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Sub-Category:

Middle and Inner Ear Malformations

Number of Conditions: 1

Congenital Cholesteatoma

Specialty: Ear

Category: Congenital and Developmental Ear Disorders

Sub-category: Middle and Inner Ear Malformations

Symptoms:
hearing loss; recurrent ear infections; persistent drainage from the ear; balance issues in some cases; facial nerve weakness (rare)

Root Cause:
Abnormal collection of keratinizing squamous epithelium within the middle ear present from birth, typically due to incomplete resorption of embryonic cells.

How it's Diagnosed: videos
Clinical examination, otoscopy to visualize the mass, imaging studies such as CT or MRI to assess extent and complications.

Treatment:
Surgical removal of the cholesteatoma to prevent complications, followed by regular follow-ups.

Medications:
Antibiotic ear drops (e.g., ciprofloxacin ) may be prescribed to manage infections. Pain relief medications like acetaminophen or ibuprofen may be used post-surgery.

Prevalence: How common the health condition is within a specific population.
Rare, accounting for 2–5% of all cholesteatomas.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
No specific risk factors identified; occurs sporadically without familial or environmental influence.

Prognosis: The expected outcome or course of the condition over time.
Good with early diagnosis and surgical treatment; untreated cases may lead to hearing loss, infection, or intracranial complications.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Hearing loss, tympanic membrane perforation, facial nerve paralysis, labyrinthitis, intracranial abscess, or meningitis.