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[Deafness in juvenile relapsing polychondritis. A case report].
1Service d'oto-rhino-laryngologie, Centre hospitalier Universitaire, Faculté de médecine, Université de Sherbrooke, Québec, Canada.
The Journal of Otolaryngology
|August 1, 1989
Summary
Relapsing polychondritis, an autoimmune cartilage disease, can affect hearing. This case highlights a rare pediatric instance of unilateral hearing loss in a 12-year-old girl.
Area of Science:
- Rheumatology
- Otolaryngology
- Genetics
Background:
- Relapsing polychondritis (RP) is a rare systemic autoimmune disorder characterized by progressive inflammation and destruction of cartilaginous structures throughout the body.
- While typically affecting adults, RP can manifest in pediatric populations, presenting unique diagnostic and management challenges.
- The audio-vestibular system, particularly the inner ear, is frequently involved in RP, often leading to sensorineural hearing loss and vestibular dysfunction, usually bilateral.
Observation:
- This report details a 12-year-old female patient presenting with unilateral sensorineural hearing loss.
- The patient's presentation is atypical given the typical age of onset and bilateral involvement often seen in pediatric cases of relapsing polychondritis.
- Clinical examination and diagnostic workup were performed to evaluate the cause of the hearing loss and assess for other signs of relapsing polychondritis.
Findings:
- The case study focuses on a pediatric patient with unilateral sensorineural hearing loss, a less common manifestation of relapsing polychondritis.
- A comprehensive literature review was conducted to compare this case with previously reported pediatric instances of RP involving the audio-vestibular system.
- Analysis of the findings aims to elucidate the spectrum of RP presentation in children and identify potential risk factors or unique characteristics.
Implications:
- This case underscores the importance of considering relapsing polychondritis in the differential diagnosis of pediatric sensorineural hearing loss, even with unilateral presentation.
- Understanding the variability in pediatric RP presentation can aid in earlier diagnosis and intervention, potentially preventing further audio-vestibular damage.
- Further research into pediatric relapsing polychondritis is warranted to better define its epidemiology, clinical course, and long-term outcomes, especially regarding audio-vestibular involvement.