Hearing rehabilitation with a binaural cochlear implant in a patient with Erdheim-Chester disease.

Abstract

OBJECTIVE AND IMPORTANCE:

Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis. This paper reports an exceptional case of bilateral neural involvement, responsible for profound hearing loss. Bilateral cochlear implantation was performed.

CLINICAL PRESENTATION:

We present a 57-year-old man affected by ECD with profound bilateral hearing loss.

INTERVENTION:

The patient underwent cochlear implantation with a binaural Digisonic(®) cochlear implant, 7 years after the initial diagnosis.

RESULTS:

Speech intelligibility rose to a plateau after about 6 months of cochlear implant use. The average outcome of speech intelligibility over time was 55% for dissyllabic words without lip reading, and 70% for sentences. Perception score decreased before the patient died from ECD.

CONCLUSION:

A description of the ECD and its otological manifestations is presented. This paper reports the effective hearing rehabilitation of profound bilateral hearing loss by the means of a binaural Digisonic(®) cochlear implant.

• Authors: Charlotte Querat, Hung Thai-Van, Denis Vital Durand, François Cotton, Stéphane Gallego, Eric Truy
• Journal: Cochlear implants international (Cochlear Implants Int) Vol. 16 Issue 5 Pg. 295-300 (Sep 2015) ISSN: 1754-7628 [Electronic] England
• PMID: 25563401 (Publication Type: Case Reports, Journal Article)

Topics

• Cochlear Implantation (instrumentation)
• Cochlear Implants
• Erdheim-Chester Disease (complications)
• Fatal Outcome
• Hearing Loss, Bilateral (etiology, surgery)
• Humans
• Male
• Middle Aged
• Speech Intelligibility
• Speech Perception