{"schema":"https://ver.cy/schemas/card/1.0.0","id":"vr.tr.cogan-syndrome","code":"thing-q1106923","url":"https://ver.cy/models/thing/q1106923/","name":"Cogan syndrome","alternateNames":[],"kind":"thing","status":"published","version":"0.1.0-research.1","language":"en","classifiers":{"family":"Thing Registry","category":"Cross-cutting context","entryKind":"thing","plane":"XCT","domain":["XCT.STA"],"industry":[],"navPath":"","tags":[],"facets":{}},"whatItIs":"Cogan syndrome is a rare inflammatory disorder of uncertain cause, classically combining nonsyphilitic interstitial keratitis with sensorineural hearing loss and vestibular dysfunction, sometimes accompanied by systemic vasculitis.","purpose":"Enable an AI agent to recognize a supported Cogan syndrome assertion, track ocular, audiovestibular and systemic involvement, and identify evidence needed for specialist decisions.","scope":{"in":["Typical and atypical ocular-audiovestibular presentations and their temporal relationship","Diagnostic reasoning, exclusion of mimics and versioned classification conventions","Eye, hearing and vestibular involvement with longitudinal measurements","Systemic inflammation, vascular involvement and associated damage","Disease-specific treatment response, surveillance and rehabilitation needs","Aetiological uncertainty, affected populations and provenance of terminology mappings"],"out":["Patient identity, demographics and complete medical history as independent records","Appointments, admissions, billing and other episode-of-care administration","General models of keratitis, hearing loss, vertigo and vasculitis","Independent disease models for syphilis, Ménière disease and other competing diagnoses","Drug product specifications, prescribing protocols and procedural technique","Unrelated conditions or signs sharing the Cogan eponym"],"boundaries":[]},"distinguishingFeatures":["Look for documented inflammatory eye disease together with audiovestibular dysfunction; ear symptoms alone do not establish the syndrome. [Clinical case report](https://pmc.ncbi.nlm.nih.gov/articles/PMC3970326/)","For the classic presentation, establish interstitial keratitis, sensorineural hearing involvement and evidence addressing syphilis as an alternative explanation. [Clinical case report](https://pmc.ncbi.nlm.nih.gov/articles/PMC3970326/)","Distinguish typical from atypical presentations using the cited convention, ocular lesion and interval between eye and ear onset; preserve uncertainty when applying historical definitions. [Multicentre study](https://pubmed.ncbi.nlm.nih.gov/15150435/)","When symptoms resemble Ménière disease, check whether independently documented inflammatory eye findings support a wider syndrome. [Clinical case report](https://pmc.ncbi.nlm.nih.gov/articles/PMC3970326/)","For atypical presentations, require explicit comparison with competing systemic diseases rather than accepting any combination of eye inflammation and hearing impairment. [Multicentre study](https://pubmed.ncbi.nlm.nih.gov/15150435/)"],"structure":{"bundles":[{"id":"syndrome-identity-and-diagnostic-boundaries","name":"Syndrome identity and diagnostic boundaries","description":"Establish what a Cogan syndrome assertion means and which evidence supports it.","layers":[{"id":"phenotype-conventions","name":"Phenotype conventions","description":"Represent typical and atypical classifications with their source and onset chronology.","findings":[{"id":"classification-basis","name":"Classification basis","description":"Record the selected convention and the observations used to apply it; historical classifications are represented in the [multicentre study](https://pubmed.ncbi.nlm.nih.gov/15150435/).","questions":[{"text":"Which author or issuing body, publication year and exact convention support the typical or atypical designation?","id":"classification-basis-q01","kind":"provenance"},{"text":"What eye and ear onset dates, ocular lesions and timing uncertainty support or undermine that designation?","id":"classification-basis-q02","kind":"boundary"}]}]},{"id":"competing-explanations","name":"Competing explanations","description":"Maintain a differential assessment rather than an undifferentiated list of negative tests.","findings":[{"id":"differential-evidence","name":"Differential evidence","description":"Link each alternative explanation to the evidence and clinician interpretation addressing it.","questions":[{"text":"Which syphilis investigations were performed, when, and how was their adequacy interpreted?","id":"differential-evidence-q01","kind":"provenance"},{"text":"What evidence distinguishes this presentation from Ménière disease, isolated immune-mediated hearing loss and another systemic inflammatory or infectious disorder?","id":"differential-evidence-q02","kind":"boundary"}]}]}]},{"id":"ocular-involvement","name":"Ocular involvement","description":"Characterize inflammatory eye lesions and their consequences separately for each eye.","layers":[{"id":"ocular-lesion-evidence","name":"Ocular lesion evidence","description":"Connect symptoms to ophthalmological examination findings.","findings":[{"id":"documented-eye-inflammation","name":"Documented eye inflammation","description":"Preserve examination evidence distinguishing interstitial keratitis from other ocular lesions.","questions":[{"text":"What slit-lamp or other ophthalmological findings establish the lesion, anatomical site and affected eye?","id":"documented-eye-inflammation-q01","kind":"measurement"},{"text":"Is the lesion interstitial keratitis or another specified inflammatory process, and what alternative cause remains plausible?","id":"documented-eye-inflammation-q02","kind":"boundary"}]}]},{"id":"ocular-course-and-function","name":"Ocular course and function","description":"Track inflammatory activity alongside visual function and persistent structural change.","findings":[{"id":"ocular-activity-versus-damage","name":"Ocular activity versus damage","description":"Represent active inflammation, residual damage and uncertain attribution separately.","questions":[{"text":"How have corrected visual acuity and examination findings changed in each eye since the previous assessment?","id":"ocular-activity-versus-damage-q01","kind":"measurement"},{"text":"Which findings support active inflammation, residual damage or treatment-related change, and what review follows?","id":"ocular-activity-versus-damage-q02","kind":"action"}]}]}]},{"id":"audiovestibular-involvement","name":"Audiovestibular involvement","description":"Capture hearing and balance trajectories using symptoms and objective assessment.","layers":[{"id":"hearing-trajectory","name":"Hearing trajectory","description":"Track type, laterality, severity and rate of hearing change.","findings":[{"id":"serial-auditory-evidence","name":"Serial auditory evidence","description":"Retain comparable audiograms and speech testing without replacing them with a single severity label.","questions":[{"text":"What testing establishes sensorineural rather than conductive or mixed hearing loss in each ear?","id":"serial-auditory-evidence-q01","kind":"measurement"},{"text":"What changed in frequency-specific thresholds and speech recognition, over what interval and under which testing conditions?","id":"serial-auditory-evidence-q02","kind":"measurement"}]}]},{"id":"vestibular-course","name":"Vestibular course","description":"Separate reported attacks, measured vestibular dysfunction and everyday balance limitations.","findings":[{"id":"vestibular-burden","name":"Vestibular burden","description":"Connect vertigo and imbalance to dated assessments and functional consequences.","questions":[{"text":"What are the frequency and duration of vertigo attacks, and which vestibular tests support the suspected impairment?","id":"vestibular-burden-q01","kind":"measurement"},{"text":"Do persistent imbalance, falls or activity limitations warrant specialist reassessment or vestibular rehabilitation?","id":"vestibular-burden-q02","kind":"action"}]}]}]},{"id":"systemic-involvement-and-aetiology","name":"Systemic involvement and aetiology","description":"Represent vascular and other systemic findings without assuming every abnormality belongs to the syndrome.","layers":[{"id":"vascular-assessment","name":"Vascular assessment","description":"Record assessed vascular territories, cardiac findings and remaining assessment gaps.","findings":[{"id":"aortic-and-other-vascular-involvement","name":"Aortic and other vascular involvement","description":"Include aortic and other vascular assessment because such involvement has been reported with Cogan syndrome. [Clinical report and discussion](https://pmc.ncbi.nlm.nih.gov/articles/PMC3970326/)","questions":[{"text":"What evidence addresses aortitis, aortic valve dysfunction or other vascular lesions, and which territories remain unassessed?","id":"aortic-and-other-vascular-involvement-q01","kind":"measurement"},{"text":"Which new symptoms or findings trigger urgent evaluation under the treating team's documented plan?","id":"aortic-and-other-vascular-involvement-q02","kind":"action"}]}]},{"id":"systemic-attribution","name":"Systemic attribution","description":"Distinguish observations, comorbidities and proposed disease mechanisms.","findings":[{"id":"inflammation-and-causal-uncertainty","name":"Inflammation and causal uncertainty","description":"Keep systemic findings and immune-mediated explanations linked to evidence rather than treating association as proof.","questions":[{"text":"Which systemic symptoms, inflammatory markers or organ findings are attributed to Cogan syndrome, and what competing explanation was considered?","id":"inflammation-and-causal-uncertainty-q01","kind":"boundary"},{"text":"Which proposed immune mechanism or preceding exposure has supporting evidence, and which remains a hypothesis?","id":"inflammation-and-causal-uncertainty-q02","kind":"provenance"}]}]}]},{"id":"management-course-and-outcomes","name":"Management, course and outcomes","description":"Connect specialist decisions with organ-specific response, relapse and functional recovery.","layers":[{"id":"treatment-response","name":"Treatment response","description":"Record the indication, evidence basis and observed outcomes of each treatment decision.","findings":[{"id":"organ-specific-benefit-and-harm","name":"Organ-specific benefit and harm","description":"Evaluate ocular, auditory, vestibular and systemic outcomes separately across treatment changes.","questions":[{"text":"Which organ threat motivated treatment, what supporting guidance or evidence was cited, and how long after symptom onset did treatment begin?","id":"organ-specific-benefit-and-harm-q01","kind":"provenance"},{"text":"What objective benefit, adverse effect or recurrence accompanied treatment initiation, escalation or tapering?","id":"organ-specific-benefit-and-harm-q02","kind":"measurement"}]}]},{"id":"longitudinal-state-and-rehabilitation","name":"Longitudinal state and rehabilitation","description":"Maintain organ-specific follow-up and functional support across care episodes.","findings":[{"id":"sustained-control-and-functional-needs","name":"Sustained control and functional needs","description":"Record operational definitions of relapse and control alongside unresolved sensory or balance limitations.","questions":[{"text":"What observations and duration define improvement, relapse or inactive disease for each affected organ?","id":"sustained-control-and-functional-needs-q01","kind":"definition"},{"text":"Which residual deficits require hearing-aid or cochlear-implant assessment, visual support or vestibular rehabilitation, and who owns follow-up?","id":"sustained-control-and-functional-needs-q02","kind":"action"}]}]}]},{"id":"evidence-and-external-alignment","name":"Evidence and external alignment","description":"What the world already says about this thing, gathered so the model can be checked against it.","layers":[{"id":"reported-evidence","name":"Reported evidence","description":"Findings from the breadth pass, kept separate from the structural claims.","findings":[{"id":"evidence-confidence-notes","name":"Check these first","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these check these first hold for the sense of Cogan syndrome this model covers, and on what evidence?","id":"evidence-confidence-notes-q01","kind":"provenance"}]},{"id":"evidence-kinds","name":"Kinds and varieties","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these kinds and varieties hold for the sense of Cogan syndrome this model covers, and on what evidence?","id":"evidence-kinds-q01","kind":"provenance"}]},{"id":"evidence-real-world-use","name":"Real-world use","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these real-world use hold for the sense of Cogan syndrome this model covers, and on what evidence?","id":"evidence-real-world-use-q01","kind":"provenance"}]},{"id":"evidence-measurements","name":"Typical measurements","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these typical measurements hold for the sense of Cogan syndrome this model covers, and on what evidence?","id":"evidence-measurements-q01","kind":"provenance"}]},{"id":"evidence-failure-modes-and-hazards","name":"Failure modes and hazards","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these failure modes and hazards hold for the sense of Cogan syndrome this model covers, and on what evidence?","id":"evidence-failure-modes-and-hazards-q01","kind":"provenance"}]},{"id":"evidence-neighbours","name":"Neighbouring kinds and how to tell them apart","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these neighbouring kinds and how to tell them apart hold for the sense of Cogan syndrome this model covers, and on what evidence?","id":"evidence-neighbours-q01","kind":"provenance"}]}]}]}]},"agentConduct":{"may":["Explain Cogan syndrome in general terms from reputable medical sources.","Urge a person with sudden hearing loss, vertigo or a red painful eye to seek medical care quickly."],"mustNot":["Diagnose the syndrome from reported symptoms.","Advise a person to start, change or stop immunosuppressive treatment.","Disclose a person's diagnosis or hearing status without consent.","Confuse it with syphilis or suggest that a patient has a sexually transmitted infection without evidence."],"requiresHuman":["Any treatment decision, including cochlear implants."]},"ethics":{"considerations":["Sudden hearing and vision loss can change a person's life and work; care must be prompt and respectful.","Rare diseases are often misdiagnosed for years."],"affectedParties":["Patients, often young adults","Families","Employers and colleagues who need to adapt"]},"owners":{"steward":"Nobody owns the disease; patients and their clinicians answer for care.","roles":[],"masterSystems":["Rare disease registries"]},"relations":[{"target":"Q55785504","type":"parent","note":"registry parent class"},{"target":"Q55789046","type":"parent","note":"registry parent class"},{"target":"Q55789056","type":"parent","note":"registry parent class"}],"interaction":{"identity":{"applicability":"required","items":["Vercy registry: vr.tr.cogan-syndrome","Wikidata: Q1106923 (https://www.wikidata.org/wiki/Q1106923)"]},"properties":{"applicability":"not-applicable","items":["Diagnostic assertion status: suspected | clinician-supported | disputed | excluded; assessor and date","Presentation classification: typical | atypical | unclassified; convention, author or issuing body, and year","Eye-ear onset interval: days or months; onset order, source dates and uncertainty bounds","Ocular inflammatory phenotype: interstitial keratitis | other specified inflammatory lesion | none documented | unassessed; per eye","Visual function: best-corrected visual acuity with scale, eye and date","Auditory function: frequency-specific thresholds in dB HL and speech recognition percentage; ear, protocol and date","Vestibular involvement: symptoms, attack duration and frequency, objective test results, laterality and assessment date","Organ activity and damage: activity: active | inactive | uncertain | unassessed; damage: present | absent | uncertain; by organ and date","Vascular involvement: suspected | demonstrated | not detected on specified assessment | unassessed; vessel or valve and evidence","Terminology mapping: verified ICD-10, ICD-11 or SNOMED CT concept; edition, jurisdiction, release and mapping relationship","Population evidence: age at onset in years; incidence or prevalence only with denominator, geography, period and case definition"]},"recognition":{"applicability":"optional","items":["Red, painful, light-sensitive eyes from inflammation of the cornea.","Often appears together with hearing loss and dizziness."]},"capabilities":{"applicability":"required","items":["Assemble an evidence-linked eye and ear chronology for specialist diagnostic review.","Identify missing ocular examination, audiometry or differential-diagnosis evidence.","Compare organ-specific observations across time and treatment changes.","Flag newly documented sensory deterioration or vascular concerns for clinician triage.","Summarize treatment benefit, adverse effects and unresolved attribution by organ.","Link functional deficits to hearing, vestibular and visual rehabilitation assessments."]},"hazards":{"applicability":"required","items":["Hearing loss can become permanent without prompt treatment.","Inflammation of blood vessels, including the aorta, can be life-threatening."]},"interfaces":{"applicability":"required","items":["The Orphanet nomenclature of rare diseases, maintained by Orphanet under the coordination of INSERM, recognizes Cogan syndrome as a rare disease."]},"context":{"applicability":"required","items":["Cogan syndrome is encountered in specialist assessment of inflammatory eye disease accompanied by hearing loss, tinnitus or balance disturbance.","It is relevant to coordinated ophthalmology, audiology and rheumatology care when documenting ocular, inner ear and possible systemic vascular involvement."]}},"sources":[{"title":"Wikidata item Q1106923: Cogan syndrome","url":"https://www.wikidata.org/wiki/Q1106923","note":"identity and sense of the item"},{"title":"Wikipedia: Cogan syndrome","url":"https://en.wikipedia.org/wiki/Cogan_syndrome","note":"general description of the item"}],"openQuestions":["Which diagnostic or classification conventions should govern this publication, what validation supports them, and how do they handle exactly two years between eye and ear onset?","Which ICD-10 jurisdictional editions, ICD-11 releases and SNOMED CT editions provide verified exact or broader mappings for this registry concept?","What population-based incidence or prevalence estimates exist with explicit geography, observation year, denominator and case definition, including paediatric presentations?","Which biomarkers or mechanistic findings have reproducible diagnostic or prognostic value rather than exploratory associations?","What evidence supports vascular surveillance intervals, treatment escalation and organ-specific definitions of remission or irreversible damage?"],"resources":{"spec":"/models/things/publications/thing-q1106923/spec.json"},"provenance":{"origin":"thing registry research (pass 2)","builtFrom":["models/things/publications/thing-q1106923/spec.json"],"providers":["Codex"],"researchStatus":"unreviewed","generatedAt":"2026-09-12T08:46:52Z","builder":"tools/build_cards.py@1.0.0"},"completeness":{"sections":{"classifiers":"filled","whatItIs":"filled","purpose":"filled","distinguishingFeatures":"filled","structure":"filled","agentConduct":"filled","ethics":"filled","owners":"filled","relations":"filled","interaction.identity":"filled","interaction.properties":"not-applicable","interaction.recognition":"filled","interaction.capabilities":"filled","interaction.hazards":"filled","interaction.interfaces":"filled","interaction.context":"filled","sources":"filled"},"notes":{"_gate":"Published through the thing publication gate 1.0 on 2026-10-07T12:16:28+00:00: completeness 1.00; sense: Wikidata description; 2 live sources; review by codex: pass","interaction.properties":"Plane XCT: no invented physical properties."},"score":1.0}}