Background. Cogan syndrome is a rare systemic vasculitis with approximately 450 cases reported in the literature [1, p. 1]. Cogan syndrome primarily affects young Caucasian adults in their 20s and 30s, with no significant differences by gender or race [2, p. 1]. Typical Cogan syndrome is defined using Cogan's original criteria with the following three conditions: (1) ocular symptoms, mainly non-syphilitic interstitial keratitis; (2) audiovestibular symptoms similar to those of Meniere's syndrome (sudden onset of tinnitus and vertigo, accompanied by progressive bilateral sensorineural hearing loss); and (3) an interval between the onset of ocular and audiovestibular manifestations of less than 2 years [3, p. 1].
According to the criteria of Haynes et al., patients with any of the following symptoms are classified as having atypical Cogan syndrome: (1) inflammatory ocular manifestations, including episcleritis, scleritis, retinal artery occlusion, choroiditis, retinal haemorrhage, papilloedema, exophthalmos or tenonitis, with or without interstitial keratitis; patients with isolated conjunctivitis, subconjunctival haemorrhage or iritis were also classified as having atypical Cogan syndrome if these inflammatory ocular manifestations were associated with Meniere-like episodes within an interval of 2 years; (2) typical ocular manifestations associated, within 2 years, with audiovestibular symptoms different from Meniere-like episodes; or (3) a delay of more than 2 years between the onset of typical ocular and audiovestibular manifestations. The atypical forms are usually underdiagnosed, delaying treatment [4, p. 1].
The treatment of Cogan syndrome is based on corticosteroids and immunomodulatory therapy, with promising prognosis, particularly with an early start that can improve the odds of recovering hearing loss [5, p. 1].
Case presentation. An 18-year-old female presented with left ear discomfort and progressive hearing loss without vestibular symptoms. She was diagnosed with secretory otitis media and received nine antibiotic courses with no improvement. Audiometry indicated bilateral severe mixed hearing loss, predominantly conductive. Computed tomography revealed bilateral middle ear effusion and mastoiditis. Tympanic membrane shunting procedure had no effect. Within two months after disease onset, patient also experienced two episodes of right eye pain, redness, and blurred vision. Slit-lamp examination identified interstitial keratitis that was successfully treated with dexamethasone-tobramycin eye drops. Laboratory data showed mild leukocytosis, elevated erythrocyte sedimentation rate (48 mm/h) and C-reactive protein (7.2 mg/l). Infectious (tuberculosis, syphilis, borreliosis) and immunological (rheumatoid factor, anti-cyclic citrullinated peptide antibodies, antinuclear antibodies, anti-proteinase 3 and anti-myeloperoxidase antibodies, HLA-B27, angiotensin converting enzyme) panel was negative. Chest computed tomography, cardiac, abdominal, pelvic ultrasound were normal. Three months post-presentation, Cogan syndrome was suspected, and prednisolone 40 mg/day was initiated, resulting in regression of middle ear effusion (complete on the left, partial on the right) and improved hearing after two weeks. Audiometry improved from severe to mild hearing loss (left) and severe to moderate (right). Methotrexate was added, and prednisolone gradually tapered to 5 mg/day. At 3 months, audiometry showed slight further improvement. At 9- and 15-month follow-ups, audiometric data stabilized with persistent moderate right-sided hearing loss. Balloon dilation of the right Eustachian tube yielded no benefit. Due to patient’s stable condition, an attempt to discontinue steroids and methotrexate was made. At 2-year follow-up, patient had no new ocular, auditory, or systemic symptoms, normal inflammatory markers, and stable audiogram data.
Conclusions. Although Cogan syndrome usually presents with acute vestibular symptoms and subsequent sensorineural hearing loss, this case highlights atypical presentation with middle ear involvement and mixed hearing loss, without Meniere-like symptoms. Diagnosis relied on recurrent interstitial keratitis, simultaneous ocular/auditory involvement, and exclusion of alternative causes (e.g., granulomatosis with polyangiitis). The patient responsed positively to steroid and methotrexate therapy, while otologic pressure-equalization procedures were ineffective.
References:
1. Nasrollahi T., Borrelli M., Lin H.W., Djalilian H.R. Cogan Syndrome: A Case Study and Review of the Literature. Ear Nose Throat J. 2023. Vol. 102, no. 9. P. 35S-39S. doi: 10.1177/01455613231187765.
2. Wang Y., Tang S., Shao C., Liu Y. Cogan's syndrome is more than just keratitis: a case-based literature review. BMC Ophthalmol. 2023. Vol. 23, no. 1. P. 212. doi: 10.1186/s12886-023-02966-6.
3. Sasano Y., Mochizuki F., Komori M.A. Pediatric Case of Cogan's Syndrome With Internal Otitis. Cureus. 2024. Vol. 16, no. 8. P. e66742. doi: 10.7759/cureus.66742. PMID: 39268327.
4. Tirelli G., Tomietto P., Quatela E., Perrino F., Nicastro L., Cattin L., Carretta R. Sudden hearing loss and Crohn disease: when Cogan syndrome must be suspected. Am J Otolaryngol. 2015. Vol. 36, no. 4. P. 590-7. doi: 10.1016/j.amjoto.2015.02.013.
5. El Bouhmadi K., Allaoui A., El Khattab M., Youbi M., Anajar S., Essaadi M., Snoussi K., Hajjij A. Atypical Cogan syndrome: a case report. J Med Case Rep. 2024. Vol. 18, no. 1. P. 551. doi: 10.1186/s13256-024-04904-6.
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