Our story begins...

When our sixth child, Tim, was eighteen months old, he was a very quiet toddler. He had yet to begin babbling, in fact, he made no sounds at all. Since he had been in speech therapy since the age of eight months, we were sure that this was due to his oral motor problems. He had always had a weak suck, and feeding problems. Still, everything I read on speech delays said you should check the hearing. Our NICU follow up clinic had done their rough "evaluation", as had the developmental center's speech and language pathologist. The pediatrician thought he was hearing fine. But I insisted we check, just in case "things were a little muffled".

A few months before this, my husband and I had seen the movie "Mr. Holland's Opus" in which the starring couple discover their son is deaf at about the age of one year. Rick and I thought this was very funny, thinking only morons would not realize their son was deaf until that age! We would surely notice if Tim was even slightly hearing impaired. So it was with total confidence that I took Tim, alone, to the audiologist's office on that April day. I sat in a soundproof room with him, and sounds were put over loudspeakers in each corner of the room. At the same time, objects above the speakers lit up and did a little dance. Tim turned to even the quietest of them. I felt a little bit silly for even being there and wasting the audiologist trainee's time. Then she announced that she would send the sounds without the visual cues. No problem, thought I. But oddly enough, Tim seemed to not get the game now. He failed to turn to even the most blaring sounds. The trainee called for the audiologist, who repeated the sounds. Still, Tim did not flinch, blink, or otherwise show any recognition of sound. And it was at that moment that I realized our little boy was deaf.

The primary thought on my mind was to not look surprised. I thought of those idiots in the movie, and didn't want to be compared. I tried to be very nonchalant when the results were being shown to me. I took Tim to the car and called my husband on the car phone. Rick started telling me how his day was going, and I cut in saying, "I just took Tim for his hearing test" to which Rick replied, "oh, yeah, how was that?" I had to repeat the failed results twice to a man as incredulous as I was. A few days later, an ABR was performed. An Auditory B rain Stem Response is a test in which the child is sedated (Tim was VERY asleep!) and EEG monitoring is performed as sounds are emitted directly into the ear canal. The electrodes measure the brain's response to sound. The nice apart about this test is that it is very objective, and therefore very accurate. It doesn't matter if the child is in the mood to co-operate or not. The bad part is that the test confirmed a severe to profound bilateral sensorineural hearing loss. We were given a booklet "When Your Perfect Child is Deaf" and left feeling somewhat stunned. At the same time, we had a real sense that a year and a half had been wasted, and did not want to lose another day. We stopped at Barnes & Noble bookstore on the way home and bought books on sign language.

Early on, we were encouraged to consider cochlear implants. There are a lot of reasons to not rush into this major decision. We were encouraged by much that we read, and knew the key to Tim's success in life was language. Initially we were just using signed English-a signed word for each English word, including tense and endings. While we still are using that at this time (it's all we know), we know we want to learn and use American Sign Language.

That would seem like the conclusion, right? Wrong. I have talked to many parents over the last year and a half, and our stories are similar. All of us thought there were times it seemed our child could hear. I expressed this to Tim's audiologists, who both assured me this was a normal parental reaction. But as time went by, my husband and I were more and more convinced. Finally, on a day when Tim and I were picking up new ear molds, I spoke to Tim, without signing, and told him to bring me a blue block. He did just that. And a surprised audiologist took him into the sound booth and obtained PERFECTLY NORMAL hearing results, all the way down to a 10dB whisper. Did I rejoice? Oddly enough, no. Because I knew that Tim had not been miraculously healed. He is deaf sometimes, and hearing others. It took several months to confirm this in testing situations. Finally, when all acknowledged that this was highly abnormal, we took Tim to Mayo Clinic for a thorough workup. Even the experts there had never seen a case quite like Tim's. Besides the hearing, we also observe great variation in his muscle tone, and other problems. Now, thanks to the work of Dr. John Shoffner at Scottish Rite Children's Hospital in Atlanta, Georgia, we have an answer. Tim suffers from a mitochondrial myopathy (disease), specifically a Complex I defect. It does affect his hearing, but not consistently. In other words, his cochlea is fine. But his auditory nerve is not.

Are we happy we did not get a cochlear implant? You better believe it. His cochlea is normal, though testing did not show that. He failed all the tests that are administered to children going through a CI screening process. He was a perfect candidate. His hearing aids did not show improvement in sound room testing. We would have done it. I am not idiot enough to assume that there are many other children in this situation. But I do know now that other infants and toddlers with ABR's showing profound hearing loss do not actually have as great a degree of loss as testing shows. I continue to have strong reservations about the early implantation of children. There is an increased push to implant even earlier (less than a year of age) which is very alarming. We had no idea what Tim's "deafness" really was until he was three years old.

I DO recognize the success that many families have had with cochlear implants, and wish them all the best. I continue to have reservations about the long term effects of having silicone implants near the brain of a child.

Now our youngest son, Danny, shows a hearing loss. Been there, done that! Currently, he is in an "at risk" kindergarten, moving on to first grade in the fall. And he is no longer wearing a hearing aid, as his hearing tests vary vastly in whether they indeed "aid" him or not. He is completely verbal, and we are working on articulation.

I'd love to hear from other parents of deaf kids, kids with metabolic or mitochondrial disorders exhibiting odd hearing, or anyone who is deaf or works with the deaf.