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Cochlear implantation in chronic demyelinating inflammatory polyneuropathy.

Sarah E Mowry1,2, Sarah King3

  • 1a Faculty at Augusta University , GA , USA.

Cochlear Implants International
|December 25, 2016
PubMed
Summary

Chronic inflammatory demyelinating polyneuropathy (CDIP) can cause sudden hearing loss. Cochlear implantation in a CDIP patient with profound hearing loss showed partial speech understanding improvement, suggesting potential eighth nerve recovery.

Keywords:
Auditory neuropathyChronic inflammatory demyelinating polyneuropathyCochlear implantElectrophysiologyHearing losseABR

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Area of Science:

  • Neurology
  • Otolaryngology

Background:

  • Chronic inflammatory demyelinating polyneuropathy (CDIP) is a rare autoimmune disorder affecting peripheral nerves.
  • Cochleovestibular dysfunction, including sensorineural hearing loss (SNHL), is an uncommon manifestation of CDIP.

Observation:

  • A 49-year-old woman with CDIP experienced rapid bilateral SNHL unresponsive to steroids.
  • She subsequently developed ascending motor palsy, diagnosed as CDIP and treated successfully with plasmapheresis and IVIg.
  • Pre-implantation audiological evaluation revealed profound hearing loss with no auditory brainstem response (ABR).

Findings:

  • The patient underwent unilateral cochlear implantation with a perimodiolar electrode.
  • One year post-implantation, she achieved a pure-tone average (PTA) of 20 dB and 40% AzBio sentence recognition.
  • Electrically evoked auditory brainstem response (eABR) indicated a neuropathy pattern.

Implications:

  • This case highlights the rarity of severe SNHL in CDIP and the potential, albeit limited, benefit of cochlear implantation.
  • Suboptimal speech discrimination may result from incomplete eighth nerve remyelination or axonal loss.
  • Further research is needed to understand the pathophysiology and optimize management of cochleovestibular dysfunction in CDIP.