--- title: "Types of Cholesteatoma - Congenital & Acquired Cholesteatoma" description: "ENT notes on Types of Cholesteatoma." source: "The ENT Resident" author: "Dr. Mausumi Das" canonical_url: "https://www.theentresident.com/ent-notes-lectures/cholesteatoma-types-congenital-acquired" categories: ["Otology", "Short Questions in ENT"] video: "https://www.youtube.com/watch?v=dys5YyJAPGE" image: "https://media.theentresident.com/og-images/cholesteatoma-types-congenital-acquired.png" --- # Types of Cholesteatoma - Congenital & Acquired Cholesteatoma ENT notes on Types of Cholesteatoma. Watch the full video lecture on YouTube: https://www.youtube.com/watch?v=dys5YyJAPGE ## 🦻 Types of Cholesteatoma Cholesteatoma is broadly classified into **two major types**: - **Congenital cholesteatoma** - **Acquired cholesteatoma** - Primary acquired cholesteatoma - Secondary acquired cholesteatoma --- ### πŸ‘Ά Congenital Cholesteatoma #### πŸ“– 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 β†’ accumulation of squamous epithelium in middle ear β†’ 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 β†’ can mimic CSOM > What is Levenson’s Criteria for diagnosis of Congenital Cholesteatoma? --- #### πŸ“Š Potsic Staging System (Congenital Cholesteatoma) | Stage | Description | | --- | --- | | **Stage I** | Single quadrant involvement; no ossicular or mastoid involvement | | **Stage II** | Multiple quadrants involved; no ossicular or mastoid involvement | | **Stage III** | Ossicular involvement without mastoid extension | | **Stage IV** | Mastoid extension irrespective of other findings | --- ### 🟑 Primary Acquired Cholesteatoma #### πŸ“– Why β€œPrimary”? 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? --- #### πŸ”„ Genesis of Primary Acquired Cholesteatoma - **Eustachian tube dysfunction** β†’ persistent negative middle ear pressure - Leads to: - **Pars flaccida / posterosuperior pars tensa retraction pocket** - Keratin accumulation β†’ cholesteatoma Other mechanisms: - Repeated subclinical infections β†’ **metaplasia of middle ear mucosa** - **Basal layer proliferation** of epithelium --- ### πŸ”΄ Secondary Acquired Cholesteatoma #### πŸ“– 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? --- #### πŸ”„ Genesis of Secondary Acquired Cholesteatoma Two common pathways: - **Repeated infections through perforation** β†’ metaplasia of middle ear mucosa β†’ cholesteatoma formation - **Acute necrotizing otitis media** β†’ large central or marginal perforation β†’ **epithelial migration through perforation** β†’ cholesteatoma --- ### πŸ“ Summary Table | Type | Tympanic Membrane | Key Mechanism | | --- | --- | --- | | **Congenital** | Intact | Persistent embryonic epithelial rests | | **Primary Acquired** | Intact | Retraction pocket due to ET dysfunction | | **Secondary Acquired** | Perforated | Epithelial migration or metaplasia | --- ### πŸ“Œ Exam Pearls - Intact TM + pearly white mass β†’ **Congenital cholesteatoma** - Pars flaccida retraction β†’ **Primary acquired cholesteatoma** - Marginal perforation + cholesteatoma β†’ **Secondary acquired cholesteatoma** --- ## About This Note This free ENT note on Types of Cholesteatoma - Congenital & Acquired Cholesteatoma was written by Dr. Mausumi Das and is paired with a corresponding video lecture. 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