Birth & Diagnosis
Our son was born in 1995, our much-wanted first child. He was born to a speech therapist mother and electronics engineer father, so when the doctor questioned the look of his ears in the delivery room, we were not too worried. Hearing loss and technology (e.g. hearing aids) were things we knew a bit about and felt comfortable with. Besides, we were much more concerned with the fact that we had a brand new baby!!
An ear, nose and throat specialist visited us first thing in the morning and confirmed the diagnosis: bilateral aural atresia and microtia (our son had no ear canals on either side, and underdeveloped outer ears). He recommended that we take a couple of weeks to get ourselves organized, and then go see him in his office.
In the meantime we searched through some of our textbooks and on the internet for information, but found very little. The follow-up visit at the ear, nose and throat specialist (otherwise called E.N.T. or otolaryngologist) resulted in a referral for an auditory brainstem response test, or ABR. This test determines the level of hearing by the brain�s responses to sounds, and doesn�t require the child to respond in any way. There was a waiting list of 2 to 3 months for this test. We knew what the degree of our son's hearing loss probably was, because complete atresia generally causes approximately a 60dB loss, but we did not know for sure until he had the ABR at three months old (at which time our suspicions were confirmed).
The E.N.T. did not refer us to an audiologist (hearing specialist) or stress the importance of getting a hearing aid as soon as possible, but we knew from my speech therapy background that this was the next step, and we pursued it on our own rather than waiting for the ABR test. We contacted our local health unit audiologist, who referred us to a private audiologist with experience fitting bone conduction hearing aids (Vancouver Hearing Centre: they were excellent!). We also contacted a local service provider for children with hearing loss (Elks Family Hearing Resource Centre: also excellent!) and were assigned a speech therapist (or Speech-Language Pathologist) who did home visits and provided us with information. Jesse got his hearing aid at approximately 2 months old, one month before he had the ABR test.
The Hearing Aid
The bone conduction hearing aid we bought for our son was the typical set-up, mounted on a metal headband. The audiologists showed us an article questioning the wisdom of using such a high-pressure head band on a young child, and recommended using a soft cloth head-band instead. After a few prototypes, we devised a band made of grey spandex. The oscillator rested against his head behind his ear, and stayed on with double-sided wig tape. The hearing aid itself was in a little bag that we could clip to his shoulder or the front of his shirt. We had to move the hearing aid depending on our son's position, so it didn't get wet when he spit up (which he did frequently!).
Our son showed a response to the hearing aid right away. When we first put it on, he would start to smile and then pout and then smile and pout. If we put it on when he was crying or fussing, he would suddenly settle at the sound of our voices. It was quite a wonderful thing to experience, actually, and reassured us that he did have some hearing and could benefit from a hearing aid.
Our son became accustomed to his hearing aid very quickly, perhaps because he was so young. We were very conscientious about putting it on all of the time, and not making exceptions (e.g. for taking pictures). We figured it was like part of his body, and he was entitled to have it as much as he could. At age five, he continues to wear his hearing aid all of the time, with the exception of bath-time, water play, and when he is sleeping. He insists on wearing it while he is falling asleep at night, and we remove it afterwards.
It was not until our son was about 18 months old that we learned of the increased incidence of heart and kidney deformities in children with ear deformities. We went to genetic counseling to determine whether or not our son had a syndrome (they felt he likely did not), and at that time he was referred for an ultrasound of his kidneys and a consult with a cardiologist. Everything was found to be normal. He was never referred for a CAT scan, which seems to be unusual compared to families we've spoken with from the U.S. We do plan to pursue this, as children with atresia and microtia are also at higher risk for developing cholostreatomas (a growth in the middle ear).
We have received ongoing services through the Elks Family Hearing Resource Centre, including sign language classes, a parent support group (which was very helpful in providing information, as well as the feeling that we were not alone), play groups with hard-of-hearing peers, and individual sessions when required. Our son's hearing was initially monitored through the private audiology clinic where he was fitted for his hearing aid, and then later by the local health unit audiologist because it was more convenient. I feel that although the "system" here was not as organized or efficient as it could have been in directing us (and thus other parents) to the right resources initially, once we got to them the services have been excellent.
Speech and Language Development
Our son�s speech and language have developed normally, and actually a little ahead of schedule. We used sign language with him, which was not as difficult as for some other families, because his mother was already somewhat fluent in sign. He started signing at about a year old. At 18 months, his language was a combination of speech and signs, and by age two, he had dropped most of his signs and used speech as his main method of expressing himself. We continued to sign to our son for awhile, to make it easier for him to understand us, but as his language level has surpassed our signing abilities, we unfortunately do not use much sign anymore. It is still a tool that we make use of in noisy situations when he has a lot of difficulty hearing.
Our son's speech has always been quite easy to understand (he had some difficulty with "sh," which cleared up with a little work when he was 4). He mispronounces new words occasionally, usually indicating his has heard them wrong, but is able to imitate them correctly once we point out the error.
In a quiet environment, such as at home, our son does not miss very much because of his hearing loss. With his aid on and without background noise, he is able to hear the doorbell, the phone, any conversation directed to him, a bird singing, water in a creek, etc. He overhears some of other people�s conversations, but this is more difficult for him than for individuals with no hearing loss. He has significantly more difficulty hearing in a noisy environment, such as at the mall or at a party. He tends to rely much more on visual information in these situations (watching the speaker's face closely, or asking people to sign).
Our son is not able to localize sounds at all (tell where a sound is coming from), because he does not have sound information coming in to two different ears (we can localize sounds because our brain calculates tiny differences in loudness and timing between one ear and the other; when a sound comes from the right, it will arrive at our right ear slightly before the left ear, and will be slightly quieter by the time it arrives at the left ear). This is mostly a concern right now when we are outside around traffic; he hears an engine start up, but has to look around to find it rather than turning immediately. I fear a little for his safety, but it is something he will learn to compensate for. He also is not great at hide and seek because he misses out on any sound cues that allow us to hear which direction someone goes to hide!
Our son spent 7 months (March to December) attending a neighbourhood preschool and using an FM system (mostly for circle time). It took some time for him to become comfortable staying at preschool by himself, but he adjusted to the FM system quite quickly. We found that his preschool teachers were happy to do whatever they could to meet his needs. The biggest difficulty was with equipment; our son�s hearing aid had to be sent away twice during that period of time, and the "loaner" hearing aid we borrowed was not compatible with the FM system. Then the FM system itself was not always working correctly. It was actually a good trial period for us to get used to these equipment issues before starting elementary school.
Our son has started kindergarten and is doing well. It is definitely a challenging listening environment. He uses the FM system whenever appropriate, and does well with it. It is more difficult when the children are playing at different centres, as there is a lot of background noise. In these situations, he often does not hear his peers calling his name to get his attention, and certainly is not able to "overhear" the conversations of others. His hearing resource teacher is very helpful at making the classroom as accessible as we can for him.
Concerns and Hopes for the Future
One thing we are exploring for our son is the BAHA� (bone anchored hearing aid). We have been gathering information, and will probably pursue this sometime in the next year or two. We are not planning on reconstructive surgery while he is a child; he will have the option of pursuing this when he is older if he chooses.
Our son is a happy, bright, affectionate kid. He is sometimes cautious of new situations and change, but generally has good self-esteem. He enjoys talking and playing with his brother, his peers and any willing adult. We feel positive about future�he�s gonna be fine!