Cystic cochleo-vestibular anomaly with dysplastic semicircular canals
Diagnosis almost certain
Presentation
Left sensorineural hearing loss (SNHL) since birth
Patient Data
Age: 9 years
Gender: Female
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- 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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