Cystic cochleo-vestibular anomaly with dysplastic semicircular canals

Case contributed by Dr Mohammad A. ElBeialy


Left sensorineural hearing loss (SNHL) since birth

Patient Data

Age: 9 years
Gender: Female
  • abnormal configuration of the left cochlea and vestibule which appear rudimentary with loss of their internal architecture and appear cystic.
  • the semicircular canals appear dysplastic and of relatively small size compared to the contra-lateral side.
  • normal right inner ear structures with intact vestibulo-cochlear system.
  • no cerebello-pontine angle mass lesion.
  • well-aerated aero-tympanic cavities and mastoid air cells on either side.

Case Discussion

Diagnosis - Left cystic cochleo-vestibular anomaly (CCVA) with dysplastic semicircular canals.

The Differential diagnosis includes

  • common cavity - featureless common cavity with rudimentary cochlea, vestibule & semicircular canals
  • cochlear aplasia - absent cochlea. The vestibule may be normal or hypoplastic.
  • labyrinthine ossificans - acquired SNHL, usually post- meningitis. Dense bone fills membranous labyrinth including cochlea
  • large endolymphatic sac anomaly (LESA) - cascading bilateral SNHL in 1st years of life. Bilateral large endolymphatic duct & sac with mild cochlear dysrnorphism.
  • labyrinthine aplasia - absence of entire inner ear including cochlea, vestibule and semicircular canals (SCCs).
  • SCC dysplasia - spectrum of anomalies; the most common appearance is short, dilated lateral SCC and  vestibule forming single cavity 
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Case information

rID: 25086
Published: 3rd Oct 2013
Last edited: 17th Dec 2015
System: Head & Neck
Inclusion in quiz mode: Included

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