Rare cause of bilateral sudden deafness

In this paper, we describe the case of a 62-year-old female with recurring episodes of sudden deafness with vertigo and facial paresis. Within a month's time, this resulted in bilateral deafness and vestibular areflexia. Erroneously, the patient was diagnosed with sudden deafness of unknown ori...

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Bibliographic Details
Published in:BMJ case reports 2016-10, Vol.2016, p.bcr2016216004
Main Authors: Vos, F I, Merkus, P, van Nieuwkerk, E B J, Hensen, E F
Format: Article
Language:English
Subjects:
Cochlear Implantation
Diagnostic Errors
Europe (West)
Female
Hearing Loss, Sensorineural - etiology
Hearing Loss, Sudden - etiology
Humans
Magnetic Resonance Imaging
Middle Aged
Polychondritis, Relapsing - complications
Polychondritis, Relapsing - diagnosis
Unusual Presentation of More Common Disease/Injury
White
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Summary:In this paper, we describe the case of a 62-year-old female with recurring episodes of sudden deafness with vertigo and facial paresis. Within a month's time, this resulted in bilateral deafness and vestibular areflexia. Erroneously, the patient was diagnosed with sudden deafness of unknown origin and subsequently with neuroborreliosis (Lyme disease). The true diagnosis of relapsing polychondritis (RP) was revealed 9 months after initial presentation. The diagnostic delay is in part explained by the fact that, by definition, the disease has to relapse before the diagnosis can be made, but also by its pluriform clinical presentation. Timely identification of RP as the cause of this profound sensorineural hearing loss proved to be important. It was key in instigating adequate follow-up, and allowed for cochlear implantation before total cochlear obliteration, which might have hampered optimal hearing rehabilitation.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2016-216004