Question

Define congenital cholesteatoma. Discuss the investigations and management of congenital cholesteatoma.

Hint:

Surgical removal is the mainstay of treatment for congenital cholesteatoma, and timely intervention can prevent complications such as hearing loss and facial nerve damage.

Answer 1

Step 1: Definition of Congenital Cholesteatoma.
Congenital cholesteatoma is a rare, non-cancerous growth of skin cells that occurs in the middle ear, typically behind the tympanic membrane (eardrum). Unlike acquired cholesteatoma, which results from chronic ear infections, congenital cholesteatoma is present at birth, developing from epithelial cells trapped in the middle ear during fetal development. These cells may proliferate, forming a mass that can erode nearby structures such as the ossicles or the mastoid.

Step 2: Clinical Features of Congenital Cholesteatoma.
- Painless otorrhea: A common presenting symptom is a foul-smelling discharge from the ear, which is typically non-inflammatory.
- Hearing loss: Conductive hearing loss is often present due to the mass affecting the ossicular chain or the tympanic membrane.
- No history of chronic ear infections: Unlike acquired cholesteatoma, patients with congenital cholesteatoma usually do not have a history of recurrent ear infections.
- Presence of a white mass: On examination, a white, pearly mass may be visible behind the tympanic membrane during otoscopy, especially if the eardrum is perforated.

Step 3: Investigations for Congenital Cholesteatoma.
Several investigations are performed to confirm the diagnosis and assess the extent of the disease:
- Audiometry: To assess the degree of hearing loss, which is usually conductive in nature.
- CT scan of the temporal bone: This imaging modality is critical in evaluating the extent of the cholesteatoma, identifying erosion of the ossicles or mastoid, and assessing for any involvement of the inner ear or other surrounding structures.
- MRI: MRI with gadolinium contrast may be used to better delineate the cholesteatoma and to assess the involvement of the facial nerve or brain structures if there is suspicion of complications.
- Tympanometry: This test helps in evaluating the middle ear pressure and mobility of the eardrum, which may be reduced in cholesteatoma cases due to ossicular disruption.

Step 4: Management of Congenital Cholesteatoma.
The management of congenital cholesteatoma is primarily surgical, with the goal of removing the cholesteatoma and preventing further complications. The treatment options include:
- Surgical removal: The mainstay of treatment is the complete removal of the cholesteatoma. This may be done through a tympanoplasty, mastoidectomy, or a combined approach, depending on the location and extent of the cholesteatoma.
- Preservation of hearing: Efforts are made to preserve hearing by reconstructing the ossicles if they have been damaged. In some cases, ossicular chain reconstruction or the use of prostheses may be necessary.
- Follow-up care: Regular follow-up is required to monitor for recurrence of the cholesteatoma, as it can reoccur even after surgical removal. - Antibiotics: If there is evidence of secondary infection, antibiotics may be prescribed to treat any associated infection in the ear.

Step 5: Conclusion.
Congenital cholesteatoma is a rare but important condition that can lead to hearing loss and other complications if not treated promptly. Early diagnosis and surgical management are key to preventing further damage to the middle ear and preserving hearing. Regular monitoring after surgery is essential to ensure there is no recurrence.