Hearing Loss, Bilateral (Causes Other Than Tumors) Clinical Trial
Official title:
Auditory Brainstem Implant (ABI) in Children With No Cochleae or Auditory Nerves
This study is a feasibility study of the Nucleus 24 ABI in children without NF2. It will be conducted as a repeated-measures, single subject experiment in order to accommodate the known variability in cochlear implant clinical results. Data obtained in the course of this study will be compared with the existing published outcome data from children with normal hearing and with cochlear implants (CIs). These comparisons may provide additional useful information about the progress of ABI subjects' performance over time. Also, this may allow the results of the current study to be utilized to design future pivotal studies of the device in the pediatric population.
The purpose of this study is to evaluate the clinical safety and efficacy of the Nucleus™ 24 Auditory Brainstem Implant (ABI) in pediatric patients who do not have Neurofibromatosis Type 2 (NF2); specifically, children with total hearing loss due to severe cochlear anomalies, cochlear nerve disorders or failed cochlear implantation. These conditions can include: developmental or acquired cochlear nerve deficiency (CND), cochlear aplasia, post-meningitis cochlear ossification, or cochlear malformation. This study proposes to implant up to 10 pre-linguistic young children (18 mos to 5 yrs. of age) and up to 10 post-linguistic children (<21 yrs of age) with the Nucleus 24 Multichannel ABI in an attempt to demonstrate safety of the surgical procedure and device stimulation and the potential for auditory benefit beyond that experienced with a CI. These children would not qualify for the Nucleus ABI device, as their etiology is not an approved indication such as neurofibromatosis Type 2(NF2). Further, these children do not benefit from conventional hearing aids or cochlear implants. Two groups of children will be included: Group 1 will include prelingiustic deaf children ages 18 months - 5 years and Group 2 will include postlinguistic deaf children < 21 years of age. Post-operative evaluations will be conducted at the initial activation and at 1, 3, 6, 12, 18, 24, 30, and 36-month intervals post-activation in order to demonstrate the emergence of open-set speech perception abilities prior to study cessation. Experience in the CI literature has shown that a minimum of 2 years of device usage is required for children with pre-lingual hearing impairment to develop some open-set speech perception abilities. While Group 2 is post-linguistic and some may have had significant hearing prior to becoming deaf, the same time intervals will be used for consistency. Blinding or masking procedures are not included in the design, as the presence/absence of a brainstem implant cannot be easily concealed from the device recipients and/or clinical investigators. ;