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Cochlear implantation in cochlear nerve deficiency: A systematic review.
Alexa N Pearce1, Peter Eckard1, Andrew R Mangan1
1Department of Otolaryngology-Head and Neck Surgery, Division of Head and Neck Oncology and Surgery, University of Arkansas for Medical Sciences, Little Rock, AR, United States of America.
American Journal of Otolaryngology
|June 28, 2025
Summary
Cochlear implantation (CI) can improve hearing in children with cochlear nerve deficiency (CND), but results vary. Counseling on potential limitations and alternative treatments like auditory brainstem implantation is crucial.
Area of Science:
- Otolaryngology
- Neuroscience
- Audiology
Background:
- Cochlear nerve deficiency (CND) presents unique challenges for hearing restoration.
- Cochlear implantation (CI) is a potential treatment, but outcomes in CND patients require further investigation.
Purpose of the Study:
- To systematically review the literature on outcomes following cochlear implantation in patients diagnosed with cochlear nerve deficiency.
Main Methods:
- A comprehensive literature search was conducted using PubMed, MEDLINE, and Google Scholar for studies published between January 2000 and December 2023.
- The search focused on articles detailing cochlear implantation in patients with cochlear nerve agenesis or deficiency, adhering to PRISMA guidelines.
Main Results:
- The review included 28 articles encompassing 514 patients and 513 cochlear implantations.
- Significant improvements were observed in average post-operative Categories of Auditory Performance (CAP) from 0.5 to 3.6 and hearing thresholds from 101.3 dB to 42.5 dB.
- While 85.5% had no pre-operative auditory brain response, only 4.2% showed no response post-implantation.
Conclusions:
- Cochlear implantation offers potential benefits for hearing and speech development in children with CND.
- Outcomes can be variable, particularly in cases of severe cochlear nerve hypoplasia or aplasia.
- Pre-operative counseling regarding potential limitations and alternative options, such as auditory brainstem implantation, is recommended.
