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Semicircular canal dehiscence syndrome
Disease definition
A rare otorhinolaryngologic disease characterized by the uni- or bilateral dehiscence of the bone(s) overlying the superior (most common), lateral or posterior semicircular canal(s). Patients present audiological (autophony, aural fullness, conductive hearing loss, pulsatile tinnitus) and/or vestibular symptoms (sound or pressure-evoked oscillopsia or vertigo, characteristic vertical-torsional eye movements), depending on which semicircular canal is affected. Posterior SCD syndrome is associated with high-riding jugular bulb and fibrous dysplasia, while lateral SCD syndrome is associated with chronic otitis media and cholesteatoma, with or without audiological and vestibular symptoms.
ORPHA:420402
Classification level: Disorder- Synonym(s):
- SCD syndrome
- Prevalence: Unknown
- Inheritance: Not applicable
- Age of onset: All ages
- ICD-10: H83.8
- OMIM: -
- UMLS: -
- MeSH: -
- GARD: -
- MedDRA: -
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