Cochlear implantation versus auditory brainstem implantation in children with auditory nerve deficiencies.

BACKGROUND: Cochlear nerve deficiency is one of the known causes of congenital sensorineural hearing loss. Management of hearing loss in children with cochlear nerve deficiency poses a multidimensional challenge. The absent or hypoplastic cochlear nerve may prevent electrical stimulation from reaching the brainstem and the auditory cortex. A deficient cochlear nerve can be associated with other inner ear malformations, which may diminish the success of cochlear implantation in those children. Promising results in adults after auditory brainstem implantation led to the expansion of candidacy to include the pediatric populations who were contraindicated for CIs. OBJECTIVE: To review the outcomes of cochlear implantation versus that of auditory brainstem implantation in children with various conditions of the auditory nerve. METHODS: This retrospective chart review study comprised two pediatric groups. The first group consisted of seven ABI recipients with cochlear nerve aplasia and the second group consisted of another seven children with cochlear nerve deficiencies who underwent CI surgery. The participants' auditory skills and speech outcomes were assessed using different tests selected from the Evaluation of Auditory Responses to Speech (EARS) test battery. RESULTS: There were some individual variations in outcomes depending on the status of the auditory nerve. The mean CAP score of the ABI group was 2.87, while the mean SIR score was 0.62. On the other hand, the mean CAP score of the CI group was 1.29, while the mean SIR score was 0.42. CONCLUSION: Our results are in good agreement with the reported auditory perception and speech and language development outcomes of pediatric auditory brainstem implantation. We added to the growing body of literature on the importance of verifying and identifying the status of the cochlear nerve in the decision-making process of the surgical management of those pediatric groups.

Audiologic Outcome of Cochlear Implantation in Children With Cochlear Nerve Deficiency.

OBJECTIVE/HYPOTHESIS: The aim of this study was to investigate cochlear implantation (CI) outcome in children with nerve deficiency. STUDY DESIGN: Retrospective chart review. METHODS: A total of seven children with prelingual profound deficiency (hypoplasia or aplasia) were included. A control group of 10 CI children with no cochlear nerve anomalies was also included. In addition to implant stimulation levels, children's performance on pure-tone audiometry, speech reception measure, and auditory and speech skills ratings were compared across groups. Additionally, pre- and postoperative audiologic results were evaluated for the group with nerve deficiency. RESULTS: In general, children with nerve deficiency performed poorer than those without nerve deficiency on all tested measures. Stimulation levels were considerably higher and more variable than the control group. Results further showed that performance was dependent on the diameter of the internal auditory canal. CONCLUSION: Overall, cochlear implantation outcome in children with auditory nerve deficiency is poorer and extremely more variable than those without nerve deficiency. However, three of the patients had a noticeable improvement in auditory performance postimplantation suggesting that CI is a viable option in this population but expected benefit can be dependent on the status of the cochlear nerve.

Auditory and language skills development after cochlear implantation in children with multiple disabilities.

BACKGROUND: Cochlear implantation (CI) in children with additional disabilities can be a fundamental and supportive intervention. Although, there may be some positive impacts of CI on children with multiple disabilities such as better outcomes of communication skills, development, and quality of life, the families of those children complain from the post-implant habilitation efforts that considered as a burden. OBJECTIVE: To investigate the outcomes of CI children with different co-disabilities through using the Meaningful Auditory Integration Scale (MAIS) and the Meaningful Use of Speech Scale (MUSS) as outcome measurement tools. METHODS: The study sample comprised 25 hearing-impaired children with co-disability who received cochlear implantation. Age and gender-matched control group of 25 cochlear-implanted children without any other disability has been also included. The participants' auditory skills and speech outcomes were assessed using MAIS and MUSS tests. RESULTS: There was a statistically significant difference in the different outcomes measure between the two groups. However, the outcomes of some multiple disabilities subgroups were comparable to the control group. Around 40% of the participants with co-disabilities experienced advancement in their methods of communication from behavior to oral mode. CONCLUSION: Cochlear-implanted children with multiple disabilities showed variable degrees of auditory and speech outcomes. The degree of benefits depends on the type of the co-disability. Long-term follow-up is recommended for those children.