Disorders · Introduction

Superior semicircular canal dehiscence

A pinhole of missing bone over the superior canal turns the inner ear into a leaky instrument — the patient grows dizzy at loud sounds and hears their own eyes move. A third window, hiding in plain sight.

What SSCD is

Trainee

SSCD is the prototypical third-window syndrome: a dehiscence of the bone over the superior semicircular canal lets acoustic and pressure energy be shunted abnormally, producing sound- and pressure-induced vertigo (Tullio and Hennebert), autophony and bone-conduction hyperacusis, with a low-frequency air–bone gap.1

By the numbers

A dehiscence is found in roughly 0.5% of temporal bones, with critically thin bone in more — a developmental substrate that usually needs a second hit (trauma or raised pressure) to become symptomatic.2

~0.5%of temporal bones show a dehiscence
~1.4%show critically thin (near-dehiscent) bone
40s–50stypical age at presentation
1998year Minor first described the syndrome

Temporal-bone survey

  • Normal bone98.1%
  • Critically thin1.4%
  • Dehiscent0.5%

A dehiscence is found in ~1 in 200 temporal bones; the symptomatic syndrome is rarer still — which is why a scan alone cannot make the diagnosis.

How this chapter is organised

Key points

  • A bony defect over the superior canal creates a third window into the inner ear.
  • Sound/pressure-induced vertigo (Tullio, Hennebert), autophony and bone-conduction hyperacusis are the hallmarks.
  • It causes a low-frequency air–bone gap with PRESERVED reflexes and supranormal bone conduction — not otosclerosis.
  • VEMP shows low cervical thresholds / high ocular amplitudes; CT (Pöschl/Stenvers) confirms the defect.
  • Diagnosis needs concordant symptoms, physiology and imaging — never a scan alone.