My Baby's Mild - Moderate Hearing Loss

Our first child, Lena, was born in 2007. Most hospitals in the US do standard hearing screening on all newborns. Newborn Screening Laws by State. Lena received a "fail" on the left ear and "pass" on the right. Since the test is not conclusive we were told that it was probably due to fluid in the ear from giving birth and it would go away on its own. We really weren't worried about the test since the baby was healthy and normal in every way and the pregnancy and delivery were free from complications. We were referred to the health department to do a follow-up test to confirm the original results. This test was done a month later and the results did not improve, they actually were worse with both ears now failing. Since we were living within driving distance to Johns Hopkins Hospital in Baltimore, MD we decided to have her more extensive testing done there. Going to Johns Hopkins was stressful, but we felt that they were the best for pediatric otolaryngology and since it was available to us we were going to give her the best. On the day of the testing we were told to wake her up early, don't feed her and keep her awake until the appointment. We are talking about a 2 month old, hungry, tired and in the car for almost 2 hours in heavy traffic. Needless to say, I was rattled by the time we arrived. She needed to be asleep for the testing (they don't like to sedate infants) so when they were ready for her we fed her and she passed out tired. I was able to hold her during the testing, which kept me calm and it was all painless for her. The audiologist performed a series of tests including an ABR (Auditory Brainstem Response), Otoacoustic Emissions, and Typanometry. The tests confirmed the type of hearing loss and the severity.

In addition to the hearing tests, we went back to the hospital to have a series of tests to find out if there were any other medical conditions to be concerned about. She had the following tests:
Eye Exam (important to correct sight for lip reading and to rule out structural abnormalities)
CT scan to look for inner ear structural abnormalities
Renal Ultrasound to check the kidneys
EKG to check the heart
All of these tests came back normal confirming that her hearing loss was an isolated form and did not increase her chances of having other problems.

We found out that Lena's type of hearing loss is often caused by a genetic mutation of the genes connexin 26 or connexin 30. We decided to proceed with genetic testing. We felt it was important to know if it was genetic, not only because we planned on having more children, but also in search of a cause. Her genetic testing came back negative which was good because it reduced the risk of it occurring in other children, however, we weren't provided with a cause.

Photo By: Emory519

The ride home from Johns Hopkins after the hearing testing was emotional. Having our fears confirmed was difficult. This was our first child, she was very healthy otherwise. My emotions were different from my husbands. I felt a lot of guilt and questioned every little thing in the pregnancy and delivery. What did I do wrong in the pregnancy? What caused this? Is she going to be able to live a normal, productive life? The permanency of the loss was devastating. Knowing that she would never hear certain sounds and even aided, it wouldn't be the same. I cried, my husband, I think, was more angry, but not at me, just angry. We are probably lucky that we are both practical and logical. We started talking about how we felt, how we would deal with it. James continually reassured me that I had done nothing wrong. Maybe it was easier because we still had this new infant to figure out so we were forced to move on quickly. Our families were all supportive (which we found out is often rare) and they listened to us, understood that it was permanent and she would likely have hearing aids her whole life. It is harder when people ask if it will get better, will she grow out of it. Nope, according to the tests, the hair cells in her inner ear are damaged and it can't be fixed.

By the time the genetic testing was complete we had come to terms with Lena's condition and we knew the results would not change our decision to have more children. The meeting with the genetic counselor was interesting; she explained what the chances were of future children to have a hearing loss. She estimated a 25% risk factor, but that was just a guess since the cause of Lena's hearing loss was unknown. We were already set that this disability would not prevent us from growing our family.

2 years later, the hearing loss is kind of like a program running in the background, you know it's there, but it's not a defining focus in our lives. Dealing with the loss has become second nature, the hearing aids go on every morning, we speak clearly, point out sounds, use eye contact, work on pronunciation, introduce new words, etc. While the emotional storm has passed, there are still worries that I have (like all mothers). My biggest right now is her speech development. It is still too early to know what are normal 2 year old things that correct on their own and what might be due to the hearing loss. We notice she is still having trouble with "f" and "s" sounds and it is being watched by the speech therapist. We look forward to when she will be old enough to tell us what she is hearing which will also help with the programming of the hearing aids.

While there is certainly no right or wrong way to get hearing aids I found that we were not prepared and lacked information when trying to make decisions. I want to briefly mention a few of the challenges we encountered.

Understand the services offered in your state before using your insurance. Early Hearing Detection and Intervention by State We lived in WV, had doctors in MD and I started the hearing aid process in MD with a local audiology office. Luckily we opted to get loaner hearing aids which were free and returnable (except for the ear molds). We paid for the ear molds out of pocket. I have found that most insurance plans offer very limited, if any, coverage for hearing aids. Once we were in the WV Birth to Three program, we found out that Lena would qualify for hearing aids through their program. This was almost jeopardized since I had started the process, but because we paid for everything out of pocket we were able to switch audiologists and use the one in the program. While it is critical to get the baby aided as soon as possible, it is also important to do what's best for the family financially. So, know that loaner aids are available. Oticon Loaner Bank Program. We got ours through Oticon and they were the Safran model. Check the state program before purchasing anything so you don't disqualify yourself. Lena ended up with Widex Inteo hearing aids that were provided by the state program. The cost of those hearing aids: about $3000 each. Our insurance would have only covered about $1000 total leaving us with a huge bill.

The second challenge was in understanding how audiologist offices work. One thing to keep in mind is that most audiologists do not carry all of the hearing aid makes available. We started out with Oticon and ended up with Widex because the new audiologist didn't use Oticon. I have been happy with the Widex Inteo hearing aids.

We made a lot of trips to the audiologist. Young babies grow quickly and we were getting new ear molds every 2-3 months. Also, we were tuning the hearing aids. They are initially tuned based on the ABR report, but as you start observing your baby you can get a feel for if they are too soft or too loud. Now we are getting molds every 6-8 months.

Each state is going to have a different program offering early intervention services. We started out in WV and now live in TX, so I have experienced two different programs. All of the people we have worked with have been very caring and professional.

When we first started, services were geared mostly towards teaching us techniques to help Lena learn to use her hearing. In addition we had a developmental specialist working with us to make sure that Lena was on track in other developmental areas just in case there might be a disability that had not been identified. Current services are moving towards speech therapy now that she is talking.

Decisions that parents must make can be difficult, but remember that you know your child and your family best. With a mild-moderate hearing loss we faced the special challenge of being pulled in two directions; to go either all oral, or use sign language. One of our support services was from the state school for the Deaf and she encouraged sign language, although we were hesitant. We wanted Lena to learn to talk and function in the hearing world. We decided to do limited sign language with Lena (see my lens for more details). Basically, we used the true ASL signs, but did single "baby" words and included the spoken word with the sign. We chose to do this to lessen frustration in communication and to allow that she might have a speech delay. Once Lena started talking about 50 words, we dropped the signs completely and asked her to use her words and not rely on signs.

Photo by: Maíra Soares

I wanted to share our story (so far) because I think that sometimes children with a mild-moderate hearing loss fall through the cracks when it comes to support and services. They deceive us because they can appear very normal and fully functional. They aren't deaf, so they don't get the extent of services that a severe or profoundly deaf child would need. They have their own special needs and because the level of hearing loss can vary, the services must be tailored to the child's specific loss.

Photo By: Maíra Soares