A Case of Relapsing Polychondritis That Presented with Bilateral Hearing Loss

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  • 両側難聴を呈した再発性多発軟骨炎例
  • リョウガワ ナンチョウ オ テイシタ サイハツセイ タハツ ナンコツエンレイ

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Abstract

<p>Relapsing polychondritis (RP) is a multi-system cartilaginous disease that is characterized by chronic inflammation and cartilage deformities. The auricular cartilage, nasal septum, and laryngeal cartilage are the most common sites of inflammation in this disease. However, inner ear symptoms are rare. We report the case of a patient with RP who presented with the initial symptom of bilateral hearing loss.</p><p>The patient was a 57-year-old woman who was referred to our hospital with the chief complaint of progressive bilateral hearing loss despite steroid treatment. Results of evaluation of the auditory steady-state response (ASSR) suggested bilateral hearing loss; otoacoustic emission (OAE) testing indicated inner ear impairment. The auricular cartilage, which is the most common site of inflammation in cases of RP, was normal. Caloric testing indicated mild canal paresis in the left ear and evaluation of the ocular vestibular evoked myogenic potentials (oVEMP) showed a mild decrease in response on the right side. Laboratory tests revealed increased values of inflammatory markers (serum C-reactive protein level and erythrocyte sedimentation rate) and high serum levels of type II collagen antibody, which supported the diagnosis of RP. Positron emission tomography-computed tomography (PET-CT) demonstrated accumulation of fluorodeoxyglucose in many systemic cartilaginous sites, but not in the auricular cartilage. Histopathological examination of the thyroid cartilage confirmed the diagnosis of RP. With the administration of immunosuppressants in addition to a steroid, the hearing loss recovered significantly, and pure tone audiometry and OAE testing indicated recovery of inner ear function.</p><p>RP with hearing loss as the initial symptom is rare. Moreover, it was difficult to diagnose RP in our patient reported herein, in the absence of auricular inflammation. While the hearing loss due to inner ear damage was reversible in this patient, treatment delay could have let to treatment failure. Recovery of the OAE response indicated that the main site of inflammation was the outer hair cells. In such cases, otolaryngologists must consider the diagnosis of RP and make efforts to obtain an early diagnosis and provide prompt treatment.</p>

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