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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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Frequently Asked Questions

What is the full form of SSCD?

SSCD stands for Superior Semicircular Canal Dehiscence. It is a condition where a thin or absent section of bone over the superior semicircular canal creates an abnormal third window in the inner ear. This opening can disrupt pressure balance, causing sound- or pressure-induced vertigo, oscillating vision, and hearing sensitivity to internal body sounds.

In the context of the National Clinical Classification (NCC), SSCD still expands to Superior Semicircular Canal Dehiscence. The abbreviation and meaning do not change under NCC terminology. It remains a recognized vestibular disorder involving a bony defect that alters inner ear fluid mechanics and pressure transmission.

Within NCC terminology, SD SW expands to Semicircular Dehiscence, Superior Canal. This is a classification label used to describe a bony defect at the superior semicircular canal site. It aligns with clinical naming conventions but is formatted to meet NCC’s structured coding hierarchy for inner ear and vestibular pathologies.

SSCD refers to a bony opening over the superior semicircular canal in the inner ear. This defect creates abnormal pressure sensitivity, allowing sound or physical strain to stimulate balance signals inappropriately. People may notice dizziness from loud noise, ear fullness, echoing, pulsatile sensations, or hearing internal sounds like eye or joint movement.

Yes, SSCD can be serious if symptoms interfere with daily life or go undiagnosed. It may lead to chronic imbalance, noise-triggered vertigo, reduced quality of life, and hearing distortion. While not always dangerous, persistent pressure sensitivity warrants specialist evaluation to prevent symptom progression and support safe, effective management.

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