaments, mucosal folds, ossicles, and walls of the
middle ear separating do not play the role of barriers but guide
the growth of cholesteatoma into distinct pathways
throughout the middle ear cleft.
■ Identification of pathway is not possible in advanced stage.

32. Routes of spread

33. Clinical presentation
■ Foul smelling discharge
■ Hearing loss
■ Persistent otitis externa
■ Vertigo, tinnitus
■ Balance disorder
■ Facial paralysis.
■ Mastoid abscess
■ Headache, otalgia, vomiting, seizure

34. Diagnosis
■ Otoscopic examination:

35. Audiometric evaluation
■ Pure tone audiometry- CHL with
good word recognition score.
■ A conductive deficit more than
40 dB indicates ossicular
discontinuity.

36. CT Scan
■ Not to diagnose
■ To learn disease extent.
■ HRCT of the temporal bone
is indispensable to
otologists for surgical
planning
■ Reading a cd rather than a
plate is more informative.

37. What to look for in a CT-Scan
■ extent of disease
■ possible osseous destruction
■ middle ear hypoplasia,
■ jugular bulb variations
■ bony dehiscence of the facial
nerve
■ sclerotic or diploic mastoids
■ anterior sigmoid sinuses
■ other complications like fistula
or dehiscence.

38. Limitation of a CT Scan & Cone beam
CT
■ similarities in the density of
CT scans for cholesteatoma,
granulation tissue, fibrous
tissue,mucosal edema, and
effusion greatly limit the
ability of HRCT to distinguish
among these disease
entities.
■ Exposure to radiation
Morphologic examination of the temporal bone by cone beam
computed tomography: Comparison with multislice helical computed
tomography
Author links open overlay panelM.Dahmani-
CausseaM.MarxaO.DeguineaB.FraysseaB.LepagebB.Escudéc
https://doi.org/10.1016/j.anorl.2011.02.016
There was no significant
difference in morphologic
assessment of the temporal
bones on the two techniques of
CBCT and MSCT. CBCT delivered
22 times less radiation than
MSCT .

39. DW-MRI
■ Diffusion-weighted (DW) sequences are highly promising in
differentiating recurrent cholesteatoma from granulation tissue
DW MRI depends on the difference in diffusion of water
molecules in different biological tissues. Water molecules in
cholesteatoma are less mobile giving rise to a hyperintense
signal. In granulation tissue, water molecules are more mobile
and thus appear less intense on DW sequence.
■ Currently, single-shot echo-planar DWI is the most used DWI
technique.

40. DW-MRI

41. Treatment
■ Treatment is essentialy surgical.
■ Choice of surgery will depend
upon-
1. Extent of disease
2. Available facilities
3. Surgeon’s expertise
4. Patients willingness towards long
term follow up

42. Patient counselling

43. Cont…

44. Objective of surgery
■ 1.Total eradication of cholesteatoma to
obtain a safe and dry ear.
■ 2.Maintain the best condition for a
successful wound healing process in the
ear.
■ 3. Restore or maintain the best functional
status of hearing.

45. Surgical Procedures
■ 1) A CWD mastoidectomy
■ 2) CWU mastoidectomy
■ 3) Other procedures:
• Reconstruction of the ear canal defect..
• Atticoantral mastoid obliteration can be done
after CWU or CWD.
• Ossicular reconstruction must be decided.

46. Transcanal anterior
Atticotomy
■ indicated for a cholesteatoma
with limited involvement of the
middle ear, with intact ossicular
chain and/or a healthy
epitympanum.
■ Reconstruction of the resulting
cavity is done with cartilage and
perichondrium.

47. CWD Mastoidectomy
■ Indications
• Cholesteatoma of an only hearing ear,
• A major erosion of the posterior bony canal wall,
• A history of vertigo due to a labyrinthine fistula,
• A poor Eustachian tube function,
• A sclerotic mastoid with limited access to the epitympanum .
• Patient non-compliant for follow-up.

48. CWD
■ Advantages
■ • Relatively short duration of
the surgery
■ • Easy detection of the
postoperative residual disease.
■ reduced rate of recurrences;
■ the facial recess is well
exteriorized as well as the
attic.
■ • Any postoperative
cholesteatoma regrowth can
readily dealt.
■ Disadvantages
■ • Hearing reconstruction is less
successful.
■ • Open cavity: the mastoid bowl
maintenance can be a lifelong
problem. Unpleasant
■ appearance of the meatoplasty
■ Wet cavity
■ Difficulty in fitting hearing aid

49. CWU Mastoidectomy
In modern otosurgery, CWU must be the
first choice for most cholesteatoma cases.
■ Disadvantages
■ • Long duration of the surgical
procedure in extended pathologies.
■ • Unsatisfactory exposure and high rate
of residual disease.
■ • Staging and multiple surgical looks

50. Endoscopic and Microscopic
■ endoscope assisted-microsurgery
allows the use of the most
efficient tools to face difficult
sites of localization of the
cholesteatoma, and allow its
complete removal.

51. Follow-up
■ 1. A surgical second-look procedure should be proposed always
for patients when a complete removal of the disease during the
primary surgery was uncertain.
■ 2. MRI evaluation may be appropriate to avoid a surgical second
look for patients when the otologist was sure that all the
disease has been completely excised by his first surgery and
when an unequivocal normal microscopic examination is
observed during the first 6 months of the postoperative period.

52. Complication of cholesteatoma
■ Otogenic intracranial complication:
meningitis,
Otitic hydrocephalus,
lateral sinus thrombosis,
cavernous sinus thrombosis.
intracranial sepsis-extradural, epidural,
subdural, peri-sigmoid sinus and cerebral
abscess formation.

53. Complication of cholesteatoma
■ Labyrinthine fistula
■ Labyrinthitis
■ Facial nerve paralysis

54. Atypical cholesteatoma
Congenital Cholesteatoma Localized to the Tip of the Mastoid
Bone: A Case Report and Possible Etiology
Seok Min Hong, Jun Ho Lee, Chan Hum Park, and Hyung-Jong Kim

55. conclusion
Recent advances in biomolecular research have enhanced our
understanding of the etiopathogenesis of acquired cholesteatoma.
Complex and hybrid procedure.
Treatment is surgical.
Choice of surgery is variable
Follow up is essential

56. Thank
you