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Updated: Apr 18, 2026
Author Spotlight: Optimizing EAS with Long Electrodes for Enhanced Cochlear Coverage and Hearing Preservation
Published on: October 11, 2024
Hearing rehabilitation with a binaural cochlear implant in a patient with Erdheim-Chester disease
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.
