FRCS (ORL-HNS) · SECTION 1 SBA

Endolymphatic sac tumour

A patient with von Hippel–Lindau disease develops unilateral tinnitus, progressive sensorineural hearing loss and episodic vertigo. CT shows destructive retrolabyrinthine temporal-bone change and MRI shows a strongly enhancing lesion. What is the most likely diagnosis?

CHOOSE ONE ANSWER

Best of five

  1. A. Otosclerosis
  2. B. Endolymphatic sac tumour
  3. C. Cholesteatoma
  4. D. Vestibular neuritis
  5. E. Glomus tympanicum
ANSWER AND REASONING

B. Endolymphatic sac tumour

These hypervascular locally destructive tumours have a strong association with von Hippel–Lindau disease and can cause early irreversible hearing loss despite small size.

Why every option is right or wrong

A. Otosclerosis: Otosclerosis causes fenestral or cochlear sclerosis rather than an enhancing destructive posterior petrous lesion.

B. Endolymphatic sac tumour: These hypervascular locally destructive tumours have a strong association with von Hippel–Lindau disease and can cause early irreversible hearing loss despite small size.

C. Cholesteatoma: Cholesteatoma restricts diffusion but is not typically intensely enhancing or linked to VHL.

D. Vestibular neuritis: This does not produce a destructive mass or progressive hearing loss.

E. Glomus tympanicum: This arises on the cochlear promontory and presents as a retrotympanic vascular mass.

What if the scenario changed?

If bilateral lesions were present, careful assessment for a germline VHL syndrome and associated tumours would be especially important.

EDUCATIONAL USE

Independent Clinora educational preparation material; not official JCIE content, an accredited programme, clinical advice, or a substitute for current guidance, local policy and specialist judgement.

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