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Superior Semicircular Canal Dehiscence (SSCD)

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About the Disease

Superior Semicircular Canal Dehiscence (SSCD) is a rare inner ear condition that leads to vestibular and auditory symptoms. It occurs due to the absence or thinning of the bony part of the labyrinth that overlies the superior semicircular canal.

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Symptoms

  1.  Vertigo and Oscillopsia: Triggered by loud noises and activities that alter intracranial or middle ear pressure, such as straining, sneezing, or coughing.
  2.  Autophony: Amplification of one’s own voice.
  3.  Hypersensitivity to Noises: Increased sensitivity to sound.
  4.  Conductive Hearing Loss: Revealed during audiometry.
  5.  Oscillopsia: Swaying sensation of the eyes or horizon, particularly on an upward plane.

Additional Symptoms

  •  Hearing one’s eyes move
  •  Distorted sensation of sound in the affected ear during activities like running
  •  Bone-conducted sounds amplify the effects of dehiscence
  •  Air-transmitted sounds to the cochlea are reduced by dehiscence
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Diagnosis

  •  Videonystagmography:
    • Vestibular evaluation, which may show nystagmus on VNG during Valsalva maneuver and vibration testing. 
  • Audiometry
    • Conductive hearing loss in audiometry.
  • Vestibular Evoked Myogenic Potential (VEMP):
    •  Loud noises provoke short-latency relaxation potential in the ipsilateral sternocleidomastoid muscle.
    •  The VEMP response will be below the normal threshold, and the amplitude of the VEMP waveform will be larger for equal stimuli intensities in the affected ear.
  • CT Imaging:
    • High-resolution CT scan of temporal bones can reveal the breach in the bone covering the superior canal. Careful analysis is required to avoid missing the thin layer of uninjured bone.
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Treatment

Surgical Correction:

  • Middle Cranial Fossa Approach: This approach involves closing the superior canal with soft fibrous tissue and bone patty, holding the fascia in place. It provides long-term relief from both vestibular and auditory symptoms. Although the functionality of the operated canal may diminish, the other semicircular canals function normally, causing little effect on the overall balance of the patient.

Managing SSCD:

  •  Trigger Avoidance: Some patients find relief by avoiding triggers such as loud noises.
  •  Surgery: For severe symptoms like constant disequilibrium, autophony, extreme sound intolerance, and pulsatile oscillopsia, surgery may be necessary.

By addressing the root cause of SSCD, patients can significantly improve their quality of life and alleviate the distressing symptoms associated with this condition.

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