Auditory Brainstem Implantation in Children and Non-Neurofibromatosis Type 2 Patients

Abstract

On the 18th of September 2009, a group of health care professionals and scientists involved in implantation of the auditory system attended a meeting convened by the Hacettepe Cochlear Implant Group. The aim of the meeting was to have a detailed discussion on the pressing and pertinent issues around auditory brainstem implantation (ABI) in children and in non-neurofibromatosis Type 2 (NF2) cases and to reach a consensus based on these discussions. surgery, experience of individual ABI centers, intraoperative issues, and rehabilitation with the final session devoted to discussion of the issues raised. The centers presented their experience of ABI in children who had hearing impairment because of congenital abnormalities or pathology where cochlear implantation (CI) was either contraindicated or the possibility of successful placement of the electrode was unlikely. Altogether, 61 children with various types of inner ear malformations, cochleovestibular nerve (CVN) anomalies, cochlear ossification, and bilateral cochlear fractures with cochlear nerve avulsion were presented by different groups. The following topics were discussed, and a consensus was obtained at the end of the meeting on the following issues. IN WHICH CHILDREN AND NON-NF2 PATIENTS IS THE ABI A VIABLE INTERVENTION?Two patient categories were identified: 1) Prelingual patients with inner ear malformations and cochlear nerve hypoplasia/aplasia. ABI provides auditory perception in most patients. The potential for speech and language acquisition in the longer term will depend on the age of implantation, the presence or absence of additional disabilities, and the other established factors seen in CI. It also was concluded that open set speech discrimination is possible in selected cases. In addition, prelingually deafened children because of meningitis with total ossification of both cochleas also should be included in this group. 2) Individuals deafened postlingually because of meningitis, temporal bone fractures with cochlear nerve avulsion, otosclerosis with gross cochlear destruction, or unmanageable facial nerve stimulation with CI. As with CI, the duration of severe or profound deafness is a prognostic indicator, and with short

Recent Rehabilitation Experience with Pediatric ABI Users

“…All children used their ABI regularly on a daily basis, and similar to the findings in the literature, they were found to develop awareness toward environmental sounds and speech sounds after 1-month to 3-month intervals [11][12][13][14] . Functionality of auditory perception skills was mostly evaluated with MAIS/IT-MAIS in researches; however, in our research, FAPCI was conducted to determine auditory functioning and compared with normally hearing population.…”

Usefulness of MRI and EABR Testing for Predicting CI Outcomes Immediately After Cochlear Implantation in Cases With Cochlear Nerve Deficiency

“…Colletti et al reported that in the population of congenital deaf children with CND, auditory performance evaluated by category of auditory performance (CAP) score was significantly higher in patients with ABI than in those with CI (8). The indication for auditory brainstem implantation should be decided carefully in prelingually deaf children, however, because the long-term outcomes of ABI have not been established in this population (7). Given that the more peripheral stimulation from CIs usually provides much better speech discrimination than achieved with ABI (9), it is recommended that children with CND who have sufficient inner ear space for electrode insertion should undergo cochlear implantation initially, with auditory brainstem implantation then considered if CI outcomes are poor.…”

“…Recently, as the effectiveness and safety of an auditory brainstem implant (ABI) has been established in postlingually deaf adults with neurofibromatosis type 2 (NF2), the use of ABI has expanded to non-NF2 patients including prelingually deaf children with congenital malformation (7). Because an ABI bypasses the cochlear nerve and directly stimulates the cochlear nucleus, a patient with CND may benefit from ABI.…”

“…The consensus statement published in 2011 emphasized that ABI surgery should preferably not be done later than 3 years of age in prelingually deaf patients (7). Therefore, when cochlear implantation precedes auditory brainstem implantation, precise early prediction of CI outcomes is important to determine if there may be an indication for ABI.…”

Early Intervention for Hearing Impaired Children. Istanbul, Turkey, May 21-22, 2013: Abstracts

“…A corner audiogram with profound hearing thresholds at the low frequencies only is likely to indicate a vibrotactile response. Management: As there is no inner ear development, auditory brainstem implantation (ABI) is the only option for hearing restoration [Sennaroglu et al, 2011]. The electrode is placed into the lateral recess to directly stimulate the cochlear nuclei via retrosigmoid approach.…”