Related Experiment Video
Updated: Jul 29, 2026
Performing Intracochlear Electrocochleography During Cochlear Implantation
Published on: March 8, 2022
[Cochlear implantation in children with inner ear malformation and postoperative performance]
Insights
Cochlear implantation in children with cochlear malformations shows promising speech reception and word acquisition. However, challenges like surgical complications and poor phonetic expression require further investigation for improved outcomes.
Area of Science:
- Otolaryngology
- Pediatric Surgery
- Audiology
Background:
- Cochlear implantation is increasingly used for children with cochlear malformations.
- Limited case reports exist, particularly in Japan, highlighting a need for more data.
Observation:
- The study analyzed five children (six ears) with congenital inner ear malformations, including common cavity and incomplete partition deformities.
- Surgical approaches varied based on malformation type: transmastoid facial recess for incomplete partition and transmastoid labyrinthotomy for common cavity.
- Complications observed included facial twitching, cerebrospinal fluid gusher, and flap-related issues, with one case requiring explantation due to infection.
Findings:
- All implanted children demonstrated satisfactory speech reception and word acquisition.
- Facial twitching was noted when the electrode array entered the internal auditory canal.
- Cerebrospinal fluid gusher and flap breakdown were managed with revision surgery or explantation.
Implications:
- Cochlear implantation can yield positive results in speech perception for children with cochlear malformations.
- Careful surgical technique and management of complications are crucial for successful outcomes.
- Further research is needed to address limitations in phonetic expression and optimize long-term results.
Abstract:
Cochlear implantation in children with cochlear malformation is on the rise. However, only one case has been reported in Japan. The objective of this paper is to report the authors' experiences with cochlear implants in children with cochlear malformations and, to investigate the morphological classification of cochlear malformation, surgical procedure, complications, and speech perceptions after implantation. Five children (6 ears, including 1 Usher's syndrome and 1 CHARGE association) with congenital inner ear malformations were implanted with multichannel cochlear implants (Nucleus Mini 22 device) between 1994 and 1998. Malformations included common cavity deformity 2, and incomplete partition 4. On the other hand, 4 cases with incomplete partition deformity underwent cochlear implantation by the transmastoid facial recess approach, 2 patients with common cavity deformity received implantation by the transmastoid labyrinthotomy approach. Complications such as facial twitching, cerebrospinal fluid (CSF) gusger, and flap-related problems were encountered. Facial twitching occurred in 1 of 2 patients with common cavity where the electrode array extended into the internal auditory canal. CSF gusher was encountered in 1 of 4 patients with incomplete partition, and revision surgery was performed one week later. One patient had delayed cochlear implant infection and a split pericranial flap was used to cover the defect in the skin flap. Within 6 months, however, the revised pedestal site broke down because of recurrent flap infection. Finally, the cochlear implant was explanted, and the patient underwent a new cochlear implantation in the contralateral ear. All the patients had satisfactory speech reception and word acquisition, but still poor phonetic expression.
