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Congenital Aural Atresia

The Day My Life Was Changed

"It's just oxygen, don't worry!" were the last five words I heard before I went under to have my whole world changed.  I woke up sever hours later in the most excruciating pain of my life, but with a new meaning of, "I can hearing you loud and clear!"  I had just had my world changed, in many ways.

From the time I was a month old, I have been shuttled to doctors of every king.  One of the endless number of doctors' names I have long forgotten discovered that my left ear canal was full of bone.  Back in those days, most doctors told my parents, "If it is not life threatening, you should just wait until she is older to make her own decision about having it corrected."  I cannot tell you the hundreds of times I have heard that in my life, and every time I felt the same way.  I would not be here wasting my parents, yours, or my time on this if I did not plan on really having it done!  Eighty percent of the doctors I have encountered have been eye doctors, but two were just for the ear that had been just hanging on the side of my face unusable for seventeen years.  The doctor that I saw in ... (that I wish was just another name on the list of doctor names I have forgotten) gave me two options.  That was the first time I remember there was actually something they could do to fix my ear.  Both would be life changing but which one was better for me?  Drilling a hole in the side of my head or sticking a screw in, then after five months of recovery attach a box and hear better than most Americans?  Or, go see two specialists in Tampa who could possible drill the bone out, reconstruct my canal, and give me a new ear drum?  What were the risks I was facing with both?  What would be the difference in my actual hearing abilities?  I felt like I was on cloud nine.  Drilling the bone out?  What a splendid idea.  No ugly box and screw in my head for the next seventy years?  With those options, my choice was made a little easier.  Of course, unnamed doctor busted my new found excitement with the risks and complications: brain fluid drainage, canal collapsing, and about a hundred other risks that seemed to be coming out like the word vomit. I finally decided to talk all those other doctors advice and wait, weigh my options, and ask my family for their opinions. In August, I decided I was finally ready to go through with the surgery.  The only problem was, unnamed doctor lost his surgical privileges in my county of residence, and I was not willing to wait for his partner to get approved by my insurance.  So the road I had not even considered giving the slightest possibility looked like my only hope.

A month later, my dad and I made the two hour and twenty minute, 152.37 mile drive to Tampa General Hospital on November 2 to meet Dr Loren Bartels.  That day was the first time I would meet the man that would change my life, in the elevator on the ride up to his office.  He would end up being the one to give me the gift of being able to hear like everyone else, to have the option of having people walk on my left side without pretending to hear them, or have it be a black hole of secrets that many people have lost inside.  Within a week, my dad had scheduled my surgery for exactly one month later.  The thirty day wait felt like it was my arch nemesis that could never be defeated.  I felt overwhelmed and had the feeling that I was having to wade through too many things than I could handle.  Two trips up to Tampa General with my dad in the days before my surgery felt as if I was making a cross country trip every time I had to get in the car at 5:30 in the morning.  Ever hear, "Wow, you look like you just got hit by a truck?"  Those are the exact words I would use to explain how I felt walking into TGH on the bone chilling morning of December 10th.  I knew that within a few short hours I would be under the microscope of Dr Bartels.  The nerves were more than present and the inevitable pee test was lurking in the back of my head since they had not even ushered a needle within five hundred feet of me the day before, which was full of tests.  The time seemed to drag by like the feeling I used to get when I was five and had to go to sleep on Christmas Eve after tracking Santa on the computer with my family.  Finally, 10:30 am made it's well anticipated and anxiously awaited arrival, and it was my turn to be wheeled into the OR # 7!

Waking up, I felt like Wonder Woman with bionic hearing. If someone across the room had dropped a pin, it would have sounded like a crash of thunder in my ear.  Knowing that I would be waking in indescribable pain, my mom and younger sister purchased me the most adorable talking bear.  The only problem was, the volume of his cute and cuddle voice made my brain shake! After a full, dragged on week of recovery my newly constructed eardrum was finally ready to hear that cuddly brown bear's voice after I clapped his hands and made my mom smile.

Having to wait two weeks to make that two hour and twenty seven minute drive again it felt like my surgery day all over again.  Only this time, I would be graced with the exact words, "Your recovery is going better than I hoped."

I want to thank you Dr Bartels, from the bottom of my heart for giving me the gift of hearing.  Though, it is really loud at times.  I can never truly thank you enough!



Effects of Aural Atresia on Speech Development and Learning

The following is a summary of published literature on a specific ear related topic. The reference to the original publication is found at the end of this summary.

Aural Atresia
Congenital aural atresia is a birth defect that consists of absence or a severely narrow external ear canal. The eardrum membrane and hearing bones are commonly also deformed and other abnormalities may co-exist in the middle ear. It is almost always accompanied by a malformed (microtia) or absent (anotia) external ear. Atresia and microtia are common congenital malformations, with the incidence of microtia reported to be 0.5 to 3 per 10000 live births, and congenital aural atresia is reported in 55% to 93% among individuals with microtia. Microtia is unilateral in most patients, more commonly in boys and more commonly on the right side.

While children with bilateral hearing loss are known to be at increased risk of speech and language delays as well as poor performance in school, the clinical significance of single sided hearing loss is not universally recognized. Common thought has been that children with single sided hearing loss were thought to develop speech and language normally and most have not received hearing aids. However, a number of small studies of children with single sided hearing loss have shown delays in word acquisition in younger children, increased grade failure rates, and a higher probability of needing speech and language services. Significant deficits for these children exist in language comprehension and oral expression.

While these findings are significant, it is unclear if they can be extrapolated to children with unilateral congenital aural atresia because most of the children in prior studies of single sided deafness had profound sensory hearing loss whereas the children with congenital aural atresia typically have normal inner ears and their problem is limited to the middle ear and external ear canal. Also unclear was whether the side of the congenital ear canal atresia might impact development of speech and language.

For this study, the authors reviewed records by audiologists, speech pathologists, and psychologists looking at the incidence of speech and/or language delay, the incidence of needing speech therapy, and other educational interventions. Also checked were parental reports of social problems.

Seventy-four children were identified with singled sided congenital absence of the ear canal: 48 on the right-side; 19 on the left-side, and 7 with bilateral absence. Children with this birth defect demonstrated high rates of utilizing speech therapy (86% among bilateral, 43% among unilateral). Reports of school problems were more common among children with right-sided (31%) than those with left-sided ear canal abnormalities(11%). Special educational interventions were common in all groups (33% right, 21% left, 43% bilateral). In the case of bilateral AA, all children who received additional interventions were enrolled in schools for the hearing impaired, without any other specified learning deficiencies.

Conclusions and Relevance Children with single sided abnormalities of the outer ear canal are at greater risk of speech and learning difficulties than previously appreciated, with rates similar to children with unilateral inner ear (sensory) hearing loss. This group of children has an obvious need for more study of how best to assist them.

Read the original article: [1]


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