Beginning the Process

On Tuesday we met with the coordinator at the CDRC Pediatric Hearing Amplification Service at Doernbecher Children’s Hospital. This is our first step in beginning the process to determine if Ben is a candidate for a Cochlear Implant. Just because he is deaf does not automatically make an implant possible. He will need to undergo several tests to determine the cause of his deafness and to be certain an implant will be effective.

The first test Ben will need is a CAT scan (x-ray) and MRI of the inner ear. These will show the condition of the cochlea and the auditory nerve. The doctor will be looking to see if the cochlea has any ossification (bony growth) or is malformed. Neither of these conditions would prevent an implant, but may determine how well the implant will work or be a deciding factor of which ear to implant. Though the implant is placed within the cochlea, sound must be transmitted to the brain via the auditory nerve. If this nerve is not intact or is not present, the implant will not be possible in that ear. Ben will have his MRI and CAT scan on April 3rd.

We also discussed possible causes for Ben’s deafness. Unfortunately, congenital deafness can be one of the symptoms of a syndrome, with other symptoms showing up later in life. The doctor suggested our first step be genetic counseling. A very common cause of congenital deafness is a genetic condition called Connexin 26. Connexin kids tend to do very well with cochlear implants. Their hearing anatomy is all present and intact. The only problem is that the hair cells along the basilar membrane, which translate mechanical sound energy into nerve impulses, are defective, due to a lack of Connexin protein. Finding out Ben has Connexin 26 would be positive.

When Ben is older, around six or seven months, he will undergo some cognitive testing. These tests will be used to help determine the possible results of the implant. If Ben has developmental delays, speech and language may be harder for him to grasp, even with an implant.

Doernbecher Hospital performed about 55 implants last year. All of those parents went though this process. All of those parents survived. All of those kids are now hearing. We are riding on the optimism they provide.