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Published on: February 29, 2020
Congenital malformation of the inner ear and pediatric cochlear implantation
Emmanuel A M Mylanus1, Liselotte J C Rotteveel, Rens L Leeuw
1Department Otorhinolaryngology, University Medical Center, Nijmegen, The Netherlands. e.mylanus@kno-azn.nl
Insights
Cochlear implantation in children with inner ear malformations shows promising results, even in severe cases like common cavity deformities. However, surgical challenges and uncertain outcomes necessitate thorough parental counseling.
Area of Science:
- Otolaryngology
- Pediatric Surgery
- Audiology
Background:
- Inner ear malformations present unique challenges for hearing restoration.
- Cochlear implantation is a viable option for profound hearing loss in children.
- Understanding surgical nuances and outcomes in this population is crucial.
Purpose of the Study:
- To evaluate the surgical aspects and performance outcomes of cochlear implantation in pediatric patients with inner ear malformations.
- To analyze complications and speech perception results in this specific patient group.
Main Methods:
- Retrospective analysis of 13 pediatric patients undergoing cochlear implantation.
- Inclusion of a literature review on similar cases.
- Evaluation of surgical data, postoperative follow-up, and audiometric results.
Main Results:
- No major surgical complications were reported.
- Challenges included locating the scala tympani in cases with abnormal cochlear position and middle ear disease.
- Cerebrospinal fluid gushers and aberrant facial nerves occurred but without sequelae.
- Patients with mild malformations (e.g., incomplete partition) showed good speech perception.
- A child with a severe common cavity malformation achieved open-set speech perception.
Conclusions:
- Cochlear implantation can yield positive outcomes in children with diverse inner ear malformations, including severe ones.
- The incidence of aberrant facial nerves is significant in severe cochlear malformations (up to 27%).
- Speech perception results vary, and preoperative counseling must address the inherent uncertainties, especially in complex cases.
Objectives:
To study the surgical aspects and performance outcome of cochlear implantation in children with malformed inner ears.
Study Design:
Clinical and audiometric evaluation in 13 patients.
Methods:
Patient data concerning surgery, postoperative follow-up, and pre- and postimplantation audiometry were obtained from the cochlear implant center's database and evaluated. A review of the literature has been included.
Setting:
Tertiary referral center.
Patients:
The patients had a variety of inner ear malformations and profound hearing loss. One patient with recurrent meningitis had a severe cochlear malformation (common cavity).
Results:
Major complications did not occur. In one patient with an abnormal position of the cochlea and concurring middle ear disease, it was difficult to find the scala tympani during surgery. A cerebrospinal fluid gusher was encountered in two patients and an aberrant facial nerve in another, which did not lead to any complications. The patients with mild cochlear malformation such as an incomplete partition demonstrated a good performance in speech perception tests. Even the child with the common cavity deformity had some open-set speech perception 1 year after implantation.
Conclusions:
Viewing the patients from this study and patients from a review of the literature concerning cochlear implantation in children with malformed inner ears including severe cochlear malformations, the occurrence of an aberrant facial nerve was 17%, which increases to 27% if one reviews the surgical findings in children with severe malformed cochleae such as a common cavity or a severe cochlear hypoplasia. In the latter patients, results in speech perception vary. Although the result of cochlear implantation may be promising, as in our patient with a common cavity, during preoperative counseling the child's parents must be informed that the result is uncertain.
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