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Published on: January 9, 2019
The Therapeutic Dilemma of Cochlear Nerve Deficiency: Cochlear or Brainstem Implantation?
Liliana Colletti1, Giacomo Colletti2, Marco Mandalà2
1ENT Department, University of Verona, Verona, Italy.
Insights
Children with cochlear nerve deficiency who received auditory brainstem implants (ABI) showed significant speech development. Cochlear implants (CI) did not yield speech understanding in these children, though complication rates were similar.
Area of Science:
- Otolaryngology
- Neuroscience
- Pediatric audiology
Background:
- Cochlear nerve deficiency presents a unique challenge for hearing restoration in children.
- Traditional cochlear implants (CI) may not provide sufficient auditory input when the cochlear nerve is absent or underdeveloped.
Purpose of the Study:
- To compare the auditory and speech outcomes of children with cochlear nerve deficiency who received either auditory brainstem implants (ABI) or cochlear implants (CI).
Main Methods:
- A retrospective cohort study compared two age-matched groups of children with cochlear nerve deficiency.
- Performance was assessed using the Category of Auditory Performance scale, with a mean follow-up of 5 years.
- Imaging confirmed absent or small cochlear nerves in both groups.
Main Results:
- Children with CI demonstrated limited auditory performance (0-3 CAP scores), requiring manual communication and visual aids.
- Children with ABI achieved higher auditory performance (2-7 CAP scores), enabling open-set speech perception and verbal language acquisition.
- No significant difference in complication rates was observed between ABI and CI groups.
Conclusions:
- Auditory brainstem implants offer a viable solution for hearing restoration and speech development in children with cochlear nerve deficiency.
- Cochlear implants are less effective for this specific population, failing to promote speech understanding.
- ABI facilitates mainstream education and oral communication, with comparable safety to CI.
Objective:
To compare the outcomes between 2 age-matched cohorts of children with cochlear nerve deficiency: those receiving auditory brainstem implants (group A) or cochlear implants (group B).
Study Design:
Retrospective cohort study.
Setting:
Tertiary referral center.
Subjects And Methods:
Subjects were selected from a pool of 537 children fitted with cochlear implants (n = 443) or auditory brainstem implants (n = 94) over the past 14 years. Performance, examined with the Category of Auditory Performance scale, and complications were compared with a mean follow-up of 5 years.
Results:
All children had bilateral profound sensorineural hearing loss and cochlear nerve deficiency. Magnetic resonance imaging documented an absent cochlear nerve (n = 12) and a small cochlear nerve (n = 8) in group A and an absent cochlear nerve (n = 11) and a small cochlear nerve (n = 9) in group B (P = 1.000). Children with cochlear implants had Category of Auditory Performance scores spanning from 0 to 3 levels of performance, and all required manual communication mode and visual supplementation. Children with auditory brainstem implants had Category of Auditory Performance scores spanning from 2 to 7, and most patients demonstrated behavioral responses irrespective of inner ear malformations and an absent cochlear nerve or small cochlear nerve (P < .001).
Conclusions:
In children with cochlear nerve deficiency, patients fitted with cochlear implants did not develop speech understanding and production. Those fitted with auditory brainstem implants had the opportunity to develop open-set speech perception, acquiring verbal language competence using oral communication exclusively and participating in mainstream education. The overall complication rate of auditory brainstem implants was not greater than that of cochlear implants.
