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