/semicircular canal dehiscence syndrome

semicircular canal dehiscence syndrome

Rare Disease

MONDO:0018484

Also Known As

Minor's syndromeMinorbs syndromeSCD syndromeSuperior Semicircular Canal Dehiscencecanal dehiscence syndromesuperior canal dehiscencesuperior canal syndromesuperior semicircular canal dehiscence syndromethird mobile window syndromesuperior semicircular canal dehiscence

Definition

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.