This resource is in its pre-alpha development stage. Content is actively being curated and may be incomplete or subject to change.

Cogan Syndrome

Autoimmune MONDO:0015453 Autoimmune Disease Vasculitis Inner Ear Diseases

Rare autoimmune vasculitis characterized by ocular inflammation and audiovestibular involvement, often with systemic vasculitis.

0
Mappings
0
Definitions
0
Inheritance
6
Pathophysiology
2
Histopathology
17
Phenotypes
0
Genes
4
Treatments
2
Subtypes
3
Differentials
0
Datasets
0
Trials
◆

Subtypes

2
Typical Cogan syndrome
Non-syphilitic interstitial keratitis with audiovestibular symptoms.
Show evidence (2 references)
PMID:24979832 SUPPORT Human Clinical
"typical form with non-syphilitic interstitial keratitis and audiovestibular symptoms"
Typical Cogan syndrome is defined by interstitial keratitis with audiovestibular symptoms.
PMID:28583785 SUPPORT Human Clinical
"classically defined as the combination of nonsyphilitic interstitial keratitis and audiovestibular symptoms resembling Meniere's disease, both of them developed in an interval of less than two years."
Case report reiterates the classic definition of typical Cogan syndrome.
Atypical Cogan syndrome
Ocular involvement affecting structures other than the cornea.
Show evidence (1 reference)
PMID:24979832 SUPPORT Human Clinical
"an "atypical" form with ocular involvement affecting structures other than the cornea"
Atypical Cogan syndrome is defined by non-corneal ocular involvement.
⚙

Pathophysiology

6
Autoantibodies target corneal antigens
Autoantibodies recognize corneal antigens.
corneal epithelial cell link
Show evidence (1 reference)
PMID:22846458 SUPPORT Human Clinical
"auto-antibodies against corneal, inner ear and endothelial antigens"
Review includes corneal antigens among autoantibody targets.
Autoantibodies target inner ear antigens
Autoantibodies recognize inner ear antigens.
sensory epithelial cell link
Show evidence (2 references)
PMID:22846458 SUPPORT Human Clinical
"auto-antibodies against corneal, inner ear and endothelial antigens"
Review includes inner ear antigens among autoantibody targets.
PMID:12354474 SUPPORT Human Clinical
"Autoantibodies to inner ear and endothelial antigens in Cogan's syndrome."
Study reports autoantibodies directed at inner ear antigens.
Autoantibodies bind CD148 on inner ear sensory epithelium and endothelial cells
Autoantibodies recognize CD148/DEP-1 expressed on inner ear sensory epithelium and endothelial cells.
sensory epithelial cell link endothelial cell link
Show evidence (1 reference)
PMID:12354474 SUPPORT Human Clinical
"The peptide sequence shows similarity also with the cell-density enhanced protein tyrosine phosphatase-1 (DEP-1/CD148), which is expressed on the sensory epithelia of the inner ear and on endothelial cells."
Autoantibody target CD148 is expressed on inner ear sensory epithelium and endothelial cells.
Autoantibodies bind connexin 26
Autoantibodies recognize connexin 26 in the inner ear.
sensory epithelial cell link
Show evidence (1 reference)
PMID:12354474 SUPPORT Human Clinical
"The same antibodies bound connexin 26"
Autoantibodies bind connexin 26, linking autoimmune response to inner ear targets.
Autoantibodies target endothelial antigens
Autoantibodies recognize endothelial antigens.
endothelial cell link
Show evidence (2 references)
PMID:22846458 SUPPORT Human Clinical
"auto-antibodies against corneal, inner ear and endothelial antigens"
Review includes endothelial antigens among autoantibody targets.
PMID:12354474 SUPPORT Human Clinical
"Autoantibodies to inner ear and endothelial antigens in Cogan's syndrome."
Study reports autoantibodies directed at endothelial antigens.
Systemic vasculitic inflammation
Downstream systemic vasculitic inflammation involving multiple organs.
immune system process link ↕ DYSREGULATED inflammatory response link ↕ DYSREGULATED
Show evidence (2 references)
PMID:22846458 SUPPORT Human Clinical
"Cogan's syndrome is a rare autoimmune vasculitis"
Review describes Cogan syndrome as an autoimmune vasculitis.
PMID:24979832 SUPPORT Human Clinical
"Cogan's syndrome (CS) is a rare autoimmune vasculitis characterized by ocular inflammation and sensorineural hearing loss."
Review links autoimmune vasculitis to ocular and audiovestibular manifestations.
✶

Histopathology

2
Endolymphatic hydrops
Histology of the temporal bone shows endolymphatic hydrops.
Show evidence (1 reference)
PMID:38973571 SUPPORT Human Clinical
"findings revealed endolymphatic hydrops"
Temporal bone histopathology demonstrates endolymphatic hydrops in atypical Cogan syndrome.
Inner ear degenerative changes
Degenerative changes are seen in multiple inner ear structures.
Show evidence (1 reference)
PMID:38973571 SUPPORT Human Clinical
"degenerative changes in various parts of the inner ear"
Temporal bone histology shows degenerative changes across inner ear structures.
●

Phenotypes

17
Cardiovascular(8) Ear(4) Eye(4) Other(1)
Cardiovascular 8
Retinal vasculitis OCCASIONAL Retinal vasculitis (HP:0025188)
Show evidence (1 reference)
PMID:39280458 SUPPORT Human Clinical
"Uveitis with retinal vasculitis was observed in both eyes"
Case report documents bilateral retinal vasculitis in atypical Cogan syndrome.
Aortic regurgitation RARE Aortic regurgitation (HP:0001659)
Show evidence (1 reference)
PMID:40843044 SUPPORT Human Clinical
"presented with newly diagnosed, severe aortic regurgitation (AR)"
Case report documents severe aortic regurgitation in Cogan syndrome.
Coronary artery stenosis RARE Coronary artery stenosis (HP:0005145)
Show evidence (1 reference)
PMID:39328241 SUPPORT Human Clinical
"recurrent coronary artery stenosis"
Case report describes recurrent coronary artery stenosis in Cogan syndrome.
Vasculitis FREQUENT Vasculitis (HP:0002633)
Show evidence (3 references)
PMID:22846458 SUPPORT Human Clinical
"Approximately 70% of patients have systemic disease, of which vasculitis is considered the pathological mechanism."
Review reports frequent systemic vasculitis.
PMID:12354474 SUPPORT Human Clinical
"characterised by sensorineural hearing loss, episcleritis, and vasculitis."
Study includes vasculitis among defining features.
PMID:27330475 SUPPORT Human Clinical
"Almost half of patients develop systemic manifestations."
Case report notes systemic manifestations, consistent with vasculitic disease.
Large vessel vasculitis OCCASIONAL Large vessel vasculitis (HP:0005310)
Show evidence (3 references)
PMID:28583785 SUPPORT Human Clinical
"late-onset Cogan's syndrome associated with a large-vessel vasculitis."
Case report documents large-vessel vasculitis in Cogan syndrome.
PMID:29489984 SUPPORT Human Clinical
"The inflammation of aortic wall, named aortitis, is a rare condition"
Aortitis is a large-vessel inflammatory manifestation reported in Cogan syndrome.
PMID:27330475 SUPPORT Human Clinical
"severe medium and large vessel vasculitis"
Case report describes severe large-vessel vasculitis in Cogan syndrome.
Medium vessel vasculitis OCCASIONAL Medium vessel vasculitis (HP:6000658)
Show evidence (1 reference)
PMID:27330475 SUPPORT Human Clinical
"severe medium and large vessel vasculitis"
Case report documents medium-vessel vasculitis as a systemic manifestation.
Arteritis OCCASIONAL Arteritis (HP:0012089)
Show evidence (3 references)
PMID:39999281 SUPPORT Human Clinical
"multiple arteritis involving the coronary artery"
Case report describes multiple arteritis with coronary involvement in Cogan syndrome.
PMID:40054904 SUPPORT Human Clinical
"Cogan syndrome, a rare multisystemic autoimmune vasculitis, can result in coronaritis"
Case report notes coronaritis in Cogan syndrome.
PMID:39328241 SUPPORT Human Clinical
"Cogan's syndrome (CS) is recognized as a form of variable vasculitis."
Case report reiterates vasculitis classification in Cogan syndrome.
Acute coronary syndrome RARE Acute coronary syndrome (HP:0033678)
Show evidence (2 references)
PMID:40054904 SUPPORT Human Clinical
"can result in coronaritis and precipitate acute coronary syndrome."
Case report describes acute coronary syndrome in Cogan syndrome with coronaritis.
PMID:39007044 SUPPORT Human Clinical
"anterior ST-elevation myocardial infarction"
Case report documents STEMI as an acute coronary syndrome manifestation in Cogan syndrome.
Ear 4
Sensorineural hearing impairment FREQUENT Sensorineural hearing impairment (HP:0000407)
Show evidence (5 references)
PMID:24979832 SUPPORT Human Clinical
"Cogan's syndrome (CS) is a rare autoimmune vasculitis characterized by ocular inflammation and sensorineural hearing loss."
Review notes sensorineural hearing loss as a defining feature.
PMID:41473897 SUPPORT Human Clinical
"Bilateral, asymmetric hearing impairment manifested as sudden sensorineural hearing loss"
Case series reports sudden sensorineural hearing loss in Cogan syndrome.
PMID:40866103 SUPPORT Human Clinical
"remote history of chronic iritis and bilateral sensorineural hearing loss"
Case report documents bilateral sensorineural hearing loss in atypical Cogan syndrome.
+ 2 more references
Vertigo FREQUENT Vertigo (HP:0002321)
Show evidence (3 references)
PMID:39455380 SUPPORT Human Clinical
"It is characterised by the sudden onset of continuous rotational vertigo associated with tinnitus"
French protocol describes sudden continuous rotational vertigo in cochleo-vestibular involvement.
PMID:39543746 SUPPORT Human Clinical
"isolated and fluctuating audiovestibular Ménière-like symptoms"
Case report notes Menière-like audiovestibular symptoms consistent with vertigo.
PMID:39280458 SUPPORT Human Clinical
"Inner ear symptoms include sensorineural hearing loss, rotatory vertigo, and tinnitus"
Case report summary reiterates rotatory vertigo as an inner ear symptom.
Tinnitus FREQUENT Tinnitus (HP:0000360)
Show evidence (3 references)
PMID:39455380 SUPPORT Human Clinical
"It is characterised by the sudden onset of continuous rotational vertigo associated with tinnitus"
French protocol notes tinnitus as part of cochleo-vestibular involvement.
PMID:39543746 SUPPORT Human Clinical
"isolated and fluctuating audiovestibular Ménière-like symptoms"
Case report describes Menière-like symptoms consistent with tinnitus.
PMID:39280458 SUPPORT Human Clinical
"Inner ear symptoms include sensorineural hearing loss, rotatory vertigo, and tinnitus"
Case report summary includes tinnitus among inner ear symptoms.
Absent vestibular function OCCASIONAL Absent vestibular function (HP:0008555)
Show evidence (3 references)
PMID:41473897 SUPPORT Human Clinical
"all patients experienced loss of bilateral vestibular function"
Case series reports loss of bilateral vestibular function in Cogan syndrome.
PMID:39268327 SUPPORT Human Clinical
"bilateral vestibular dysfunction"
Pediatric case report documents bilateral vestibular dysfunction.
PMID:36502531 SUPPORT Human Clinical
"Cogan's syndrome (CS) is a rare inflammatory disease characterized by interstitial keratitis or uveitis, vestibular impairment, and progressive hearing loss, commonly bilateral."
Pediatric review notes vestibular impairment as a key manifestation.
Eye 4
Keratitis FREQUENT Keratitis (HP:0000491)
Show evidence (3 references)
PMID:24979832 SUPPORT Human Clinical
"typical form with non-syphilitic interstitial keratitis and audiovestibular symptoms"
Typical Cogan syndrome includes interstitial keratitis.
PMID:39328241 SUPPORT Human Clinical
"history of non-syphilis keratitis"
Case report documents keratitis in Cogan syndrome.
PMID:36502531 SUPPORT Human Clinical
"Cogan's syndrome (CS) is a rare inflammatory disease characterized by interstitial keratitis or uveitis, vestibular impairment, and progressive hearing loss, commonly bilateral."
Pediatric review notes interstitial keratitis as a defining manifestation.
Uveitis OCCASIONAL Uveitis (HP:0000554)
Show evidence (1 reference)
PMID:36502531 SUPPORT Human Clinical
"Cogan's syndrome (CS) is a rare inflammatory disease characterized by interstitial keratitis or uveitis, vestibular impairment, and progressive hearing loss, commonly bilateral."
Pediatric review highlights uveitis as a core ocular manifestation.
Episcleritis OCCASIONAL Episcleritis (HP:0100534)
Show evidence (1 reference)
PMID:12354474 SUPPORT Human Clinical
"characterised by sensorineural hearing loss, episcleritis, and vasculitis."
Study lists episcleritis as a characteristic clinical feature.
Panuveitis OCCASIONAL Panuveitis (HP:0012121)
Show evidence (1 reference)
PMID:39268327 SUPPORT Human Clinical
"The patient had panuveitis"
Pediatric case report documents panuveitis in Cogan syndrome.
Other 1
Aortitis OCCASIONAL
Show evidence (2 references)
PMID:29489984 SUPPORT Human Clinical
"The inflammation of aortic wall, named aortitis, is a rare condition that can be caused by a number of pathologies, mainly inflammatory or infectious in nature. In this context, the occurrence of combined audiovestibular and/or ocular manifestations eventually led to the diagnosis of Cogan's syndrome"
Case report ties aortitis to Cogan syndrome diagnosis.
PMID:36502531 SUPPORT Human Clinical
"Also, aortic involvement was present in 19.5% of patients in the literature."
Pediatric systematic review reports aortic involvement frequency.
💊

Treatments

4
Corticosteroid therapy MAXO:0000058
Corticosteroids are first-line therapy for Cogan syndrome.
Show evidence (2 references)
PMID:22846458 SUPPORT Human Clinical
"Corticosteroids are the first line of treatment"
Review identifies corticosteroids as first-line therapy.
PMID:36502531 SUPPORT Human Clinical
"Although glucocorticoids are fundamental treatment options"
Pediatric review highlights glucocorticoids as fundamental therapy.
Immunosuppressive therapy MAXO:0000058
Immunosuppressive agents are used for refractory or systemic disease.
Show evidence (3 references)
PMID:41473897 SUPPORT Human Clinical
"immunosuppressive therapy (methotrexate, cyclophosphamide, cyclosporin A)"
Case series reports use of multiple immunosuppressive agents.
PMID:40866103 SUPPORT Human Clinical
"she was started on systemic corticosteroids with rapid improvement in visual acuity and resolution of symptoms."
Case report illustrates systemic therapy response in atypical Cogan syndrome.
PMID:36502531 SUPPORT Human Clinical
"treated with corticosteroids and methotrexate."
Pediatric cases used methotrexate as an immunosuppressive agent.
TNF-alpha inhibitor therapy MAXO:0000058
TNF-alpha blockers represent a novel immunosuppressive option.
Show evidence (3 references)
PMID:22846458 SUPPORT Human Clinical
"Tumour necrosis factor (TNF)-alpha blockers are a category of immunosuppressive agents representing a recent novel therapeutic option in Cogan's syndrome."
Review notes TNF-alpha blockers as a therapeutic option.
PMID:39455380 SUPPORT Human Clinical
"Combination with anti-TNF therapy should be promptly discussed."
Protocol recommends prompt consideration of anti-TNF therapy.
PMID:41473897 SUPPORT Human Clinical
"biological treatment (infliximab, adalimumab)"
Case series reports biologic anti-TNF agents used in Cogan syndrome.
Cochlear device implantation MAXO:0009025
Cochlear implantation is used for hearing rehabilitation in severe hearing loss.
Show evidence (2 references)
PMID:41473897 SUPPORT Human Clinical
"four patients were treated with cochlear implantation and achieved long-term stable speech perception."
Case series reports cochlear implantation with stable long-term outcomes.
PMID:36502531 SUPPORT Human Clinical
"One patient had a cochlear implant"
Pediatric case report includes cochlear implantation.
🔀

Differential Diagnoses

3

Conditions with similar clinical presentations that must be differentiated from Cogan Syndrome:

Meniere disease MONDO:0007972
Overlapping Features Audiovestibular symptoms can mimic Meniere disease.
Show evidence (1 reference)
PMID:22846458 SUPPORT Human Clinical
"audiovestibular symptoms similar to those of Meniere's syndrome."
Review notes audiovestibular symptoms resembling Meniere disease, supporting it as a differential.
Takayasu arteritis Not Yet Curated MONDO:0017991
Overlapping Features Large-vessel vasculitis can overlap with systemic Cogan syndrome.
Show evidence (1 reference)
PMID:39455380 SUPPORT Human Clinical
"Association with other autoimmune diseases, particularly other forms of vasculitis such as polyarteritis nodosa or Takayasu's arteritis, is possible."
Protocol notes association with Takayasu arteritis.
Polyarteritis nodosa Not Yet Curated MONDO:0019170
Overlapping Features Medium-vessel vasculitis can be associated with Cogan syndrome.
Show evidence (1 reference)
PMID:39455380 SUPPORT Human Clinical
"Association with other autoimmune diseases, particularly other forms of vasculitis such as polyarteritis nodosa or Takayasu's arteritis, is possible."
Protocol notes association with polyarteritis nodosa.
{ }

Source YAML

click to show 
name: Cogan Syndrome
creation_date: '2026-02-04T15:55:07Z'
updated_date: '2026-02-04T15:55:07Z'
category: Autoimmune
synonyms:
- Cogan syndrome
- Cogan's syndrome
description: >-
  Rare autoimmune vasculitis characterized by ocular inflammation and
  audiovestibular involvement, often with systemic vasculitis.
disease_term:
  preferred_term: Cogan syndrome
  term:
    id: MONDO:0015453
    label: Cogan syndrome
parents:
- Autoimmune Disease
- Vasculitis
- Inner Ear Diseases
prevalence:
- population: Pediatric cases in literature
  notes: Systematic review identified 34 articles describing 44 pediatric patients.
  evidence:
  - reference: PMID:36502531
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "In the literature, 34 articles describing 44 pediatric patients with CS were identified."
    explanation: Systematic review quantifies reported pediatric cases in the literature.
has_subtypes:
- name: Typical Cogan syndrome
  description: Non-syphilitic interstitial keratitis with audiovestibular symptoms.
  evidence:
  - reference: PMID:24979832
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "typical form with non-syphilitic interstitial keratitis and audiovestibular symptoms"
    explanation: Typical Cogan syndrome is defined by interstitial keratitis with audiovestibular symptoms.
  - reference: PMID:28583785
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "classically defined as the combination of nonsyphilitic interstitial keratitis and audiovestibular symptoms resembling Meniere's disease, both of them developed in an interval of less than two years."
    explanation: Case report reiterates the classic definition of typical Cogan syndrome.
- name: Atypical Cogan syndrome
  description: Ocular involvement affecting structures other than the cornea.
  evidence:
  - reference: PMID:24979832
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "an \"atypical\" form with ocular involvement affecting structures other than the cornea"
    explanation: Atypical Cogan syndrome is defined by non-corneal ocular involvement.
pathophysiology:
- name: Autoantibodies target corneal antigens
  description: Autoantibodies recognize corneal antigens.
  cell_types:
  - preferred_term: corneal epithelial cell
    term:
      id: CL:0000575
      label: corneal epithelial cell
  evidence:
  - reference: PMID:22846458
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "auto-antibodies against corneal, inner ear and endothelial antigens"
    explanation: Review includes corneal antigens among autoantibody targets.
- name: Autoantibodies target inner ear antigens
  description: Autoantibodies recognize inner ear antigens.
  cell_types:
  - preferred_term: sensory epithelial cell
    term:
      id: CL:0000098
      label: sensory epithelial cell
  evidence:
  - reference: PMID:22846458
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "auto-antibodies against corneal, inner ear and endothelial antigens"
    explanation: Review includes inner ear antigens among autoantibody targets.
  - reference: PMID:12354474
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Autoantibodies to inner ear and endothelial antigens in Cogan's syndrome."
    explanation: Study reports autoantibodies directed at inner ear antigens.
- name: Autoantibodies bind CD148 on inner ear sensory epithelium and endothelial cells
  description: Autoantibodies recognize CD148/DEP-1 expressed on inner ear sensory epithelium and endothelial cells.
  cell_types:
  - preferred_term: sensory epithelial cell
    term:
      id: CL:0000098
      label: sensory epithelial cell
  - preferred_term: endothelial cell
    term:
      id: CL:0000115
      label: endothelial cell
  evidence:
  - reference: PMID:12354474
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The peptide sequence shows similarity also with the cell-density enhanced protein tyrosine phosphatase-1 (DEP-1/CD148), which is expressed on the sensory epithelia of the inner ear and on endothelial cells."
    explanation: Autoantibody target CD148 is expressed on inner ear sensory epithelium and endothelial cells.
- name: Autoantibodies bind connexin 26
  description: Autoantibodies recognize connexin 26 in the inner ear.
  cell_types:
  - preferred_term: sensory epithelial cell
    term:
      id: CL:0000098
      label: sensory epithelial cell
  evidence:
  - reference: PMID:12354474
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The same antibodies bound connexin 26"
    explanation: Autoantibodies bind connexin 26, linking autoimmune response to inner ear targets.
- name: Autoantibodies target endothelial antigens
  description: Autoantibodies recognize endothelial antigens.
  cell_types:
  - preferred_term: endothelial cell
    term:
      id: CL:0000115
      label: endothelial cell
  evidence:
  - reference: PMID:22846458
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "auto-antibodies against corneal, inner ear and endothelial antigens"
    explanation: Review includes endothelial antigens among autoantibody targets.
  - reference: PMID:12354474
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Autoantibodies to inner ear and endothelial antigens in Cogan's syndrome."
    explanation: Study reports autoantibodies directed at endothelial antigens.
- name: Systemic vasculitic inflammation
  description: Downstream systemic vasculitic inflammation involving multiple organs.
  biological_processes:
  - preferred_term: immune system process
    term:
      id: GO:0002376
      label: immune system process
    modifier: DYSREGULATED
  - preferred_term: inflammatory response
    term:
      id: GO:0006954
      label: inflammatory response
    modifier: DYSREGULATED
  evidence:
  - reference: PMID:22846458
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Cogan's syndrome is a rare autoimmune vasculitis"
    explanation: Review describes Cogan syndrome as an autoimmune vasculitis.
  - reference: PMID:24979832
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Cogan's syndrome (CS) is a rare autoimmune vasculitis characterized by ocular inflammation and sensorineural hearing loss."
    explanation: Review links autoimmune vasculitis to ocular and audiovestibular manifestations.
histopathology:
- name: Endolymphatic hydrops
  description: Histology of the temporal bone shows endolymphatic hydrops.
  evidence:
  - reference: PMID:38973571
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "findings revealed endolymphatic hydrops"
    explanation: Temporal bone histopathology demonstrates endolymphatic hydrops in atypical Cogan syndrome.
- name: Inner ear degenerative changes
  description: Degenerative changes are seen in multiple inner ear structures.
  evidence:
  - reference: PMID:38973571
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "degenerative changes in various parts of the inner ear"
    explanation: Temporal bone histology shows degenerative changes across inner ear structures.
phenotypes:
- name: Keratitis
  category: Ophthalmologic
  frequency: FREQUENT
  description: Interstitial keratitis in typical Cogan syndrome.
  phenotype_term:
    preferred_term: Keratitis
    term:
      id: HP:0000491
      label: Keratitis
  evidence:
  - reference: PMID:24979832
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "typical form with non-syphilitic interstitial keratitis and audiovestibular symptoms"
    explanation: Typical Cogan syndrome includes interstitial keratitis.
  - reference: PMID:39328241
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "history of non-syphilis keratitis"
    explanation: Case report documents keratitis in Cogan syndrome.
  - reference: PMID:36502531
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Cogan's syndrome (CS) is a rare inflammatory disease characterized by interstitial keratitis or uveitis, vestibular impairment, and progressive hearing loss, commonly bilateral."
    explanation: Pediatric review notes interstitial keratitis as a defining manifestation.
- name: Uveitis
  category: Ophthalmologic
  frequency: OCCASIONAL
  phenotype_term:
    preferred_term: Uveitis
    term:
      id: HP:0000554
      label: Uveitis
  evidence:
  - reference: PMID:36502531
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Cogan's syndrome (CS) is a rare inflammatory disease characterized by interstitial keratitis or uveitis, vestibular impairment, and progressive hearing loss, commonly bilateral."
    explanation: Pediatric review highlights uveitis as a core ocular manifestation.
- name: Sensorineural hearing impairment
  category: Auditory
  frequency: FREQUENT
  phenotype_term:
    preferred_term: Sensorineural hearing impairment
    term:
      id: HP:0000407
      label: Sensorineural hearing impairment
  evidence:
  - reference: PMID:24979832
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Cogan's syndrome (CS) is a rare autoimmune vasculitis characterized by ocular inflammation and sensorineural hearing loss."
    explanation: Review notes sensorineural hearing loss as a defining feature.
  - reference: PMID:41473897
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Bilateral, asymmetric hearing impairment manifested as sudden sensorineural hearing loss"
    explanation: Case series reports sudden sensorineural hearing loss in Cogan syndrome.
  - reference: PMID:40866103
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "remote history of chronic iritis and bilateral sensorineural hearing loss"
    explanation: Case report documents bilateral sensorineural hearing loss in atypical Cogan syndrome.
  - reference: PMID:39280458
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Hearing tests revealed impaired hearing in both ears."
    explanation: Case report shows bilateral hearing impairment in atypical Cogan syndrome.
  - reference: PMID:36502531
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Cogan's syndrome (CS) is a rare inflammatory disease characterized by interstitial keratitis or uveitis, vestibular impairment, and progressive hearing loss, commonly bilateral."
    explanation: Pediatric review emphasizes progressive hearing loss as a defining feature.
- name: Vertigo
  category: Neurologic
  frequency: FREQUENT
  phenotype_term:
    preferred_term: Vertigo
    term:
      id: HP:0002321
      label: Vertigo
  evidence:
  - reference: PMID:39455380
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "It is characterised by the sudden onset of continuous rotational vertigo associated with tinnitus"
    explanation: French protocol describes sudden continuous rotational vertigo in cochleo-vestibular involvement.
  - reference: PMID:39543746
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "isolated and fluctuating audiovestibular Ménière-like symptoms"
    explanation: Case report notes Menière-like audiovestibular symptoms consistent with vertigo.
  - reference: PMID:39280458
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Inner ear symptoms include sensorineural hearing loss, rotatory vertigo, and tinnitus"
    explanation: Case report summary reiterates rotatory vertigo as an inner ear symptom.
- name: Tinnitus
  category: Auditory
  frequency: FREQUENT
  phenotype_term:
    preferred_term: Tinnitus
    term:
      id: HP:0000360
      label: Tinnitus
  evidence:
  - reference: PMID:39455380
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "It is characterised by the sudden onset of continuous rotational vertigo associated with tinnitus"
    explanation: French protocol notes tinnitus as part of cochleo-vestibular involvement.
  - reference: PMID:39543746
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "isolated and fluctuating audiovestibular Ménière-like symptoms"
    explanation: Case report describes Menière-like symptoms consistent with tinnitus.
  - reference: PMID:39280458
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Inner ear symptoms include sensorineural hearing loss, rotatory vertigo, and tinnitus"
    explanation: Case report summary includes tinnitus among inner ear symptoms.
- name: Absent vestibular function
  category: Neurologic
  frequency: OCCASIONAL
  phenotype_term:
    preferred_term: Absent vestibular function
    term:
      id: HP:0008555
      label: Absent vestibular function
  evidence:
  - reference: PMID:41473897
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "all patients experienced loss of bilateral vestibular function"
    explanation: Case series reports loss of bilateral vestibular function in Cogan syndrome.
  - reference: PMID:39268327
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "bilateral vestibular dysfunction"
    explanation: Pediatric case report documents bilateral vestibular dysfunction.
  - reference: PMID:36502531
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Cogan's syndrome (CS) is a rare inflammatory disease characterized by interstitial keratitis or uveitis, vestibular impairment, and progressive hearing loss, commonly bilateral."
    explanation: Pediatric review notes vestibular impairment as a key manifestation.
- name: Episcleritis
  category: Ophthalmologic
  frequency: OCCASIONAL
  phenotype_term:
    preferred_term: Episcleritis
    term:
      id: HP:0100534
      label: Episcleritis
  evidence:
  - reference: PMID:12354474
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "characterised by sensorineural hearing loss, episcleritis, and vasculitis."
    explanation: Study lists episcleritis as a characteristic clinical feature.
- name: Panuveitis
  category: Ophthalmologic
  frequency: OCCASIONAL
  phenotype_term:
    preferred_term: Panuveitis
    term:
      id: HP:0012121
      label: Panuveitis
  evidence:
  - reference: PMID:39268327
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The patient had panuveitis"
    explanation: Pediatric case report documents panuveitis in Cogan syndrome.
- name: Retinal vasculitis
  category: Ophthalmologic
  frequency: OCCASIONAL
  phenotype_term:
    preferred_term: Retinal vasculitis
    term:
      id: HP:0025188
      label: Retinal vasculitis
  evidence:
  - reference: PMID:39280458
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Uveitis with retinal vasculitis was observed in both eyes"
    explanation: Case report documents bilateral retinal vasculitis in atypical Cogan syndrome.
- name: Aortic regurgitation
  category: Cardiovascular
  frequency: RARE
  phenotype_term:
    preferred_term: Aortic regurgitation
    term:
      id: HP:0001659
      label: Aortic regurgitation
  evidence:
  - reference: PMID:40843044
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "presented with newly diagnosed, severe aortic regurgitation (AR)"
    explanation: Case report documents severe aortic regurgitation in Cogan syndrome.
- name: Aortitis
  category: Cardiovascular
  frequency: OCCASIONAL
  description: Aortic wall inflammation has been reported with Cogan syndrome.
  evidence:
  - reference: PMID:29489984
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The inflammation of aortic wall, named aortitis, is a rare condition that can be caused by a number of pathologies, mainly inflammatory or infectious in nature. In this context, the occurrence of combined audiovestibular and/or ocular manifestations eventually led to the diagnosis of Cogan's syndrome"
    explanation: Case report ties aortitis to Cogan syndrome diagnosis.
  - reference: PMID:36502531
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Also, aortic involvement was present in 19.5% of patients in the literature."
    explanation: Pediatric systematic review reports aortic involvement frequency.
- name: Coronary artery stenosis
  category: Cardiovascular
  frequency: RARE
  phenotype_term:
    preferred_term: Coronary artery stenosis
    term:
      id: HP:0005145
      label: Coronary artery stenosis
  evidence:
  - reference: PMID:39328241
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "recurrent coronary artery stenosis"
    explanation: Case report describes recurrent coronary artery stenosis in Cogan syndrome.
- name: Vasculitis
  category: Cardiovascular
  frequency: FREQUENT
  phenotype_term:
    preferred_term: Vasculitis
    term:
      id: HP:0002633
      label: Vasculitis
  evidence:
  - reference: PMID:22846458
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Approximately 70% of patients have systemic disease, of which vasculitis is considered the pathological mechanism."
    explanation: Review reports frequent systemic vasculitis.
  - reference: PMID:12354474
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "characterised by sensorineural hearing loss, episcleritis, and vasculitis."
    explanation: Study includes vasculitis among defining features.
  - reference: PMID:27330475
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Almost half of patients develop systemic manifestations."
    explanation: Case report notes systemic manifestations, consistent with vasculitic disease.
- name: Large vessel vasculitis
  category: Cardiovascular
  frequency: OCCASIONAL
  phenotype_term:
    preferred_term: Large vessel vasculitis
    term:
      id: HP:0005310
      label: Large vessel vasculitis
  evidence:
  - reference: PMID:28583785
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "late-onset Cogan's syndrome associated with a large-vessel vasculitis."
    explanation: Case report documents large-vessel vasculitis in Cogan syndrome.
  - reference: PMID:29489984
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The inflammation of aortic wall, named aortitis, is a rare condition"
    explanation: Aortitis is a large-vessel inflammatory manifestation reported in Cogan syndrome.
  - reference: PMID:27330475
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "severe medium and large vessel vasculitis"
    explanation: Case report describes severe large-vessel vasculitis in Cogan syndrome.
- name: Medium vessel vasculitis
  category: Cardiovascular
  frequency: OCCASIONAL
  phenotype_term:
    preferred_term: Medium vessel vasculitis
    term:
      id: HP:6000658
      label: Medium vessel vasculitis
  evidence:
  - reference: PMID:27330475
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "severe medium and large vessel vasculitis"
    explanation: Case report documents medium-vessel vasculitis as a systemic manifestation.
- name: Arteritis
  category: Cardiovascular
  frequency: OCCASIONAL
  description: Systemic arteritis with coronary involvement has been reported.
  phenotype_term:
    preferred_term: Arteritis
    term:
      id: HP:0012089
      label: Arteritis
  evidence:
  - reference: PMID:39999281
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "multiple arteritis involving the coronary artery"
    explanation: Case report describes multiple arteritis with coronary involvement in Cogan syndrome.
  - reference: PMID:40054904
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Cogan syndrome, a rare multisystemic autoimmune vasculitis, can result in coronaritis"
    explanation: Case report notes coronaritis in Cogan syndrome.
  - reference: PMID:39328241
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Cogan's syndrome (CS) is recognized as a form of variable vasculitis."
    explanation: Case report reiterates vasculitis classification in Cogan syndrome.
- name: Acute coronary syndrome
  category: Cardiovascular
  frequency: RARE
  description: Acute coronary syndrome can occur due to coronaritis in systemic vasculitis.
  phenotype_term:
    preferred_term: Acute coronary syndrome
    term:
      id: HP:0033678
      label: Acute coronary syndrome
  evidence:
  - reference: PMID:40054904
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "can result in coronaritis and precipitate acute coronary syndrome."
    explanation: Case report describes acute coronary syndrome in Cogan syndrome with coronaritis.
  - reference: PMID:39007044
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "anterior ST-elevation myocardial infarction"
    explanation: Case report documents STEMI as an acute coronary syndrome manifestation in Cogan syndrome.
diagnosis:
- name: FDG PET/CT for arteritis evaluation
  description: FDG PET/CT can identify arteritis with coronary involvement and guide anti-inflammatory management.
  diagnosis_term:
    preferred_term: positron emission tomography procedure
    term:
      id: MAXO:0000137
      label: positron emission tomography procedure
  evidence:
  - reference: PMID:39999281
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "18 F-FDG PET/CT showed multiple arteritis involving the coronary artery"
    explanation: Case report demonstrates PET/CT detection of arteritis with coronary involvement.
  - reference: PMID:39328241
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Positron emission tomography/computed tomography revealed an elevated uptake of (18)F-fluorodeoxyglucose"
    explanation: Case report shows FDG PET/CT uptake in large vessels during Cogan syndrome.
differential_diagnoses:
- name: Meniere disease
  description: Audiovestibular symptoms can mimic Meniere disease.
  disease_term:
    preferred_term: Meniere disease
    term:
      id: MONDO:0007972
      label: Meniere disease
  evidence:
  - reference: PMID:22846458
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "audiovestibular symptoms similar to those of Meniere's syndrome."
    explanation: Review notes audiovestibular symptoms resembling Meniere disease, supporting it as a differential.
- name: Takayasu arteritis
  description: Large-vessel vasculitis can overlap with systemic Cogan syndrome.
  disease_term:
    preferred_term: Takayasu arteritis
    term:
      id: MONDO:0017991
      label: Takayasu arteritis
  evidence:
  - reference: PMID:39455380
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Association with other autoimmune diseases, particularly other forms of vasculitis such as polyarteritis nodosa or Takayasu's arteritis, is possible."
    explanation: Protocol notes association with Takayasu arteritis.
- name: Polyarteritis nodosa
  description: Medium-vessel vasculitis can be associated with Cogan syndrome.
  disease_term:
    preferred_term: polyarteritis nodosa
    term:
      id: MONDO:0019170
      label: polyarteritis nodosa
  evidence:
  - reference: PMID:39455380
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Association with other autoimmune diseases, particularly other forms of vasculitis such as polyarteritis nodosa or Takayasu's arteritis, is possible."
    explanation: Protocol notes association with polyarteritis nodosa.
biochemical: []
genetic: []
environmental: []
treatments:
- name: Corticosteroid therapy
  description: Corticosteroids are first-line therapy for Cogan syndrome.
  evidence:
  - reference: PMID:22846458
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Corticosteroids are the first line of treatment"
    explanation: Review identifies corticosteroids as first-line therapy.
  - reference: PMID:36502531
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Although glucocorticoids are fundamental treatment options"
    explanation: Pediatric review highlights glucocorticoids as fundamental therapy.
  treatment_term:
    preferred_term: pharmacotherapy
    term:
      id: MAXO:0000058
      label: pharmacotherapy
    qualifiers:
    - predicate:
        preferred_term: therapeutic agent
        term:
          id: NCIT:C2259
          label: Therapeutic Agent
      value:
        preferred_term: prednisone
        term:
          id: CHEBI:8382
          label: prednisone
- name: Immunosuppressive therapy
  description: Immunosuppressive agents are used for refractory or systemic disease.
  evidence:
  - reference: PMID:41473897
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "immunosuppressive therapy (methotrexate, cyclophosphamide, cyclosporin A)"
    explanation: Case series reports use of multiple immunosuppressive agents.
  - reference: PMID:40866103
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "she was started on systemic corticosteroids with rapid improvement in visual acuity and resolution of symptoms."
    explanation: Case report illustrates systemic therapy response in atypical Cogan syndrome.
  - reference: PMID:36502531
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "treated with corticosteroids and methotrexate."
    explanation: Pediatric cases used methotrexate as an immunosuppressive agent.
  treatment_term:
    preferred_term: pharmacotherapy
    term:
      id: MAXO:0000058
      label: pharmacotherapy
    qualifiers:
    - predicate:
        preferred_term: therapeutic agent
        term:
          id: NCIT:C2259
          label: Therapeutic Agent
      value:
        preferred_term: methotrexate
        term:
          id: CHEBI:44185
          label: methotrexate
    - predicate:
        preferred_term: therapeutic agent
        term:
          id: NCIT:C2259
          label: Therapeutic Agent
      value:
        preferred_term: cyclophosphamide
        term:
          id: CHEBI:4027
          label: cyclophosphamide
    - predicate:
        preferred_term: therapeutic agent
        term:
          id: NCIT:C2259
          label: Therapeutic Agent
      value:
        preferred_term: cyclosporin A
        term:
          id: CHEBI:4031
          label: cyclosporin A
- name: TNF-alpha inhibitor therapy
  description: TNF-alpha blockers represent a novel immunosuppressive option.
  evidence:
  - reference: PMID:22846458
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Tumour necrosis factor (TNF)-alpha blockers are a category of immunosuppressive agents representing a recent novel therapeutic option in Cogan's syndrome."
    explanation: Review notes TNF-alpha blockers as a therapeutic option.
  - reference: PMID:39455380
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Combination with anti-TNF therapy should be promptly discussed."
    explanation: Protocol recommends prompt consideration of anti-TNF therapy.
  - reference: PMID:41473897
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "biological treatment (infliximab, adalimumab)"
    explanation: Case series reports biologic anti-TNF agents used in Cogan syndrome.
  treatment_term:
    preferred_term: pharmacotherapy
    term:
      id: MAXO:0000058
      label: pharmacotherapy
- name: Cochlear device implantation
  description: Cochlear implantation is used for hearing rehabilitation in severe hearing loss.
  evidence:
  - reference: PMID:41473897
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "four patients were treated with cochlear implantation and achieved long-term stable speech perception."
    explanation: Case series reports cochlear implantation with stable long-term outcomes.
  - reference: PMID:36502531
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "One patient had a cochlear implant"
    explanation: Pediatric case report includes cochlear implantation.
  treatment_term:
    preferred_term: cochlear device implantation
    term:
      id: MAXO:0009025
      label: cochlear device implantation
datasets: []
clinical_trials: []