semicircular canal dehiscence syndrome
MONDO:0018484
Semicircular canal dehiscence (SCD) syndrome is 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.
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Superior canal dehiscence syndrome
The semicircular canal dehiscence (SCD) is a category of rare neurotological diseases/disorders affecting the inner ears, comprising the superior, lateral and posterior semi-circular ducts. These SCDs induce SCD syndromes (SCDSs), which define specific sets of hearing and balance symptoms. This entry mainly deals with the superior SCDS. The superior semicircular canal dehiscence syndrome (SSCDS) is a set of hearing and balance symptoms induced by a rare disease/disorder of the inner ear's superior semicircular canal/duct. The symptoms are caused by a thinning or complete absence of the part of the temporal bone overlying the superior semicircular canal of the vestibular system. There is evidence that this rare defect, or susceptibility, is congenital. There are also numerous cases of symptoms arising after physical trauma to the head. It was first described in 1998 by Lloyd B. Minor of Johns Hopkins University in Baltimore.
Background only. Canonical identity remains MONDO; clinical-trial relationships come from ClinicalTrials.gov.Drugs being studied
Disease identity and terminology: MONDO. Trial relationships: ClinicalTrials.gov.