ATRESIA MICROTIA & YOUR CHILD
Introduction: About this book
Dedication
This book is dedicated to the amazing parents of those children with CAAM who have entrusted me to care for their children—and those yet to come. The trust you have placed in me and my team is one of my life’s most cherished compliments. This book is written in the spirit of bravely and honestly communicating treatment options while seeking to achieve the best results for your children.
And with special thanks and admiration to my oldest daughter, Caitlin Roberson, whose wordsmithing skills are amazing and have launched her professional career as well as improved this book immensely.
Letter to the Reader
Dear Reader,
As a young boy growing up in the mountains of Western North Carolina, one of my favorite times of year was late spring. During that time, my grandad let me accompany him as he prepared his fields for planting. He lived on a small farm and used a horse and plow. He depended on his crops for food and income.
Mostly, I simply sat on the laboring horse as he walked behind me in the fresh earth, holding the handles of the plow. The sunshine was hot, and I watched as we went back and forth across his long, rectangular field. A barbed wire fence made of split wooden fence posts about 6 feet apart bordered the field. When it was time for me to help “man” the plow, Grandad would take me down from my perch on the horse to place his strong hands over mine and walk behind me, steering the blade through the soft warm earth. Our goal was to make grooves in the ground—called furrows—for seeds that would grow into his crop as the summer progressed.
Grandad taught me that the best way to plow a straight furrow was to aim at a fence post, keeping it firmly in view throughout the entire length of the field.
If I did not follow his advice, my furrow curved this way and that—creating chaos in the field (and re-plowing for Grandad!). Grandad had the love, wisdom, and presence of mind to teach me that life is no different—seeing the finish from the start allows us to do our best without wasting effort or getting off track.
If you want your child to hear, to speak, to listen, and to thrive in a hearing world despite the hearing impairment he or she was born with—or if you are gathering information about CAAM and its treatment options—this book is for you.
Due to the rarity of CAAM, few physicians understand the best treatment options, and correct information can be scarce. Parents sometimes receive misleading advice given by people who aren’t up to date on state-of-the-art treatment techniques for this rare condition.
By reading this text, attending, or watching recorded proceedings of one of our conferences, you will access a knowledge base some parents don’t have access to for years. Over the past two decades, we have made significant progress in the treatment of CAAM.
This thorough and succinct book will help you assemble the team that will overcome the effects of hearing impairment and cosmetic deformity from CAAM in your child’s life.
The views expressed in this book result from my personal evaluation of and treatment recommendations for more than 5,000 children in more than 100 international conferences for parents with children who have CAAM; from many children evaluated at the International Center for Atresia & Microtia Repair in Portola Valley, California; and from surgeries I have personally performed on more than 3,000 children and adults with CAAM from more than 50 countries. The recommendations shared in this book have been strongly shaped by my experience as the father of three wonderful children.
I want to help you make a strong start as you begin to see what your child can be—as you begin to see your child’s fence post—in short, seeing his or her end from the beginning.
With best wishes for success,
Joe Roberson, Jr., M.D.
This book could make a big difference in your child’s life.
This book is for you if:
Due to the rarity of CAAM, many physicians are not up to date on the latest available solutions, and solid advice can be scarce. However, in the past two decades, huge strides have been made, and there is hope in almost all situations. In this book, you’ll gain knowledge some parents need years to gain. Its contents include:
Please note this book gives general guidance only. Individual treatment plans need to be made by surgical specialists. |
Introduction
Why I Approach Being a Doctor as a Dad
When I see children with hearing loss and look in their parents’ eyes, I know exactly how they feel. Some are angry and frightened, while others feel helpless. Others are almost fierce in their insistence on hope. It doesn’t matter which part of the world we’re in—these universal emotions are natural parts of every parent’s process when caring for children with critical needs.
I know because I’ve been in those parents’ shoes.
A Newlywed, First-Time Dad, and Budding Surgeon
I fell in love with my wife, Julia, when we were both young. We married the week after I completed college, right before I started medical school. With a partner I adored and a deeply meaningful career, I didn’t think my plate could get fuller.
I knew I was wrong when Julia shared we were pregnant. We became a family on June 7, 1985, when Caitlin Crist Roberson entered the world. I imagine every parent remembers the moment they first hold their newborn. Time was wonderful, and it seemed to fly.
The Day Things Changed
Twenty-eight months later, I was a surgeon-in-training in my ninth year of training after college. I loved interacting with patients. During this phase of training, I moved through different areas of surgery to complete my broad education before specializing in conditions of the ear. At that time, I served as a head of the emergency room (ER) and found it infinitely fascinating how many conditions our team of medical professionals seemed to be able to treat. Yes, the 24-hour shifts were grueling, but I had regular time off with Julia and Caitlin at least once a week. I loved those days. We’d go for hikes and stroller walks. Sometimes, we’d take my motorcycle to get ice cream. We lived on the third floor of married-student housing, and our second child was on the way. Life was exciting, demanding, and good.
One weekend, I felt sick after arriving home from one of my 24-hour shifts in the ER—likely from something I caught at the hospital. After vomiting, I opened the third-story floor-to-ceiling window in our tiny bathroom to air the room. I knew this wasn’t safe for Caitlin, but Julia was suffering from severe pregnancy sickness, and I didn’t want the smell to make her more sick.
The decision went against an agreement Julia and I had made several months before, when a friend of Caitlin’s died of complications from falling out of a one-story window. I decided to tell Julia about the open window later since she was napping in our bedroom. Exhausted from work and from the virus, I fell asleep in the other room with Caitlin on my chest.
“She’s Fallen Out the Window”
Several hours later, I jolted awake to the sound of Julia screaming Caitlin’s name. While I slept, our daughter had run down the hall to see my wife while Julia was in the bathroom. (Julia hadn’t yet put on her glasses and hadn’t noticed the open window.) When Caitlin’s socks encountered the linoleum, she slipped, and her body bumped through the exposed window screen.
As I ran to the bathroom, I already knew what I would see … or rather, wouldn’t.
“She’s fallen out the window,” Julia repeated over and over.
There Caitlin was, three stories down, lying unconscious on hard, clay ground.
I cannot describe the anguish I felt in that moment, nor how rapidly those first few moments passed. Before I knew it, I was down the staircase, around the building, and weeping as I held Caitlin in my arms.
“What are you doing?” Julia screamed, coming around the corner just behind me. “Why aren’t you doing something?”
It was then that I realized Caitlin had literally just fallen.
From Dad to Daughter’s Doctor
Looking at Caitlin’s lifeless frame, for a moment, I was frozen. Was Caitlin still alive? If she was, had I paralyzed her if she had trauma to her spinal column when I picked her up?
“You’ve been trained to know what to do,” Julia said—her intuitive wisdom as practical as it’s always been. “Just do it.”
The doctor in me took over.
“Call an ambulance,” I said, checking her pulse. It was present but low. And Caitlin wasn’t breathing.
Operating on automatic, I gave her mouth-to-mouth.
No response. Try again.
No response. Try again.
No response. Try again, heart sinking.
After agonizing moments, her breathing and heart rate began to return, but Caitlin couldn’t breathe on her own without mouth-to-mouth for quite some time. We waited for what seemed like hours for the ambulance, and Caitlin remained unconscious. I continued giving her mouth-to-mouth.
Would the hours and hours I’d spent away from my family in the hospital save Caitlin?
A Patient in My Own Hospital
When we arrived at the hospital, we raced into the emergency room. What a role reversal. When I’d been there last, I was a doctor; everything was in order; and I felt in control.
The same IVs, lights, and beds were still there, but everything else had changed.
One thing I knew—my family desperately needed my colleagues’ help.
After evaluation in the ER trauma room, Caitlin was taken to the Intensive Care Unit on the second floor. Since Julia was so dehydrated from her sickness, she was admitted three floors above. I promised her, there on the fifth floor, that I’d do everything I could to make sure our daughter got the best care.
For the next blur of hours and days, I wandered between the second and fifth floors, sometimes with Julia, sometimes without. My main memory is the constant sensation that I’d walk through fire to help my daughter and wife. I was grateful for my colleagues, trained professionals who I knew I could trust.
It was an experience of the hospital process from the other side… and a lesson of what I’d intellectually known for years—having a loved one, especially a child, who is suffering produces the most excruciating emotions a human can have.
Integrating the Experience as a Doctor
I share this, dear reader, so you know that I speak to you as a surgeon-scientist and a dad.
Amazingly, miraculously, Caitlin not only survived but has since thrived—without lasting injury. While she’s a happy, accomplished woman today, I will never forget what it was like to be her parent as we evaluated treatment options, made critical decisions, and navigated her healing process. Perhaps my keenest memory is how crucial communication with loved ones was, as well as simple explanations from the medical teams in times of severe stress.
I know what it’s like to navigate traumatic situations and make life-altering decisions for your child. I know what it’s like to crave information and advice. What treatment options are available? What is the likelihood of success? What are the different risks of different choices? Even for me—someone who “speaks medicine”—the choices were almost overwhelming. As I treat my patients now, I realize how critical accurate, respectful, and truthful communication is to parents with children in medical situations.
I also know how invaluable it is to have the partnership of trustworthy medical experts who are motivated to do everything in their power to treat your child.
Understanding the Needs of Children with CAAM
Like other doctors, I first learned about CAAM during my residency in the late 1980s. In the 1990s, I noticed that many CAAM patients had serious problems in normal hearing situations, even after they received treatment. At first, I didn’t think much of this. After all, correcting CAAM is one of the most difficult surgeries to complete. It’s so hard, in fact, that many ear surgeons do not recommend the procedure to patients.
I’d learned through working with children needing cochlear implants that early intervention dramatically improved hearing and language results—especially in the first few months and years of life. As CAAM patients then received treatment typically between 10 and 12 years of age, I wondered if treatment timing was a contributing factor to the poor outcomes I observed.
At the time, CAAM treatment required a series of five or more separate surgeries. The first four surgeries corrected the appearance of the outer ear and caused a significant defect where cartilage was harvested from the chest to make the ear. The final surgery restored the missing ear canal. This sequence had never been varied.
During the early years of my career I was fortunate to treat a type of brain tumor that involves the hearing nerve. In the mid portion of my professional years, I was blessed to be involved in the early days of an implantable device that can bring hearing to deaf individuals called a Cochlear Implant. Needing a new challenge in 2003, I started the International Center for Atresia & Microtia Repair at Global Hearing, my organization, to develop better ways to treat CAAM. As you will see, different specialists are involved in evaluating and treating the condition. By bringing these experts together under one roof, I hoped we would collaborate to develop new and innovative solutions.
In 2004, I invited Dr. John Reinisch to speak at the hearing impairment conference that my team has hosted for 27 years now. We now do multiple conferences like this around the world each year (see atresiarepair.com for an up-to-date list). He spoke about a new technique he had developed for surgically correcting the outer ear. While listening, I realized an important thing—the ear canal could be surgically corrected before the outer ear was reconstructed using Dr. Reinisch’s new technique. (Soon thereafter, I realized that these two separate surgeries might be conducted together on the same day, but we’ll talk about that more later.) Dr. Reinisch confirmed that his portion of the procedure could be performed successfully regardless of whether the ear canal surgery was completed before or after his work on the outer ear. I began reconstructing the ear canals of CAAM patients before the outer ear surgery—for the first time in the world early in my career.
When I shared the success of the ear canal-first procedures in an article in 2009, this novel approach received opposition.[i] It disrupted existing methods and threatened to reduce the need for multiple procedures to a single surgery. Today, a decade later, the strategy has consistently demonstrated improved results in treating CAAM.
I’m happy to report we’ve seen a marked improvement in the hearing and language development of patients who undergo atresia repair when they’re three to five years old.
Giving someone the gift of hearing—whether it’s in one ear or both—is a huge deal. It proves to be a rewarding experience, and I understand that parents put profound trust in the doctors they choose to treat their children. Figuring out a treatment plan for your child’s CAAM can feel overwhelming. This condition is almost always unexpected and discovered at birth. Parents face a new condition they, and sometimes their doctors, have never seen nor know much about, and accurate information can be hard to find. I intend to rectify that information gap in this book.
In the following pages, I’ll help you clearly understand the choices and outcomes you have before you, and I will explain in frank and honest terms what I would do for my own child and why.
I hope you get to meet our incredible team members. I owe so much to the staff, care providers, physicians, and surgeons at Global Hearing who advance the art and science of treating this condition.
What Is CAAM?
Congenital aural atresia and microtia (CAAM) doesn’t develop over time. It’s an ear abnormality that is present from birth.
To understand CAAM, you need to know how hearing works in a normal ear. The outer ear focuses sound energy down the open ear canal. The tiny vibrations of sound cause a vibration in the eardrum—a delicate, thin, living tissue membrane at the end of the ear canal that separates the middle ear from the outside world with air on both sides. The vibrations of the eardrum are transferred to three middle ear bones, which act as a lever to amplify the sound energy and transfer that energy to the fluid-filled inner ear. The fluid of the inner ear receives the pressure wave, which enters the snail-shaped cochlea. In the cochlea, nerve endings are stimulated by the pressure wave brought by the middle ear bones, and electrical impulses are sent to the brain. These impulses are perceived and processed by the brain as sound.
The anatomy of a normal hearing system: the outer ear (auricle), ear canal, ear drum, middle ear (containing the three middle ear bones), and the inner ear (consisting of the snail-shaped cochlea and hearing nerve, which transmits sound to the brain).
With atresia, infants can’t hear normal sounds because they don’t have a normal ear canal and eardrum. Bone occupies the space where the ear canal usually is, and the eardrum is missing.
This image shows the ear of a child with atresia (missing ear canal and eardrum) and microtia (missing outer ear).
In almost all cases of atresia, children have middle ear bones, which you now know play a role in processing sound. However, the middle ear bones are usually partly malformed and fused to the abnormal surrounding bone, which prevents them from vibrating. Given the absence of the eardrum as well, the normal hearing structures are useless without intervention. Fortunately, the inner ear and the hearing nerve are almost always normal. In simple terms, it is often possible to restore hearing by surgically opening the ear canal, mobilizing the middle ear bones, and making an eardrum and/or by other treatment options summarized in this book that can bypass the lack of an ear canal/eardrum/middle ear bone system.
Children born with abnormal outer ears, or without any ears at all, have a condition called microtia. Because the outer ear is usually responsible for catching sound waves, this condition also contributes to hearing loss. How it looks can have significant social and psychological effects throughout children’s lives. (Note: Aural Atresia and Microtia usually occur together. Atresia can occur as an isolated issue with a normal outer ear. Microtia, however, is always accompanied by abnormalities of the ear canal and never occurs alone.)
Many doctors don’t realize atresia and microtia are related but separate conditions.
The options for microtia treatment (ear reconstruction) are straightforward. It’s important to coordinate atresia repair, which is more complicated and requires much more specialized equipment and surgical techniques than microtia repair, when investigating one’s choices for treatment. For the first time in history, our physicians have been able to combine surgical treatment on the same day for both conditions.
This Book’s Important Points
If you’re reading this book, chances are good that your child has CAAM. If that’s the case, chances are also high that you’re stressed, thinking about this often, and perhaps not sure what to do. You’re probably worried about what to do while also determined to do whatever it takes to treat to your child. Therefore, this section summarizes this book’s most important points.
On Emotional Care and Family Support
- Talk with your family. How are your emotions and heart doing? Dealing with a child’s health issues can be hard, especially at first. Almost all parents are shocked when they learn their infant has CAAM, and the time and effort it takes to learn about the condition can be stressful. It is normal to experience varying degrees of anger, guilt, helplessness, denial, fear, sorrow, and grief.
- Check in with your partner. Parents move through their unique emotional journeys at different rates. Don’t forget to check in with your partner and family members to see where they are in their journey.
- Don’t let emotions get the better of you. While emotions are important to process, they also can impede timely decision-making. It’s common for one parent to need longer than the other to process their reaction to their child’s condition. In the case of CAAM, where waiting to treat the condition can impact the child’s entire life, it’s important to balance empathy and support with action.
- Know this isn’t your fault. CAAM is generally not caused by either parent. While we’ll discuss possible causes for the condition later, know that this is not your fault.
On Evaluating Treatment Options
- See the finish from the start. You’re about to face many important decisions. Knowing what you want for your child long term will help you be confident as you weigh different options.
- Education is critical. Parents who want their children with CAAM to hear, speak, and develop at the highest level possible must become knowledgeable about CAAM to make prompt decisions about treatment, therapy, and education.
- Hearing matters more. It is easy to focus on the way the ear looks. As your child grows up, however, the functional deficit of untreated hearing impairment is a bigger issue in the grand scheme of life. I have many adult CAAM patients who treated the cosmetic deformity of microtia but did not treat their hearing loss, and now they wish they had. Many didn’t have the options we have today, others acted on poor information, but some simply ignored the problem.
- Single sided CAAM will have severe hearing implications later in life. We require two ears to develop and function normally (see later in the book). Since children can hear fairly well in quiet with single sided CAAM in early years, some doctors tell parents not to worry about hearing. That advice is wrong.
- Bilateral CAAM is a hearing emergency. Children with CAAM in both ears (about 10% of patients worldwide) MUST have hearing treatment started within the first months following birth to allow normal development.
Do not overlook treatment for single-sided CAAM! The advice that “one-ear hearing is enough” is wrong.
On Making Decisions
- The window to fix hearing loss is limited. You must intervene and treat hearing loss in the first few years of life, or you miss the treatment window forever.
- Act fast. Timing is key to the successful treatment of CAAM—the earlier you seek treatment, the better.
Hearing loss will impact your child for his or her entire life if left untreated. This is the most important message to take from this book.
On Non-Family Relationships and Community Support
- Connect with the CAAM community. Along with talking to your partner, family, or friends about your emotions, it’s good to connect with other parents too. Many of Global Hearing’s patients and families are grateful for advice they received from others who walked the road ahead of them and shared experiences and advice. (You can find many support communities on the internet, many of which occasionally meet in person. Please note this book does not necessarily espouse the advice given in these groups.) If you have the courage to reach out, you will be amazed at how much it can help you. The cycle may also repeat as you have the chance to help others.
The Most Important Points of All
- Hearing loss is a disability. Normal hearing and brain function require two ears. Early hearing—and in the case of hearing loss, hearing treatment—produces the best results. When treatment is delayed beyond the early years of life, the impact is lifelong and cannot be reversed with treatment at late ages.
- The sooner your child gets treatment, the better. Recent studies have shown another alarming phenomenon: Long-term hearing deficits may remain even after asymmetric hearing loss has been treated if the correction was not completed early in life within the “critical time window.”[ii]
- Your child requires a personalized care plan. While this book provides a framework for evaluation and education about the options for treatment, your child’s hearing loss will require highly customized care to be effective. Since the anatomy of each child with CAAM varies, options for repair and success vary. Only a qualified specialist can help you create your own personal treatment plan.
- Be confident in your child’s future. As hard as it may be to believe, there are worse things in life than CAAM. Treatment can be time-consuming, difficult, and expensive, but, ultimately, can lead to normal development without limitation for your child.
- Communicate your confidence to your child. While it is important to acknowledge and talk about your feelings, don’t give your child a sense that CAAM limits them. When families honestly and courageously accept their situation and execute a timely treatment plan, their children can experience rich and uninhibited lives.
How to Use This Book
This book is intended to be a manual for parents of children with CAAM. It aggregates insights that my team has collected while evaluating more than 5,000 children at 100 international locations while performing more than 3,000 CAAM surgeries over the course of more than 20 years. You can read this book from start to finish in the order we wrote it. We also purposefully designed the content so you can skip from section to section as you need different information throughout your child’s journey.
The first two chapters explain how normal hearing and language develop, and what goes awry in the case of a child with CAAM. The subsequent chapters explore testing and evaluation, treatment options, and results. These chapters follow a sequence I have refined and found maximally effective over many years of parent conference presentations and communication. If you are more of a visual learner, send us an email at atresiarepair@calear.com and we will give you access to a video of the latest CAAM lecture.
In the end, your child will need a customized treatment plan that requires consultation with a medical professional. By reading and understanding the information in this book, you will be prepared to understand your choices and determine the optimal plan of action for your child in consultation with a specialist.
Chapter in Review
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“My Life Could Have Been So Different”
Global Hearing received this letter from a woman with single-sided CAAM, who we have not treated. She accurately describes some of the challenges she faces with the disability associated with single-sided hearing loss. I suggest you read it now. I will also prompt you to return to it later after you know more about CAAM. You will then understand why she had such difficulty in some hearing situations.
Dear Dr. Joe Roberson,
I just watched your video on microtia and atresia and am in tears. I’ve always felt so alone and almost crazy. After watching your video, I realize I’m not.
My life could have been so different.
At an early age, my mother suspected something was wrong and took me to many specialists. They all said she shouldn’t worry—my hearing is fine; my left ear was just smaller than the right.
In school, I was constantly in trouble. When we read books out loud in class, each student continued where the previous reader left off. I was never able to do that.
I was told I was naughty. Teachers asked why I was not listening.
Children teased me and said I was dumb. I couldn’t follow conversations, and I constantly asked what they were talking about. I did not know that I couldn’t hear.
I started believing I was just not good enough.
I ended up hating school so much that I skipped almost every second day. In my last year, I started refusing to go. I studied at home and only went to school for exams. I completed school, not well, but I passed.
At some point in my adult life, I realized my hearing is not normal. I had it tested and found that I was deaf on the left side. I’ve been through a few operations to make the ear canal bigger. My third child was born with microtia and atresia of the left side [Note: This is very unusual as only a very small percentage of CAAM affects successive generations]. I was devastated. I felt responsible for him being this way.
Only now, at the age of 40, do I truly realize the profound impact my hearing impairment has had on my entire life. I look back and it breaks my heart.
I could have become so much more.
I still get anxious about being in a group of people, and I feel incompetent in conversations. Even though I’m now able to hear, it feels I’m lacking in cognitive ability. This has killed any chance I had of being confident.
I’m writing to thank you for educating people on the importance of hearing, especially from an early age. I have so many emotional scars from all that I endured. NO CHILD should ever have to go through this.
— A Mother with CAAM