Auditory Brainstem Implant (ABI) in Pediatric Non-Neurofibromatosis Type 2 Subjects

Exclusion criteria

• . Pre- or post-linguistic child currently making significant progress with CI - This will be considered if a child is progressing along the expected speech reception hierarchy (SRI-Q) as detailed by Wang et al (50). Even for the very young children (18 months of age with 6 months of use), nearly all children with a good auditory signal from their CI will have reached ceiling effects on the IT-MAIS and have pattern perception beyond chance using the ESP (50). Moreover, there will evidence of improvement in these metrics over time.
• . MRI evidence of one of the following:
• . Clear surgical reason for poor CI performance that can be remediated with revision CI or contralateral surgery rather than ABI.
• . Intractable seizures or progressive, deteriorating neurological disorder
• . Unable to participate in behavioral testing and mapping with their CI. If this appears to be an age effect, ABI will be delayed until we can be assured that the child will be able to participate, as reliable objective measures of mapping are currently not available for mapping these devices.
• . Lack of potential for spoken language development. This will be considered the case when evidence of the following exist:
• . Unable to tolerate general anesthesia (cardiac, pulmonary, bleeding diathesis, etc.).
• . Need for brainstem irradiation