Rare and Fascinating Hearing Disorders

Rare and Fascinating Hearing Disorders

This week on the Sound Advice blog, we highlight a different rare hearing condition each day.

Superior Semicircular Canal Dehiscence (SSCD)

SSCD is a rare medical condition of the inner ear which leads to hearing and balance disorders in those affected. The symptoms are caused by a thinning or complete absence of part of the temporal bone overlying the superior semicircular canal of the vestibular system. This could result from the slow erosion of the bone, or physical trauma to the skull.

SSCD can affect both hearing and balance to different extents in different people.

Symptoms of SSCD include:

  • Autophony: person’s own speech or other self-generated noises (e.g. heartbeat, eye movements, creaking joints, chewing) are heard unusually loudly in the affected ear
  • dizziness/vertigo
  • tullio phenomenon – sound-induced vertigo, disequilibrium or dizziness
  • hyperacusis: the over-sensitivity to sound
  • Low-frequency conductive hearing loss
  • A feeling of fullness in the affected ear
  • Pulsatile tinnitus
  • “brain fog”
  • Fatigue
  • headache/migraine