Case #9

Case #7

• External ears:
  • Tiny preauricular pits
  • Soft tissue in the bony segment of the left external auditory canal
  • The tympanic membranes are normal in appearance
• Middle ears/mastoids:
  • Dysmorphic appearance of the bilateral malleus and incus with widening of the malleoincudal articulation laterally
  • No normal stapes is visualized on either side with possible dysmorphic stapes elements adjacent to a stenotic-appearing oval window on the right
  • The tympanic segment of the facial nerve is uncovered and partially overlies the oval window bilaterally
  • The middle ear cavity is well-aerated and formed bilaterally
  • Bilateral enlarged Eustachian tubes
• Inner ears:
  • Unwound appearance of the cochlea with anteromedial displacement of the hypoplastic middle and apical turns
  • The apical portion of the modiolus appears hypoplastic bilaterally
  • Dysmorphic, incompletely formed, bulbous appearing bilateral posterior semicircular canals with possible dehiscence of the overlying jugular plate on the right
  • Asymmetric narrowing of the right lateral semicircular canal
  • Bony uncovering at the apex of the right superior semicircular canal
  • Enlarged left vestibular aqueduct

Images

Branchio-oto-renal syndrome (BOR)

Background

Imaging Findings