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X-linked Malformation and Cochlear Implantation.

Henrik Smeds1, Jeremy Wales, Filip Asp

  • 1*Department of Clinical Science, Intervention and Technology, Karolinska Institutet †Department of Otolaryngology, Karolinska University Hospital, Stockholm ‡Department of Signals and Systems, Chalmers University of Technology, Gothenburg, Sweden §Department of Special Needs Education, University of Oslo, Oslo, Norway ||Department of Radiology, Karolinska University Hospital ¶Department of Molecular Medicine and Surgery, Karolinska Institutet **Department of Clinical Genetics ††Department of Social Work in Health, Karolinska University Hospital, Stockholm, Sweden.

Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [And] European Academy of Otology and Neurotology
|October 26, 2016
PubMed
Summary

Cochlear implantation is safe for children with X-linked inner ear malformations. While hearing and speech develop, outcomes are generally lower than in typical pediatric cochlear implant recipients.

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Area of Science:

  • Otolaryngology
  • Genetics
  • Pediatric Audiology

Background:

  • X-linked inner ear malformation, often associated with POU3F4 mutations, causes severe-profound mixed hearing loss.
  • Incomplete Partition type 3 is a specific cochlear malformation observed in these children.

Purpose of the Study:

  • To assess the safety and efficacy of cochlear implantation (CI) for hearing rehabilitation in children with X-linked inner ear malformations.
  • To evaluate the impact of CI on hearing and spoken language development in this specific pediatric population.

Main Methods:

  • Retrospective review of ten children with Incomplete Partition type 3 cochlear malformation who underwent cochlear implantation between 2007 and 2015.
  • Multidisciplinary follow-up assessing surgical events, intraoperative findings, and postoperative hearing and spoken language abilities.

Main Results:

  • Fifteen cochlear implantations were performed with no severe complications; minor issues like electrode repositioning and cerebrospinal fluid leak were managed successfully.
  • Nine out of ten children showed higher postoperative electrical stimulation levels compared to typical CI patients.
  • Eight children achieved varying degrees of spoken language development, but speech recognition scores were below average for pediatric CI recipients.

Conclusions:

  • Cochlear implantation is a safe surgical option for children with severe-profound mixed hearing loss due to POU3F4 mutation-related inner ear malformations.
  • Children with this condition can develop hearing and spoken language skills following CI, although the overall outcomes may be less favorable compared to other pediatric cases.