What is the management of cholesteatoma?

Management of Cholesteatoma

Surgical excision is the only effective treatment for cholesteatoma, as there are no viable nonsurgical therapies currently available. 1, 2, 3

Immediate Referral and Diagnosis

Any patient with suspected cholesteatoma must be referred immediately to an otolaryngologist for definitive surgical management. 4, 5 The diagnosis should be suspected in patients presenting with:

• Painless, foul-smelling purulent otorrhea (most common presentation) 5
• Hearing loss, tinnitus, and ear fullness 5
• Tympanic membrane abnormalities including retraction pockets, perforation, white keratinous debris visible behind intact membrane, or granulation tissue 4, 5
• Attic blockage and scutum erosion on careful otoscopic examination 5

Preoperative Imaging Strategy

High-resolution CT temporal bone without IV contrast is the cornerstone imaging modality for presurgical planning, providing excellent anatomic detail of bony structures and determining disease extent. 4 This imaging:

• Accurately detects ossicular erosion, scutum erosion, tegmen erosion, and lateral semicircular canal erosion 4
• Identifies potential surgical hazards and guides surgical approach decisions 4
• Should NOT be ordered with IV contrast, as it provides no additional necessary bony detail 4

MRI with non-echoplanar diffusion-weighted imaging (DWI) in the coronal plane is essential for differentiating cholesteatoma from other soft tissue pathology, including scar tissue and inflamed granulation tissue, with high sensitivity and specificity. 4

Surgical Approach Selection

The surgical technique should be tailored to the extent and location of disease:

For Limited Disease (Type 1)

• Retraction of Shrapnell's membrane or posterosuperior quadrant with minimal cholesteatoma lateral to ossicles: Transcanal technique to evert the cholesteatomatous pocket intact, followed by reconstruction of the outer attic wall 6
• Atticotomy/limited mastoidectomy (inside-outside approach through endaural incision) creates small, dry, self-cleaning cavities without need for meatoplasty or obliteration 7

For Moderate Disease (Type 2)

• Circumscribed cholesteatoma lateral and often medial to ossicles: Combined approach tympanoplasty or canal wall up mastoidectomy 6
• If luxation of matrix in toto fails, proceed to radical mastoidectomy 6

For Extensive Disease (Type 3)

• Marginal perforations with large cholesteatomas medial to ossicular chain: Canal wall down mastoidectomy is the method of choice 8, 6
• Extensive cholesteatomas with tegmen erosion, sigmoid sinus invasion, or jugular bulb involvement: Canal wall down mastoidectomy with or without ossiculoplasty, with neurosurgical intervention considered in the same sitting when intracranial complications present 8

Critical Surgical Considerations

The choice of operative procedure must account for disease extent and surgeon experience, with the primary goal being complete disease eradication while minimizing recurrence risk. 7, 6 Key anatomical hazards to anticipate include:

• Ossicular chain erosion (most common) 4
• Facial canal dehiscence 1
• Lateral semicircular canal erosion 4, 1
• Tegmen tympani or tegmen mastoideum erosion with potential brain hernia or CSF leak 8
• Sigmoid sinus or jugular bulb invasion in extensive cases 8

Postoperative Management

Intravenous antibiotics are required postoperatively, with consideration of IV steroids when indicated. 8 The polymicrobial bacterial flora typically includes H. influenzae, S. aureus, and P. aeruginosa. 5, 1

Postoperative Surveillance

CT temporal bone without IV contrast is the primary modality for detecting recurrent cholesteatoma, evaluating for abnormal, new, or enlarging lobulated material in the temporal bone region. 4

MRI with DWI and delayed postcontrast T1-weighted imaging differentiates recurrent cholesteatoma from postoperative granulation tissue and scar, which is critical for distinguishing keratinous debris from non-cholesteatoma findings postoperatively. 4

Common Pitfalls to Avoid

• Never rely on plain radiography, as it has no role in cholesteatoma diagnosis or surveillance 4
• Do not confuse cholesteatoma with simple acute otitis media or ventilation tube-associated otorrhea, as cholesteatoma requires surgical management and is characterized by white keratinous debris 5
• Avoid CT with IV contrast, as it provides no additional necessary information 4
• Do not delay referral to otolaryngology, as untreated cholesteatoma causes progressive bone erosion through enzymatic activity and pressure effects, leading to potentially fatal intracranial complications 1, 3