List II - Less Common 'Know of' Conditions

Question 1

What is a cholesteatoma?

Answer 1

• Abnormal sac of keratinising squamous epithelium and accumulation of keratin within the middle ear or mastoid air cell spaces which can become infected and also erode neighbouring structures

Question 2

How does cholesteatoma present?

Answer 2

• May be asymptomatic in its early stages
• Most commonly presents with a persistent or recurrent discharge from the ear that is often foul smelling - associated conductive hearing loss may also occur
• Rarely with progression of disease, vertigo, sensorineural hearing loss, facial nerve palsy, meningitis or intracranial abscess may develop

Question 3

How is cholesteatoma diagnosed?

Answer 3

• Diagnosis requires clear visualisation of the tympanic membrane
• If the tympanic membrane can be seen, a cholesteatoma should be suspected if there is:
• Evidence of ear discharge
• Presence of a deep retraction pocket with or without granulation tissue and skin debris
• Crust or keratin in the upper part of the tympanic membrane may be perforated
• Congenital cholesteatoma (rare) may appear as a white mass behind an intact tympanic membrane, in a person with no prior history of ear discharge, tympanic membrane perforation or surgical procedures on the ear
• If there is significant discharge occluding the tympanic membrane, referral for examination with an otomicroscope and micro-suctioning of the ear may be appropriate

Question 4

Who should be arranged for emergency admission with suspected cholesteatoma?

Answer 4

• People with cholesteatoma associated with a serious complication, including:
• Facial nerve palsy or vertigo
• Other neurological symptoms (including pain) or signs that could be associated with the development of an intra-cranial abscess or meningitis
• Semi - urgent referral to ENT should be arranged for people with a suspected cholesteatoma who do not have a serious complication

Question 5

What is a vestibular schwannoma (acoustic neuroma)?

Answer 5

• Account for 5% of intracranial tumours and 90% of cerellopontine angle tumours

Question 6

What is the classical history of a patient with a vestibular schwannoma (acoustic neuroma)?

Answer 6

• Classical history includes a combination of vertigo, hearing loss, tinnitus and an absent corneal reflex
• Features can be predicted by the affected cranial nerves:
• CNVIII - vertigo, unilateral sensorineural hearing loss, unilateral tinnitus
• CNV - absent corneal reflex
• CNVII - facial palsy

Question 7

In which condition could you see bilateral vestibular schwannomas (acoustic neuroma)?

Answer 7

• Neurofibromatosis type 2

Question 8

How should patients with a suspected vestibular schwannonma be managed?

Answer 8

• They should be referred urgently to ENT

* These tumour are often slow growing, benign and often are observed initially

Question 9

What is the investigation of choice for a vestibular schwannoma (acoustic neuroma)?

Answer 9

• MRI of the cerebellopontine angle is the investigation of choice
• Audiometry is also important as only 5% will have a normal audiogram
• Management is either with surgery, radiotherapy or observation

Question 10

An absent corneal reflex is associated with which nerve palsy?

Answer 10

• CNV

Question 11

A facial palsy is associated with which nerve palsy?

Answer 11

• CNVII