Audiology Appointment

Harper had an audiology appointment Friday afternoon. She had to have another hearing test, and we had planned on buying the hearing aids and FM system. The hearing tests take place in a soundproof booth with her sitting in a high chair and an audiologist sitting in front of her. This time I sat in there with them. There is another audiologist sitting behind a semi-one way window to conduct the test. They place microphones in Harper's ears and try to get her to turn toward the sound. She is then "rewarded" by a moving pig or moving duck. In true Harper style after three or four turns, she was bored with the rewards and wouldn't turn to look anymore. The few results that they did get were consistent with her past test, so that means that her hearing hasn't gotten worse. Yay!
However, the audiologist did not want to order the hearing aids or FM system at the appointment. Because of Harper's diagnosis of enlarged vestibular aqueducts, they would like her to use the loaner aids for six more months in case her hearing drops further. If that happens, she may need a different type of aid or only one aid in her better ear.
We also discussed with her our concerns about Harper's balance issues. She said that compared to some children with EVA Harper's case isn't nearly as severe. She said that some children have to wear a helmet because their balance is so poor. Any bump on the head for children with EVA can cause a drastic drop in hearing. We are so thankful that Harper is doing so well!

Test Results

We finally got a call from the genetics clinic regarding Harper's Pendred Syndrome test. The test came back as normal. However, the counselor told me that this doesn't mean that Harper doesn't have Pendred Syndrome; it just means that this test came back negative. She has been in contact with Dr. Richard Smith at the University of Iowa which is always ranked nationally in the top 3 in otolaryngology. He said that in the next 1-2 years that there will be several more tests available that may be more conclusive targeting different chromosomes. The counselor told me that as of now Harper's diagnosis is still large vestibular aqueducts with hearing loss.

Sing-a-long

Harper LOVES music. In the last couple of weeks, she has really started to pick up on songs. Of course, she likes some more than others. Here is a rundown of what Harper is singing these days:

• Wheels on the Bus---She does some of the motions (wipers) but only sings "all through" which sounds like ahhh foo. "The wheels on the bus go round and round...." Then I point to her and she says, "ahhhh foo" and I finish "the town"!
• Ants Go Marching--She claps, stomps around, and says the "Boom boom boom boom" part. It is absolutely precious!
• The Clean-Up Song--She loves this and sings it ALL the time. She'll just be playing or sitting in her car seat singing "Up, up, up up". Maybe this is a sign that she's going to be mama's helper!

Terrible twos???

The past couple of weeks I have wondered if Harper has already entered the "terrible twos".
Harper has done a great job wearing her hearing aids---until recently. Now, if I tell her "no", do something she doesn't like, or try to challenge her while doing her speech therapy, she will pull both of her hearing aids out! She is basically saying, "I'll show you!" She realizes that this is one way that she can exert her independence and have control of the situation. In addition Harper has become increasingly impatient. For example, she wants her breakfast IMMEDIATELY after the microwave beeps. If there is a cartoon on tv/dvd player that she doesn't like, she'll point and scream at it until we change it to one she does like.
I have been worrying if this is "normal" behavior for a fifteen month old child or if this is related to her hearing loss and a sign of frustration. I had a long talk with her speech therapist today who assured me that although it is a little early, Harper's behavior is perfectly normal and very common with hearing impaired children. Thankfully, she gave me some tips to deal with Harper's new sassy attitude, but also warned that it will get worse before it will get better. I'm going to have my hands full!

One Proud Mommy

Yesterday Harper's speech therapist wanted to try a new game. She thought that Harper would be too young to catch on, but Harper proved her wrong!
Susan lined up three toys (a monkey, car, and airplane) and told Harper to get the monkey ooo ooo ooo eee eee eee and throw it in the basket. SHE DID along with the car, airplane, horse, bus, dog, and cat!!! The first round she was using the object's name along with the "learning to listen" sound. She did so well that she played the next game only with the object's name. She only missed one!!!
After only four months wearing her aids, she is listening and learning way more than I thought. I am so proud of my baby girl!!!

Harper's First Birthday

On May 16 we celebrated Harper's first birthday. This year has FLOWN by!

We had a small party at home, but Harper had a blast! She really dug into her cake. We even had to clean it out of her nose! Notice how well she is doing with her hearing aids. The majority of the time she wears this headband instead of the hat. I bought her these cute headbands at Kids Unlimited in Cullman when she was younger and didn't have enough hair to wear a bow. I had no idea how handy they actually would be!
She got WAY too many toys, but I have to say she plays with it all. She especially loves this baby doll that she puts into the recliner and says, "Rock, rock". Luke and I got her a pink electric car. When she's old enough the stick comes out of the back and she can make it go by pressing a button on the steering wheel. She loves it!

A Start in the Right Direction

I finally talked to Dr. Robin, Harper's geneticist, concerning the CT scan results. He said that it looks like she has enlarged vestibular aqueducts. Here's a snippet I found from nicdc.nih.gov:

Research suggests that most children with enlarged vestibular aqueducts (EVA) will develop some degree of hearing loss. Scientists also are finding that five to 15 percent of children with sensorineural hearing loss, or hearing loss caused by damage to sensory cells inside the cochlea, have EVA. However, scientists do not think that EVA causes hearing loss. Instead, scientists regard EVA as an important clue about hearing loss and its possible causes. This information helps physicians talk with families about how their child's hearing loss may change over time.
The presence of EVA can be a symptom of a genetic disorder called Pendred syndrome, a cause of childhood hearing loss. According to a study by the National Institute on Deafness and Other Communication Disorders (NIDCD), approximately one-third of individuals with EVA and hearing loss have Pendred syndrome. With Pendred syndrome, the hearing loss is progressive, which means that a child will have less hearing over time. Some children may become totally deaf.
In addition to its association with hearing loss, EVA may also be linked with balance symptoms in a small percentage of people. However, the brain is very good at making up for a weak vestibular system, and most children and adults with EVA do not have a problem with their balance or have difficulty doing routine tasks.

This isn't great news but it isn't horrible news either. There are a number of syndromes that have much worse effects than Pendred (i.e. blindness, heart defects, etc.) I am very encouraged that the doctors may finally be heading in the right direction. Dr. Robin is consulting with several other doctors about the test results. He said that the genetic counselor would get back in touch with me soon.