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.