Objective: This study assesses the results of cochlear implantation in chil
dren with cochlear malformations.
Study Design: Retrospective, matched-pairs analysis of prospectively collec
ted data.
Setting: University-based regional cochlear implant center.
Patients: Seventeen children with osseous cochlear malformations who had un
dergone implantation were matched and compared with a group of children wit
h normal cochleae who had undergone implantation.
Interventions: All subjects received a multichannel cochlear implant and ha
bilitation.
Main Outcome Measures: All subjects were tested with the Early Speech Perce
ption test, and the Glendonald Auditory Speech Perception tests for words a
nd sentences. Patients were classified in a standardized speech perception
category based on performance on the Early Speech Perception test.
Results: All subjects demonstrated improving performance on all measures of
speech perception over time. Overall, the two groups showed no statistical
ly significant differences in performance at 6 and 24 months. However, subj
ects with malformed cochleae evidenced slower rates of improvement than did
their matched control subjects. Subjects with more severe malformations de
monstrated poorer performance, but this may have been attributable to preop
erative factors rather than to implant performance.
Conclusions: Children with radiographic cochlear malformations benefit from
cochlear implantation with multichannel devices. They ultimately perform a
s well as their matched counterparts with normal cochleae, although they ma
y improve more slowly over time.