ObjectiveTo 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 DesignRetrospective cohort study.SettingTertiary referral center.Subjects and MethodsSubjects 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.ResultsAll 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).ConclusionsIn 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.