Cochlear implantation in chronic demyelinating inflammatory polyneuropathy

Sarah E. Mowry, Sarah King

Research output: Contribution to journalArticle

Abstract

Objective: To describe a case of chronic inflammatory demyelinating polyneuropathy (CDIP) with bilateral sudden sensorineural hearing loss who subsequently benefited from unilateral cochlear implantation. Methods: case history review and review of the literature for the terms CDIP, hearing loss, cochleovestibular dysfunction, and cochlear implantation. Results: A 49-year-old woman presented with bilateral rapidly progressive sensorineural hearing loss (SNHL) 1 month after an upper respiratory tract infection. Hearing loss was not responsive to high-dose steroids and there were no other laboratory abnormalities or physical findings. Within 1 month, she developed ascending motor palsy, requiring long-term ventilator support. This neurologic condition was diagnosed as CDIP and she was successfully treated with plasmapheresis and intravenous immunoglobulin. Her hearing never recovered. At the time of cochlear implant, she had no response at the limits of the audiometer and obtained 0% on AzBio testing. No ABR could be recorded preoperatively. She underwent uneventful cochlear implantation with a perimodilar electrode. One year after activation, she had a PTA of 20 dB and 40% on AzBio sentence testing. Her eABR demonstrated a neuropathy pattern. Only two other cases of CDIP associated with dysfunction of the eighth nerve have been described, and neither had documented profound hearing loss. Conclusions: Severe SNHL associated with CDIP is rare. Although this patient has good access to sound, speech discrimination is poor at 1-year post implantation. This outcome may be due to incomplete recovery of myelination of the eighth nerve. Other possibilities include loss of peripheral nerve fibers due to the initial viral upper respiratory infection, which may lead to less neural substrate to stimulate.

Original languageEnglish (US)
Pages (from-to)116-120
Number of pages5
JournalCochlear Implants International
Volume18
Issue number2
DOIs
StatePublished - Mar 4 2017

Fingerprint

Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Cochlear Implantation
Sensorineural Hearing Loss
Hearing Loss
Respiratory Tract Infections
Bilateral Hearing Loss
Sudden Hearing Loss
Speech Perception
Plasmapheresis
Cochlear Implants
Intravenous Immunoglobulins
Mechanical Ventilators
Peripheral Nerves
Nerve Fibers
Paralysis
Nervous System
Hearing
Electrodes
Steroids

Keywords

  • Auditory neuropathy
  • Chronic inflammatory demyelinating polyneuropathy
  • Cochlear implant
  • Electrophysiology
  • Hearing loss
  • eABR

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Speech and Hearing

Cite this

Cochlear implantation in chronic demyelinating inflammatory polyneuropathy . / Mowry, Sarah E.; King, Sarah.

In: Cochlear Implants International, Vol. 18, No. 2, 04.03.2017, p. 116-120.

Research output: Contribution to journalArticle

Mowry, Sarah E. ; King, Sarah. / Cochlear implantation in chronic demyelinating inflammatory polyneuropathy In: Cochlear Implants International. 2017 ; Vol. 18, No. 2. pp. 116-120.
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