Bing Siebenmann dysplasia

  • Extremely rare condition characterised by total dysplasia of membranous labyrinth ( a type of vestibulocochlear dysplasia )
  • The bony labyrinth is developed normally
    • hence this condition cannot be diagnosed radiographically and not included in Jackler et al classification
  • Some suggest that it may be a severe variant of the more common Sheibe’s dysplasia
  • The patient may present with hearing loss or vestibular symptoms like vertigo and dizziness

Alternate names:

  • Complete membranous labyrinthine dysplasia

Associated disorders:

  • Jervell and Lange-Nielsen syndrome
  • Oculo auriculo  vertebral spectrum (OAVS)
  • Usher syndrome

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OAVS – Acronym

OAVS stands for: Oculo Auriculo Vertebral Spectrum (a type of craniofacial anomaly)