1. Cholesteatoma

2. Definition
Cholesteatoma is an abnormal squamous epithelium skin growth often found behind the eardrum in the middle ear.
It can erode and destroy structures within the middle ear and cause hearing loss as well as many CNS complications including brain abscess and meningitis.
Cholesteatomas are not classified as either tumors or cancer

3. Congenital Cholesteatoma Primary Acquired Cholesteatoma
Classification
Congenital Cholesteatoma
Primary Acquired Cholesteatoma
Secondary Acquired Cholesteatoma

4. Congenital Cholesteatoma
Squamous epithelium trapped within the temporal bone during embryogenesis
Typically develop in the anterior mesotympanum
Usually identified in early childhood
Unlike other cholesteatomas, the congenital type is usually identified behind an intact, normal-appearing tympanic membrane. The child often has no history of recurrent suppurative ear disease, previous otologic surgery, or tympanic membrane perforation.

5. Primary Acquired Cholesteatoma
Develops as a result of medial retraction of the tympanic membrane.
Medial retraction of the tympanic membrane continues beyond the heads of the ossicles and into the posterior epitympanum.
A variant of primary acquired cholesteatoma occurs when posterior quadrant of the tympanic membrane retract into the posterior middle ear which usually produce facial nerve lesion and destruction of the ossicles.

6. Secondary Acquired Cholesteatoma
Usually caused by injury to the tympanic membrane (surgery, perforation, infection, otitis media)
Deep retraction pockets can also trap desquamated epithelium which can then lead to secondary acquired cholesteatoma

7. Symptoms and History Painless ear discharge (hallmark symptom)
Hearing loss
Otitis Externa
Ear pain
Balance disruption
Tinnitus
Vertigo
Bleeding from ear
Facial nerve weakness
Previous history of middle ear diseases

8. Diagnosis
Diagnosis can usually be made based on physical examination (Otoscopy examination)
Imaging reserved for questionable diagnosis such as when patient has symptoms of dizziness or facial weakness and to further delineate anatomy
CT is the preferred modality:
Detects subtle bony lesions
Evaluate the extent of the cholesteatoma
MRI is done when CT is non-diagnostic
Pneumatic otoscopy should be performed. (Pneumatic otoscopy will usually result in nystagmus and vertigo)

9. Diagnosis Continued
If cholesteatoma is suspected, patient should receive an audiology exam to evaluate the degree of hearing loss
Consultation with an otolaryngologist is mandatory.
Pneumatic otoscopy should be performed. (Pneumatic otoscopy will usually result in nystagmus and vertigo)

10. Otoscopic Appearance

11. Otoscopy Appearance: Examination of the left external auditory canal shows a thickened tympanic membrane with crusting and erythema in the superior aspect at the pars flaccida in reaction to the underlying cholesteatoma. A small portion of the malleus is visualized centrally. There is tympanosclerosis noted inferiorly at the anterior and posterior aspects as well. Red arrow denotes superior orientation. L1

12. Otoscopy Appearance: Examination of the left external auditory canal shows a clear canal with a translucent drum inferiorly. The malleus is visualized from the umbo to the lateral process. There is a retracted pars flaccida with a white mass indicative of cholesteatoma in the typical attic area. L3

13. Treatment
Topical Antibiotics to control infection and stop ear drainage
Cholesteatomas should be excised unless contraindicated
Surgery is generally outpatient
Unfortunately, 5-40% of cholesteatoma operations are unsuccessful as cholesteatoma either persist or recur months after surgery.
Therefore, close monitoring is crucial.
(Absence of hearing in the contralateral ear is relatively contraindicated)