European Archives of Oto-Rhino-Laryngology

, Volume 272, Issue 3, pp 583–589

Outcome of cochlear implantation in children with cochlear malformations



The objective of the study was the evaluation of outcomes of cochlear implantation (CI) in children with cochlear malformations. A retrospective case–control study was conducted in a tertiary referral centre. The patients were children with inner ear malformation judged by high-resolution computed tomography and magnetic resonance imaging treated with uni- or bilateral CI and a follow-up period of at least 3 years. They were matched with a control group of children operated for other reasons. The patients were operated by one of two surgeons using similar techniques including a standard perimodiolar electrode in all cases. The intervention was therapeutic and rehabilitative. The main outcome measures were category of auditory performance (CAP) and speech intelligibility rating (SIR). Eighteen children were diagnosed with cochlear malformations (12 % of children receiving CI). No statistical differences regarding CAP and SIR scores were found between the two groups. Only one child was diagnosed with a common cavity and performed below average. Children with auditory neuropathy performed beyond average. Children with cochlear malformations performed equally to children without malformation in the long term. Standard perimodiolar electrodes can be used despite cochlear malformations. The most important factors determining the outcome is the age of the child at the time of implantation and duration of hearing loss before CI. Awareness towards an increased risk of complications in case of inner ear malformations is recommended.


Cochlear implantation Inner ear malformation Category of auditory performance (CAP) Speech intelligibility rating (SIR) Complication Control group 

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Jesper Bille
    • 1
  • Vibeke Fink-Jensen
    • 2
  • Therese Ovesen
    • 1
  1. 1.Department of Otorhinolaryngology, Head and Neck SurgeryAarhus University HospitalAarhus CDenmark
  2. 2.Department of NeuroradiologyAarhus University HospitalAarhus CDenmark

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