In Netherlands, cholesteatoma is managed by ents. Cholesteatoma is an abnormal, expansile collection of keratinizing squamous epithelium within the middle ear or mastoid cavity that gradually erodes surrounding bone. Annual incidence in adults is roughly 9 per 100,000 (Tos 1988); pediatric incidence about 3 per 100,000, with bilateral disease in 10-15% of children.
Cholesteatoma (ICD-10: H71) is a destructive epithelial cyst within the middle ear cleft (tympanic cavity, attic, antrum, mastoid air cells) lined by keratinizing stratified squamous epithelium. The lesion is not a true neoplasm but produces matrix expansion, periostitis, and bone erosion through release of osteolytic cytokines (IL-1, IL-6, TNF-alpha, RANKL) by the perimatrix. Cholesteatomas are classified by the EAONO/JOS 2017 consensus as congenital (white pearl behind an intact eardrum with no history of otorrhea or eardrum surgery) or acquired, with acquired forms subdivided into primary acquired (retraction-pocket-derived, most commonly Prussak's space or pars flaccida) and secondary acquired (following tympanic-membrane perforation, trauma, or post-surgical implantation). Stage is assigned by anatomic extent and reflects surgical complexity and prognosis: Stage I (limited to one site, attic or mesotympanum), Stage II (two or more sites), Stage III (extracranial complications such as labyrinthine fistula or facial nerve involvement), and Stage IV (intracranial complications).
The key symptoms of Cholesteatoma are: Chronic, foul-smelling otorrhea that does not respond to repeated courses of antibiotics — the hallmark complaint, present in 60-80% of acquired cases., Progressive conductive hearing loss of 20-40 dB caused by ossicular erosion (typically incus long process and stapes superstructure)., Retraction pocket or perforation in the pars flaccida or posterior-superior quadrant visible at otoscopy, often containing white keratin debris., White pearl visible behind an intact tympanic membrane on otoscopy in congenital cholesteatoma, classically in the anterior-superior quadrant in children age 2-10., Ear fullness, pressure, and intermittent dull pain in chronic disease; severe pain is uncommon and suggests complication., Vertigo or imbalance, particularly with Valsalva maneuver or pressure changes — pneumatic-otoscopy-induced vertigo (positive fistula test) suggests labyrinthine fistula., Facial weakness ranging from subtle asymmetry to House-Brackmann grade III-VI palsy when the lesion erodes the fallopian canal..
Diagnosis combines clinical otoscopic examination with audiometry and selected imaging. Microsuction with a binocular operating microscope or oto-endoscope is the cornerstone of clinical assessment: removal of keratin debris and granulation tissue exposes the retraction pocket, perforation, or pearl. Documented findings include site (pars flaccida attic, pars tensa, posterior-superior, anterior-superior), extent (limited or extensive), bone erosion, and any visible middle-ear structures (ossicles, facial canal). Pure-tone audiometry quantifies conductive, mixed, or rarely sensorineural hearing loss and informs ossicular reconstruction planning. Imaging is required preoperatively for all cases. Non-contrast high-resolution CT of the temporal bones (0.6 mm slices) maps anatomy: tegmen tympani thickness, sigmoid sinus position, facial canal integrity, scutum erosion, ossicular continuity, lateral semicircular canal fistula, and mastoid pneumatization. CT identifies bony erosion patterns but cannot reliably distinguish granulation tissue from cholesteatoma matrix. Non-echo-planar diffusion-weighted MRI is the modern standard for distinguishing cholesteatoma from inflammation: cholesteatomas show restricted diffusion (bright on b1000) due to densely packed keratin, with sensitivity 90% and specificity 95% for lesions over 3 mm (De Foer 2008). This imaging has reduced the need for routine second-look surgery in many centers. Pneumatic otoscopy and the fistula test (positive vertigo or nystagmus with pressure changes) suggest labyrinthine fistula. Facial nerve function is documented with House-Brackmann grading. In children, examination under anesthesia may be required.
Complete surgical removal at the primary procedure leads to a disease-free, dry ear in 60-85% of adult patients and 50-75% of pediatric patients at 5 years. Hearing outcomes depend on ossicular status at time of surgery and reconstruction method: closure of the air-bone gap to within 20 dB is achieved in 50-70% of cases with PORP and 30-50% with TORP. Recurrence rates differ by approach: 20-40% with canal-wall-up versus 5-15% with canal-wall-down; pediatric recurrence rates are higher because of faster epithelial growth and more aggressive disease. Facial palsy resolves to House-Brackmann grade I-II in 70-85% of cases when surgery occurs within 2 weeks of onset. Intracranial complications occur in under 1% of treated cases in high-income countries but remain substantially more common in low-resource settings with untreated disease. Lifelong follow-up is required because recurrent or residual disease can present years after apparently complete surgery.
Cholesteatoma requires evaluation by an otologist or general otolaryngologist with cholesteatoma surgical volume. The diagnosis is made in clinic but management is operative; delay risks ossicular destruction, hearing loss, facial nerve injury, and intracranial complications. Pediatric cases benefit from a pediatric otolaryngology service. Recurrent or extensive disease, only-hearing ear, and skull-base involvement need a neurotologist at a tertiary center.
Find specialists →Postoperative discharge usually next day for simple tympanoplasty, 1-2 days after mastoidectomy. Outer dressing removal and gentle ear cleaning at 2 weeks. Audiogram at 6-8 weeks. Graft take confirmed at 6 weeks. Hearing improvement evident from 8-12 weeks and stabilizes by 6 months. Canal-wall-down cavity-care visits every 6-12 months; canal-wall-up DWI surveillance at 12 and 24 months. Second-look ossiculoplasty, when planned, performed 6-12 months after primary surgery.
Resume light activity within 1-2 weeks of surgery. Avoid heavy lifting, straining, and contact sports for 4-6 weeks while the graft heals. Air travel is generally allowed after 2-4 weeks at the surgeon's discretion. Scuba diving requires individualized assessment by an otologist — most patients with prior cholesteatoma are advised against deep diving because of barotrauma risk and the presence of a mastoid cavity.
Choose an otologist performing at least 40 mastoidectomy and tympanoplasty procedures per year, with access to non-EPI DWI MRI surveillance and high-resolution temporal bone CT. Pediatric cases benefit from surgeons trained in both pediatric and otologic surgery. Endoscopic ear surgery experience is desirable for limited attic disease. Multidisciplinary cleft and craniofacial teams improve outcomes in patients with cleft palate.
Medically reviewed by AIHealz Medical Editorial Board · May 13, 2026
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