Semicircular canal dehiscence syndrome
Synonyms: SCD syndrome
- Type
- Clinical syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Semicircular canal dehiscence syndrome | SCD syndrome |
| fr | Syndrome de déhiscence du canal semi-circulaire | |
| de | Bogengangsdehiszenz-Syndrom | SCD-Syndrom |
| es | Síndrome de dehiscencia del canal semicircular | Síndrome DCS |
| it | Sindrome da deiscenza del canale semicircolare | Sindrome SCD |
| nl | Dehiscentie van canalis semicircularis-syndroom | 'Semicircular canal deshiscence'-syndroom · SCD-syndroom |
| pt | Síndrome de deiscência do canal semicircular | Síndrome SCD |
| pl | Zespół przetoki kanału półkolistego | Zespół SCD · Zespół rozejścia się kanału półkolistego |
| cs | Syndrom dehiscence semicirkulárního kanálku | = en |
| uk | Синдром дегісценції півколового каналу | Синдром SCD |
| zh | 半规管裂隙综合征 | SCD综合征 |
| tr | Semisirküler kanal dehissansı sendromu | SCD sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | H83.8 | NTBT | attributed | validated |
| ICD-11 | AB31.3 | BTNT | attributed | validated |
| MONDO | 0018484 | E | validated | |
| MeSH | D000084322 | E | validated | |
| UMLS | C4708600 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:98036Rare otorhinolaryngologic disease
rare otorhinolaryngological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/420402
- This record as JSON: /data/d/420402.json