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Published on: February 29, 2020
Bilateral implantation in children with cochleovestibular nerve hypoplasia
1ENT Department, Armand Trousseau Children's Hospital, 75012 Paris, France. n.oker@hotmail.fr
Insights
Sequential bilateral cochlear implantation (CI) in children with inner ear malformation improved hearing thresholds. Bilateral CI may benefit children with cochleovestibular nerve hypoplasia when unilateral implantation yields limited results.
Area of Science:
- Otolaryngology
- Pediatric Audiology
- Neurosurgery
Background:
- Inner ear malformations, including cochleovestibular nerve hypoplasia, present significant challenges for hearing rehabilitation in children.
- Profound bilateral congenital hearing loss necessitates advanced audiological interventions.
Purpose of the Study:
- To evaluate the outcomes of sequential bilateral cochlear implantation (CI) in pediatric patients with inner ear malformations.
- To assess the impact of bilateral CI on auditory thresholds and speech perception in cases of cochleovestibular nerve hypoplasia.
Main Methods:
- Retrospective case study involving two children with bilateral profound hearing loss and inner ear malformations.
- Sequential bilateral cochlear implantation performed at different ages, with audiological and speech perception assessments post-implantation.
Main Results:
- Unilateral cochlear implantation resulted in mean hearing thresholds of 60 dB and 70 dB.
- Sequential bilateral cochlear implantation improved mean hearing thresholds to 40 dB and 55 dB.
- Case 1 demonstrated significant speech perception improvements (83% CSW, 70% OSW) after one year, while Case 2 showed no significant linguistic evolution at six months.
Conclusions:
- Sequential bilateral cochlear implantation can enhance audiological outcomes in children with inner ear malformations.
- Bilateral CI should be considered for patients with cochleovestibular nerve hypoplasia experiencing limited benefits from unilateral implantation.
Abstract:
To report on the outcomes of sequential bilateral cochlear implantation (CI) in children with inner ear malformation. The study design is a retrospective case study. The setting is a tertiary reference center. Two children presenting a profound bilateral congenital hearing loss with bilateral hypoplasia of the cochleovestibular nerves and hypoplasic external semicircular canal had a cochlear implant at respectively 16 months and 33 months. A second implant was proposed at respectively 17 and 20 months after the first implant. The main outcome measures are audiometry, perceptive results in closed and open set words (CSW and OSW) and oral production at follow-up. The first cochlear implant gave respectively mean thresholds at 60 dB and 70 dB. Bilateral CI showed mean threshold at respectively 40 dB and 55 dB. In case 1, perceptive assessment was 83% and 70% in respectively CSW and OSW with oral production and comprehension of sentences after 1 year follow-up. In case 2, the perceptive assessment showed no perceptive or linguistic evolution at 6 months follow-up. In cochleovestibular nerve hypoplasia, bilateral implantation could be discussed in cases of limited result after unilateral implant.
