Abby's First Booth Test

My mother-in-law and I headed back to Ohio for Abigail's first sound booth hearing test this past weekend. I had tried to schedule her hearing test, gastro appt. (for the high triglycerides that popped on her original bloodwork), genetic eval and ear molds all for the same day since we were coming from Pittsburgh. Everything went very smoothly, but I am slightly confused about her hearing test results. This new test indicated that Abigail only had a mild/moderate loss rather than the initial moderately-severe loss that her ABR (Auditory Brainstem Response) test showed. While I am incredibly happy that her loss is not as bad as we had originally thought, I am a bit concerned that we are going to be programming her hearing aids to correspond to these new results. The sound booth test is different than I expected and I am not sure how accurate it is. The entire test is based on subjective interpretation of Abby's reactions to sound. I will have to do some research to put my fears at ease, but for now I am cautiously happy about these results. I can't wait to tell Ben.

Below is a picture of a standard speech audiogram--the object pictures are placed on the audiogram where their typical sound decibel would be and the letters are representative of where those sounds would be in the speech banana. I have included a picture of Abigail's first hearing test audiogram as well so that you can understand exactly what we think she is hearing right now.

The A symbols refer to Abby's aided hearing (what she hears with her hearing aids on), the circle symbols refer to what she hears unaided with her right and the X refers to what she hears unaided with her left ear. The gray shaded banana shaped area is referred to as the speech banana--where most of the sounds of our speech exist. If she is able to hear above that speech banana, she should be able to develop speech normally. As you can see her aided results put her above the speech banana and just below the highest frequency sounds of "f," "s," "th."

Abby's Hearing Aids are Finally In!

Abigail's hearing aids came in just one week after our first ear mold impressions. Our audiologist had pre-programmed them specifically for Abigail's hearing loss and disabled the exterior volume button (so that Abigail wouldn't be able to turn them up/off accidentally). We arrived very eager and couldn't wait to see what they looked like, we were also very excited to see her reaction when she finally heard our voices for the first time! The audiologist spent almost an hour explaining our warranty, operation, how to care for the aids, etc. Oticon (the manufacturer of our hearing aids) will replace the hearing aids one time in the first year regardless of the cause of damage or loss. After that we were advised to talk to our home owners' insurance provider to see if we could set up some sort of rider on our policy to protect their loss. Since her hearing aids were $4000 and our health insurance only pays for a new pair once every four years, we need to have some sort of backup protection.



Our audiologist put them in for the first time and turned them on. Abigail seemed startled and started to cry. This made me really sad as I had expected giant smiles from this little two month old baby who was truly "hearing" for the first time. Our audiologist then took them out so that we could practice putting them in and taking them out by ourselves. It was really hard, I felt like I couldn't get the mold in her ear and they just kept whistling at me. The whistling was really surprising to us, I guess we never really understand how much feedback there would be when the aids were turned on. Feedback is a result of the sound that the aid amplifies leaking out of the inner ear and amplified a second time by the aid--it sounds similar to the feedback you sometimes hear on a microphone in an auditorium. Young babies are very susceptible to this feedback since they aren't able to support themselves yet--feedback is intensified by being too close to something or someone else (i.e. being held). The feedback was so bad that I left the office that day crying. My expectations were unrealistic. I did not expect that this new world of sound would be scary or annoying to her, I did not expect their to be so much work involved in caring for her aids and last but not least, I did not expect that their would be so much feedback for poor little ears.



I didn't know how I was going to do this, we had just left and I already hated her aids.

Let's get ear molds!

We went in for our first set of ear mold impressions and are getting even closer to getting Abigail's ears aided. We met with our audiologist to do the impressions and sign paperwork for the hearing aids. The process for taking the impressions was very quick. The audiologist took a small cotton ball attached to a string and pushed it into Abigail's ear canal, then used a two-part epoxy to fill her ear canal. The epoxy hardens very quickly, after only a few minutes, and is removed by the strings. The result is a perfect impression of the inside of Abigail's ear!

Our audiologist sends it off to Microsonic and we have ear molds in less than one week!

Ear molds come in many different colors including sparkles, confetti and bright neon colors. We chose to get lilac colored ear molds to match Abby's pink hearing aids.

Our Questions Answered

Abigail's genetic profile came back on December 15, 2008. The tests revealed that Abigail's hearing loss is the result of a Connexin 26 gene mutation. Connexin 26 is a protein that helps different cells pass molecules between another, specifically it helps to cycle potassium through the cochlea and the inner ear. This results in a build-up of potassium and resulting nerve damage of the cochlea hair cells. In about 2/3 of patients this damage causes severe to profound hearing loss. In Abigail's case, she retained a greater amount of her hearing resulting in only a moderate-to-severe loss. Since this particular mutation is recessive, both my husband and I must be carriers to have passed the recessive trait on. We have no incidence of hearing loss on either side of our families and have an older child without hearing impairment.

Our question is finally answered--we have a reason. I was incredibly sad, but also relieved; sad that this was something that we had passed on and could potentially pass on to our other children, but also relieved to finally know that there was nothing that I did wrong--nothing that I could have done differently to prevent this. Since her hearing loss was determined to be genetic we were provided with an ENT that specialized in genetic hearing loss in addition to a geneticist.

State Funded Aid

The state of Ohio has an umbrella health care organization that exists to financially support all kids with medical handicaps, Bureau for Children with Medical Handicaps (BCMH). BCMH also provides support to special needs children that require ongoing medical treatments including diabetes, heart defects, chronic lung disease, cancer and hearing loss. The financial support is intended not only to help families without insurance, but also to supplement insurance to meet any co-pays or services not covered. This includes the cost of hearing aids and services associated with hearing loss.

While families must meet certain income requirements for treatment services, any and all diagnostic services are covered regardless of income. This means that any screenings, tests, x-rays, specialist appointments leading up to a diagnosis are covered 100% by BCMH. Your insurance will be the first payer, but any out of pocket costs will be picked up by BCMH. In addition BCMH provides extremely low-cost payment plans for hearing aids, public nurse visits and access to a variety of specialists.

I am not sure of state-funded organizations in different states as our experience is solely in Ohio. However, The National Dissemination Center for Children with Disabilities has an awesome website that allows you to search for agencies by state.

During that first meeting with our caseworker, we filled out the initial paperwork for BCMH to apply for financial support for diagnostic services. Within 4 weeks we received a letter of approval that we would be required to submit to all doctors or providers (i.e. children's hospitals, labs, etc.) that Abigail would be receiving care from.

The Question of Cost

I wanted to take a couple of posts to explain everything that we have learned about funding Abigail's treatment and hearing aids. This first post I will talk solely about the overall cost and insurance policies, while I will try to provide as much information about state-funded assistance programs in the next post.

Shortly after Abigail's HOH diagnosis we had a meeting with our caseworker to discuss our insurance and how we intended to fund the purchase of Abigail's hearing aids. Since Abigail has a bilateral loss (a hearing loss in both ears) we would be purchasing two hearing aids in addition to two ear molds (click here for an interesting article on ear molds). Hearing aids range in price from $1000 USD for a quality basic hearing aid to over $3000 USD for a state of the art hearing instrument. The Oticon hearing aids that were chosen for Abigail, the VIGO PRO, cost approximately $2000 per hearing aid. This makes our total about $3600 for her aids alone.

The cost of an ear mold impression ranges from about $80 USD to $175 USD depending on the material that the ear mold is constructed from. The cost is constructed of two sub costs--the cost of the actual impression being taken by an audiologist and the cost that the company charges for the construction of the ear mold. Dayton Childrens' Audiology department sends their ear mold impressions to a company in Pennsylvania called Microsonic. The cost of Abby's ear molds are $100 a piece, bringing our grand total for ear molds and aids to $4200.

Although the financial cost was overwhelming to us, we were certain that our insurance would be there for our daughter and help pay a portion of the cost. This is where our caseworker began to explain that most insurance providers do not pay for hearing aids, regardless of whether they are for children. I found this fact absolutely appalling and plan on writing more about this later on, but lucky for us we had one of the few insurance providers that paid for hearing aids in full. The only stipulation is that the child must possess a hearing loss significant enough to affect proper speech development. We would not be required to pay any portion of her hearing aids, which was a relief to us.

Since we an Active Duty Air Force family we have the military insurance program, Tricare. While most Active Duty families have free healthcare at military treatment facilities, we have chosen to pay out of pocket for a more PPO style Tricare program. However, it wouldn't have mattered which Tricare option we had chosen because Tricare is one of the few insurance providers that pay for hearing aids. In the next post I will talk about Ohio's state funded program, BCMH, that can help parents of deaf and HOH children whose insurance does not provide the same financial help that we were lucky to have.

Pink Hearing Aids?

The ENT gave us the medical clearance for hearing aids, so we headed back to the audiologist to get busy learning all about our options. Abigail's hearing loss falls in the moderate-severe range so we believe that she will benefit greatly from aids. We had little to no input on what hearing aids Abigail would be receiving. At first, I was slightly annoyed--Why shouldn't we choose the aids or at least be asked our opinion. However, this feeling quickly faded when I realized that I know absolutely nothing about hearing aids, or even how our ears work. Once our audiologist started explaining all the benefits of the hearing aids that she would receive, I was confident in her ability to choose the best aid devices for our daughter. The features were all so technical that I truly was relieved that she had done all the research for us and picked the perfect aids.


Abigail would receive the Oticon Vigo Pro, a hearing aid perfect for infants. It has several nice features for little ones to grow with. It has the ability to have an optional wireless FM boot, it has no external on/off switch to prevent little hands from turning off their aids by themselves, it has smart technology to amplify the sounds you want to hear while dampening background noise.

The only choice we had left to make was the color! I thought long and hard about this because our insurance only pays for our hearing aids once every four years unless there is a medically necessary reason for upgrading (I plan on talking about insurance in a future post). The Vigo Pro comes in 10 different colors including skin colors. I originally thought that I would pick skin color aids so that they wouldn't be as noticeable, but the more I thought about it, the more I wanted them to be a sign of pride for her. I want her to love them, to want to wear them, to consider them a cute accessory that she can't wait to put on! That's when it hit me, I decided on soft pink ice--a pearlized baby pink. I hope that she will love them and that they will be a perfect addition for her!