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Related Experiment Videos

Paget's disease and cochlear implantation.

A Bacciu1, E Pasanisi, V Vincenti

  • 1Department of Otolaryngology, University of Parma, Via Gramsci, 14, 43100 Parma, Italy. andreabacciu@yahoo.it

The Journal of Laryngology and Otology
|November 20, 2004
PubMed
Summary

Cochlear implantation successfully restored hearing in a patient with Paget's disease of the skull, a condition previously unreported for this procedure. The patient achieved significant speech perception, demonstrating the viability of this treatment for hearing loss caused by the bone disorder.

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Area of Science:

  • Otolaryngology
  • Neurosurgery
  • Genetics

Background:

  • Paget's disease of bone is a common skeletal disorder of unknown cause, marked by abnormal bone remodeling.
  • Skull involvement can lead to sensorineural hearing loss and profound deafness.
  • No prior reports exist of cochlear implantation in patients with Paget's disease.

Observation:

  • A 77-year-old male with Paget's disease of the skull and a 20-year history of progressive bilateral sensorineural hearing loss was evaluated.
  • The patient underwent cochlear implantation with a Nucleus 24 Contour electrode array.

Findings:

  • The surgical procedure and postoperative recovery were uneventful.
  • At 10 months post-implantation, the patient demonstrated excellent open-set speech perception.

Related Experiment Videos

  • Word recognition was 100% in quiet and 96% in noise; sentence recognition was 98% in quiet and 94% in noise (+10 dB SNR).

    • Implications:

    • Cochlear implantation is a safe and effective treatment for hearing loss in patients with Paget's disease of the skull.
    • This case expands the indications for cochlear implantation in complex otological cases.
    • Further research may explore long-term outcomes and efficacy in a larger patient cohort.