Aliases & Classifications for Inner Ear Disease

MalaCards integrated aliases for Inner Ear Disease:

Name: Inner Ear Disease 12 15 17
Labyrinthine Dysfunction 12 15 71 32
Diseases of Inner Ear 71 32
Abnormality of the Inner Ear 6
Labyrinthine Disorder 71
Labyrinthine Disease 12
Labyrinth Diseases 43

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Ear diseases


External Ids:

Disease Ontology 12 DOID:2952 DOID:566
ICD9CM 34 386.5 386.50
MeSH 43 D007759
SNOMED-CT 67 20425006 5239005
UMLS 71 C0022890 C0155514 C0494559

Summaries for Inner Ear Disease

MalaCards based summary : Inner Ear Disease, also known as labyrinthine dysfunction, is related to autoimmune inner ear disease and usher syndrome, and has symptoms including vertigo and earache. An important gene associated with Inner Ear Disease is COCH (Cochlin), and among its related pathways/superpathways are Matrix Metalloproteinases and amb2 Integrin signaling. The drugs Dexamethasone acetate and Dexamethasone have been mentioned in the context of this disorder. Affiliated tissues include bone, eye and testes, and related phenotypes are immune system and nervous system

Wikipedia : 74 The inner ear (internal ear, auris interna) is the innermost part of the vertebrate ear. In vertebrates,... more...

Related Diseases for Inner Ear Disease

Diseases in the Inner Ear Disease family:

Autoimmune Inner Ear Disease

Diseases related to Inner Ear Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 201)
# Related Disease Score Top Affiliating Genes
1 autoimmune inner ear disease 35.1 TNF TIMP1 MPO MMP9
2 usher syndrome 32.7 SLC26A4 MYO7A MYH9 GJB2 COCH
3 superior semicircular canal dehiscence 32.6 RTEL1 COCH ASPRV1
4 sensorineural hearing loss 31.9 TNF SLC26A4 MYO7A MYH9 GJB2 COCH
5 branchiootic syndrome 1 31.7 SLC26A4 MYO7A GJB2
6 auditory system disease 31.6 SLC26A4 RTEL1 MYO7A MIR96 GJB2 FOXI1
7 cogan syndrome 31.4 GJB2 COCH CACNA1F
8 benign paroxysmal positional nystagmus 31.2 RTEL1 HECTD4 CMTR2
9 vestibular neuronitis 31.1 RTEL1 HECTD4 CMTR2 ASPRV1
10 labyrinthitis 30.9 SLC26A4 MYO7A GJB2 COCH
11 otosclerosis 30.9 SLC26A4 RTEL1 GJB2 COCH
12 otodental dysplasia 30.9 FGF3 FADD
13 ear malformation 30.8 SLC26A4 GJB2 FOXI1 FGF3
14 vestibular disease 30.7 SLC26A4 RTEL1 MYO7A GJB2 FOXI1 COCH
15 peripheral vertigo 30.6 SLC26A4 RTEL1 GJB2 FOXI1 COCH
16 eye disease 30.5 TNF MYO7A MMP9 ERCC6 CACNA1F
17 bilateral hyperactive labyrinth 11.5
18 unilateral hyperactive labyrinth 11.5
19 bilateral hypoactive labyrinth 11.5
20 unilateral hypoactive labyrinth 11.4
21 tietz albinism-deafness syndrome 11.3
22 branchiooculofacial syndrome 11.1
23 optic atrophy 3, autosomal dominant 11.1
24 donnai-barrow syndrome 11.1
25 vici syndrome 11.1
26 thiamine-responsive megaloblastic anemia syndrome 11.1
27 usher syndrome, type i 11.1
28 usher syndrome, type iiia 11.1
29 cerebellar ataxia, deafness, and narcolepsy, autosomal dominant 11.1
30 fibrochondrogenesis 11.1
31 myh-9 related disease 11.1
32 alport syndrome 11.1
33 sptlc1-related hereditary sensory neuropathy 11.1
34 mondini dysplasia 11.1
35 schindler disease 11.1
36 trnt1 deficiency 11.1
37 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 10.7
38 microscopic polyangiitis 10.7 TNF MPO
39 pendred syndrome/nonsyndromic enlarged vestibular aqueduct 10.7 SLC26A4 FOXI1
40 cryptogenic organizing pneumonia 10.7 TNF TIMP1 MMP9
41 deafness, autosomal dominant 17 10.7 MYO7A MYH9 COCH
42 collagenous colitis 10.7 TIMP1 MPO MMP9
43 mononeuropathy 10.7 TNF MPO HECTD4
44 gingival disease 10.7 TNF TIMP1 MMP9
45 deafness, autosomal dominant 9 10.7 MYO7A GJB2 COCH
46 deafness, autosomal dominant 25 10.7 MYO7A GJB2 COCH
47 decubitus ulcer 10.7 TNF MMP9 HECTD4
48 deafness, autosomal dominant 11 10.7 MYO7A GJB2 COCH
49 preterm premature rupture of the membranes 10.7 TNF TIMP1 MMP9
50 systolic heart failure 10.7 TNF TIMP1 MMP9

Comorbidity relations with Inner Ear Disease via Phenotypic Disease Network (PDN):


Hypertension, Essential Labyrinthitis
Transient Cerebral Ischemia

Graphical network of the top 20 diseases related to Inner Ear Disease:



Diseases related to Inner Ear Disease

Symptoms & Phenotypes for Inner Ear Disease

UMLS symptoms related to Inner Ear Disease:


vertigo, earache

MGI Mouse Phenotypes related to Inner Ear Disease:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 10.06 ERCC6 FADD FGF3 GJB2 MMP9 MPO
2 nervous system MP:0003631 10.03 CACNA1F ERCC6 FADD FGF3 FOXI1 GJB2
3 hearing/vestibular/ear MP:0005377 9.97 COCH ERCC6 FGF3 FOXI1 GJB2 MYH9
4 no phenotypic analysis MP:0003012 9.76 AQP6 CACNA1F FADD FGF3 GJB2 MYH9
5 reproductive system MP:0005389 9.61 FADD FGF3 FOXI1 GJB2 MMP9 MYO7A
6 skeleton MP:0005390 9.32 CACNA1F ERCC6 FADD FGF3 FOXI1 GJB2

Drugs & Therapeutics for Inner Ear Disease

Drugs for Inner Ear Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 61)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 4 1177-87-3
2
Dexamethasone Approved, Investigational, Vet_approved Phase 4 50-02-2 5743
3
Cisplatin Approved Phase 4 15663-27-1 84093 441203 2767
4 BB 1101 Phase 4
5 Antiemetics Phase 4
6 Gastrointestinal Agents Phase 4
7 Hormone Antagonists Phase 4
8 Autonomic Agents Phase 4
9 Anti-Inflammatory Agents Phase 4
10 Antineoplastic Agents, Hormonal Phase 4
11 glucocorticoids Phase 4
12 Hormones Phase 4
13 HIV Protease Inhibitors Phase 4
14
protease inhibitors Phase 4
15 Dexamethasone 21-phosphate Phase 4
16
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 5755
17
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 6741
18
Methylprednisolone hemisuccinate Approved Phase 2, Phase 3 2921-57-5
19
Prednisolone phosphate Approved, Vet_approved Phase 2, Phase 3 302-25-0
20 Prednisolone acetate Approved, Vet_approved Phase 2, Phase 3 52-21-1
21
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
22
leucovorin Approved Phase 3 58-05-9 6006 143
23
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
24
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
25
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
26
Prednisolone hemisuccinate Experimental Phase 2, Phase 3 2920-86-7
27 Anti-Bacterial Agents Phase 2, Phase 3
28 Methylprednisolone Acetate Phase 2, Phase 3
29 Anti-Infective Agents Phase 2, Phase 3
30 Neuroprotective Agents Phase 2, Phase 3
31 Immunologic Factors Phase 3
32 Immunosuppressive Agents Phase 3
33 Folic Acid Antagonists Phase 3
34 Folate Phase 3
35 Dermatologic Agents Phase 3
36 Vitamin B9 Phase 3
37 Antimetabolites Phase 3
38 Vitamin B Complex Phase 3
39 Antirheumatic Agents Phase 3
40 Pharmaceutical Solutions Phase 2
41 Antibodies, Monoclonal Phase 1, Phase 2
42 Immunoglobulins Phase 1, Phase 2
43 Antibodies Phase 1, Phase 2
44 Interleukin 1 Receptor Antagonist Protein Phase 1, Phase 2
45
Nitric Oxide Approved 10102-43-9 145068
46
Carbon monoxide Approved, Investigational 630-08-0 281
47 Anesthetics
48 Hemostatics
49 Vasoconstrictor Agents
50 Arginine Vasopressin

Interventional clinical trials:

(show all 25)
# Name Status NCT ID Phase Drugs
1 Prevention of Cisplatin-Induced Hearing Loss by Intratympanic Dexamethasone Treatment. Completed NCT01372904 Phase 4 Dexamethasone Phosphate
2 Effect of the Preoperative Administration of Dexamethasone in the Respiratory Function and Postoperative Symptoms in Women Undergoing Mastectomy for Cancer. Results of a Randomized Clinical Trial. Unknown status NCT02305173 Phase 3 Intravenous Dexamethasone;intravenous salin water
3 Effectiveness of Transtympanic Steroids in Unilateral Ménière's Disease: a Randomised Controlled Double-Blind Trial Completed NCT00802529 Phase 2, Phase 3 Methylprednisolone;Gentamicin
4 A Prospective, Randomized, Double Blind, Placebo-controlled, Multicenter, Phase 3 Efficacy and Safety Study of OTO-104 Given as a Single Intratympanic Injection in Subjects With Unilateral Meniere's Disease Recruiting NCT03664674 Phase 3 OTO-104;Placebo
5 Autoimmunity in Inner Ear Disease Terminated NCT00000361 Phase 3 Corticosteroids;Methotrexate
6 An Open-label Study of the Effects of Gevokizumab in Corticosteroid-resistant Subjects With Autoimmune Inner Ear Disease Completed NCT01950312 Phase 2 gevokizumab
7 A Phase I/II Open-label Study of the Effects of Anakinra in Corticosteroid-resistant Subjects With Autoimmune Inner Ear Disease Completed NCT01267994 Phase 1, Phase 2 Anakinra
8 A Phase II Placebo Controlled Study of the Effects of Anakinra (Kineret) in Subjects With Autoimmune Inner Ear Disease Disease (AIED) Recruiting NCT03587701 Phase 2 Anakinra 100Mg/0.67Ml Inj Syringe (Period 1);Placebo injection (Period 1);Anakinra 100Mg/0.67Ml Inj Syringe (Period 2);Placebo injection (Period 2)
9 Safety and Efficacy of Intratympanically Delivered Golimumab for Stabilization of Hearing in Patients With Autoimmune Inner Ear Disease: An Open-label Proof-of-concept Clinical Trial Terminated NCT01526174 Phase 1, Phase 2 Golimumab
10 A Phase I & II Open-label Study of the Effects of Anakinra (Kineret) in Corticosteroid-resistant Subjects With Sudden Sensorineural Heaing Loss (SSNHL) Terminated NCT02414152 Phase 1, Phase 2 anakinra
11 A Randomised Double Blind, Multiple Dose, Pharmacokinetic and Safety Study of SENS 401 in Healthy Subjects Completed NCT03071003 Phase 1 SENS-401;Placebo
12 An Open-label, Single-dose, Pharmacokinetic Study of SENS-218 in Healthy Adult, Caucasian Subjects. Completed NCT02772796 Phase 1 SENS-218
13 INTRATYMPANIC STEROID TREATMENT FOR THE PREVENTION OF INNER EAR TOXICITY ASSOCIATED WITH SYSTEMIC TREATMENT WITH CISPLATIN. Unknown status NCT01285674 Intra-tympanic Cisplatinum
14 Comparison of Clinical and Game-based Rehabilitation for Balance Impairments and Gaze Dysfunction in Clients With Vestibular Disorders Unknown status NCT02134444
15 Unilateral Peripheral Vestibular Dysfunction: Reeducation and Spatial Orientation. Completed NCT03171181
16 Vasopressin and V2 Receptor in Meniere's Disease Completed NCT00599560
17 Effectiveness of Osteopathic Manipulative Treatment (OMT) and Vestibular Rehabilitation Therapy (VRT) Alone or in Combination on Balance and Visual Function in Individuals With Vertigo and Somatic Dysfunction Completed NCT01529151
18 Clinical Study of Endolymphatic Sac Drainage With or Without Steroids for Intractable Meniere's Disease Completed NCT00500474
19 Hyperbaric Oxygen Therapy for Adult Onset Post Traumatic Stress Disorder Completed NCT03466554
20 Clinical Trial Evaluation of a Sensory Enrichment Multimodal Device (SEMD) on Physical Therapy Patients With Disequilibrium Completed NCT01483937
21 The Effect of Hyperbaric Oxygen Therapy on Patients Suffering From Neurologic Deficiency Due Traumatic Brain Injury Completed NCT00715052
22 First-in-Human Early Feasibility Study of Safety, Tolerability and Efficacy for a Multichannel Vestibular Implant in Individuals With Bilateral Severe-to-Profound Loss of Vestibular Sensation Recruiting NCT02725463
23 Exposure of Taxi Drivers to Ultrafine Particles and Black Carbon Within Their Vehicles: Determinants of In-vehicle Exposure and Short Term Respiratory Impact. Recruiting NCT03839537
24 Strength Training on Pancreatic Cancer Withdrawn NCT01395563
25 Refractory Eustachian Tube Dysfunction: Are the Symptoms Related to Endolymphatic Hydrops? Withdrawn NCT01661777 Nasal steroid;Diuretic;Antihistamine

Search NIH Clinical Center for Inner Ear Disease

Cochrane evidence based reviews: labyrinth diseases

Genetic Tests for Inner Ear Disease

Anatomical Context for Inner Ear Disease

MalaCards organs/tissues related to Inner Ear Disease:

40
Bone, Eye, Testes, Brain, Thyroid, T Cells, Smooth Muscle

Publications for Inner Ear Disease

Articles related to Inner Ear Disease:

(show top 50) (show all 459)
# Title Authors PMID Year
1
On the Relationship Between Menière's Disease and Endolymphatic Hydrops. 61
31746815 2020
2
Anatomical and Functional Consequences of Microneedle Perforation of Round Window Membrane. 61
31789795 2020
3
An update on autoimmune inner ear disease: A systematic review of pharmacotherapy. 61
31733712 2020
4
Demystifying autoimmune inner ear disease. 61
31605190 2019
5
Pediatric Autoimmune Inner Ear Disease: A Rare, But Treatable Condition. 61
31609997 2019
6
Expression of aquaporins in inner ear disease. 61
31593306 2019
7
The natural progression of low-frequency hearing loss in patients who meet hybrid implant system candidacy criteria. 61
31393603 2019
8
Endolymphatic hydrops induced by different mechanisms responds differentially to spironolactone: a rationale for understanding the diversity of treatment responses in hydropic inner ear disease. 61
31145014 2019
9
Hepatic vasculitis mimicking multiple liver abscesses in Cogan's Syndrome. 61
31308353 2019
10
miR‑10b‑3p, miR‑8112 and let‑7j as potential biomarkers for autoimmune inner ear diseases. 61
31115534 2019
11
l-Thyroxine does not prevent immunemediated sensorineural hearing loss in autoimmune thyroid diseases. 61
30107874 2019
12
Using Machine Learning to Predict Sensorineural Hearing Loss Based on Perilymph Micro RNA Expression Profile. 61
30833669 2019
13
Immune-Mediated Inner Ear Disease: Diagnostic and therapeutic approaches. 61
29525415 2019
14
Emerging options in immune-mediated hearing loss. 61
30828626 2019
15
Inner ear pathologies impair sodium-regulated ion transport in Meniere's disease. 61
30390121 2019
16
Long-term outcomes of triple semicircular canal plugging for the treatment of intractable Meniere's disease: A single center experience of 361 cases. 61
31640108 2019
17
Proteome of normal human perilymph and perilymph from people with disabling vertigo. 61
31185063 2019
18
Fluctuating Sensorineural Hearing Loss. 61
31315108 2019
19
[Recent immunology research of Meniere's disease]. 61
30585012 2018
20
Azathioprine reduces the risk of audiometric relapse in immune-mediated hearing loss. 61
29502794 2018
21
Can positron emission tomography support the characterization of immune-mediated inner ear disease? 61
30032987 2018
22
Feasibility of microRNA profiling in human inner ear perilymph. 61
29781875 2018
23
Ear Involvement in Inflammatory Bowel Disease: A Review of the Literature. 61
29977417 2018
24
The Reliability and Validity of "Dokuz Eylül University Meniere's Disease Disability Scale". 61
29283101 2018
25
Evidence of surgical treatments for intractable Meniere's disease. 61
28760332 2018
26
Imaging in autoimmune inner-ear disease and endolymphatic hydrops, bone cement for improving hearing outcomes in stapes surgery, and the reporting of results. External ear canal cholesteatoma: a hypothesis. 61
30019670 2018
27
Intratympanic gadolinium magnetic resonance imaging supports the role of endolymphatic hydrops in the pathogenesis of immune-mediated inner-ear disease. 61
29888688 2018
28
The Clinician Faced With Anticochlear Antibodies: What to Do With Them? 61
29116948 2018
29
Autoimmune inner ear disease (AIED): A diagnostic challenge. 61
30376736 2018
30
Decompensated labyrinthine weakness presenting as de novo peripheral vertigo: a discrete clinical entity? 61
29343311 2018
31
Bilateral Vestibular Weakness. 61
29904366 2018
32
Heat Shock Proteins in Human Perilymph: Implications for Cochlear Implantation. 61
29227447 2018
33
Long-term delivery of brain-derived neurotrophic factor (BDNF) from nanoporous silica nanoparticles improves the survival of spiral ganglion neurons in vitro. 61
29584754 2018
34
Autoimmune inner ear disease secondary to Hashimoto's thyroiditis: a case report. 61
30181832 2018
35
Audiovestibular Symptoms in Systemic Autoimmune Diseases. 61
30211232 2018
36
Update on Vertigo in Autoimmune Disorders, from Diagnosis to Treatment. 61
30356417 2018
37
Oxidative Stress in the Blood Labyrinthine Barrier in the Macula Utricle of Meniere's Disease Patients. 61
30233382 2018
38
Gene Therapy in Mouse Models of Deafness and Balance Dysfunction. 61
30210291 2018
39
Autoimmune Inner Ear Disease: Immune Biomarkers, Audiovestibular Aspects, and Therapeutic Modalities of Cogan's Syndrome. 61
29850616 2018
40
Evaluation and Treatment of Acute and Subacute Hearing Loss: A Review of Pharmacotherapy. 61
29023930 2017
41
Longitudinal up-regulation of endolymphatic hydrops in patients with Meniere's disease during medical treatment. 61
29299506 2017
42
The Seasonal Variation of Benign Paroxysmal Positional Vertigo. 61
28796081 2017
43
The Balance of Tissue Inhibitor of Metalloproteinase-1 and Matrix Metalloproteinase-9 in the Autoimmune Inner Ear Disease Patients. 61
28696822 2017
44
Anisomorphic cortical reorganization in asymmetric sensorineural hearing loss. 61
28515283 2017
45
The clinical manifestations of vestibular migraine: A review. 61
28285826 2017
46
Diagnosis of endolymphatic hydrops by means of 3T magnetic resonance imaging after intratympanic administration of gadolinium. 61
28017456 2017
47
Mobile tablet audiometry in fluctuating autoimmune ear disease. 61
28270231 2017
48
Clinically relevant human temporal bone measurements using novel high-resolution cone-beam CT. 61
29937832 2017
49
Cochlear Impairment and Autoimmune Ear Disorder in a Patient with Breast Cancer. 61
28458811 2017
50
Autoimmune Ear Disease: Clinical and Diagnostic Relevance in Cogan's Sydrome. 61
28458810 2017

Variations for Inner Ear Disease

ClinVar genetic disease variations for Inner Ear Disease:

6 ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 46;XX;t(4;15)(q31.3;q15)Translocation Uncertain significance 267810

Expression for Inner Ear Disease

Search GEO for disease gene expression data for Inner Ear Disease.

Pathways for Inner Ear Disease

Pathways related to Inner Ear Disease according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.33 TNF TIMP1 MMP9
2 10.81 TNF MPO MMP9
3 10.72 TNF TIMP1 RTEL1 MMP9
4 10.46 TNF MPO

GO Terms for Inner Ear Disease

Biological processes related to Inner Ear Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 death-inducing signaling complex assembly GO:0071550 9.16 TNF FADD
2 necroptotic signaling pathway GO:0097527 8.96 TNF FADD
3 sensory perception of sound GO:0007605 8.92 SLC26A4 MYO7A GJB2 COCH

Molecular functions related to Inner Ear Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protease binding GO:0002020 9.13 TNF TIMP1 FADD
2 actin-dependent ATPase activity GO:0030898 8.62 MYO7A MYH9

Sources for Inner Ear Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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