ATRESIA MICROTIA & YOUR CHILD
Chapter 6
Other Resources
Several repositories of information exist with resources available for you to explore. We have spent a significant amount of time and energy on our website, which contains more information for your use. Patients who are scheduled for surgery or who have had surgery previously will find sections giving instructions for both surgery preparation and postoperative care. Should you schedule surgery with us, you will receive all appointment times and information via email prior to your visit to our facilities. Many people find the FAQ section of our website helpful for exploring general questions and investigating unique and unusual situations that might pertain to you or your child.
Website: www.atresiarepair.com
Social media platforms can be fantastic to learn more as well, and we actively support the following:
Evaluation Summary Timeline
At Birth:
- Hearing testing of both ears (whether or not they are affected by CAAM!) as soon as possible following birth; complete all normal newborn screening testing as recommended by your birth center
- Soft Band BCHA fitting as early as possible in life, preferably <6 months old (mandatory in bilateral CAAM, optional in unilateral CAAM)
0 – 2.5 Years:
- Put together your care team:
- Pediatrician – general physical examination, routine check-ups, immunizations, treat any ear infections in non-CAAM ear (if applicable), genetic testing a applicable, test for syndromes (if applicable)
- Audiologist – establish care and monitor hearing, fit with BCHA device if needed; preferably pediatric audiologist familiar with hearing testing in children
- Otologist – contact to establish care, begin filling in HEAR MAPS acronym evaluation system
- Plastic Surgeon – select outer ear reconstructive method by 2.5 years of age, and contact plastic surgeon of your choosing to establish care
- ± Craniofacial Surgeon – if evaluation or treatment is needed for jaw and/or facial reconstruction
- At approximately 1 year of age, consult with speech pathologist if available to ensure speech & language skills are age-appropriate
- Continue with regular speech & language therapy if recommended by speech pathologist
- Consider attending one of GHI’s international Atresia Microtia Conferences for an in-person consultation with our team
- Address any other medical conditions (cardiac, etc.) that must be treated prior to elective ear surgery
2.5 Years:
- Complete diagnostic testing
- Temporal Bone CT Scan – performed no earlier than 2.5 years of age
- New Audiogram – including air and bone conduction levels for both ears (whether or not they are affected by CAAM!)
- Send diagnostic testing to otologist for evaluation and treatment plan
- Select treatment plan and schedule surgery
Affected Ear/s:
Right
Left
Bilateral
HEAR MAPS Score: ( Right / Left ) Ear
H__.__ E___ A___ R___ M___ A___ P___ S___
Hearing
Bone Conduction/Nerve Function: ____
Air Conduction: ____
External Ear
Grade 1 / 2 / 3 / 4
Atresia Score — CT
1–10: ____
Complete Atresia / Partial Canal
Remnant Lobe
Normal
Reduced
Absent
Displaced
Mandible
Normal
Mild reduced
Moderate reduced
Severe reduced
Asymmetry of facial soft tissue
Normal
Mild reduced
Moderate reduced
Severe reduced
Paralysis of facial nerve
Normal
Mild reduced
Moderately reduced
Severe reduced
No movement, normal muscle tone
No movement, poor muscle tone
Syndromes
None identified to date
Other: ______________________________________________
Associated Conditions
None
Yes: _____________________________________
Partial ear canal
Bilateral CAAM
Cholesteatoma
Fibrous IS joint
Hemi-facial microsomia/facial asymmetry
Jaw abnormalities & correction
Ear infections
PET’s
Tonsil and adenoid enlargement
Sleep Apnea
HEAR MAPS Score: ( Right / Left ) Ear
H__.__ E___ A___ R___ M___ A___ P___ S___
Hearing
Bone Conduction/Nerve Function: ____
Air Conduction: ____
External Ear
Grade 1 / 2 / 3 / 4
Atresia Score — CT
1–10: ____
Complete Atresia / Partial Canal
Remnant Lobe
Normal
Reduced
Absent
Displaced
Mandible
Normal
Mild reduced
Moderate reduced
Severe reduced
Asymmetry of facial soft tissue
Normal
Mild reduced
Moderate reduced
Severe reduced
Paralysis of facial nerve
Normal
Mild reduced
Moderately reduced
Severe reduced
No movement, normal muscle tone
No movement, poor muscle tone
Syndromes
None identified to date
Other: ______________________________________________
Associated Conditions
None
Yes: _____________________________________
Partial ear canal
Bilateral CAAM
Cholesteatoma
Fibrous IS joint
Hemi-facial microsomia/facial asymmetry
Jaw abnormalities & correction
Ear infections
PET’s
Tonsil and adenoid enlargement
Sleep Apnea
About Dr. Joe Roberson
At the start of his career, Dr. Joseph B. Roberson, Jr., MD served as the Director of Otology-Neurotology of the Skull Base Surgery Program at Stanford University. During his 10 years at the university, he focused on hearing-related brain tumors and cochlear implants as well as a small number of CAAM patients. Since 2004, he has served as Chief Executive of the California Ear Institute Medical Group and its many related medical entities.
In the 1990s, there was still a great deal of room for improvement in the treatment of CAAM, and the need for a center of excellence for the condition. Dr. Roberson began to focus on CAAM and established the International Center for Atresia & Microtia Repair and Global Hearing to respond to this need and to focus on this condition with a goal of improving results.
In 2002, Dr. Roberson and his wife started The Let Them Hear Foundation, a non-profit Christian organization that helps treat deafness in children and adults. Since then, the foundation has provided assistance to set up multiple cochlear implant programs around the world, including training surgeons and staff. More than 100 Surgeons have been trained through this program while Dr. Roberson has performed more than 100 cochlear implants in international venues personally. Due to the programs LTHF initiated, more than 5,000 deaf children in countries around the world have received the gift of hearing through a cochlear implant.
At this time, Dr. Roberson’s main focus is the surgical care of children and adults with CAAM. He has treated over 3,000 patients from over 55 counties around the world. He still cares for many patients with a wide variety of ear- and skull-related disorders.
A Personal Note from Dr. Roberson
I like difficult problems, especially if they involve a situation in the operating room and they deal with issues children face—and even more if others don’t do them well and there is an opportunity to improve results. In my opinion, the surgical correction of CAAM is the hardest challenge ear surgeons face. It’s so difficult, in fact, that many ear surgeons do not even recommend surgery.
During the early part of my career (in addition to CAAM), I focused on refining the process of cochlear implantation and surgery for a type of brain tumor called an acoustic neuroma (vestibular schwannoma) while serving as the Director of the Otology-Neurotology and Skull Base Surgery Program at Stanford University. I enjoyed teaching young trainee surgeons for just under a decade during my tenure at the university and have continued to train surgeons here at CEI who have finished prior training at numerous prestigious otolaryngology training programs. As the year 2000 approached, I was drawn more and more to CAAM. I think, in part, this was because improved surgical techniques had been successfully developed for each of the areas of my early focus, and now could be treated with excellent results that many surgeons are capable of providing. It was my desire to produce the same excellent outcomes for CAAM and to develop the procedures to make this possible.
We like to think we had a part in the overall progress of developing treatment of many of the conditions I specialized in early in my career. For example, through the Let Them Hear Foundation, numerous cochlear implant programs have been established internationally, where local surgeons and staff in international sites can receive training in treating certain types of deafness. The foundation has helped spread the expertise to implant these miraculous devices that can treat deafness in both children and adults. We have been, and continue to be, privileged to provide hearing both directly and indirectly to thousands of deaf children as a result (see www.lethemhear.org for more information). Another book I have written details a parent’s decisions in the treatment of sensorineural deafness using cochlear implants and encapsulates many of the areas I participated in developing in this period of my career (in Hear for Life: Dr. Joe’s Guide to Your Child’s Hearing Loss). A third publication deals with some miraculous and remarkable stories that have resulted from our LTHF activities (in Let Them Hear: An Ear Surgeon’s Joyful Experience with Enabling People to Hear for the First Time).
In the early 2000s, I began to focus on treating CAAM even more, with the hopes of achieving the same results and surgical advances in treatment. I now travel to multiple international countries each year to host conferences for parents and children affected by CAAM. In 2003, I founded the California Ear Institute Medical Group, which expanded CEI, originally founded in 1968. Moving into the non-university-affiliated healthcare environment has allowed better development and focus on CAAM and its treatment. Since 2004, when CEI and associated entities became a separate organization, I have been privileged to operate on patients from more than 55 countries with this condition.
CAAM is rare, which means few doctors have significant experience with its treatment. That fact also leads to something we see far too often: doctors who mean well (but who don’t actually know a lot about CAAM) frequently give misleading or wrong advice to parents. They don’t mean to harm, of course, but parents must avoid the mistake of thinking all advice received from a physician is correct. Since you are reading this book, you have already started the process of getting accurate and up-to-date information—good job!
The need for a center of excellence for CAAM was obvious, as the results we saw in the treatment of CAAM in the 1990s needed improvement. It was a challenge I accepted. Having a center capable of making all aspects of treatment available is the best way I know of to focus on new and innovative solutions—something we are proud to have achieved in the last two decades. Our physicians see a large volume of this condition and can collaborate freely to maximize outcomes as a result. As you have seen in the text, several disciplines are needed to evaluate and treat this condition. All those individuals are brought together under one roof at the International Center for Atresia & Microtia Repair at the CEI Medical Group. I hope you get to meet them—they are incredible. I owe so much to the staff, care providers, physicians, and surgeons at CEIMG who work so diligently to advance this art and science! We believe there is no greater honor, calling, responsibility, or privilege than to be entrusted with the care of children with CAAM—and perhaps, your own child. This is the highest compliment you can give us, and we are overwhelmingly grateful for that trust.
Endnotes
[i] Roberson, J. B., Reinisch, J., Colen, T. Y., & Lewin, S. (2009). Atresia repair before microtia reconstruction: comparison of early with standard surgical timing. Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, 30(6), 771–776.
[ii] Kaplan, A. B., Kozin, E. D., Remenschneider, A., Eftekhari, K., Jung, D. H., Polley, D. B., & Lee, D. J. (2016). Amblyaudia: Review of Pathophysiology, Clinical Presentation, and Treatment of a New Diagnosis. Ymhn, 154(2), 247–255.
[iii] Lieu, J. E. C., Tye-Murray, N., Karzon, R. K., & Piccirillo, J. F. (2010). Unilateral Hearing Loss Is Associated With Worse Speech-Language Scores in Children. Pediatrics, 125(6), e1348–e1355.
[iv] Roberson, J. B., Jr, Goldsztein, H., Balaker, A., Schendel, S. A., & Reinisch, J. F. (2013). International Journal of Pediatric Otorhinolaryngology. International Journal of Pediatric Otorhinolaryngology, 77(9), 1551–1554.
[v] R.A. Jahrsdoerfer, J.W. Yeakley, E.A. Aguilar, R.R. Cole, L.C. Gray, Grading system for the selection of patients with congenital aural atresia, Am. J. Otol. 13 (1992) 6–12.
[vi] Goldsztein, H., & Roberson, J. B. (2013). Anatomical Facial Nerve Findings in 209 Consecutive Atresia Cases. Otolaryngology--Head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery, 1–5.
[vii] Goldsztein, H., Ort, S., Roberson, J. B., Jr, & Reinisch, J. (2012). Scalp as split thickness skin graft donor site for congenital atresia repair. The Laryngoscope, pp. 1-3.
[viii] Roberson, J. B. Combined Atresia Microtia (CAM) Repair – a new technique for reconstruction of form and function in congenital atresia and microtia. In Press, Microtia Repair. Editors Reinisch J, Tahiri Y.
[ix] Anthropomorphic growth study of the head. Cleft Palate Craniofac J, 1992 vol. 29(4) pp. 303-308.
[x] Service, G. J., & Roberson, J. B. (2010). Alternative placement of the floating mass transducer in implanting the MED-EL Vibrant Soundbridge. Operative Techniques in Otolaryngology-Head and Neck Surgery, 21(3), 194–196.
[xi] Balaker, A. E., Roberson, J. B., & Goldsztein, H. (2014). Fibrous Incudostapedial Joint in Congenital Aural Atresia. Otolaryngology--Head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery.