We report on a rare case of cochlear implantation in a patient affected by relapsing polychondritis (RP), which over time induced cochlear fibrosis/ossification and deterioration of previously excellent hearing performance. The clinical course was determined by CT scan, electrophysiology, and speech perception data. We conclude that RP is a severe autoimmune connective disorder that can cause profound sensorineural hearing loss. Cochlear implantation in these patients can provide excellent performance. Continuation of therapy may improve prognosis, but relapses involving inner ear structures can determine fibrosis/ossification of the modiolus and interfere with cochlear implant use.
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http://dx.doi.org/10.3109/00016489.2010.545077 | DOI Listing |
Case Rep Pediatr
January 2025
Department of Internal Medicine, Lebanese Hospital Geitaoui University Medical Center, Beirut, Lebanon.
Red ear syndrome (RES) is a rare clinical entity presenting with paroxysmal erythema of one or both ears associated with a burning sensation or earache. The onset of symptoms could be either spontaneous or triggered by touch, stress, coughing, sneezing, neck movements, chewing, and combing hair. While most cases are usually described in adults, it can rarely present in children.
View Article and Find Full Text PDFInt J Rheum Dis
January 2025
Department of Internal Medicine, Hedi Chaker University Hospital, Sfax, Tunisia.
Int J Med Sci
January 2025
Department of Rheumatism and Immunology, Tianjin First Central hospital, Tianjin, China.
Autoimmune inner ear disease (AIED) is a rare condition characterized by immune-mediated damage to the inner ear, leading to progressive sensorineural hearing loss (SNHL) and vestibular symptoms such as vertigo and tinnitus. This study investigates the pathogenesis and therapeutic strategies for AIED through the analysis of three cases with different underlying autoimmune disorders: rheumatoid arthritis, relapsing polychondritis, and IgG4-related disease. The etiology of AIED involves complex immunopathological mechanisms, including molecular mimicry and the "bystander effect," with specific autoantibodies, such as those against heat shock protein 70 (HSP70), playing a potential role in cochlear damage.
View Article and Find Full Text PDFInt J Dermatol
December 2024
Department of Dermatology, International University of Health and Welfare, Chiba, Japan.
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