Mehdee Hassan, MBBS, MS, Sr Lecturer in

ENT
 Named by Johannes Mueller in 1838
• Erroneous belief that one of the primary
components of the tumor was fat
• “a pearly tumor of fat…among sheets of
polyhedral cells”
 More appropriate name has been
suggested to be keratoma to
describe tumor composition

2
 Expanding lesion of the temporal bone
composed of
• Cystic content: desquamated keratin center
• Matrix: keratinizing stratified squamous
epithelium
• Perimatrix: granulation tissue that secretes
multiple proteolytic enzymes capable of
bone destruction
 May develop anywhere within
pneumatized portions of the temporal
bone, most frequent locations:
• Middle ear space
• Mastoid
3
 Congenital
 Acquired
• Primary
• Secondary

For Cholesteatoma Formation

 Multiple theories proposed regarding etiology
behind tumor formation
 Proposed mechanisms remain theories

4
 Definition (Levenson, 1989)
• 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 were not grounds
for exclusion as was the case in original
definition

5
 Pathogenesis theories
• Failure of involution of ectodermal epithelial
thickening that is present during fetal
development in proximity to geniculate
ganglion
• Metaplasia of the middle ear mucosa

6
Anterosuperior >Posterosuper
quadrant
ior quadrant

7
Large congenital cholesteatoma

ossicular erosion

cholesteatoma
8
 Common factor: keratinizing
squamous epithelium has grown
beyond its normal limits

9
 Ultimately form due to underlying
Eustachian tube dysfunction that
causes retraction of pars flaccida
• Results in poor aeration of epitympanic space
which draws pars flaccida medially on top of
malleus neck, forming retraction pocket
• Normal migratory pattern of the tympanic
membrane epithelium altered by retraction
pocket
• Enhances potential accumulation of keratin

10
 Primary Acquired
Cholesteatomas
Pars flaccida retraction Pars tensa retraction

11
12
• Implantation theory
Squamous epithelium implanted in the middle ear as a
result of surgery, foreign body, blast injury, etc.

• Metaplasia theory
Desquamated epithelium is transformed to keratinized
stratified squamous epithelium secondary to chronic or
recurrent otitis media

• Epithelial invasion theory

Squamous epithelium migrates along perforation edge
medially along undersurface of tympanic membrane
destroying the columnar epithelium

• Papillary ingrowth theory

Inflammatory reaction in Prussack’s space with an intact
pars flaccida (likely secondary to poor ventilation) may
cause break in basal membrane allowing cord of epithelial
cells to start inward proliferation 13
14
 Named based on position relative to
superior and inferior aspect of external
auditory canal (EAC)
• Epitympanum: superior to superior limit
of EAC
• Mesotympanum: bound superiorly by
superior limit of EAC and inferiorly by
inferior limit of EAC
• Hypotympanum: inferior to inferior limit
of EAC
15
16
 Lies above the level of the short
process of the malleus
 Contents:
• Head of the malleus
• Body of the incus
• Associated ligaments and mucosal folds

17
 Contents:
• Stapes
• Long process of the incus
• Handle of the malleus
• Oval and round windows
 Eustachian tube exits from the anterior aspect
 Two recesses extend posteriorly that are often not
visible directly
• Facial recess
 Lateral to facial nerve
 Bounded by the fossa incudis superiorly
 Bounded by the chorda tympani nerve laterally
• Sinus tympani
 Lies between the facial nerve and the medial
wall of the mesotympanum
18
 Lies inferior and medial to the floor of
the bony ear canal
 Irregular bony groove that is seldom
involved by cholesteatoma

19
 Annular ligament sends fibrous bands from
anterior and posterior tympanic spines that
meet at neck of malleus and make up
middle layer of pars tensa
 Dehiscent area in the tympanic bone
(Notch of Rivinus) lies above fibrous bands
 Dense fibers that form middle layer of pars
tensa do not extend to pars flaccida
• Lack of structural support predisposes
Shrapnell’s membrane to retract when
negative middle ear pressure is present
secondary to Eustachian tube dysfunction 20
 Cholesteatomas of
epitympanum start
in Prussack’s space
between pars
flaccida and neck
of malleus with
upper boundary
being the lateral
mallear fold

21
 Predictable in that they are
channeled along characteristic
pathways by:
• Ligaments
• Folds
• Ossicles

22
 Posterior
epitympanum
 Posterior
mesotympanum
 Anterior
epitympanum

23
 Posterior epitympanic cholesteatoma
passing through superior incudal space
and aditus ad antrum

24
 Posterior mesotympanic cholesteatoma
invading the sinus tympani and facial
recess

25
 Anterior epitympanic cholesteatoma with
extension to geniculate ganglion

26
 History
• Detailed otologic history
 Hearing loss
 Otorrhea
 Otalgia
 Nasal obstruction
 Tinnitus
 Vertigo
• Previous history of middle ear disease
 Chronic otitis media
 Tympanic membrane perforation
 Prior surgery 27
 Head and neck examination
 Otologic examination
• Otomicroscopy is essential in evaluating the
extent of disease
• Clean ear thoroughly of otorrhea and debris
with cotton-tipped applicators or suction
• Culture wet, infected ears and treat with topical
and/or oral antibiotics
• Pneumatic otoscopy should be performed in
every patient with cholesteatoma
 Positive fistula (pneumatic otoscopy will
result in nystagmus and vertigo) response
suggests erosion of the semicircular canals
28
 Hearing evaluation to assess for
conductive hearing loss
• Pure tone audiometry with air and bone
conduction
• Speech reception thresholds
• Word recognition
 512Hz tuning fork exam
• Always correlate with audiometry results
 Tympanometry
• May suggest decreased compliance or TM
perforation

29
 Degree of conductive loss will vary
considerably depending on the extent of
disease
• Moderate conductive deficit in excess of
40 dB indicates ossicular discontinuity,
usually from erosion of the long process of the
incus or capitulum of the stapes
• Mild conductive deafness may be
present with extensive disease if
cholesteatoma sac transmits sound
directly to stapes or footplate

30
 Preoperative imaging with computed
tomographies (CTs) of temporal
bones (2mm -section without
contrast in axial and coronal planes)
• Allows for evaluation of anatomy
• May reveal evidence of the extent of the
disease
• Screen for asymptomatic complications

31
 CT is not essential for preoperative
evaluation
 Should be obtained for:
• Revision cases due to altered landmarks
from previous surgery
• Chronic suppurative otitis media
• Suspected congenital abnormalities
• Cases of cholesteatoma in which
sensorineural hearing loss, vestibular
symptoms, or other complication evidence
exists

32
 Preoperative counseling is an absolute
necessity prior to surgery
 Primary objective of surgery is a safe
dry ear which is accomplished by:
• Treating all supervening complications
• Removing diseased bone, mucosa, granulation
polyps, and cholesteatoma
• Preserving as much normal anatomy as
possible
 Improvement of hearing is a secondary
goal
33
 Possible adverse outcomes must be
discussed
• Facial paralysis
• Vertigo
• Further hearing loss
• Tinnitus
 Patient should understand that long-
term follow-up will be necessary and
that they may need additional surgeries

34
 Tympanostomy tube for early
retraction pockets

 Surgical exploration for retraction

persistence
35
 Treated surgically with primary goal
of total eradication of cholesteatoma
to obtain a safe and dry ear
 Patients with unacceptable risk of
anesthesia need local care

36
 Canal-wall-down procedures (CWD)
 Canal-wall-up procedure (CWU)
 Transcanal anterior atticotomy
 Bondy modified radical procedure

37
 Prior to the advent of the tympanoplasty,
all cholesteatoma surgery was performed
using CWD approach
 Procedure involves:
• Taking down posterior canal wall to level of
vertical facial nerve
• Exteriorizing the mastoid into external auditory
canal
 Epitympanum is obliterated with removal
of scutum, head of malleus and incus

38
 Classic CWD operation is the modified
radical mastoidectomy in which middle ear
space is preserved
 Radical mastoidectomy is CWD operation
in which:
• Middle ear space is eliminated
• Eustachian tube is plugged
 Meatoplasty should be large enough to
allow good aeration of mastoid cavity and
permit easy visualization to facilitate
postoperative care and self cleaning

39
 Indications for CWD approach:
• Cholesteatoma in an only hearing ear
• Significant erosion of the posterior bony canal
wall
• History of vertigo suggesting a labyrinthine
fistula
• Recurrent cholesteatoma after canal-wall-up
surgery
• Poor eustachian tube function
• Sclerotic mastoid with limited access to
epitympanum

40
 Advantages:
• Residual disease is easily detected
• Recurrent disease is rare
• Facial recess is exteriorized
 Disadvantages:
• Open cavity created: Takes longer to heal
• Mastoid bowl maintenance can be a lifelong
problem
• Shallow middle ear space makes OCR difficult
• Dry ear precautions are essential

41
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43
44
45
46
 CWU procedure developed to avoid problems
and maintenance necessary with CWD
procedures
 CWU consists of preservation of posterior bony
external auditory canal wall during simple
mastoidectomy with or without a posterior
tympanotomy
 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
 Procedure should be adapted to extent of
disease as well as skill of otologist

47
 CWU may be indicated in patients
with large pneumatized mastoid and
well aerated middle ear space
• Suggests good eustachian tube function
 CWU procedures are contraindicated
in:
• Only hearing ear
• Patients with labyrinthine fistula
• Long-standing ear disease
• Poor eustachian tube function

48
 Advantages:
• Rapid healing time
• Easier long-term care
• Hearing aids easier to fit
• No water precautions
 Disadvantages:
• Technically more difficult
• Staged operation often necessary
• Recurrent disease possible
• Residual disease harder to detect

49
50
51
 Indicated for limited cholesteatoma
involving middle ear, ossicular chain,
and epitympanum
 If extent of the cholesteatoma is
unknown approach can be combined
with CWU mastoidectomy or
extended to CWD procedure

52
 Procedure involves:
• Elevation of tympanomeatal flap via
endaural incision with removal of scutum to
limits of the cholesteatoma
• Aditus obliteration with muscle, fascia,
cartilage or bone prior to reconstruction of
the middle ear space
• Reconstruction of lateral attic wall with bone
or cartilage is optional
 May lead to retraction disease and possible
recurrence in patients with poor eustachian tube
function

53
54
 Useful for attic and mastoid cholesteatoma
that does not involve middle ear space and
lateral to ossicles
 Like modern modified radical
mastoidectomy with exception that middle
ear space is not entered
 Mastoid should be poorly developed for
creation of a small cavity
 Eustachian tube function should be
adequate with intact pars tensa and
aerated middle ear space.
 Rarely used today 55
 In 2002 Shohet et. al. reports
recidivism rates with CWU as high as
• Adults = 36%
• Children = 67%

 Approximately 30% of cases for

cholesteatoma will be CWD
• House Ear Institute 1982
• St. Joseph’s Hospital in Phoenix, AZ 2003

56
 Syms et. al. examined surgical approach
to manage cholesteatoma- retrospective
review of 486 cases in 2003
• 68.5% CWU: 31.5% CWD
• CWU had “second look operation”
 Residual cholesteatoma found in 26.9%
second procedures
 Residual cholesteatoma found in 2.7% third
procedures

57
 Kos et. al. examined long-term
implications of CWD- retrospective
review in 2004
• 259 cases with follow-up range of 1 to 24
years (mean = 7 years)
• Recurrent cholesteatoma in 6.1%
• TM perforation in 7.3%
• Dry, self-cleaning ears in 95%
• Otorrhea in 5%

58
 Kos et. al. (continued)
• Hearing threshold
 Improved in 30.7%
 Unchanged in 41.3%
 Decreased in 28%
• Sensorineural hearing loss greater than
60dB in 2 patients
• Facial paralysis in 1 patient
• Vertigo in 4 patients

59
 In 2005 Gantz et. al. reported 130
cases of canal wall reconstruction
tympanomastoidectomy with mastoid
obliteration
• No evidence of recurrence = 98.5%
• Recurrence treated with CWD (1.5%)
• Second look ossiculoplasty in 78%
• Post-operative wound infection was 14.3%
for first 42 patients
 Decreased rate to 4.5% in last 88 patients with 2
days of post-operative IV antibiotics

60
 Canal Wall Reconstruction technique
• Complete cortical mastoidectomy with opening of
facial recess and removal of incus and malleus
head
• Posterior canal wall skin elevated, annulus
elevated
• Microsagittal saw used to cut posterior canal wall
• Cholesteatoma removed
• Posterior canal wall bone replaced
 Cortical bone chips used to block attic and mastoid
from tympanum
 Bone pate’ holds bone chips in place

61
62
 In 2005, Godinho et. al. reported 42
cases of Canal Wall Window (CWW)
performed for pediatric cholesteatoma
• CWW compared to CWU and CWD
 CWW converted to CWD in 14%
 Dry ear results
 CWW = 94%, CWD = 92%, CWU = 90%
 Recidivation rate at 1 year
 CWW = 19.5%, CWD = 0%, CWU = 7.7%
 CWW provided similar post-operative hearing to
CWU, rather than increased air-bone gap seen with
CWD

63
 CWW effective for posterior superior
cholesteatoma
• Increases visibility
• Conduit for instrument manipulation
 CWW technique
• CWU mastoidectomy performed
• Lateral epitympanotomy
• Slit drilled from lateral cortex to medial
aspect of canal wall at annulus

64
65
 CWW reconstruction
• Tragal cartilage placed in defect after
scoring proximal perichondrium and
cartilage
• Cortical bone graft may be used as an
alternative

66
67
  Infection
 Otorrhea
 Bone destruction
 Subperiosteal abscesses
 Hearing loss
 Facial nerve paresis or paralysis
 Labyrinthine fistula
 Intracranial complications
mplications are caused by expansion and infect

68
 Conductive HL: ossicular chain erosion (30%)
• Erosion of lenticular process and/or stapes
superstructure may produce 50dB conductive
hearing loss
• Hearing loss varies despite disease extent
(natural myringostapediopexy, transmission of
sound through cholesteatoma sac)
 Sensorineural HL: involvement of labyrinth
 Following surgery, 30% have further
impairment due to:
• Extent of disease present
• Complications in healing process

69
 Incidence: as high as 10%
 Symptom: Sensorineural hearing loss
and/or vertigo induced by noise or
pressure change
 Common site: horizontal semicircular
canal, basal turn of cochlea
 Diagnosis: Fine cut temporal bone CT
(1mm)
 Management: modified radical
mastoidectomy with management of
matrix overlying fistula

70
 May develop:
• Acutely secondary to infection
• Slowly from chronic expansion of cholesteatoma
 Temporal bone CT: localize the nerve involvement
 Most common site: geniculate ganglion due to
disease in the anterior epitympanum
 Management: Needs immediate surgery
• Removal of cholesteatoma and infected material
with decompression of the nerve (mastoidectomy,
middle fossa approach)
• Administration of intravenous antibiotics and high-
dose steroids
• Iatrogenic injury to the nerve during surgery should
be immediately repaired with decompression of
nerve proximal and distal to site of injury
71
 Potentially life-threatening
 Incidence: as high as 1%
 Complications
• Extradural abscess
• Subdural empyema
• Lateral sinus thrombosis
• Meningitis
• Brain abscess
 Symptom:
• Suppurative malodorous otorrhea
• Chronic headache
• Fever
• Otalgia 72
 Management:
• Presence of mental status changes with
nuchal rigidity or cranial neuropathies
warrant neurosurgical consultation with
urgent intervention
• Epidural abscess, subdural empyema,
meningitis and cerebral abscesses should
be treated immediately prior to definitive
otologic management of ear disease

73
 Pathogenesis of cholesteatoma remains uncertain
 Essential to possess basic knowledge of the
important anatomic and functional characteristics
of the middle ear for successful management of
cholesteatomas
 Careful and thorough evaluations are the key to
early diagnosis and treatment
 Treatment is surgical with primary goal to
eradicate disease and provide a safe and dry ear
 Surgical approaches must be customized to each
patient depending on extent of disease
 Surgeon must be aware of serious and potentially
life-threatening complications of cholesteatomas

74
 Treatment should be tailored to case
 Absolute CWD indications
• Cholesteatoma in an only hearing ear
• Significant erosion of the posterior bony
canal wall
• History of vertigo suggesting a labyrinthine
fistula
• Recurrent cholesteatoma after canal-wall-up
surgery
• Poor eustachian tube function
• Sclerotic mastoid with limited access to
epitympanum
 No harm in starting CWU and converting to
CWD as necessary
 Know your surgical limitations 75
 inflammation of the
mucosal lining of the
mastoid antrum and
mastoid air cell system
inside the mastoid process,
that portion of he temporal
bone of the skull that is
behind the ear and which
contains open, air-
containing spaces.
1. HOST FACTORS
2. MICROBIAL FACTORS
 Children less than 2 years.
 Immature immune system.
 H/o Acute Otitis Media.
 Incomplete pneumatization of the
mastoid process.
 Streptococcus pyogenes
 Staphylococcus aureus
 Streptococcus pneumoniae 
 Haemophilus influenzae
 Pseudomonas aeruginosa
 Group A beta-haemolytic streptococci
 Moraxella catarrhalis
 Mycobacterium species
 Temporal bone is intimately associated
with the middle ear space by its
connection through the narrow aditus
and antrum.

 Because of the continuity of the middle

ear space with the mastoid cavity, all
patients with otitis media exhibit some
degree of mastoid inflammation.
 The antrum and mastoid air cells are
lined with respiratory epithelium that
swells when infection is present.
 Blockage of the antrum by inflamed
mucosa entraps infection within the
air cells by inhibiting drainage and
precluding re-aeration from the
middle-ear side.
Continued inflammation,
hyperaemia, and accumulation of
purulent debris
cause
•venous stasis
•local acidosis
•dissolution of calcium from the bony
septae
 As the acute inflammation
subsides, the healing processes
replace the mucoperiosteum
with maturing granulation
tissue. Fibrosing osteitis
appears in areas of bone
destruction. With healing, the
osteoplastic activity
results in the production of
dense, compact bone.
 5 STAGES
• Hyperaemia of the mucosal lining of the
mastoid air cells
• Transudation and exudation of fluid and/or
pus within the cells
• Necrosis of bone by loss of vascularity of
the septa
• Cell wall loss with coalescence into abscess
cavities
• Extension of the inflammatory process to
contiguous areas
 Pain and swelling in the mastoid area
• Persistence of pain is a warning sign of mastoid
disease localized deep in or behind the ear
• typically worse at night

 Fever
• High grade fever
• Persistence of fever, particularly when the
patient is receiving adequate and appropriate
antimicrobial agents
o Deafness

o Ear discharge
• Profuse, mucopurulent or purulent.
• Persistence of discharge beyond 3 weeks in a
case of acute otitis media points to mastoiditis.

o Poor feeding, fever, irritability, or diarrhoea

in infants

o Pinna turned forwards and outwards.

 Redness and tenderness behind the ear.
• Tenderness and inflammation over mastoid
process is the most consistent sign of acute
surgical mastoiditis.
• tenderness should be compared with the
healthy side.

 Swelling over the mastoid

• smooth ironed out feel
• to be compared with the other side
 Sagging of the
posterosuperior meatal
wall due to periosteitis of
bony wall between the
antrum and bony canal.

 Nipple-like protrusion of
the central tympanic
membrane, usually
oozing pus.
 TM congested and
small perforation may
be seen.
 Light house sign-
Pulsatile ear discharge.
 Mastoid reservoir sign-
immediate filling of the
deep auditory meatus
with pus after cleaning
or mopping of the pus.
 Otitis externa/ Furunculosis

 Mastoid trauma

 Postauricular lymphadenopathy

 Suppuration of the mastoid region

 Infected sebaceous cyst

 Furuncle of meatus of the ear
• Differentiated from acute mastoiditis by
1. Absence of preceding acute otitis media
2. Painful movements of pinna
3. Swelling of the meatus confined to
cartilaginous part only
4. Discharge is never
mucoid/mucopurulent
5. Enlargement of pre/post auricular LN
6. Conductive hearing loss is mild
7. TM normal
8. X-ray mastoid with clear air cell system
excludes acute mastoiditis.
 Complete blood count
• Polymorphonuclear leucocytosis
• Raised ESR
 Ear swab for C/S
 X-ray mastoid- clouding of air cells due to
collection of exudates.
 Pure Tone Audiometry- shows conductive
HL
 Conductive HL on PTA

Clouding of mastoid
air cells
 CT SCAN
• Opacification of the mastoid
air cells and middle ear by
inflammatory swelling of
mucosa and by collection of
fluid
• Loss of sharpness or
visibility of mastoid cell
walls due to
demineralization, atrophy,
or necrosis of bony septa
• Haziness or distortion of the
mastoid outline, possibly
with visible defects of the
tegmen or mastoid cortex
1. Subperiosteal abscess.
2. Labyrinthitis.
3. Facial paralysis
4. Periosteitis
5. Extra dural abscess
6. Subdural abscess
7. Meningitis
8. Brain abscess
9. Lateral sinus thrombophlebitis
10. Otitic hydrocephalus
 Post auricular abscess
• Most common
• Pinna displaced forward, outward and
downward
 Zygomatic
abscess
• Infection of
zygomatic air cells
• Swelling in front of
and above the pinna
• Associated swelling
of upper eyelid
• Pus collects
superficial or deep
to temporalis
muscle
 Bezold abscess
• Swelling in the upper part of neck
• Sudden onset pain, fever, tender neck swelling
and torticollis
• D/D-
 Acute upper jugular lymphadenitis
 Abscess/mass in lower parotid gland
 Infected branchial cyst
 Parapharyngeal abscess
• CT Scan for diagnosis
• Treatment-
 Intra venous antibiotics
 Drainage of pus
 Cortical mastoidectomy
 Citelli abscess
• Behind the mastoid
towards the occiput

 Meatal abscess/
Lucs abscess
• Swelling in the deep
part of bony meatus
• It may burst and
drain through the
meatus
 MEDICAL
• Until microbiology information is available, the following
principles guide the selection

(1) the antimicrobial must be appropriate to cover the

invasive strains of bacteria most common for AOM

(2) the selected antibiotic should cross the blood-brain

barrier

(3) the selected therapeutic spectrum should include

consideration of MDRSP organisms that are prevalent in
the individual’s community.

Specific microbiologic diagnoses should be treated with

appropriate antibiotics.
Myringotomy
Its aim is to exenterate all the mastoid air
cells and remove any pockets of pus. It is
done when-
Subperiosteal abscess.
Sagging of posterosuperior meatal wall.
Positive reservoir sign
Mastoiditis leading to complications
 A postaural incision is placed a few
millimeters from the postaural sulcus.
 The incision is deepened through the
periosteum to the bone.
 The periosteum is lifted from the
underlying bone with periosteal
elevators to expose the spine of
Henle, the MacEwen triangle, and the
posterior bony margin of the meatus.
 The periosteum is elevated forward as
far as the lateral end of the posterior
bony meatal wall, backward for a few
millimeters, and upward (simultaneously
pushing up the temporalis muscle) to
the level of the upper attachment of the
pinna. A Mollison self-retaining
haemostatic mastoid retractor is
inserted to hold the soft tissues away
from the underlying exposed bone.
 Drilling is commenced posterior to the
posterior canal wall in a vertical direction.
A triangle-shaped excavation is created,
with the superior limit bounded by the
extension of the linea temporalis (which
becomes the floor of the middle fossa as
one drills deeper), the posterior margin
bounded by the sigmoid sinus, and the
anterior margin bounded by the thinned
wall of the posterior external ear canal
 The mastoid cortex is now removed
over the MacEwen triangle
  all cells in all directions are opened
by drilling gently through their
separating trabecula. Clearing all
cells from the sinodural angle is
particularly important
 Closure of the wound is with
interrupted sutures
 Injury to the facial nerve
 Dislocation of the incus
 Penetration of the middle or posterior
fossa
 Rupture of the sigmoid sinus
 Labyrinthine transgression and
destruction