2. Definition:
ā¢ Chronic pyogenic infection of middle ear cleft lasting
for >3 months with cholesteatoma & granulation
tissue in attic or postero-superior quadrant of pars
tensa
ā¢ Unsafe/ Dangerous : Higher chances of complication
due to bone erosion
ā¢ Hallmark of Disease : Cholesteatoma/granulations
3. Cholesteatoma
ā¢ Johannes MĆ¼ller ( 1858)
ā¢ Defined as a three dimensional sac lined by
matrix of keratinizing stratified squamous
epithelium that rests on a thin layer of fibrous
tissue and contains desquamated keratin debris
which grows at the expense of surrounding bone
ā¢ Not a tumor and has no cholesterol ( Ludman )
ā¢ Better term : Epidermosis ( Tumarkin )
4. Cholesteatoma :
ā¢It is a three dimensional epidermal and connective
tissue structure , formed due to accumulation of
exfoliated Keratin in middle ear cleft . It has the
capacity for progressive independent growth at
the expense of underlying bone .
- Abramson , 1977
It has been accurately described as Keratoma
- by Schuknecht
6. Histologically
ā¢ Cystic Content
o is composed of fully differentiated anucleate keratin squames.
ā¢ Matrix
o contains keratinizing squamous epithelium lining a cyst like
structure.
ā¢ Perimatrix
o known as lamina propria
o peripheral part of cholesteatoma consists of granulation tissue
and cholesterol granules.
o This layer is in contact with the bone. It is the granulation tissue
which releases enzymes that cause bone destruction.
7. Causes of bone destruction
ā¢ Hyperaemic decalcification
ā¢ Osteoclastic bone resorption
ā Acid phosphatase ,collagenase, acid proteases
proteolytic enzymes, leukotrienes, cytokines
ā¢Pressure necrosis
ā¢Bacterial toxins
9. Congenital Cholesteatoma
ā¦ Definition (Levenson, 1989) ā 5 points
White mass medial to normal tympanic membrane
Normal pars flaccida and pars tensa
No prior history of otorrhea or perforations
No prior otologic procedures
Prior bouts of otitis media not grounds for exclusion
11. Teedās theory ā Failure of involution of
embryonic cell rest
ā¦ Proposed in 1936
ā¦ The embryonic ectodermal epithelial cell rests
that is present during fetal development in
proximity to the geniculate ganglion fails to
involute.
ā¦ Persistence of embryonic squamous cell rests in the
temporal bone led to the formation of congenital
cholesteatoma.
ā¦ Presence of squamous cell rests in the temporal
bone ā fairly common. Usually they involute at a
later date to become mature middle ear lining
12. Wendtās Squamous Metaplastic theory
ā¦ This was first proposed by Wendt in 1873
ā¦ The attic area of the middle ear cavity is lined by
pavement epithelium.
ā¦ According to Wendt, this pavement epithelium
undergoes squamous metaplasia in response to
infection thus forming a nidus for cholesteatoma
formation.
13. Michaelās epidermoid formation theory
ā¦ Michaels in 1980s ā fetal human temporal bones
ā¦ Identified squamous cell tuft present from 10-33 wk
of gestation.
ā¦ This āepidermoid formationā was noted in
anterosuperior wall of ME cleft.
ā¦ Failure of involution could be basis of cholesteatoma
in anterosuperior mesotympanum
14. Reudiās invagination theory
ā¦ First proposed by Ruedi
ā¦ Suggested in-utero infection of TM causing it to
invaginate into the middle ear cavity and produce
stratified squamous epithelium.
ā¦ These invaginations predispose to cholesteatoma
formation.
15. Congenital Cholesteatoma
ā¦ Origin remains uncertain
ā¦ Usually starts from the antero superior quadrant
ā¦ Spreads through the posterior superior quadrant,
attic and finally into the mastoid cavity
ā¦ Mean age of presentation is 4.5 yo
ā¦ M:F ratio is 3:1
ā¦ Incidence is 0.12 per 100,000 people
17. Acquired Cholesteatoma
1. Invagination / Retraction pocket (Wittmackās
theory)
āOne of the primary mechanism of
cholesteatoma formation
āDevelops in posterosuperior quadrant of Pars
tensa /Attic with adjacent canal wall erosion
18. Retraction pocket formation
Retraction pocket in pars flaccida or Postero-superior
quadrant of pars tensa due to E.T. dysfunction
cerebellopontine angle
19. Reasons for Retraction pocket in
posterosuperior quadrant :
ā¢ Poser- superior part of pars tensa is thinnest
ā¢ Rate of migration of epithelium is highest at posterosuperior
canal wall
ā¢ Poor ventilation of the postero-superioir mesotympanum
due to too much crowding due to presence of ossicles
ā¢ Presence of negative pressure in the posterosuperior
mesotympanum because of :
- Relatively large and deep posterior pouch
- Proximity of the aditus ( due to large volume of the mastoid
antrum )
20. 2. Basal cell hyperplasia (Ruedi)
Hyperplasia of basal cells in epithelial layer of T.M. &
their invasion of sub-epithelial tissues .
The cause for hyperplasia is subclinical inflammation
in the attic .
21. 3. Primary squamous metaplasia( Ruede ,
Tumarkin and McGuckin)
Transformation of middle ear mucosa into squamous
epithelium due to infection without TM perforation
22. 4. Secondary squamous
metaplasia( Wendt and Sade )
Transformation of middle ear mucosa into squamous
epithelium due to infection via T.M. perforation
24. 6. Tertiary / Post-traumatic cholesteatoma
ā¢ Mechanisms:
1. Epithelial entrapment in fracture line
2. Ingrowth of epithelium through fracture line
3. Traumatic implantation of epithelium into
middle ear
4. Entrapment of epithelium medial to E.A.C.
stenosis
30. Tos classification ā Pars Flaccida Retractions:
ā¢Grade 1 ā Simple Attic dimple
ā¢Grade 2 ā Pars flaccida retracted maximally and
drapped over neck of malleus
ā¢Grade 3 ā As grade 3 with erosion of outer attic wall
ā¢Grade 4 ā Deep Retraction with unreachable
accumulated keratin
38. Spread of cholesteatoma
- In the middle ear cleft, cholesteatoma follows
the path of least resistance and causes enzymatic
bone destruction.
- The growth of attic cholesteatoma is limited by
the mucosal folds and suspensory ligaments of
the ossicles.
- Attic cholesteatoma first invades Prussakās
space (lateral most portion of epitympanum) and
then into the recesses of epitympanum
posteriorly, lateral to the body of incus.
39. ā¢Inferiorly it goes into the middle ear via pouch of
von Troltsch. (anatomical spaces between
the malleolar folds & the tympanic membrane )
ā¢ Anteriorly cholesteatoma enters into the
protympanum.
ā¢An attic cholesteatoma thus extends posteriorly
into the aditus, antrum and
ā¢mastoid, inferiorly into the mesotympanum and
medially surrounds the incus and head of the
malleus
40. Prussak space :
ā¢ Prussak space is a
subcomponent of the
lateral epitympanic
ā¢ Boundaries :
ā¢ lateral: pars flaccida of
the tympanic membrane
ā¢ medial: neck of
the malleus
ā¢ superior: lateral malleal
ligament fold
ā¢ inferior: lateral (short)
process of the malleus
41. Destruction of bone :
ā¢ Cholesteatoma destroys the bones, which come in its way
such as ear ossicles, bony labyrinth, canal of facial nerve,
sinus plate and tegmen tympani.
ā¢ Formerly bone destruction was believed to be due to
pressure necrosis. Currently, bone destruction has been
attributed to enzymes.
ā¢ They are liberated by osteoclasts and mononuclear
inflammatory cells (associated with cholesteatoma) and
include as collagenase, acid phosphatase and proteolytic
enzymes.
42. Investigations
ā¢ Examination under microscope
ā¢ Ear discharge swab: for culture and sensitivity
ā¢ Pure tone audiometry
ā¢ X-ray mastoid : B/L 300 lateral oblique
(Schuller)
ā¢ CT scan: revision surgery, complications,
children
43. Advantages of E.U.M.
ā¢ Confirmation of otoscopic findings
ā¢ Epithelial migration from margin of perforation
ā¢ Cholesteatoma & granulations
ā¢ Adhesions & tympanosclerosis
ā¢ Assessment of ossicular chain integrity
ā¢ Collection of discharge for culture sensitivity
44. Uses of X-ray mastoid
1. Position of dural & sinus plates
2. Type of pneumatization : Cellular (80%), Diploic
(<1%), Sclerotic (20%)
3. Cholesteatoma (cotton wool appearance)
4. Bone destruction: presence & extent
5. Mastoid cavity
53. Management
History:
1. Hearing loss
2. Otorrhea: malodorous
3. Otalgia
4. Tinnitus
5. Vertigo
Progressive unilateral hearing loss with a chronic foul smelling
otorrhea should raise suspicion.
ā¦ Previous history of middle ear disease
1. Chronic otitis media
2. Tympanic membrane perforation: Pars flaccida
3. Prior surgery
54. Examination:
ā¦ Otoscopy
ā¦ Microscopy
ā¦ Positive fistula (pneumatic otoscopy will result in
nystagmus and vertigo) response suggests erosion
of the semicircular canals or cochlea
ā¦ 512Hz tuning fork exam
- Always relate with audiometry results
56. Imaging
ā¦ Preoperative imaging with computed tomographies (CTs
) of temporal bones (2mm ) section without contrast in
axial and coronal planes.
1. Allows for evaluation of anatomy
2. May reveal evidence of the extent
3. Screen for asymptomatic complications
57. Medical - Conservative
ā¢ Topical ear drops + frequent suction clearance
ā¢ Indications:
ā Early disease with shallow retraction pocket
ā Only hearing ear with cholesteatoma
ā Elderly patients
ā Pts who are not fit for surgery under G.A.
ā Pts who can regularly come for follow up
Treatment Options
58. Management
Cholesteatoma is a surgical problem
Goals of surgery include:
1) To make the ear safe by eradicating the
cholesteatoma and infection
2) To conserve residual hearing Improvement of
hearing when possible
3) To provide acceptable cosmetic appearance
4) To reconstruct the ear in a manner that reduces
the chances of recurrence
59. Surgeries
ā¢ Grommet insertion (to manage early
retraction pockets)
ā¢ Canal wall down procedure
ā¢ Canal wall up procedure
60. Canal wall down procedure
ā¢ Most commonly used
ā¢ Good access
ā¢ Middle ear space reduced
ā¢ Large cavity (cavity problems)
61. ā¦ Classic CWD operation is the modified
radical mastoidectomy in which middle ear
space is preserved
ā¦ Radical mastoidectomy is CWD operation
in which: Middle ear space is eliminated ,
Eustachian tube is plugged
62. Canal wall down procedure
ā¢ Posterior canal wall is reduced up to the
level of vertical portion of facial nerve
ā¢ Middle ear, attic, antrum,and mastoid are
exteriorized and made into a single cavity
ā¢ Attic is obliterated
ā¢ Large meatoplasty is performed
63. Indications of canal wall down
ā¢ Cholesteatoma in the only hearing ear
ā¢ Erosion of posterior canal wall
ā¢ H/o vertigo suggesting labyrinthine fistula
ā¢ Recurrent cholesteatoma after canal wall
up surgery
ā¢ Poor Eustachean tube function
ā¢ Sclerosed mastoid with poor access to
attic region
64. Canal wall down procedure :
Advantages :
ā¢ Residual disease is easily
detected
ā¢ Recurrent disease
is rare
ā¢ Facial recess is
exteriorized
Disadvantages :
ā¢ Open cavity is created
ā¢ Takes longer time to heal
ā¢ Mastoid bowl maintenance
can be life long problem
ā¢ Shallow middle ear space
makes OCR (Ossicular Chain
Reconstruction) difficult
ā¢ Dry ear precautions are
essential
65. Bondyās operation
ā¦ A type of modified radical mastoidectomy in which
the mastoid cavity is exteriorized without disturbing
the intact ossicular chain and pars tensa.
ā¦ Indication: epitympanic cholesteatoma with intact
ossicular chain, normal pars tensa, and good
hearing.
ā¦ The advantages of the technique are one-stage
surgery with preservation of preoperative hearing
levels, which is not possible with any other
procedure.
66. Canal wall up surgery
ā¢ More conservative procedure
ā¢ Posterior canal wall is retained
ā¢ Middle ear space is maintained
ā¢ Normal physiology is maintained
ā¢ Usually is performed as a staged
procedure
67. Canal -Wall Up :
ā¦ CWU procedure developed to avoid problems and
maintenance necessary with CWD procedures
ā¦ CWU consists of preservation of posterior bony
external auditory canal wall during simple
mastoidectomy with or without a posterior with
tympanotomy
ā¦ Staged procedure often necessary with a scheduled
second look operation at 6 to 18 months for:
- Removal of residual cholesteatoma
- Ossicular chain reconstruction if necessary
ā¦ Procedure should be adapted to extent of disease as
well as skill of otologist
68. Indications of canal wall up procedure
ā¢ In patients with well pneumatized mastoid
ā¢ Patients with good Eustachean tube
function
ā¢ In patientās with limited disease (confined
to the attic)
69. Contraindications of CWU
ā¢ Patients with poor Eustachean tube
function
ā¢ Patient with labyrinthine involvement
ā¢ Long standing ear disease
ā¢ Patients with intra cranial complications
70. Advantages of CWU
ā¢ Rapid healing time
ā¢ No cavity problems
ā¢ Hearing aids can be used without
problems