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Endolymphatic Sac Tumour

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  • 45-year-old patient presented with sensorineural hearing loss, tinnitus and dizziness for 2 months.
  • MRI showed enhancement within the vesitubular aqueduct, vestibule and proximal semicircular canals.
  • CT showed widening of the vestibular aqueduct with a moth-eaten margin (red arrows).

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  • 30-year-old patient with a prior left cerebellar haemangioblastoma developed a lesion in the right petrous bone.
  • The lesion centred in the right vestibular aqueduct, which was T2-hyperintense and enhanced after gadolinium, was compatible with an endolymphatic sac tumour.
  • The patient was diagnosed with von Hippel Lindau.

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  • A 20-year-old patient presented with a worsening left facial nerve palsy.
  • CT showed an expansile left petrous bone lesion eroding the facial canal (and lateral semicircular canal).
  • MRI showed a T1- and T2-hyperintense lesions with central enhancement without diffusion restriction.
  • Following resection, an ELST was diagnosed. There were no other retinal or abdominal manifestations of vHL.