Cholesteatoma

WHAT IS IT? 

Cholesteatomas are collections of squamous cell debris that form a mass (sometimes described as pearly) behind the tympanic membrane. They can either be congenital or can be an acquired primary lesion following infection, trauma, or surgery of the middle ear. Primary cholesteatomas result of chronic negative pressure in the middle ear causing retraction pockets in the tympanic membrane that become cystic; as the squamous cell debris accumulates, a cholesteatoma is formed. Secondary cholesteatomas occur after squamous epithelium migrates to or is implanted in the middle ear (‘skin in the wrong place”).

The arrow above marks a common site of cholesteatoma formation (source)
The black arrow above marks a common site of cholesteatoma formation (source)
WHY IS IT A PROBLEM?

They can cause hearing loss due to erosion in auditory ossicles. They can produce lytic enzymes that are responsible for this erosion. lf a mass grows sufficiently large. it can erode into the vestibular apparatus or facial nerve, causing vertigo or facial palsies.

WHAT MAKES US SUSPECT IT?

Risk factors: male child (age ~4,5), down syndrome, turner syndorme, cleft palate, craniofacial anomaly, family history of chronic middle ear disease/cholesteatoma.

Chief concern (CC): may be asymptomatic in early stage. Most common early symptoms include

  • hearing loss
  • intermittent ear discharge (otorrhea)
HOW DO WE CONFIRM A DIAGNOSIS?

Diagnosis of acquired cholesteatoma established by visual examination showing retraction pocket or granular tissue on surface of drum (otoscopy)

Visual appliance of cholesteatoma when looking inside the ear (source)
Visual appliance of cholesteatoma when looking inside the ear (source)
HOW DO WE TREAT IT? 

Mastoid surgery is treatment of choice (to remove cholesteatoma) and subsequent reconstruction surgery of the ossicles and tympanic membrane.

HOW WELL DO THE PATIENTS DO?

Recurrence and residual lesions post-surgery is common

WAS THERE A WAY TO PREVENT IT? 

Prevention methods for this condition are not clear

WHAT ELSE ARE WE WORRIED ABOUT? 

Progression leading to permanent hearing loss or facial palsy.

OTHER HY FACTS?
FURTHER READING

DynaMed

UpToDate

Page Updated: 02.02.2106