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Author Spotlight: Advancements in Impedance Monitoring for Cochlear Implant Surgery
Published on: August 4, 2023
Clinical outcomes following cochlear implantation in children with inner ear anomalies
Amal Isaiah1, Daniel Lee2, Felicity Lenes-Voit1
1Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Insights
Cochlear implantation (CI) in children with inner ear malformations (IEMs) is safe but may yield lower speech perception. Outcomes are comparable to normal anatomy only when an enlarged vestibular aqueduct is present.
Area of Science:
- Otolaryngology
- Pediatric Audiology
- Medical Imaging
Background:
- Congenital hearing loss affects many children.
- Inner ear malformations (IEMs) occur in a significant proportion of pediatric cochlear implant (CI) candidates.
- Limited data exists on surgical and speech outcomes for CI in children with IEMs.
Purpose of the Study:
- To report surgical and speech outcomes of cochlear implantation in children with inner ear malformations.
- To determine the prevalence of IEMs in children evaluated for CI.
- To identify specific IEMs associated with varying speech perception outcomes.
Main Methods:
- Retrospective chart review of children (age 1-18) evaluated for CI between 1986-2014.
- Inclusion criteria: bilateral severe-profound sensorineural hearing loss, limited benefit from amplification; Exclusion criteria: neurodevelopmental disorders, lack of speech assessment.
- Outcome measures included imaging findings (MRI/CT), intraoperative complications, and speech perception (closed-set, open-set, none).
Main Results:
- Prevalence of IEMs was 27% (102/381), with 79% being bilateral.
- Cochlear dysplasia was the most common anomaly (30%).
- Surgery was challenging in 24% of cases. Cochlear dysplasia, vestibular dysplasia, and cochlear nerve hypoplasia correlated with poor speech perception (0-23%), while enlarged vestibular aqueduct showed outcomes similar to normal anatomy (65%).
Conclusions:
- Cochlear implantation is a safe procedure for children with IEMs.
- Speech perception outcomes in children with IEMs are generally lower than in those with normal inner ear anatomy.
- Enlarged vestibular aqueduct is an exception, with outcomes comparable to children without IEMs.
Objective:
A significant proportion of children with congenital hearing loss who are candidates for cochlear implants (CIs) may have inner ear malformations (IEMs). Surgical and speech outcomes following CI in these children have not been widely reported.
Methods:
The charts of children who were evaluated for a CI between 1/1/1986 and 12/31/2014 at a university-based tertiary level pediatric cochlear implant center were reviewed. Principal inclusion criteria included (i) age 1-18 years, (ii) history of bilateral severe to profound sensorineural hearing loss, and (iii) limited benefit from binaural amplification. Exclusion criteria included (i) underlying diagnosis of neurodevelopmental disorder and (ii) lack of follow up for speech assessment if a CI was performed. The following outcome measures were reviewed: (i) imaging findings with magnetic resonance imaging or high resolution computed tomography, (ii) intraoperative complications, and (iii) speech perception categorized as the ability to perceive closed set, open set, or none.
Results:
The prevalence of IEMs was 27% (102 of 381), of which 79% were bilateral. Cochlear dysplasia accounted for 30% (40 of 136) of the anomalies. Seventy-eight of the 102 patients received a CI (78%). Surgery was noted to be challenging in 24% (19 of 78), with a perilymphatic gusher being the most common intraoperative finding. Cochlear dysplasia, vestibular dysplasia and cochlear nerve hypoplasia were associated with poor speech perception (open OR closed set speech recognition scores, 0-23%), although the outcomes in children with enlarged vestibular aqueduct were similar to those of children with normal inner ear anatomy (65%).
Conclusions:
Cochlear implantation is safe in children with IEMs. However, the speech perception outcomes are notably below those of patients with normal anatomy, with the exception of when an enlarged vestibular aqueduct is present.
