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DOI: 10.1055/s-0042-1746856
From normacusis to surditas and vestibular loss within 6 months: Differential diagnosis and management
Introduction:There are different causes for the occurrence of labyrinth hemorrhages. Autoimmune diseases of the inner ear are very rare with an estimated annual incidence of <5/100 000. Case history:We present a 42-year-old female patient, who initially came to our clinic in 10/2020 with unilateral deafness and vestibular loss. In 8/2020 she experienced sudden vertigo and rapidly progressive hearing loss. An MRI showed a hemorrhage in the right labyrinth. The recommended early CI fitting was initially not accepted. In 02/2021, the patient presented again with sudden mild to moderate hearing loss on the contralateral side and vertigo attacks for initially 3-4 minutes duration, progressing into permanent vertigo. An early MRI showed renewed hemorrhage on the right and no abnormal findings on th left. A sequential MRI (incl. hydrops imaging) after complete deafness on the left showed intralabyrinthine hemorrhage on the left and secondary hydrops in th right side. Differential diagnosic work-up including a PET-CT did not reveal a cause for the labyrinth hemorrhage. Hearing rehabilitation using CI took place on 3/2021 and 4/2021. In 7/2021 the patient presented with swelling / redness of the nose.The cartilage biopsy showed no inflammatory infiltrates. On 11/2021 she presented with an isolated unilateral auricular perichondritis. Conclusion:We consider relapsing polychondritis (RP) as very likely and as the cause of the meta-chroneous bilateral intralabyrinthine hemorrhage. RP is a rare disease (peak in the 5th decade of life). Men and women are affected with equal frequency. In RP, the ear, nose and larynx / tracheal cartilage can be involved and should be considered as a rare differential diagnosis of autoimmune diseases that involve the inner ear, eyes and brain.
Conflict of Interest
The author declares that there is no conflict of interest.
Conflict of Interest
The author declares that there is no conflict of interest.
Publication History
Article published online:
24 May 2022
© 2022. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).
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