Cogan’s syndrome
Description
● Definition: Cogan's syndrome (CS) is a rare, suspected autoimmune condition that is typified by vestibuloauditory symptoms (tinnitus, vertigo, and hearing loss) and inflammatory ocular illness (traditionally non-syphilitic interstitial keratitis, IK).
●Epidemiology:
➣ First reported by David G. Cogan in 1945, mostly affects young white adults, and has no inherited pattern.
➣ <200 instances have been documented globally.
●Classification:
➣Acute audiovestibular symptoms within two years combined with non-syphilitic IK is typical of Cogan's syndrome.
➣ Other inflammatory eye conditions (such as conjunctivitis, scleritis, episcleritis, or uveitis) or a gap of more than two years between ocular and audiovestibular symptoms are examples of atypical Cogan's syndrome.
➣ Pathogenesis: Possibly brought on by a viral prodrome, this condition is thought to be autoimmune-mediated vasculitis affecting small and medium arteries.
● Diagnosis:
• The classic triad: acute vestibuloauditory dysfunction (SNHL, vertigo, tinnitus), and non-syphilitic IK.
•This case: bilateral IK + progressive bilateral SNHL + vestibular symptoms, which ultimately developed into classic Cogan's syndrome after an initial unusual presentation (conjunctivitis).
Autoimmune inner ear disease (AIED) is one differential diagnosis.
↑ Labyrinthitis caused by viruses (HSV, VZV, CMV).
→ Ménière's disease (but CS → bilateral progression, severe vestibular loss, protracted).
- Neurosyphilis (negative serology rule out).
Pathogenesis: Probably an autoimmune-mediated vasculitis that affects the vestibular system, cochlea, and eye tissue.
• The potential involvement of viral triggers, in this case prodromal pharyngitis.
Aortitis, vasculitis, musculoskeletal, neurological, renal, or gastrointestinal involvement are examples of systemic symptoms, which occur in around 30% of cases.
Examining and investigating:
➣ ENT: no cutaneous herpes lesions, severe left vestibular paresis.
➣Audiometry: modest left sensorineural hearing loss that progresses to severe/profound unilateral hearing loss, followed by minor right high-frequency loss.
➣ During caloric testing, bilateral vestibular paresis was subsequently found after left vestibular paresis was established.
➣ABR: left-side delayed waves I, III, and V.
➣HR-MRI: inflammatory alterations in the proximal facial nerve, vestibular nerve, cochlea, and semicircular canals.
➣Ophthalmology: bilateral interstitial keratitis develops after conjunctivitis.
➣Laboratory: all viral, autoimmune, and infectious panels were negative (HIV, EBV, CMV, ➣HBV, HCV, syphilis, Lyme, ANA, ANCA, etc.); leukocytosis (13,640/mm³); and increased ESR (70 mm/h).
➣ Abdominal USG, echo, and CXR systemic evaluation: normal.
● Case Presentation: Patient: White female patient, age 31.
➣ First signs:
• Topical corticosteroids are used to treat bilateral conjunctivitis.
• After pharyngeal inflammation, vertigo, tinnitus, and left-sided hearing loss may occur.
Protocol
● Clinical Education
➣First line of treatment: topical corticosteroids, vitamin B, antiemetics, and vasoactive medications.
•If the condition worsens, valacyclovir (for a suspected viral aetiology), high-dose IV prednisone (60 mg/day), and then combined immunosuppression (methotrexate, cyclophosphamide, cyclosporine, low-dose corticosteroids, aspirin, and folate) may be used.
➣Progression:
• Increasingly severe/profound, high-frequency loss due to worsening left hearing loss.
• The onset of high-frequency sensorineural hearing loss on the right side.
• Bilateral vestibular paresis resulting in ataxia and oscillopsia.
• Eye condition: conjunctivitis at first, followed by bilateral interstitial keratitis.
➣Response:
• Immunosuppressive medication produced a partial improvement: vestibular symptoms (bilateral paresis) continued, ocular inflammation subsided, and right hearing stabilised or improved, but left hearing remained severely damaged.
● Cogan's Syndrome Issues and How They Are Handled
➣ Vestibulo-Auditory Symptoms: vertigo, tinnitus, dizziness, and hearing loss
→Issues include tinnitus, vertigo, dizziness, ataxia, oscillopsia, vestibular paresis, and progressive sensorineural hearing loss (typically bilateral).
• Management: To decrease inflammation and stop progression, systemic corticosteroids, such as prednisone, are used as first therapies. Levosulpiride and other anti-emetic and neuroleptic medications are used to treat nausea and vertigo. To enhance inner ear blood flow and nerve function, vasoactive medications (such pentoxifylline) and vitamin supplements may be used.
• Difficulties: Corticosteroids include hazards such as iatrogenic Cushing's syndrome and only provide temporary advantages. Treatment may not stop hearing loss from becoming worse. Although they have dangers and varying benefits, immunosuppressants (methotrexate, cyclophosphamide, and cyclosporine) are used as corticosteroid-sparing medicines to reduce autoimmune activity.
•Additional interventions: For those with severe hearing loss, cochlear implantation may be explored.
➣ Ocular Inflammation (Interstitial Keratitis, Conjunctivitis)
→ Issues: Initially bilateral conjunctivitis, which develops into interstitial keratitis, a characteristic of the illness.
•Management: Systemic corticosteroids if inflammation is significant or systemic, and topical corticosteroids to lessen inflammation in the eyes. It is essential to have regular ophthalmologic evaluations.
•Difficulties: Controlling steroid side effects is essential, and ocular problems may persist even after therapy.
➣ Autoimmunity and Systemic Involvement
→ Issues: About 30% of patients may develop systemic vasculitis, aortitis, musculoskeletal discomfort, and neurological symptoms, which might make therapy more difficult.
• Treatment: Immunosuppressive medications to regulate systemic autoimmune reactions. monitoring using chest X-rays, echocardiograms, and other imaging techniques to identify systemic issues early.
➣ Diagnostic Difficulty
→ Issues: The diagnosis is difficult because of the diversity of symptoms, the absence of certain diagnostic tests, and the overlap with other illnesses like Ménière's disease.
• Management: The clinical association of vestibuloauditory and ocular symptoms, the exclusion of infections, and the response to corticosteroids are the basis for diagnosis. To detect the progression of the condition, vestibuloauditory examination must be performed on a regular and extended basis (monthly at first, then less often).
● Interventions in Nursing (Deduced from Management Difficulties)
➣ Symptom Monitoring: To identify deterioration or treatment side effects, regular evaluations of hearing, balance, vertigo symptoms, and ocular indicators are conducted.
➣ Medication Administration and Monitoring: Give immunosuppressants and corticosteroids, keep an eye out for adverse effects (such Cushing's syndrome or immunosuppression risk), and encourage adherence.
➣ Patient Education: Inform patients about the possible adverse effects of immunosuppressants and steroids, the value of follow-up, and how to report changes in vision or hearing as soon as possible.
➣ Supportive Care: Handle vertigo-related symptoms such nausea and ataxia-related falls; offer safety precautions and assistive technology as required.
➣ Care Coordination: Arrange interdisciplinary treatment and assist in referring patients to specialists (otorhinolaryngologists, ophthalmologists, and rheumatologists).
➣ Emotional Assistance: Offer psychological assistance because chronic illnesses and sensory impairments impact quality of life.
Notes
for more details visit https://pmc.ncbi.nlm.nih.gov/articles/PMC2808685/
