Introduction: Auditory brainstem implantation (ABI) may be beneficial for deaf children with cochlear nerve deficiency (CND) that fail to benefit from cochlear implantation (CI). ABIs could provide auditory awareness, speech perception and language development, thereby improving educational achievement and quality of life. We introduce the largest US clinical trial of ABIs in children with CND.
Methods: Five pre-linguistic deaf children (ages 2-5 years) with CND underwent retrosigmoid craniotomy and electrode array placement on the dorsal cochlear nucleus within the foramen of Luschka. Auditory brainstem responses (ABRs) were recorded intraoperatively to confirm placement of the investigational device. Device mapping was planned at 4 weeks and comprehensive auditory and speech perception testing carried out at planned intervals.
Results: Intraoperative ABR confirmed responses in 4 of 5 children. Perioperative complications were limited to 1 case each of CSF leak and aseptic meningitis that resolved without sequelae. All patients developed sound detection with 4/5 patients having speech detection thresholds of < 20 dB HL. Parental reports (IT-MAIS) demonstrated 3 of 5 children having scores of > 50%. Three of 5 children can identify songs with > 75% accuracy. Early speech perception testing shows 2 of 5 children with greater than chance closed set speech perception (ESP-monosyllables). These results continue to evolve with time.
Conclusions: Untreated deafness profoundly impacts the course of a child’s life. We demonstrate the ABI can be safely implanted in young children with CND. Auditory awareness can be anticipated in all subjects, usually at levels that can support speech perception and language development. While still very early, receptive and expressive language skills are evolving, albeit at slower rates than children with CIs. In carefully selected children with CND, early results suggest the ABI can provide meaningful sound awareness that may significantly alter the child’s long-term quality of life.
Patient Care: This clinical trial provides the framework for, and better understanding of, the continued implantation of auditory brainstem implants for children with cochlear nerve deficiency who would otherwise be limited in ability to develop speech perception and language development. Implantation of this investigational device may significantly improve the quality of life for these patients.
Learning Objectives: By the conclusion of this session participants will understand the indications for investigational auditory brainstem device implantation, surgical considerations, and auditory functional outcomes for children with cochlear nerve deficiency.