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Pediatric cochlear implantation of children with eighth nerve deficiency
Nancy M Young1, Francine M Kim, Maura E Ryan
1Section of Otology and Neurotology, Division of Pediatric Otolaryngology, Ann & Robert H. Lurie Children's Hospital of Chicago, United States. nyoung@luriechildrens.org
Insights
Cochlear implantation can benefit some children with eighth nerve hypoplasia or aplasia. Preoperative aided auditory detection predicted better outcomes, but imaging findings did not predict post-implantation auditory performance.
Area of Science:
- Otolaryngology
- Pediatric Audiology
- Neurosurgery
Background:
- Eighth nerve hypoplasia/aplasia presents challenges for auditory development.
- Cochlear implantation is a potential intervention for hearing loss in these cases.
Purpose of the Study:
- To assess auditory outcomes in children with eighth nerve hypoplasia/aplasia following cochlear implantation.
- To identify preoperative predictors of auditory performance.
Main Methods:
- Retrospective case review of ten children with eighth nerve hypoplasia/aplasia.
- Auditory performance evaluated using neural response tests, detection/speech awareness thresholds, and speech perception levels.
- High-resolution magnetic resonance imaging (HR-MRI) used for neural assessment.
Main Results:
- Post-implantation, outcomes ranged from no sound detection to open-set speech perception in three children.
- No correlation found between imaging findings and auditory outcomes.
- Preoperative aided auditory detection significantly predicted better implant detection thresholds and Meaningful Auditory Integration Scale scores.
Conclusions:
- Cochlear implantation can provide significant auditory benefits for select children with eighth nerve hypoplasia/aplasia.
- HR-MRI is more sensitive than HR-CT for detecting neural deficiency.
- Imaging findings were not predictive of post-implantation auditory success.
Objective:
To evaluate the auditory outcomes of children implanted in an ear with eighth nerve hypoplasia or aplasia and to determine whether preoperative characteristics are predictive of auditory performance achieved.
Study Design:
retrospective case review.
Setting:
tertiary care medical center.
Patients:
ten children implanted in an ear with eighth nerve hypoplasia or aplasia, as determined by high resolution magnetic resonance imaging.
Main Outcome Measures:
Neural response test measurements, detection and speech awareness thresholds, Meaningful Auditory Integration Scale scores, as well as speech perception level achieved.
Results:
Post-implantation, three children demonstrated little to no detection of sound, three had improved detection and awareness of environmental sounds, one developed closed-set speech perception and spoken language, and three developed open-set speech perception and spoken language. No imaging findings appeared related to outcomes. Significantly better implant detection thresholds and Meaningful Auditory Integration Scale scores were found in children who had preoperative aided auditory detection (p's ≤ 0.02-0.05).
Conclusion:
Some children with eighth nerve hypoplasia or aplasia may derive significant benefit from a cochlear implant. In our study high resolution magnetic resonance imaging was more sensitive than high resolution computer tomography in detecting neural deficiency. However, no imaging findings were predictive of auditory performance level achieved post-implantation.