"Emergency" Cochlear Implantation in Labyrinthitis Ossificans Secondary to Polyarteritis Nodosa

How to Face a Rare Entity

Pietro Canzi, Federico Aprile, Marco Manfrin, Irene Avato, Marianna Magnetto, Domenico Minervini, Lorenzo Cavagna, Marco Benazzo

Research output: Contribution to journalArticle

Abstract

Polyarteritis nodosa (PAN) is a systemic vasculitis affecting the small- and medium-sized arteries that may present with hearing impairment. In rare cases, PAN may be associated with progressive labyrinthitis ossificans (LO), an otologic emergency requiring early cochlear implantation (CI) to restore hearing before the complete, irreversible cochlear ossification. We report the first case in the literature of a patient affected by PAN with bilateral sudden sensorineural hearing loss and rapid LO who underwent "emergency" bilateral simultaneous CI. This case report emphasizes the importance of an early audiological evaluation in patients with PAN when LO is suspected. Multidisciplinary approach is mandatory when facing organ-specific manifestations in patients with PAN. Detailed discussion is provided with particular regard to clinical and radiological presentation as well as CI outcomes in such a rare and challenging case.

Original languageEnglish
JournalJournal of International Advanced Otology
DOIs
Publication statusE-pub ahead of print - Dec 12 2018

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Labyrinthitis
Cochlear Implantation
Polyarteritis Nodosa
Emergencies
Bilateral Hearing Loss
Sudden Hearing Loss
Systemic Vasculitis
Sensorineural Hearing Loss
Cochlea
Hearing Loss
Osteogenesis
Hearing
Arteries

Cite this

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title = "{"}Emergency{"} Cochlear Implantation in Labyrinthitis Ossificans Secondary to Polyarteritis Nodosa: How to Face a Rare Entity",
abstract = "Polyarteritis nodosa (PAN) is a systemic vasculitis affecting the small- and medium-sized arteries that may present with hearing impairment. In rare cases, PAN may be associated with progressive labyrinthitis ossificans (LO), an otologic emergency requiring early cochlear implantation (CI) to restore hearing before the complete, irreversible cochlear ossification. We report the first case in the literature of a patient affected by PAN with bilateral sudden sensorineural hearing loss and rapid LO who underwent {"}emergency{"} bilateral simultaneous CI. This case report emphasizes the importance of an early audiological evaluation in patients with PAN when LO is suspected. Multidisciplinary approach is mandatory when facing organ-specific manifestations in patients with PAN. Detailed discussion is provided with particular regard to clinical and radiological presentation as well as CI outcomes in such a rare and challenging case.",
author = "Pietro Canzi and Federico Aprile and Marco Manfrin and Irene Avato and Marianna Magnetto and Domenico Minervini and Lorenzo Cavagna and Marco Benazzo",
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T1 - "Emergency" Cochlear Implantation in Labyrinthitis Ossificans Secondary to Polyarteritis Nodosa

T2 - How to Face a Rare Entity

AU - Canzi, Pietro

AU - Aprile, Federico

AU - Manfrin, Marco

AU - Avato, Irene

AU - Magnetto, Marianna

AU - Minervini, Domenico

AU - Cavagna, Lorenzo

AU - Benazzo, Marco

PY - 2018/12/12

Y1 - 2018/12/12

N2 - Polyarteritis nodosa (PAN) is a systemic vasculitis affecting the small- and medium-sized arteries that may present with hearing impairment. In rare cases, PAN may be associated with progressive labyrinthitis ossificans (LO), an otologic emergency requiring early cochlear implantation (CI) to restore hearing before the complete, irreversible cochlear ossification. We report the first case in the literature of a patient affected by PAN with bilateral sudden sensorineural hearing loss and rapid LO who underwent "emergency" bilateral simultaneous CI. This case report emphasizes the importance of an early audiological evaluation in patients with PAN when LO is suspected. Multidisciplinary approach is mandatory when facing organ-specific manifestations in patients with PAN. Detailed discussion is provided with particular regard to clinical and radiological presentation as well as CI outcomes in such a rare and challenging case.

AB - Polyarteritis nodosa (PAN) is a systemic vasculitis affecting the small- and medium-sized arteries that may present with hearing impairment. In rare cases, PAN may be associated with progressive labyrinthitis ossificans (LO), an otologic emergency requiring early cochlear implantation (CI) to restore hearing before the complete, irreversible cochlear ossification. We report the first case in the literature of a patient affected by PAN with bilateral sudden sensorineural hearing loss and rapid LO who underwent "emergency" bilateral simultaneous CI. This case report emphasizes the importance of an early audiological evaluation in patients with PAN when LO is suspected. Multidisciplinary approach is mandatory when facing organ-specific manifestations in patients with PAN. Detailed discussion is provided with particular regard to clinical and radiological presentation as well as CI outcomes in such a rare and challenging case.

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JO - Journal of International Advanced Otology

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