Worst outcomes if missed
Temporal bone complications
- deafness – both conductive and sensorineural hearing loss can occur
- vertigo – due to erosion of the bone over the lateral semicircular canal
- facial nerve paralysis
Cholesteatoma is a slowly progressive disease. Deafness due to a conductive hearing loss is common. The other complications occur rarely.
- intracranial abscess
- lateral sinus thrombosis
Intracranial complications are rare compared to temporal bone complications but have the potential to be fatal.
The true incidence of cholesteatoma is not known but it is thought to be in the region of 10-70 per 100,000 per year. A study from northern Europe estimated the incidence at 9.2 per 100,000 per year1. The most common cause of otorrhoea in children is acute otitis media and in adults it is due to otitis externa. The overall number of patients presenting with cholesteatoma is relatively small in comparison, with a ratio of new cases being about one child to four adults. However, due to the necessity of surgery and prolonged aftercare, these patients account for a significant proportion of an ENT department’s time.
Symptoms and signs
Cholesteatoma typically presents with a chronic intermittent or constant otorrhoea affecting one ear, which is usually not painful. Often the patient will also notice that the hearing has been poor for some time. Treatment of the ear may lead to resolution of the otorrhoea but the patient will frequently complain of recurrent ear discharge, which is usually smelly, over months or years.
Five key questions
- When did the ear discharge first begin? Cholesteatoma is usually painless.
- Is the ear infection unresponsive or refractory to treatment? Cholesteatoma usually causes chronic intermittent or constant otorrhoea which is non-responsive to appropriate treatment.
- Is the ear painful? Cholesteatoma is usually painless in comparison to otitis externa.
- Is the hearing normal? After treatment of acute otitis media or otitis externa the hearing loss usually returns to normal, whereas cholesteatoma often causes a persistent hearing loss which is usually conductive.
- Has there been any previous surgery on the ear? Previous surgery may cause implantation of squamous epithelium causing a “secondary” cholesteatoma behind an intact ear drum.
- Otitis media in children – most common cause of otorrhoea in this age group. Treat appropriately with topical and systemic antibiotics if necessary and review.
- Otitis externa in adults – treat appropriately with topical and systemic antibiotics if necessary and review.
- Tympanic membrane perforations – if associated with symptoms and signs mentioned above then refer to the ENT department. Cholesteatoma can be associated with perforations.
- Scarring of tympanic membrane or after ear surgery especially when cartilage has been used to reconstruct the eardrum can make it difficult to exclude cholesteatoma. Cholesteatoma can occur following surgery on the ear for other reasons.
Five red herrings
1. A good response to medical treatment does not exclude cholesteatoma
2. Recurrent, painless otorrhoea can occur with tympanic membrane perforations
3. Scarring and altered anatomy from previous surgery may mimic cholesteatoma
4. Cholesteatoma can occur behind an intact tympanic membrane
5. Wax over the eardrum can obscure a view of cholesteatoma
Examine the ear. If it is infected a good view of the tympanic membrane may not be possible. Wax in the ear canal can also make it difficult to see the eardrum. Treat the ear with topical antibiotic eardrops, with or without systemic antibiotics, as it may initially be difficult to determine whether this due to an otitis media or externa, and re-examine the ear in a few weeks. If you cannot see the tympanic membrane then referral to an ENT department should be performed. Should any of the complications of ear disease be apparent apart from a conductive hearing loss, the patient should be referred and seen by the ENT department on the same day. A conductive hearing loss is a complication of cholesteatoma, but in itself does not warrant referral on the same day to an ENT department.
At the ENT department, microsuction of the ear can be performed to allow a better view of the eardrum and aid treatment. Audiometry will document any hearing loss. Dependant on the clinical situation, a high resolution CT scan of the temporal bones will aid diagnosis and may reveal impending complications.
It is also important to recognise that cholesteatoma can occur behind an intact eardrum. This can sometimes be apparent as a white mass behind an intact ear drum causing bulging of the tympanic membrane.
Mr Shaun Davey is an ENT specialist registrar and Mr Andrew Bath is an ENT consultant at Norfolk and Norwich University Hospital.
1 Kemppainen HO, Puhakka HJ, Laippala PJ et al. Epidemiology and aetiology of middle-ear cholesteatoma. Acta Otolaryngologica, 1999; 119 (5): 568-572