A 45-Year-Old Woman With Unexplained Hearing Loss

A 45-year-old woman presents complaining of difficulty hearing out of her right ear. She reports that her hearing loss is worsening, and in the last couple of weeks she has also had a fairly constant headache and feels dizzy. When you examine her right ear, you note the following appearance:

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1. What is the most likely diagnosis?

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This patient has a cholesteatoma, which is a collection of epidermal and connective tissues that is located within the middle ear. Without treatment it continues to grow and is likely to be locally invasive. It can cause destruction of the bones of the middle ear. It is usually unilateral and remains one of the most common indications for otological surgery.

Cholesteatoma may be congenital, primary acquired (thought to be related to chronic middle ear infections) or secondary acquired (due to tympanic membrane damage).

Small lesions often present with a progressive conductive hearing loss. With local invasion of structures, symptoms such as headache, vertigo and facial nerve palsy may be present. The peripheral edges of the lesion can release osteolytic enzymes and the activity of these enzymes appears to be enhanced when infection is present.

Central nervous system infection can rarely occur due to the proximity of the brain, and this can manifest as meningitis, sigmoid sinus thrombosis or an epidural abscess.

2. What would be the gold standard investigation of choice?

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CT imaging is the gold standard investigation of choice. This allows the extent of the lesion to be assessed and helps the surgeon to plan the nature of the procedure required.

3. How could this patient be managed?

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Surgical intervention remains the mainstay of treatment, with the aim being to remove the lesion. Patients that are medically unfit for a general anaesthetic or who refuse treatment can opt for conservative management. This involves regular ear cleaning and prompt treatment of ear infections. Without intervention, a cholesteatoma continues to grow. Complications will depend on which structures it invades, as outlined above.

4. What are the possible complications of surgical intervention?

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Possible complications of surgical intervention include:

  • Alteration of taste (this is usually temporary and resolves after a few months)
  • Facial nerve injury
  • Hearing loss
  • Chondritis/ perichondritis
  • Persisting dizziness
  • Stenosis of the ear canal