Cholesteatoma

Question 1

What is a cholesteatoma?

Answer 1

Squamous epithelium in the middle ear or mastoid.
It p/w otorrhea and CHL.
Retraction of TM w/ squamous debris collection or a whitish mass behind an intact TM.

Question 2

What are the types of cholesteatoma?

Answer 2

1. Acquired (primary and secondary) – m/c

2. Congenital

Question 3

Primary vs secondary acquired cholesteatoma

Answer 3

Primary: forms as a retraction of the TM (usu pars flaccida)
Secondary: forms 2/2 squamous epithelial migration from the TM or implantation of squamous epithelium into ME during surgery (PET, tympanoplasty)

Question 4

What is a congenital cholesteatoma?

Answer 4

Occurs w/o TM retraction or implantation of squamous epithelial material.
Classically defined as an embryonic rest of epithelial tissue in ear w/o TM perf and w/o h/o ear infxn.

Question 5

Pathogenesis of acquired cholesteatoma

Answer 5

A/w ETD and chronic inflammation of ME (COM).

Chronic neg ME pressure -> retraction -> nl migratory pattern of squamous epith disrupted -> accumulation of keratin debris -> chronic infxn and inflammation -> further growth and migration of squamous epith and inc OC activity -> bone resorption

Local inflam response further inhibits ET fnc, increases mucosal edema and mucous secretion, and disrupts drainage pathway of t-bone

This environment fosters growth of Pseudomonas, Strep, Staph, Proteus, Enterobacter, anaerobes -> more inflammation and continues the cycle

Question 6

Pathogenesis of congenital cholesteatoma

Answer 6

Failure of involution of the epithelioid formation in the ME during fetal development

Other theories:

• Metaplasia of ME mucosa
• Direct microretractions of the TM near the malleus long process that are self-limited but leave squamous epith rests in the ME

Question 7

How can cholesteatoma be prevented?

Answer 7

• Congenital: can’t
• Secondary acquired: careful surgical technique
• Primary acquired: restoring ET fnc, providing secondary ventilation to ME, control of infxn and inflam state of ME with regular debridement of cholesteatoma sac

Question 8

How do pts present?

Answer 8

Recurrent or persistent purulent otorrhea
CHL
Tinnitus
Rarely, vertigo or dysequilibrium or facial n sx

Question 9

Imaging study of choice

Answer 9

CT

Question 10

CT findings

Answer 10

• Bone window axial and coronals with 1.5mm slices or less are ideal
• Esp useful in pts w/ hx suggestive of secondary acquired cholesteatoma but TM opaque and can’t visualize middle ear
• Erosion of bone (m/c scutum and ossicular chain)
• Erosion of the labyrinth is highly suggestive (although can be seen w/ neoplasia)

Question 11

How is CT useful in surgical planning?

Answer 11

Can indicate presence of

• Low tegmen
• Ant sigmoid sinus
• Labyrinthine erosion
• Ossicular erosion
• Petrous apex involvement

All of which affect surgeon’s approach

Question 12

Is CT a requirement prior to surgery?

Answer 12

No

Many will not get pre-op unless revision surgery, suspicion of labyrinthine fistula, or possibility of petrous apex dz

Question 13

When is MRI useful?

Answer 13

If neoplasm or encephalocele is suspected

Question 14

Typical audiogram

Answer 14

CHL

Question 15

What if the audiogram shows SNHL?

Answer 15

May be due to chronic or recurrent inflammatory process but should consider possibility of labyrinthine fistula

Question 16

Complications of cholesteatoma

Answer 16

• Chronic inflammation and infxn
• Bone erosion (ossicular chain)
• Otorrhea
• SNHL
• Dizziness
• Facial nerve injury
• Suppurative complications (acute mastoiditis, subperiosteal abscess, sigmoid sinus thrombosis, meningitis, and brain abscess)

Question 17

Nonsurgical treatment options

Answer 17

Goal: reduce level of inflammatory and infectious activity

• Remove infected debris from EAC
• Keep all water out of ears
• Topical ABX ofloxacin or neomycin-polymyxin B
• Consider topical steroids for inflammation

Question 18

What is the goal of surgery?

Answer 18

A “dry and safe” ear = permanent reversal of the processes causing bone erosion, chronic inflam, and infxn. To achieve, must remove or exteriorize all cholesteatoma matrices.

Question 19

What is Prussak’s space?

Answer 19

Area b/w pars flaccida laterally and malleus neck and lower portion of head medially
Where most cholesteatomas invade

Question 20

What is the m/c location of cholesteatoma in the ME

Answer 20

The area around the stapes superstructure and incus long process

Question 21

List the areas of the middle ear where cholesteatoma may be left behind due to difficult access

Answer 21

• Area around stapes superstructure
• Facial recess
• Sinus tympani
• Posterior hypotympanum

Question 22

How can visualization of the epitympanum be improved?

Answer 22

By removing the incus and malleus head

If tegmen is very inferior, may need to remove posterior and superior canal wall.

Question 23

What are the various air cell tracts through which cholesteatoma can invade the petrous apex?

Answer 23

• Subarcuate
• Retrolabyrinthine
• Supralabyrinthine
• Retrofacial
• Infralabyrinthine

Question 24

CWU or CWD?

Answer 24

1. Surgeon training level and experience
2. Anatomy of the pt’s t bone. Sometimes adequate exposure can be obtained w/ either.
   - Low tegmen or anterior sigmoid sinus may prompt for CWD for exposure
3. Issue of recurrent or recidivistic (residual) dz
   - ?Higher recurrent/residual dz w/ CWU esp if cholesteatoma arises from pars flaccida

Question 25

Causes of otorrhea after CWD mastoidectomy?

Answer 25

• Recurrent or residual cholesteatoma
• High facial ridge
• Presence of mucosal tissue w/in mastoid cavity

Question 26

Should treatment of cholesteatoma be staged?

Answer 26

Controversial

-2nd look surg is usu performed 8-12 months after 1st stage

Question 27

Why is cholesteatoma around the oval window typically removed towards the end of the procedure?

Answer 27

In case a perilymph fistula occurs.
MAN can then be instituted w/ fascia or other soft tissue grafts w/o risk of compromising the repair during further dissection.

Question 28

What is the rate of recurrent or residual dz at the 5 yr mark from surgery?

Answer 28

as high as 40%

Other 10 year series report 15-25%

Question 29

What are the 4 procedure options for cholesteatoma?

Answer 29

1. CWU mastoid
2. Atticotomy
3. Modified radical mastoidectomy (CWD)
4. Radical mastoidectomy (CWD) (mastoid cavity and no TM)

As you go down, risk of otorrhea rises and risk of recurrence falls.