Cholesteatoma

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Definition & Pathogenesis - The Skin Invasion

  • Definition: Destructive, expansile lesion of keratinizing squamous epithelium (skin) in middle ear, mastoid, or temporal bone.
  • Types:
    • Congenital: Embryonic epidermal rests; pearly white mass behind intact TM.

      ⭐ Congenital cholesteatoma typically presents as a white mass behind an intact tympanic membrane in a child with no history of otorrhea or perforations.

    • Acquired (Common):
      • Primary: TM retraction (pars flaccida) from chronic Eustachian tube dysfunction (ETD).
      • Secondary: Epithelial migration through pre-existing TM perforation.
  • Key Pathogenesis Theories (Acquired):
    • Invagination (Wittmaack): ETD → Negative pressure → Retraction pocket → Keratin accumulation.
    • Epithelial Migration (Ruedi): Basal cell migration through TM defect.
    • Metaplasia (Habermann): Chronic inflammation transforms mucosa (less favored).

Cholesteatoma pathogenesis theories

Clinical Features - The Silent Destroyer

  • Symptoms (often insidious):
    • Persistent, foul-smelling, scanty ear discharge (otorrhea).

      ⭐ Foul-smelling, scanty, persistent ear discharge is highly characteristic of cholesteatoma.

    • Progressive conductive hearing loss (CHL); may become sensorineural (SNHL).
    • Dull earache (otalgia); severe pain suggests complications.
    • Vertigo, tinnitus (labyrinthine involvement).
    • Facial weakness (late, ominous sign).
  • Signs (Otoscopy):
    • Attic or posterosuperior marginal perforation or deep retraction pocket.
    • Otoscopic view of cholesteatoma
    • Visible whitish/pearly keratin debris or flakes.
    • Ossicular chain erosion, granulation tissue, or aural polyp.
    • Positive fistula test (e.g., Hennebert's sign) if labyrinthine erosion.

Investigations - Spotting the Culprit

  • Otoscopy: Key initial exam. Reveals attic or posterosuperior pars tensa retraction pocket, keratin debris, granulations, or polyp.
  • Tuning Fork Tests (Rinne, Weber): Suggest conductive hearing loss (CHL).
  • Pure Tone Audiometry (PTA): Quantifies CHL; may show mixed loss if cochlea involved.
  • Microscopy: Detailed examination, allows for suction clearance.
  • CT Scan (HRCT Temporal Bone):
    • Assesses bony erosion (scutum, ossicles, tegmen, sigmoid plate).
    • Shows extent of soft tissue mass.

    ⭐ HRCT temporal bone is the gold standard imaging for assessing bony erosion and extent of cholesteatoma.

Axial HRCT: Left temporal bone cholesteatoma

  • MRI (DW-MRI): Differentiates cholesteatoma from granulation tissue or cholesterol granuloma, especially in post-operative recurrence. Useful if CT is equivocal.

Complications - Danger Zones

  • Intracranial (↑ mortality):
    • Meningitis: Most common intracranial.
    • Brain abscess: Temporal lobe, cerebellum.
    • Lateral sinus thrombophlebitis.
    • Otitic hydrocephalus (pseudotumor cerebri).
  • Extracranial:
    • Facial nerve palsy.
    • Labyrinthitis (serous/suppurative) → SNHL, vertigo.
    • Mastoiditis & subperiosteal abscess (e.g., Bezold's, Citelli's).
    • Petrositis (Gradenigo's syndrome: VI nerve palsy, retro-orbital pain, otorrhea).
  • Key Spread Pathways (Danger Zones):
    • Tegmen tympani/mastoideum → Middle cranial fossa.
    • Sinodural angle (Trautmann's triangle) → Posterior fossa, sigmoid sinus.
    • Medial wall (oval/round windows) → Inner ear.
    • Fallopian canal dehiscence → Facial nerve.

⭐ Facial nerve palsy is the most common cranial nerve palsy associated with cholesteatoma.

Management - Surgical Showdown

⭐ The primary goal of cholesteatoma surgery is to create a safe, dry, and disease-free ear; hearing preservation/reconstruction is secondary.

  • Surgical Goals: Complete disease eradication, create a permanently safe, dry, aerated ear. Hearing preservation/reconstruction is secondary.
  • Main Approaches:
-   **Canal Wall Up (CWU):** e.g., Atticotomy, Intact Canal Wall Mastoidectomy. Preserves posterior canal wall.
-   **Canal Wall Down (CWD):** e.g., Modified Radical Mastoidectomy (MRM). Removes posterior canal wall, exteriorizes disease.
  • Ossiculoplasty: Reconstructs ossicular chain if eroded; can be staged.

High-Yield Points - ⚡ Biggest Takeaways

  • Cholesteatoma is keratinizing squamous epithelium in the middle ear/mastoid.
  • Acquired type commonly arises from pars flaccida retraction (Attic).
  • Key symptoms: painless, foul-smelling otorrhea; progressive conductive hearing loss.
  • CT temporal bone is crucial for diagnosis and delineating extent.
  • Mainstay of treatment is surgical eradication (e.g., mastoidectomy).
  • Serious complications include ossicular erosion, facial palsy, and intracranial spread.
  • Congenital type: pearly white mass behind intact TM, often in anterosuperior quadrant_._

Practice Questions: Cholesteatoma

Test your understanding with these related questions

All are intracranial complications of otitis media except which of the following?

1 of 5

Flashcards: Cholesteatoma

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The most accepted theory for secondary cholesteatoma is _____ theory.

TAP TO REVEAL ANSWER

The most accepted theory for secondary cholesteatoma is _____ theory.

Habermann's

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