MCID: SPR126
MIFTS: 36

Superior Semicircular Canal Dehiscence

Categories: Bone diseases, Ear diseases, Rare diseases

Aliases & Classifications for Superior Semicircular Canal Dehiscence

MalaCards integrated aliases for Superior Semicircular Canal Dehiscence:

Name: Superior Semicircular Canal Dehiscence 12 15
Superior Semicircular Canal Dehiscence Syndrome 12 54
Superior Canal Dehiscence Syndrome 54
Third Mobile Window Syndrome 12
Canal Dehiscence Syndrome 12
Superior Canal Dehiscence 12
Superior Canal Syndrome 12
Anemia, Sickle Cell 74
Scds 54

Classifications:



External Ids:

Disease Ontology 12 DOID:0080193
UMLS 74 C0002895

Summaries for Superior Semicircular Canal Dehiscence

NIH Rare Diseases : 54 Superior semicircular canal dehiscence syndrome is a rare balance disorder characterized by auditory and/or vestibular symptoms. These might include dizziness and vertigo triggered by heavy lifting, straining, coughing or loud sounds that change the middle ear or intracranial pressure, fullness in the ear, autophony (an echo or reverberation in the ear when speaking, chewing or swallowing), hearing loss, nystagmus, or oscillopsia (the apparent motion of objects that are stationary). This condition is caused by an opening (dehiscence) in the bone that overlays the superior (uppermost) semicircular canal within the inner ear. While many patients with superior semicircular canal dehiscence syndrome are able to tolerate their symptoms and reduce or avoid triggering stimuli, others can benefit from surgical repair of the dehiscence.

MalaCards based summary : Superior Semicircular Canal Dehiscence, also known as superior semicircular canal dehiscence syndrome, is related to sickle cell disease and schnyder corneal dystrophy, and has symptoms including angina pectoris, abdominal pain and edema. An important gene associated with Superior Semicircular Canal Dehiscence is COCH (Cochlin). Affiliated tissues include bone, prostate and heart.

Disease Ontology : 12 An inner ear disease characterized by dehiscence in the bone overlying the superior semicircular canal experience with symptoms of pressure or sound-induced vertigo, bone conduction hyperacusis, and pulsatile tinnitus.

Wikipedia : 77 Superior semicircular canal dehiscence syndrome is a set of hearing and balance symptoms, related to a... more...

Related Diseases for Superior Semicircular Canal Dehiscence

Diseases related to Superior Semicircular Canal Dehiscence via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 85)
# Related Disease Score Top Affiliating Genes
1 sickle cell disease 12.3
2 schnyder corneal dystrophy 11.7
3 ehlers-danlos syndrome, spondylodysplastic type, 3 11.7
4 cardiac conduction defect 11.5
5 sickle cell anemia 11.3
6 qt interval, variation in 11.3
7 carnitine deficiency, systemic primary 11.2
8 hemoglobinopathy 11.1
9 subacute cerebellar degeneration 11.1
10 semicircular canal dehiscence syndrome 11.0
11 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 10.8
12 otosclerosis 10.7
13 pathologic nystagmus 10.6
14 rere-related disorders 10.4
15 ehlers-danlos syndrome 10.4
16 encephalocele 10.4
17 meningoencephalocele 10.4
18 vertigo, benign recurrent 10.3
19 benign paroxysmal positional nystagmus 10.3
20 hypertrophic cardiomyopathy 10.3
21 osteoporosis 10.2
22 aging 10.2
23 deafness, autosomal recessive 4, with enlarged vestibular aqueduct 10.2
24 deafness, autosomal dominant 9 10.2
25 bone fracture 10.2
26 sleep apnea 10.2
27 sensorineural hearing loss 10.2
28 acoustic neuroma 10.2
29 neurilemmoma 10.2
30 meningitis 10.2
31 hyperacusis 10.2
32 renal oncocytoma 10.2
33 otitis media 10.0
34 fibrous dysplasia 10.0
35 meningocele 10.0
36 beta-thalassemia 10.0
37 spinal stenosis 10.0
38 thalassemia 10.0
39 disease of mental health 10.0
40 infertility 10.0
41 spinocerebellar degeneration 10.0
42 acute insulin response 10.0
43 body mass index quantitative trait locus 1 10.0
44 meningioma, familial 10.0
45 human venous malformation 10.0
46 cholesteatoma of middle ear 10.0
47 spinal meningioma 10.0
48 patulous eustachian tube 10.0
49 secretory meningioma 10.0
50 peripheral vertigo 10.0 COCH RTEL1

Comorbidity relations with Superior Semicircular Canal Dehiscence via Phenotypic Disease Network (PDN):


Acute Cystitis Deficiency Anemia
Heart Disease

Graphical network of the top 20 diseases related to Superior Semicircular Canal Dehiscence:



Diseases related to Superior Semicircular Canal Dehiscence

Symptoms & Phenotypes for Superior Semicircular Canal Dehiscence

UMLS symptoms related to Superior Semicircular Canal Dehiscence:


angina pectoris, abdominal pain, edema, chest pain

Drugs & Therapeutics for Superior Semicircular Canal Dehiscence

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Vestibular Function Outcome After Cartilage Cap Occlusion Surgery Withdrawn NCT01727544 Early Phase 1

Search NIH Clinical Center for Superior Semicircular Canal Dehiscence

Inferred drug relations via UMLS 74 / NDF-RT 52 :


Genetic Tests for Superior Semicircular Canal Dehiscence

Anatomical Context for Superior Semicircular Canal Dehiscence

MalaCards organs/tissues related to Superior Semicircular Canal Dehiscence:

42
Bone, Prostate, Heart, Testes, Eye

Publications for Superior Semicircular Canal Dehiscence

Articles related to Superior Semicircular Canal Dehiscence:

(show top 50) (show all 177)
# Title Authors Year
1
Bilateral Superior Semicircular Canal Dehiscence Associated with Ehlers-Danlos Syndrome: A Report of 2 Cases. ( 30385359 )
2019
2
The Prevalence of Superior Semicircular Canal Dehiscence in Patients With Mastoid Encephalocele or Cerebrospinal Fluid Otorrhea. ( 30870362 )
2019
3
Prevalence of Superior Semicircular Canal Dehiscence on High-Resolution CT Imaging in Patients without Vestibular or Auditory Abnormalities. ( 30819770 )
2019
4
Clinical Assessment of Patients with Bilateral Superior Semicircular Canal Dehiscence. ( 30928582 )
2019
5
Skull vibration induced nystagmus in patients with superior semicircular canal dehiscence. ( 31029487 )
2019
6
Age and gender considerations on the symptomology in patients with superior semicircular canal dehiscence: A systematic review and case illustration. ( 31078378 )
2019
7
Superior semicircular canal dehiscence with concomitant otosclerosis-A literature review and case discussion. ( 30564330 )
2018
8
RESPONSE TO "MICHAEL YONG, ERICA ZAIA, BRIAN WESTERBERG, AND JANE LEA. DIAGNOSIS OF SUPERIOR SEMICIRCULAR CANAL DEHISCENCE IN THE PRESENCE OF CONCOMITANT OTOSCLEROSIS". OTOL NEUROTOL 2017;38: 1071-1075. ( 29494468 )
2018
9
Aggregating the symptoms of superior semicircular canal dehiscence syndrome. ( 29280497 )
2018
10
Audiometric and cVEMP Thresholds Show Little Correlation With Symptoms in Superior Semicircular Canal Dehiscence Syndrome. ( 30124614 )
2018
11
A Cohort Study of Hearing Outcomes Between Middle Fossa Craniotomy and Transmastoid Approach for Surgical Repair of Superior Semicircular Canal Dehiscence Syndrome. ( 30444849 )
2018
12
Flat Panel Computed Tomography in the Diagnosis of Superior Semicircular Canal Dehiscence Syndrome. ( 30570605 )
2018
13
Round window plugging in the treatment of superior semicircular canal dehiscence. ( 28990655 )
2018
14
Middle cranial fossa approach for the repair of superior semicircular canal dehiscence is associated with greater symptom resolution compared to transmastoid approach. ( 29022108 )
2018
15
Hearing eyeball and/or eyelid movements on the side of a unilateral superior semicircular canal dehiscence. ( 29116385 )
2018
16
Superior semicircular canal dehiscence: Diagnosis and management. ( 29224712 )
2018
17
The influence of the subarcuate artery in the superior semicircular canal dehiscence and its frequency on stillbirths: illustrative cases and systematic review. ( 29272988 )
2018
18
The Efficacy of Magnetic Resonance Imaging for the Diagnosis of Superior Semicircular Canal Dehiscence. ( 29283100 )
2018
19
Bone Metabolic Markers in the Clinical Assessment of Patients with Superior Semicircular Canal Dehiscence. ( 29452318 )
2018
20
Clinical Implications of the Association Between Temporal Bone Tegmen Defects and Superior Semicircular Canal Dehiscence. ( 29697584 )
2018
21
Minimally invasive surgery for superior semicircular canal dehiscence: Results of a four fenestration technique in twenty-one adults. ( 29741293 )
2018
22
Mechanisms of Hearing Loss in a Guinea Pig Model of Superior Semicircular Canal Dehiscence. ( 29853836 )
2018
23
Incidence of intraoperative hearing loss during middle cranial fossa approach for repair of superior semicircular canal dehiscence. ( 29908720 )
2018
24
Reducing Radiation Dose for High-resolution Flat-panel CT Imaging of Superior Semicircular Canal Dehiscence. ( 30113564 )
2018
25
Stenting the Superior Petrosal Sinus in a Patient With Symptomatic Superior Semicircular Canal Dehiscence. ( 30177909 )
2018
26
Can Wideband Tympanometry Be Used as a Screening Test for Superior Semicircular Canal Dehiscence? ( 30458602 )
2018
27
The Gopen-Yang Superior Semicircular Canal Dehiscence Questionnaire: development and validation of a clinical questionnaire to assess subjective symptoms in patients undergoing surgical repair of superior semicircular canal dehiscence. ( 30674366 )
2018
28
Superior Semicircular Canal Dehiscence in a Patient with Ehlers-Danlos Syndrome: A Case Report. ( 28484680 )
2017
29
Diagnosis of Superior Semicircular Canal Dehiscence in the Presence of Concomitant Otosclerosis. ( 28657952 )
2017
30
Superior semicircular canal dehiscence syndrome. ( 28084916 )
2017
31
Superior Semicircular Canal Dehiscence Syndrome - Diagnosis and Surgical Management. ( 28382131 )
2017
32
Electrocochleography summating potential seen on auditory brainstem response in a case of superior semicircular canal dehiscence. ( 28607824 )
2017
33
Outcomes of middle fossa craniotomy for the repair of superior semicircular canal dehiscence. ( 28622893 )
2017
34
Diagnostic performance of reformatted isotropic thin-section helical CT images in the detection of superior semicircular canal dehiscence. ( 28627985 )
2017
35
A Method of Locating the Dehiscence during Middle Fossa Approach for Superior Semicircular Canal Dehiscence Surgery. ( 28725523 )
2017
36
Minimally Invasive Middle Fossa Keyhole Craniectomy for Repair of Superior Semicircular Canal Dehiscence. ( 28922894 )
2017
37
Superior semicircular canal dehiscence: A new perspective. ( 29234688 )
2017
38
Temporal bone anatomy characteristics in superior semicircular canal dehiscence. ( 29937854 )
2017
39
Risk of progressive hearing loss in untreated superior semicircular canal dehiscence. ( 27739592 )
2017
40
A Comparison of Surgical Treatments for Superior Semicircular Canal Dehiscence: A Systematic Review. ( 27861193 )
2017
41
Cervical and Ocular VEMP Testing in Diagnosing Superior Semicircular Canal Dehiscence. ( 28168887 )
2017
42
Computerized Assessment of Superior Semicircular Canal Dehiscence Size using Advanced Morphological Imaging Operators. ( 28321386 )
2017
43
Superior Semicircular Canal Dehiscence Symptoms Unmasked by Ossicular Chain Reconstruction. ( 28418791 )
2017
44
Minimally Invasive Middle Fossa Keyhole Craniectomy for Repair of Superior Semicircular Canal Dehiscence. ( 28521355 )
2017
45
Pediatric superior semicircular canal dehiscence: illustrative case and systematic review. ( 28548616 )
2017
46
Retrospective cohort study on hearing outcome after transmastoid plugging in superior semicircular canal dehiscence syndrome: Our Experience. ( 26382590 )
2016
47
Outcomes of Transmastoid Surgery for Superior Semicircular Canal Dehiscence Syndrome. ( 27327868 )
2016
48
Outcomes and complications in superior semicircular canal dehiscence surgery: A systematic review. ( 26371952 )
2016
49
Bilateral Superior Semicircular Canal Dehiscence and Tullio Phenomenon. ( 26738730 )
2016
50
Ontogenetic explanation for tegmen tympani dehiscence and superior semicircular canal dehiscence association. ( 26738982 )
2016

Variations for Superior Semicircular Canal Dehiscence

Expression for Superior Semicircular Canal Dehiscence

Search GEO for disease gene expression data for Superior Semicircular Canal Dehiscence.

Pathways for Superior Semicircular Canal Dehiscence

GO Terms for Superior Semicircular Canal Dehiscence

Biological processes related to Superior Semicircular Canal Dehiscence according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 axon guidance GO:0007411 9.16 NTN1 PALLD
2 homophilic cell adhesion via plasma membrane adhesion molecules GO:0007156 8.96 CDH15 PALLD
3 cell-cell adhesion GO:0098609 8.62 CDH15 NTN1

Sources for Superior Semicircular Canal Dehiscence

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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