Cochlear implants in children with congenital inner ear malformations

Abstract

Objective: To describe clinical experiences with multichannel cochlear implantation in children with inner ear malformations, including surgical indications and techniques, imaging findings, and outcomes.

Design: A retrospective review of a series of 10 consecutive cases with a mean follow-up of 29 months, as well as a review of the literature.

Setting: Academic referral center.

Subjects: Ten children who underwent multichannel cochlear implantation for inner ear malformations. High-resolution computed tomographic scans demonstrated a common cavity deformity in 3, an incomplete cochlear partition in 4, and an enlarged vestibule in 1. Two had membranous anomalies as indicated by cerebrospinal fluid gushers at surgery, but the results of imaging were normal.

Intervention: All subjects received multichannel cochlear implants. Two subjects underwent mastoid obliteration at the time of implantation owing to preoperative recurrent meningitis or chronic otitis media with episodes of clinical mastoiditis.

Main outcome measures: The 10 subjects were evaluated for electrode insertion and stability and auditory function for up to 7 years.

Results: All 22 electrodes are functional in each child with an incomplete partition, an enlarged vestibule, or a membranous anomaly. Of 3 subjects with common cavities, 2 had full insertion of electrodes and 1 had 16 electrodes inserted. All subjects had speech awareness thresholds detected at 25 dB or better. Three (75%) of the 4 subjects with at least 30 months of experience, including 1 subject with a common cavity, have developed open-set word recognition.

Conclusions: Electrode insertion and hearing results in children with an incomplete partition, an enlarged vestibule, or a membranous anomaly are similar to those in children with normal cochleas. Specific surgical techniques are effective for children with a common cavity, and the results are less certain. Cerebrospinal fluid gushers were encountered frequently but were not difficult to control.