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Cogan syndrome (CS) is a rare autoimmune disorder of unknown origin characterized by inflammatory ocular disease (mainly interstitial keratitis) and vestibulo-auditory manifestations (mainly acute onset hearing loss, tinnitus and vertigo), in the setting of a negative work-up for syphilis, with a variable risk of developing into a systemic disease. Systemic manifestations may occur in more than 70% of cases.
Features include very common findings: Tinnitus, Keratitis, Photophobia, and Abnormal vestibular function and others; and common findings: Inner ear hearing loss (sensorineural hearing impairment), Elevated platelet count (thrombocytosis), Low red blood cell count (anemia), and Elevated white blood cell count (increased total leukocyte count) and others. 20 total HPO annotations.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Blood and immune system | 5 |
Biomarker and diagnostic research for Cogan syndrome has been reported in the published literature.
Phenotype severity distribution: 6 very common features, 6 common features.
Estimated prevalence: 1-9 in 1,000,000 (Rare).
No clinical trials have been registered for Cogan syndrome.
49 publications have been identified in PubMed for Cogan syndrome. Research spans Case Report / Case Series (63%), Review / Meta-Analysis (16%), and Epidemiology / Natural History (8%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 31 | 63% |
Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 11:55 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Common questions about Cogan syndrome
Ears | 4 | Tinnitus, Abnormal vestibular function, Vertigo |
Eyes | 4 | Keratitis, Conjunctivitis, Uveitis |
Heart and blood vessels | 1 | Aortic regurgitation |
8 |
16% |
Disease patterns and progression | 4 | 8% |
Other research | 2 | 4% |
Testing and diagnosis research | 2 | 4% |
Laboratory research | 2 | 4% |
Baigrie D (2026). [PMID: 29489227](https://pubmed.ncbi.nlm.nih.gov/29489227/). *Unknown Journal*. [Epidemiology / Natural History]
Mendes J (2026). [PMID: 42039758](https://pubmed.ncbi.nlm.nih.gov/42039758/). *Case Rep Ophthalmol*. [Case Report / Case Series]
Kaiser KP (2026). [PMID: 41499048](https://pubmed.ncbi.nlm.nih.gov/41499048/). *Int Ophthalmol*. [Case Report / Case Series]
Nagashima T (2026). [PMID: 41672532](https://pubmed.ncbi.nlm.nih.gov/41672532/). *Intern Med*. [Case Report / Case Series]
Jud P (2026). [PMID: 42031137](https://pubmed.ncbi.nlm.nih.gov/42031137/). *Joint Bone Spine*. [Case Report / Case Series]
Wu Y (2026). [PMID: 41958680](https://pubmed.ncbi.nlm.nih.gov/41958680/). *Front Immunol*. [Review / Meta-Analysis]
Pastore S (2026). [PMID: 41980717](https://pubmed.ncbi.nlm.nih.gov/41980717/). *Pediatrics*. [Case Report / Case Series]
Türkmen Ş (2026). [PMID: 42084883](https://pubmed.ncbi.nlm.nih.gov/42084883/). *Turk Arch Pediatr*. [Case Report / Case Series]
Guida S (2026). [PMID: 42269955](https://pubmed.ncbi.nlm.nih.gov/42269955/). *Clin Dermatol*. [Review / Meta-Analysis]
Haller PM (2025). [PMID: 40054898](https://pubmed.ncbi.nlm.nih.gov/40054898/). *JACC. Case reports*. [Case Report / Case Series]
AI-curated news mentioning Cogan syndrome
Updated Jul 30, 2026
Recent research highlights Cogan syndrome as a rare cause of deafness, contributing to the understanding of this condition's impact on auditory health. This discovery may inform future studies and treatment approaches for affected individuals.
A recent publication highlights an unexpected death in a young child with Cogan syndrome, raising concerns about the disease's severity and management. This case underscores the need for further research into Cogan syndrome and its implications for affected individuals.