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Cholesteatoma – Causes, Symptoms and Treatment

A cholesteatoma is a benign but aggressive growth of skin tissue in the middle ear that can destroy bone and cause permanent hearing loss if left untreated.

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Things worth knowing about "Cholesteatoma"

A cholesteatoma is a benign but aggressive growth of skin tissue in the middle ear that can destroy bone and cause permanent hearing loss if left untreated.

What Is a Cholesteatoma?

A cholesteatoma is an abnormal, non-cancerous accumulation of dead skin cells (keratinizing squamous epithelium) in the middle ear or the mastoid bone behind the ear. Despite being benign, it behaves aggressively at the local level: it grows slowly but steadily, eroding the surrounding bone structures. If left untreated, it can lead to serious complications such as permanent hearing loss, dizziness, or bacterial meningitis.

Causes

There are two main types of cholesteatoma:

Acquired cholesteatoma: The most common form. It typically develops as a result of chronic middle ear infections or persistent negative pressure in the middle ear (e.g., due to Eustachian tube dysfunction). This causes the eardrum to retract and form a pocket in which shed skin cells accumulate.
Congenital cholesteatoma: Less common. It develops from embryonic tissue remnants behind an intact eardrum, without any prior infection or surgery.

Symptoms

Symptoms often develop gradually. Typical signs of a cholesteatoma include:

One-sided, often foul-smelling ear discharge (otorrhea)
Progressive hearing loss in one ear
Feeling of pressure or pain in the ear
Dizziness or vertigo (if the balance organ is affected)
Tinnitus (ringing in the ear)
In advanced cases: facial nerve paralysis, headache, or fever indicating complications

Diagnosis

Diagnosis is made by an ear, nose, and throat (ENT) specialist. The following examinations are typically used:

Otoscopy: Direct examination of the ear canal and eardrum using an otoscope. Characteristic findings include whitish, flaky debris or a retraction pocket in the eardrum.
Audiometry: Hearing tests to assess the degree of hearing loss.
CT scan: A computed tomography (CT) scan of the temporal bone shows the exact extent of the cholesteatoma and any bone erosion.
MRI: Non-echo-planar diffusion-weighted MRI is particularly useful for distinguishing cholesteatoma from other conditions and for detecting recurrence after surgery.

Treatment

A cholesteatoma cannot be treated with medication alone. The only effective treatment is surgical removal. Depending on the extent of the disease, various surgical techniques are available:

Tympanoplasty: Reconstruction of the eardrum and ossicles (hearing bones) after removal of the cholesteatoma, with the aim of preserving or restoring hearing.
Mastoidectomy: Surgical clearance of the mastoid bone if the cholesteatoma has spread to that area.
Canal-wall-up and canal-wall-down techniques: Different surgical approaches chosen based on the extent of disease and the risk of recurrence.

Regular follow-up examinations after surgery are essential, as cholesteatomas can recur in up to 20% of cases. A planned second-look operation 6 to 12 months after the initial surgery is common practice to check the surgical site and reconstruct the hearing bones if needed.

Complications

Without treatment, a cholesteatoma can cause serious complications, including:

Complete deafness in the affected ear
Facial nerve palsy (paralysis of the facial nerve)
Labyrinthitis (inner ear infection causing severe dizziness)
Meningitis (inflammation of the membranes surrounding the brain)
Brain abscess
Mastoiditis (infection of the mastoid bone)

References

Deutsche Gesellschaft fuer Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e. V. (DGHNO-KHC): S2k-Leitlinie Cholesteatom des Kindes- und Erwachsenenalters. AWMF-Register Nr. 017-006 (2022).
Tos M. - A new pathogenesis of mesotympanic (congenital) cholesteatoma. In: Laryngoscope. 2000;110(11):1890-1897. PubMed PMID: 11081609.
Kuo C.L. - Etiopathogenesis of acquired cholesteatoma: prominent theories and recent advances in biomolecular research. In: Laryngoscope. 2015;125(1):234-240. PubMed PMID: 25290260.

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