Relapsing polychondritis associated with bilateral stapes footplate fixation: a case report

Abstract

IntroductionRelapsing polychondritis is a rare multisystem autoimmune disease of uncertain etiology characterized by recurrent episodes of inflammation and progressive destruction of cartilaginous tissues. Its respiratory, cardiovascular, renal and neurological complications are life-threatening, and it is thus important to recognize the disease and its complications early. Relapsing polychondritis may follow a slowly evolving or rapidly progressive course.Case presentationThe case of a 39-years-old Caucasian woman with a three-year history of recurrent bilateral chondritis of the auricles, nasal chondritis, seronegative polyarthritis and dermatitis is reported. She had an associated bilateral stapedial fixation and one side was successfully operated on. She also had a large septal perforation involving both the cartilaginous and bony parts. The patient first presented with severe cutaneous inflammation when she was only one month old, and so this is an illustrative case of relapsing polychondritis that slowly evolved over many years.ConclusionsRelapsing polychondritis is still a relatively uncommon condition, which explains why there is often a delayed diagnosis of the disease. It is usually difficult to examine tympanic membranes in cases of relapsing polychondritis, and, therefore stapes fixation should also be suspected when there is an associated conductive hearing loss.

Relapsing polychondritis: a review

“…Auricular collapse, closure of the external auditory meatus, eustachian tube edema or obstruction with serous otitis media can lead to conductive hearing loss. A possibility of stapedial foot plate fixation is also to be considered in these patients as the abnormality is correctable surgically [28]. The hearing loss can also be sensorineural as a result of inflammation of the vestibular structures or vasculitis of the internal auditory artery [3,5].…”

Autoimmune-Related Nasal Septum Perforation: A Case Report and Systematic Review

“…All articles except four were case reports or case series. Relapsing polychondritis, 15–20 GPA, 4,21–27 cocaine-induced midline destructive lesions (CIMDL), 26,28–32 vasculitis, 33,34 SLE, 35,36 IgG4 related, 37 pyoderma gangrenosum, 38 STING-associated vasculopathy with onset in infancy, 39 chronic rhinosinusitis, 40 and Takayasu arteritis 41 were the reported NSP-associated autoimmune diseases (Fig. 2).…”

CT Scan Showing Ossicular Chain Arthritis Associated With Ossifying Labyrinthitis as Rare Signs of Relapsing Polychondritis

“…RPC is also known to induce immune-mediated inner ear disease (IMIED) and sensorineural deafness. RPC-associated middle ear lesions are limited to chronic otitis media (2) and stapes fixation (3). We describe here the imaging results of a mixed hearing loss (HL) with normal tympanic membrane.…”