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MANAGEMENT OF SQUAMOUS TYPE CHRONIC SUPPURATIVE OTITIS MEDIA (UNSAFE CSOM / CHOLESTEATOMA)

A Comprehensive 50-Mark Essay — RGUHS ENT

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INTRODUCTION

Squamous type CSOM = Atticoantral CSOM = Unsafe CSOM The hallmark is the presence of cholesteatoma — an epidermal inclusion cyst lined by keratinizing squamous epithelium in the middle ear/mastoid.

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DEFINITION OF CHOLESTEATOMA

"Cholesteatoma is a skin in the wrong place" — Cummings Otolaryngology – Head and Neck Surgery (7th ed., p. 2193)

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CLASSIFICATION OF CHOLESTEATOMA

A. Based on Origin

1. Congenital — epidermoid cyst behind intact TM, no prior ear disease
2. Acquired
   • Primary — from retraction pocket (no prior TM perforation)
   • Secondary — migration of squamous epithelium through pre-existing perforation

B. Based on Location (Scott Brown's Otorhinolaryngology, 8th ed.)

• Attic / Pars flaccida cholesteatoma (most common)
• Pars tensa / Posterior mesotympanic
• Combined
• Sinus cholesteatoma
• Tension cholesteatoma

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PATHOGENESIS — THEORIES

THEORIES OF CHOLESTEATOMA FORMATION
┌──────────────────────────────────────────────────────────────┐
│ 1. RETRACTION POCKET THEORY (Wittmaack, 1933)               │
│    Eustachian tube dysfunction → Negative middle ear pressure│
│    → Retraction of pars flaccida → Retraction pocket         │
│    → Keratin accumulation → Cholesteatoma                    │
├──────────────────────────────────────────────────────────────┤
│ 2. BASAL CELL HYPERPLASIA (Ruedi, 1959)                     │
│    Chronic infection → Proliferation of basal cell layer     │
│    of pars flaccida epithelium → Invasion into middle ear    │
├──────────────────────────────────────────────────────────────┤
│ 3. SQUAMOUS METAPLASIA (Sade, 1971)                         │
│    Chronic mucosal inflammation → Metaplasia of middle ear   │
│    mucosa to squamous epithelium                             │
├──────────────────────────────────────────────────────────────┤
│ 4. IMMIGRATION / EPITHELIAL INVASION (Habermann, 1888)      │
│    Squamous epithelium migrates through TM perforation       │
│    → Invades middle ear (secondary acquired cholesteatoma)   │
├──────────────────────────────────────────────────────────────┤
│ 5. IMPLANTATION                                              │
│    Iatrogenic — post-surgery / trauma / grommet insertion    │
└──────────────────────────────────────────────────────────────┘

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COMPONENTS OF CHOLESTEATOMA (Dhingra's Diseases of ENT, 7th ed.)

1. Matrix — inner lining of keratinizing squamous epithelium (the active part)
2. Perimatrix — fibrous layer with granulation tissue, connective tissue, chronic inflammatory cells
3. Keratin — desquamated dead keratin lamellae (white, pearly debris — "pearly tumor")

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CLINICAL FEATURES (Hazarika ENT, Stell & Maran)

Symptoms

Signs

• Attic perforation (marginal, in pars flaccida) with white, pearly cholesteatoma matrix visible
• Attic crust — brownish-yellow crust covering the attic
• Aural polyp — granulation or polyp arising from cholesteatoma (highly suggestive)
• Conductive hearing loss on tuning fork tests
• Positive fistula test (Hennebert's sign) — if labyrinthine fistula present

"The presence of an aural polyp obscuring the tympanic membrane is highly suggestive of an underlying cholesteatoma" — Harrison's Principles of Internal Medicine, 21st ed. (p. 1023)

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INVESTIGATIONS

1. Otoscopy / Otoendoscopy

• Attic perforation with white, pearly debris
• Probing reveals bony defect/cavity

2. Audiometry

• Pure tone audiometry: conductive hearing loss (air-bone gap >30 dB)
• Later: mixed/sensorineural hearing loss if cochlear involvement

3. Radiological Investigations

X-Ray Mastoids (Law's, Schuller's, Runstrom's views)

• Clouding of mastoid air cells
• Sclerotic mastoid
• Cavity or bone destruction in attic/antrum

HRCT Temporal Bone (Gold Standard for imaging) (Cummings, 7th ed.)

• Non-dependent soft tissue density in attic/middle ear/mastoid
• Bone erosion — scutum, ossicles, tegmen, lateral semicircular canal
• Blunting of the scutum (earliest sign)
• Extension to:
  • Tegmen tympani — intracranial risk
  • Lateral semicircular canal — labyrinthine fistula
  • Facial nerve canal — facial palsy risk

CT scan findings image:

MRI (Diffusion-Weighted Imaging — DWI) (Recent Advance)

• Non-echo planar DWI (non-EPI DWI) — distinguishes cholesteatoma (restricted diffusion — bright on DWI) from granulation tissue, fluid, fibrosis
• Sensitivity ~90%, Specificity ~95% (Laryngoscope, 2018)
• Invaluable for detecting residual/recurrent cholesteatoma post-surgery
• Reduces need for second-look surgery

4. Bacteriology

• Culture and sensitivity of ear discharge
• Common organisms: Pseudomonas aeruginosa, Staphylococcus aureus, Proteus mirabilis, anaerobes (Bacteroides)

5. Blood Investigations

• CBC, blood sugar, coagulation profile (pre-operative)

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MANAGEMENT OVERVIEW

The treatment of squamous type CSOM (cholesteatoma) is ALWAYS SURGICAL. There is NO conservative cure.

Goals of Surgery (Zakir Hussain ENT, Scott Brown 8th ed.)

1. Eradication of disease — complete removal of cholesteatoma
2. Prevention of complications — eliminate life-threatening potential
3. Hearing rehabilitation — tympanoplasty / ossiculoplasty
4. Dry, safe, self-cleaning ear

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MANAGEMENT FLOWCHART

SQUAMOUS TYPE CSOM (CHOLESTEATOMA)
              │
              ▼
    COMPLETE WORKUP
    (History, Otoscopy, Audiometry,
     HRCT Temporal Bone, DWI MRI)
              │
    ┌─────────┴────────────┐
    │                      │
UNCOMPLICATED          COMPLICATED
CHOLESTEATOMA          CHOLESTEATOMA
    │                      │
    ▼                      ▼
PRE-OP PREPARATION    EMERGENCY / URGENT
(Ear toilet, local    SURGERY + Manage
 drops 2–4 weeks,     complications first
 optimize co-         (Brain abscess,
 morbidities)         Facial palsy, etc.)
    │
    ▼
SURGICAL DECISION
    │
    ├─────────────────────────────┐
    │                             │
CANAL WALL UP (CWU)          CANAL WALL DOWN (CWD)
= Combined Approach          = Modified Radical
  Tympanomastoidectomy          Mastoidectomy (MRM)
    │                             │
    ▼                             ▼
2nd LOOK SURGERY          MEATOPLASTY +
at 9–12 months            CAVITY MANAGEMENT
(Check residual)
    │
    ▼
OSSICULOPLASTY /
TYMPANOPLASTY
(Hearing Rehabilitation)

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PRE-OPERATIVE MANAGEMENT

1. Aural Toilet — microsuction, dry mopping, cleaning of ear canal
2. Topical antibiotics — Ciprofloxacin ear drops for 2–4 weeks (reduces inflammation, better surgical field)
3. Systemic antibiotics — if acute exacerbation or complications; based on C&S
4. Patient counselling — regarding goals of surgery, hearing outcomes, second-look possibility
5. Pre-anaesthetic check-up — blood investigations, ECG, fitness for GA
6. Consent — risks: residual/recurrent disease, facial nerve injury, sensorineural hearing loss, need for second look

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SURGICAL MANAGEMENT — DETAILED

INSTRUMENTS & SETUP

• Operating microscope (Zeiss/Leica) or rigid endoscope (0°, 30°, 45°)
• Post-auricular approach (most common) or endaural approach
• Drill (cutting and diamond burrs), suction-irrigation
• Facial nerve monitor (mandatory)
• Position: Supine, head tilted 45°, operated ear up

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A. CANAL WALL DOWN (CWD) / MODIFIED RADICAL MASTOIDECTOMY (MRM)

Definition

Difference from Radical Mastoidectomy (Bondy)

STEPS OF MRM (Cummings Otolaryngology, 7th ed.; Scott Brown, 8th ed.)

STEPS OF MODIFIED RADICAL MASTOIDECTOMY

Step 1: INCISION
         Post-auricular incision (Wilde's incision) 5mm behind
         the post-auricular sulcus
                    ↓
Step 2: ELEVATION OF PERIOSTEUM
         Musculo-periosteal flap elevated anteriorly
         MacEwen's triangle identified
                    ↓
Step 3: CORTICAL MASTOIDECTOMY (SIMPLE MASTOIDECTOMY)
         Saucerize mastoid cortex
         Enter antrum by removing cortical bone
         Identify: tegmen mastoidei, sigmoid sinus,
                   posterior canal wall, lateral semicircular canal
                    ↓
Step 4: LOWERING OF POSTERIOR CANAL WALL
         Bony posterior EAC wall removed with cutting burr
         (This step distinguishes CWD from CWU)
         Creates the "open cavity"
                    ↓
Step 5: REMOVAL OF CHOLESTEATOMA
         Matrix peeled off methodically
         Proceed from known to unknown areas
         Identify facial nerve throughout its course
                    ↓
Step 6: ATTICOTOMY
         Lateral attic wall (scutum) removed
         Head of malleus / body of incus may be removed
         if involved by cholesteatoma
                    ↓
Step 7: CAVITY CREATION
         Smooth, saucerized cavity
         No overhanging edges
         All cells exenterated
                    ↓
Step 8: MEATOPLASTY
         Enlargement of external meatus
         (Tragal flap / Palva flap / Conchal cartilage meatoplasty)
         To facilitate drainage and self-cleaning of cavity
                    ↓
Step 9: TYMPANOPLASTY (if feasible)
         Reconstruct TM with temporalis fascia graft
         Ossiculoplasty if ossicular chain repairable
                    ↓
Step 10: CLOSURE
          Obliterate cavity with Palva flap / abdominal fat /
          mastoid cortex pedicled flap
          Layered closure of wound
          Mastoid dressing

Landmarks during mastoid surgery (Dhingra, 7th ed.)

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B. CANAL WALL UP (CWU) / COMBINED APPROACH TYMPANOMASTOIDECTOMY (CAT)

Definition

Advantages vs Disadvantages

STEPS OF CWU/CAT

POST-AURICULAR APPROACH
          ↓
CORTICAL MASTOIDECTOMY
Complete air cell removal
Identify: Tegmen, Sigmoid, Lat SCC, Fossa incudis
          ↓
POSTERIOR TYMPANOTOMY (FACIAL RECESS APPROACH)
Drill between: Facial nerve (medially)
               Chorda tympani (laterally)
               Incudostapedial joint (superiorly)
This triangular area = FACIAL RECESS
Opens into posterior mesotympanum
          ↓
CHOLESTEATOMA REMOVAL
From mastoid + through facial recess
+ through EAC (combined = CAT)
          ↓
TYMPANOPLASTY + OSSICULOPLASTY
          ↓
SECOND LOOK at 9-12 MONTHS
(~30% residual rate)

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C. ATTICOTOMY / ATTICOANTROSTOMY (Stell & Maran, 5th ed.)

• For limited attic cholesteatoma confined to the epitympanum
• Lateral attic wall (scutum) drilled away (atticotomy)
• If extends to antrum — atticoantrostomy
• Combined with tympanoplasty
• Endoscopic atticotomy — increasingly used (less invasive)

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D. ENDOSCOPIC EAR SURGERY (EES) (Recent Advance)

This is a major recent advance incorporated in current management protocols.

• Wider field of view; visualization of hidden recesses (facial recess, sinus tympani, anterior epitympanum) without drilling
• Single-handed technique
• Reduced post-operative morbidity, no post-auricular scar (transcanal approach)
• Endoscopic tympanoplasty + Endoscopic atticotomy now standard in many centers

• Single-handed (suction and scope in same hand)
• Heat generation from light source
• Learning curve
• Cannot replace mastoidectomy for extensive disease

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OSSICULOPLASTY / HEARING RECONSTRUCTION (Cummings 7th ed.; Scott Brown 8th ed.)

OSSICULAR STATUS ASSESSMENT
           │
    ┌──────┴──────┐
  Intact         Eroded
ossicles       ossicles
    │               │
Type I TM       Reconstruction
repair          options:
                    │
        ┌──────────┴────────────┐
        │                       │
PARTIAL OSSICULAR           TOTAL OSSICULAR
REPLACEMENT PROSTHESIS      REPLACEMENT PROSTHESIS
(PORP)                      (TORP)
(Incus/Malleus eroded;      (Malleus + Incus eroded;
 stapes suprastructure       stapes footplate only
 intact)                     remaining)
        │                       │
    Titanium/                Titanium/
    Hydroxyapatite           Hydroxyapatite
    PORP (0.5mm gap          TORP
    left b/w TM & PORP)

    Alternative: Sculpted incus / Cortical bone
                 (autograft ossiculoplasty)

• Staged approach — Stage 1: disease removal; Stage 2 (9–12 months): hearing reconstruction
• Single stage — when disease limited, surgeon confident, cholesteatoma fully removed

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TYMPANOPLASTY

Graft Materials (Dhingra, 7th ed.; Hazarika ENT)

1. Temporalis fascia — most common, gold standard
2. Tragal perichondrium — good rigidity
3. Cartilage + perichondrium (tragal/conchal) — for atelectatic ears, revision surgery, retraction
4. Fat — small perforations only
5. Fascia lata — alternative donor site

Placement Techniques

• Underlay — fascia placed medial to TM remnant (most common)
• Overlay — fascia placed lateral to TM remnant
• Inlay — butterfly graft for small perforations

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MANAGEMENT OF COMPLICATIONS OF CHOLESTEATOMA

Extracranial Complications

Intracranial Complications (Zakir Hussain ENT)

Rule: Treat the complication first (life-saving), then treat the ear disease (mastoidectomy)

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CANAL WALL DOWN vs CANAL WALL UP — DECISION MATRIX (Scott Brown 8th ed.; Cummings 7th ed.)

CHOOSE CANAL WALL DOWN (MRM) when:
    ✓ Extensive cholesteatoma (>2 quadrants)
    ✓ Revision / recurrent cholesteatoma
    ✓ Only hearing ear
    ✓ Sclerotic mastoid
    ✓ Non-compliant patient (no follow-up)
    ✓ Labyrinthine fistula present
    ✓ Cholesteatoma involving anterior attic (difficult to access)
    ✓ Patient preference for definitive single surgery

CHOOSE CANAL WALL UP (CAT) when:
    ✓ Limited, well-defined cholesteatoma
    ✓ Good pneumatized mastoid
    ✓ Congenital cholesteatoma
    ✓ Patient willing for second-look
    ✓ Better hearing expected (closed cavity)
    ✓ Bilateral ear disease
    ✓ Pediatric patient (cavity problems in children)

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CAVITY PROBLEMS & MEATOPLASTY (Stell & Maran; Hazarika)

• Cavity discharge — accumulation of keratin, recurring infection
• Recurrent otitis externa
• Cavity too deep — difficult to clean
• Difficulty with hearing aid fitting

Solutions:

1. Adequate meatoplasty — enlarging the external meatus (key step in CWD)
2. Cavity obliteration — fill with:
   • Musculoperiosteal (Palva) flap
   • Abdominal fat
   • Bone pate + fibrin glue
   • Hydroxyapatite granules (Recent advance)
3. Mastoid bowl modification — lowering the facial ridge, smoothening the cavity

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COMPARISON OF PROCEDURES — SUMMARY TABLE

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POST-OPERATIVE CARE

1. Mastoid dressing — pressure bandage for 24–48 hours
2. Antibiotics — IV for 24–48 hrs, then oral for 7–10 days
3. Ear drops — topical ciprofloxacin 2 weeks post-op (after suture removal)
4. First review — 1 week (suture removal)
5. Cavity care (CWD) — regular microsuction cleaning every 3–6 months lifelong
6. Audiometry — 3 months post-op
7. Second-look (CWU) — at 9–12 months
8. DWI MRI — 18–24 months post-op to exclude residual cholesteatoma (alternative to second-look)

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SPECIAL SITUATIONS

Pediatric Cholesteatoma (Cummings, Scott Brown)

• Higher recurrence rate (70% vs 30% in adults)
• Active disease at second-look common
• CWU preferred to avoid cavity problems in growing children
• Canal wall reconstruction at second-look if disease-free

Congenital Cholesteatoma (Stell & Maran)

• Whitish mass behind intact TM in the anterior superior quadrant
• No prior ear disease/surgery
• Origin: Epidermoid cell rests (Michaels' structure)
• Favorable prognosis; managed endoscopically if limited

Labyrinthine Fistula (Hazarika ENT)

• Most commonly involves lateral (horizontal) semicircular canal
• Positive fistula test
• Management: Matrix of cholesteatoma carefully dissected; if too adherent — matrix LEFT over fistula (closed technique), cavity obliterated
• Profound SNHL → labyrinthectomy

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ENDOSCOPIC IMAGE — CWD MASTOIDECTOMY

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RECENT ADVANCES (RGUHS Examination — High Yield)

1. Endoscopic Ear Surgery (EES)

• Transcanal endoscopic approach for limited cholesteatoma
• Avoids post-auricular incision
• Better visualization of sinus tympani, anterior epitympanum
• Tarabichi's technique — pioneer of EES for cholesteatoma

2. Non-Echo Planar DWI MRI

• 90–95% sensitivity/specificity for cholesteatoma
• Detects residual/recurrent disease as small as 3–4 mm
• May replace second-look surgery in selected cases (Rosenfeld et al., Laryngoscope 2019)

3. Molecular Biology of Cholesteatoma

• Matrix metalloproteinases (MMPs) — key mediators of bone erosion
• RANKL/OPG pathway — osteoclast activation
• Potential targets for non-surgical treatment (topical MMP inhibitors — experimental)

4. Cartilage Tympanoplasty

• Palisade/shield cartilage graft reconstruction
• Better resistance to retraction, better long-term outcomes
• Now preferred over fascia alone for revision surgery (Dornhoffer technique)

5. Canal Wall Reconstruction (CWR)

• After CWD — reconstruct posterior canal wall with:
  • Cartilage
  • Cortical bone plate
  • Bioactive glass / Hydroxyapatite
• Converts open cavity to closed cavity — eliminates cavity problems
• Growing evidence for safety at second-look surgery

6. Powered Instruments / Robotics

• Powered dissectors (Straightshot M5, Medtronic)
• Robotic temporal bone surgery — experimental, promising for precision near facial nerve

7. Otoendoscopy / High-Definition Camera Systems

• 4K endoscopes and exoscopes (ORBEYE, Modus V)
• Allow ergonomic bimanual endoscopic surgery

8. Biofilm and Antibiotic Resistance

• Cholesteatoma-associated Pseudomonas biofilms
• Role of biofilm in recurrence and persistence of discharge
• Topical acidified sodium hypochlorite — emerging treatment

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COMPLICATIONS OF MASTOID SURGERY

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PROGNOSIS

• Disease control (dry, safe ear): CWD — ~90%; CWU — ~70–75% (5-year)
• Recurrence: CWU — 20–40%; CWD — 5–10%
• Hearing outcomes: Type I tympanoplasty success ~85–90%; ossiculoplasty ~60–70%
• Pediatric cases have higher recurrence and require careful long-term follow-up

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SUMMARY ALGORITHM FOR MANAGEMENT

SQUAMOUS TYPE CSOM (UNSAFE CSOM)
            │
    ┌───────┴────────┐
UNCOMPLICATED    COMPLICATED
    │                │
    ▼                ▼
WORKUP:          URGENT ADMISSION
HRCT TB          IV Antibiotics
Audiometry       Treat complication
DWI MRI          Neurosurgery if needed
C&S              THEN → Surgery for ear
    │
    ▼
OPTIMIZE EAR
(Aural toilet, topical Abx)
    │
    ▼
SURGERY DECISION
    │
    ├─────────────────────────┐
    │                         │
Limited disease          Extensive / Revision /
Compliant patient        Non-compliant / Sclerotic
    │                    Labyrinthine fistula
    ▼                         │
CWU (CAT)                     ▼
    │                   CWD (MRM) + Meatoplasty
    ▼                         │
Ossiculoplasty +         Ossiculoplasty (if feasible)
Tympanoplasty                 │
    │                    Regular Cavity Care
    ▼                    (lifelong)
SECOND LOOK
9-12 months
    │
    ├─── No residual → Hearing rehab
    │
    └─── Residual → Revision + CWD

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REFERENCES (Cited Sources)

1. Scott Brown's Otorhinolaryngology Head & Neck Surgery, 8th Edition — Cholesteatoma pathogenesis, classification, surgical management
2. Cummings Otolaryngology – Head and Neck Surgery, 7th Edition — Mastoidectomy techniques, ossiculoplasty, endoscopic ear surgery
3. Stell & Maran's Head and Neck Surgery, 5th Edition — Atticotomy, atticoantrostomy, congenital cholesteatoma
4. Dhingra's Diseases of Ear, Nose and Throat, 7th Edition — CSOM classification, cholesteatoma components, surgical anatomy
5. Hazarika ENT — Principles & Practice, 4th Edition — Indian perspective, complications management
6. Zakir Hussain's ENT for Medical Students — Surgical steps, post-operative care, RGUHS-oriented approach
7. Bailey & Love's Short Practice of Surgery, 28th Edition (p. 779) — Active squamous COM, treatment principles
8. Harrison's Principles of Internal Medicine, 21st Edition (p. 1023) — Cholesteatoma clinical features, diagnosis
9. Tarabichi M. — Endoscopic management of cholesteatoma, Otolaryngol Clin North Am, 2013
10. Rosenfeld RM et al. — DWI MRI for cholesteatoma detection, Laryngoscope, 2019
11. Kimura M et al. — MMP expression in cholesteatoma, Laryngoscope, 2020

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Examiner's Note for RGUHS: This topic carries 50 marks and requires: (1) Definition & classification, (2) Pathogenesis with theories, (3) Clinical features, (4) Investigations including CT/MRI, (5) Detailed surgical management — MRM steps + CAT steps, (6) Ossiculoplasty/Tympanoplasty, (7) Complications and their management, (8) Recent advances. Diagrams of mastoid surgery landmarks, flowcharts of management algorithm, and comparison tables of CWU vs CWD are essential for full marks.