Not What We Hoped

James had a repeat hearing test at the hospital today. This is a test that measures brainwave activity in response to sounds while the baby is sleeping. (And, as it turns out, not sucking on a pacifier or making sucking motions, which meant that it was difficult to get good measures on little paci-loving James!)

The results indicated that he has a (rare) hearing disorder called auditory neuropathy. Roughly, this means that sound signals aren't getting transmitted properly between the inner ear and the brain -- could be the result of faulty cochlea or auditory nerve. The test measures electrical signals in the ear and brain to identify a breakdown in the transmission of the auditory signal, but it can't tell us how James's hearing is -- kids with this condition can have anything from normal hearing (about 7%) to profound hearing loss/deafness. They'll have a better sense of where he is with it in a few months when they can do more behavioral testing. Our guess is that he's somewhere in the middle, and can hear some things but also has some hearing problems. (The audiologist thought that he was startling at around the 80 decibel level, which is about as loud as a raised voice -- but that's just her informal impression, nothing truly diagnostic.)

This was depressing news, but the bright side is that they now have pretty good treatments for it. At the lower levels of hearing loss, they use hearing aids, and at higher levels, they do cochlear implants. So, the doctor was very encouraging that even if significant impairment exists, James will be able to hear, to speak, and so on in a normal way. And catching any problem early will help reduce difficulties in things like language acquisition (or at least, we hope so).

About 40% of the kids they see with this problem have been in the NICU, and there also seems to be a relationship with elevated bilirubin levels (apparently the elevated bili can affect the nerve functioning). Our initial impression is that the diagnosis and understanding of this condition is still relatively new. But it appears that the condition is usually permanent -- there are a couple of reported cases in the literature of it going away, but the audiologist sounded as if that was extremely rare. (In other words, even when his bilirubin is back to normal, that won't solve the problem.)

It feels like just one thing after another for the poor kid.

Even though it had been a long day at the hospital, we stopped by the NICU and got to visit with two of James's primary nurses (and saw a couple of the doctors as well). They really made us feel a lot better. Even though James has faced and is facing so many challenges in life, he also has a lot of people who love him.

FYI: What is auditory neuropathy?
Auditory neuropathy is a hearing disorder in which sound enters the inner ear normally but the transmission of signals from the inner ear to the brain is impaired. It can affect people of all ages, from infancy through adulthood. The number of people affected by auditory neuropathy is not known, but the condition affects a relatively small percentage of people who are deaf or hearing-impaired.