QuestionSBA
QuestionChoice
This patient demonstrates conductive hearing loss on the right-hand side on Rinne’s and Weber’s testing. Combined with her history of foul-smelling discharge, this makes cholesteatoma (an abnormal collection of skin cells within the ear) the most likely of these options. All of the other options are causes of sensorineural hearing loss rather than conductive hearing loss.
10017166
a
Cholesteatoma
QuestionChoice
This would cause a sensorineural hearing loss and would present with clusters of unilateral hearing loss, vertigo and tinnitus.
10017170
e
Meniere’s disease
QuestionChoice
This would cause a sensorineural hearing loss and may be expected to be seen in an older patient.
10017168
c
Presbycusis
QuestionChoice
There is no mention of ototoxic drugs (e.g. aminoglycosides, loop diuretics) in the history and this would also cause a sensorineural hearing loss.
10017169
d
Ototoxicity from medication
QuestionChoice
This would cause a sensorineural hearing loss, usually associated with unilateral tinnitus and vertigo.
10017167
b
Acoustic neuroma
Concept
Chapter
# Summary
Cholesteatoma, despite its name, is neither a tumor nor related to cholesterol. It is a severe but rare complication of chronic otitis media, common in patients aged 5-15 years old. Key signs and symptoms include persistent foul-smelling discharge, headache, and otalgia. Critical investigations include examination of the tympanic membrane, which often reveals a white area in the attic behind the membrane. Management strategies focus on preventing complications, such as facial nerve palsy and central nervous system (CNS) complications, through surgical intervention.

# Definition
Cholesteatoma is a misnomer, as it is neither a tumor nor has relations to cholesterol. It is an abnormal accumulation of skin and squamous epithelium within the middle ear cleft and mastoid air cells.

# Epidemiology
Cholesteatoma is a rare condition, commonly occurring in younger patients, particularly those aged 5-15 years old. It is a severe complication of chronic otitis media.

# Aetiology
The primary cause of cholesteatoma is the abnormal accumulation of squamous epithelium and skin cells in the middle ear cleft and mastoid air cells. This unusual build-up often results from chronic otitis media.

# Signs and Symptoms
Key signs and symptoms of cholesteatoma include:
- Persistent foul-smelling discharge
- Headaches
- Otalgia

Upon examination of the tympanic membrane, a white area is often visible in the attic behind the membrane.

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# Differential Diagnosis
Differential diagnoses for cholesteatoma include:
- Chronic otitis media: Presents with recurrent ear infections, ear discharge, hearing loss, and ear pain.
- Otosclerosis: Symptoms include progressive hearing loss, tinnitus, and vertigo.
- Squamous cell carcinoma: Presents with persistent ear pain, hearing loss, and sometimes facial nerve paralysis.

# Investigations
Critical investigation for cholesteatoma includes an examination of the tympanic membrane. This examination often reveals a white area in the attic behind the tympanic membrane. More advanced investigations may include CT scans and MRIs to assess the extent of the disease.

# Management
The primary management strategy for cholesteatoma is surgical intervention to remove the abnormal skin and squamous epithelium accumulation. This approach helps prevent severe complications such as facial nerve palsy and CNS complications (including meningitis, epidural abscess, and sigmoid sinus thrombosis).

# References
[Click here for NICE CKS on cholesteatoma](https://cks.nice.org.uk/topics/cholesteatoma/)
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Picture
An example appearance of a cholesteatoma.
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Cholesteatoma.jpeg
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Cholesteatoma
Topic
24
Ear, Nose & Throat
15977
Cholesteatoma presents with conductive hearing loss and foul-smelling ear discharge due to abnormal skin cell accumulation in the middle ear.
A 47-year-old female has foul smelling discharge from her right ear with associated hearing loss. When assessing her hearing with a tuning fork, she hears the sound louder on the right, with the tuning fork placed in the middle of the forehead. On the right, the sound from the tuning fork is heard best when placed on the mastoid process, whilst it is heard best next to the external auditory meatus on the left. She denies any tinnitus or vertigo.

What is the most likely diagnosis?
a