[Autoimmune hearing loss in giant cell arteritis: a clinical case]
I V Savenko1, M Yu Boboshko1, S V Bryzgalova1
1Pavlov First Saint Petersburg State Medical University, Saint Petersburg, Russia.
Insights
Giant cell arteritis (GCA), or temporal arteritis, can cause hearing loss. This case study shows GCA presenting atypically after SARS-CoV-2 infection, leading to hearing impairment that improved with corticosteroid therapy.
Area of Science:
- Rheumatology
- Otolaryngology
- Infectious Disease
Background:
- Giant cell arteritis (GCA), a systemic vasculitis primarily affecting large vessels in individuals over 50, can present with varied symptoms.
- Cochleovestibular dysfunction, including sensorineural hearing loss, is an uncommon but recognized manifestation of GCA, potentially preceding other symptoms.
- The interplay between autoinflammatory and autoimmune processes contributes to arterial damage and potential ischemia in GCA.
Purpose of the Study:
- To present a clinical case of atypical Giant cell arteritis (GCA) manifesting after SARS-CoV-2 infection.
- To highlight the diagnostic challenges posed by GCA mimicking other conditions like granulomatosis with polyangiitis.
- To emphasize the importance of timely diagnosis and corticosteroid treatment for hearing improvement in GCA-related cochleovestibular dysfunction.
Main Methods:
- Case report detailing an atypical presentation of GCA following SARS-CoV-2 infection.
- Clinical evaluation including assessment of rhinosinusitis, otitis media with effusion, and profound mixed hearing loss.
- Diagnostic exclusion of other autoimmune conditions based on antibody levels and clinical features.
- Therapeutic intervention with urgent corticosteroid administration.
Main Results:
- The patient developed subacute rhinosinusitis and bilateral otitis media with effusion post-SARS-CoV-2 infection, leading to profound mixed hearing loss.
- Clinical presentation mimicked granulomatosis with polyangiitis, but serological markers were negative.
- Unilateral vision loss occurred 12 months after disease onset, aiding in GCA verification.
- Prompt corticosteroid therapy resolved main symptoms and significantly improved hearing.
Conclusions:
- This case illustrates an unusual GCA presentation post-SARS-CoV-2, involving middle and inner ear pathologies.
- The findings suggest a potential link between SARS-CoV-2 infection, GCA pathogenesis, and cochleovestibular dysfunction.
- Early recognition and treatment of GCA are crucial for preventing irreversible complications like hearing loss and vision loss.
Abstract:
Giant cell arteritis (GCA), also called as temporal arteritis, is a systemic vasculitis, primary affecting individuals over 50 years of age and mostly women. The disease can manifest with symptoms of systemic inflammatory response syndrome as well with symptoms of systemic vasculitis affecting mainly medium and large vessels, including branches of the carotid artery, first of all the ophthalmic and temporal. Pathogenetically, arterial damage in GCA is a combination of autoinflammatory and autoimmune processes, resulting in vascular wall remodeling with narrowing of the arterial lumen and subsequent ischemia in the affected organs and tissues. One of the uncommon symptoms of GCA, which in some cases may precede the onset of the disease, is cochleovestibular dysfunction, in particular sensorineural hearing loss, which might occur in 25-60% of GCA cases. The clinical case of untypical GCA was shown. The disease manifested after SARS-Cov-2 infection as the subacute rhinosinusitis and bilateral otitis media with effusion that finally resulted in profound mixed hearing loss. The clinical features of the disease looked like granulomatosis with polyangiitis, however, no increase in antinuclear and antineutrophil cytoplasmic antibody levels was detected. A specific clinical feature with unilateral vision loss after 12 months from the disease onset could help to verify it. Urgently corticosteroid therapy let us relieve both main symptoms and rhinosinusitis with bilateral otitis media with effusion that resulted in significant hearing improvement. It has been suggested that the combination of middle and inner ear lesions in this case could either be a consequence of the simultaneous development of GPA and GCA, or was the result of coincidence of a number of their pathogenesis links, specifically the activation of B cells, as well as excessive proliferation of monocytes/macrophages producing metalloproteinase-9.


