Types of Cholesteatoma - Congenital & Acquired Cholesteatoma

🦻 Types of Cholesteatoma

Cholesteatoma is broadly classified into two major types:

• Congenital cholesteatoma
• Acquired cholesteatoma
  • Primary acquired cholesteatoma
  • Secondary acquired cholesteatoma

👶 Congenital Cholesteatoma

📖 Definition of Congenital Cholesteatoma

Congenital cholesteatoma is an expanding cystic mass of keratinizing squamous epithelium located medial to an intact tympanic membrane.

Key point: Tympanic membrane is intact

• Assumed to be present at birth

• Usually diagnosed in infancy or early childhood

• No prior history of:

  • Otorrhea
  • Tympanic membrane perforation
  • Ear surgery

🧬 Etiopathogenesis of Congenital Cholesteatoma

• Caused by persistence of embryonic epithelial cell rests

• These rests are located in the epitympanum

• Normally regress during development

• Persistence → accumulation of squamous epithelium in middle ear → cholesteatoma

What are the common sites of Congenital Cholesteatoma?

🩺 Clinical Features of Congenital Cholesteatoma

• Pearly white mass seen medial to an intact tympanic membrane

• Conductive hearing loss

• Often detected:

  • Incidentally on routine otoscopy
  • During myringotomy

• May rarely rupture spontaneously, presenting as a discharging ear → can mimic CSOM

What is Levenson’s Criteria for diagnosis of Congenital Cholesteatoma?

📊 Potsic Staging System (Congenital Cholesteatoma)

🟡 Primary Acquired Cholesteatoma

📖 Why “Primary”?

Called primary because it develops without:

• Prior otitis media
• Tympanic membrane perforation
• Otorrhea

🧠 Theories of Pathogenesis of Primary Acquired Cholesteatoma

• Invagination (retraction pocket) theory
• Basal cell hyperplasia theory
• Metaplasia theory

What are the different theories of Cholesteatoma?

What is Sade's metaplasia theory?

What is Basal cell hyperplasia theory?

What is Retraction Pocket theory?

🔄 Genesis of Primary Acquired Cholesteatoma

• Eustachian tube dysfunction → persistent negative middle ear pressure

• Leads to:

  • Pars flaccida / posterosuperior pars tensa retraction pocket
  • Keratin accumulation → cholesteatoma

Other mechanisms:

• Repeated subclinical infections → metaplasia of middle ear mucosa
• Basal layer proliferation of epithelium

🔴 Secondary Acquired Cholesteatoma

📖 Definition of Secondary Acquired Cholesteatoma

Occurs in the presence of a pre-existing tympanic membrane perforation, commonly:

• Posterosuperior marginal perforation
• Sometimes large central perforation

🧠 Theories of Pathogenesis of Secondary Acquired Cholesteatoma

• Habermann's Epithelial Migration theory
• Metaplasia theory

What is Habermann's Theory?

🔄 Genesis of Secondary Acquired Cholesteatoma

Two common pathways:

• Repeated infections through perforation → metaplasia of middle ear mucosa → cholesteatoma formation

• Acute necrotizing otitis media → large central or marginal perforation → epithelial migration through perforation → cholesteatoma

📝 Summary Table

📌 Exam Pearls

• Intact TM + pearly white mass → Congenital cholesteatoma
• Pars flaccida retraction → Primary acquired cholesteatoma
• Marginal perforation + cholesteatoma → Secondary acquired cholesteatoma