Cochlear implantation in a child with osteogenesis imperfecta

Int J Pediatr Otorhinolaryngol. 2003 Jun;67(6):677-80. doi: 10.1016/s0165-5876(03)00073-9.


Osteogenesis imperfecta (OI) is a hereditary disease of connective tissue and affects bone, dentine, sclera, joint, tendon, blood vessels, heart valves, and skin. Approximately 50% of the adult patients with OI have associated hearing impairment. To date, only three cases of cochlear implantation in adults with OI have been reported, but none in children. We present a case of cochlear implantation in a congenitally deaf 6-year-old boy with OI. The Nucleus 24 Contour device was successfully implanted using the suprameatal approach (SMA). At 6 months post-initial stimulation there was no evidence of non-acoustic nerve excitation (i.e. facial twitching) or discomfort, and significant progress in auditory abilities was manifested by open set word identification.

Publication types

  • Case Reports

MeSH terms

  • Audiometry
  • Child
  • Cochlear Implantation*
  • Deafness / etiology*
  • Deafness / physiopathology
  • Deafness / surgery*
  • Humans
  • Male
  • Osteogenesis Imperfecta / complications*
  • Osteogenesis Imperfecta / physiopathology
  • Osteogenesis Imperfecta / surgery
  • Recovery of Function / physiology