Audiological Performance in Children with Inner Ear Malformations Before
and After Cochlear Implantation
Abstract
Objective: To evaluate the auditory perception outcomes of cochlear
implant (CI) in children with different types of inner ear malformations
(IEMs) and to compare them with CI users with the normal cochlea.
Design: Retrospective and prospective data collection. Settings:
Tertiary referral hospital. Participants: There were 274 CI users with
and without IEMs as two groups. Both groups’ chronological age at
implantation and duration of cochlear implant usage was matched (±8
months). Main outcome measures: All subjects were evaluated
preoperatively and postoperatively with Ling’s sound test and auditory
perception test battery, which includes the Meaningful Auditory
Integration Scale (MAIS), close-set Pattern Perception Test (PPT), and
open-set Sentence Recognition Test (SRT). Also, children with IEMs were
assessed for language development. Results: The incidence of IEMs were
incomplete partition-II, 40 (29.19%), incomplete partition-I, 36
(26.2%), cochlear hypoplasia, 26 (18.9%), enlarged vestibular
aqueduct, 14 (%10.2), incomplete partition-III, 10 (%7.2), common
cavity, 8 (5.8%) and dilatation of vestibule, 3 (2.1%) patients. The
significant difference was seen in Ling’s sound test and auditory
perception test battery scores of children with incomplete partition-I,
cochlear hypoplasia, and common cavity (p-value < .005).
Conclusion: IEMs group showed different progress according to the type
of ear anomaly. Although CI users with enlarged vestibular aqueduct
(EVA) had the highest scores, users with common cavity had the lowest
scores. Taking these results, caused by anatomical differences, in to
account is very critical in follow-ups and rehabilitation programs. Each
cochlear implant user should be evaluated according to his/her
individual needs.