ELLAS STORY
Ella is a bubbly, outgoing, funny, sometimes cheeky, but very lovable 10 year old girl. She has two sisters, Lucy (5) and Evie (3), who adore her, and more than anything in the world, she loves to dance. What some of you may not know, is that behind her beautiful smile, she has her own little struggle. Ella was born on the 27th of June 2005 with a congenital ear condition called Microt
ia Atresia, a condition which effects the formation of the outer ear and the development of the ear canal. What this means for Ella is that she has severe conductive hearing loss in her right ear, mild hearing loss in her left, and cosmetically an underdeveloped right ear. When Ella was born, we were quite shocked and concerned to see that her right ear was closed and underdeveloped, as this condition had not been detected during the pregnancy scans. The doctors and midwives in Albury had not seen this condition before, which was quite stressful and worrying as first time parents. However photos of Ella were emailed to the Royal Children’s Hospital in Melbourne, and within 6 hours we had a diagnosis. Ella’s first visit to see an audiologist was at 4 weeks of age, where after testing, it was determined that Ella had severe conductive hearing loss in her right ear, and at birth mild hearing loss in her left ear. We were then referred to the Microtia Clinic at the Royal Children’s hospital in Melbourne, the team there provided so much support, and have continued to every year for the past 10 years. Ella has regular ENT and audiology appointments. Thankfully, we were also put in contact with the Australian Microtia Support group and Simone at the Hear and Say Centre in Brisbane. The support and advice of many of the parents, patients and coordinators has been invaluable to Ella, and to us as a family. Ella had a CT scan at the age of 6, and to our absolute amazement we found out that her inner ear had formed and her cochlear functions well. Therefore Ella is a candidate for not only the outer ear reconstruction surgery but also the ear canal (atresia) repair to open Ella’s ear. SURGERY
After 10 years of research into the wee hours of the morning, advice from families and professionals, and in particular a visit to the annual Microtia and Atresia conference in Brisbane, we have learnt so much. Without the conference we would not have had the opportunity to meet the international professionals in this field, who generously give up their time to attend. With the information provided at the conference and our own continuous research we were able to come to a decision on what we believe is the best option for Ella to be able to reach her full potential. At the conference we were fortunate enough to meet American Surgeons Dr Joseph Roberson (Otologist) and Dr John Reinish (Plastic Surgeon). Together they perform the worlds only Combined Atresia and Microtia (CAM) surgery. This surgery is extremely complex, Dr Roberson performs the Atresia repair, he will create a new ear canal and ear drum to achieve near- normal hearing. Dr Reinish performs the Microtia repair which using a synthetic ear framework called Medpor and complex skin grafting procedure to build a new ear. The cost of the surgery, combined with post-operative care and the recovery period of 4-6 weeks in the U.S.A, will total approx. $110,000. To give Ella the gift of hearing, we have started the journey to help Ella get the surgery she needs. This will involve significant fundraising. We thank you from the bottom of our hearts for your help and support in achieving our goals! Belinda Taylor & Nicholas Morgan
MICROTIA/ ATRESIA
What is Microtia? The word Microtia is Latin for the words ‘micro’ and ‘otia’, meaning “little ear”. Microtia is a under development of the outer ear. At early stages of pregnancy, for reasons unknown, there is an arrest in development and the ear stops growing. It is often accompanied by Atresia as the outer ear and ear canal are developed at the same time. This can occur on one or both sides, in Ella’s case she has right side grade III Microtia Atresia. There are 4 Grades of Microtia:
Grade I : a slightly small ear with identifiable structures and a small but present ear canal. Grade I Microtia is not usually associated with deafness. Grade II : a partial pinna (outer ear) with a closed off ear canal. Grade II Microtia causes conductive deafness. Grade III : the pinna, ear canal and ear canal and eardrum are missing. There may be a small peanut-shaped lobe near where the pinna would be. Grade III Microtia causes conductive deafness. Grade IV : may be known as anotia and means ‘no ear’
What is Atresia? Atresia is the absence of the ear canal. This is what causes severe conductive unilateral hearing loss for Ella. UNILATERAL HEARING
Ella has unilateral hearing, meaning she can only hear from one ear. How does this affect Ella, she has difficulty:
• Localising sound
• Hearing in noisy or crowded situations
• Is unable to hear when someone speaks on her right side
• Unless the person speaking is close to her, she cannot hear from a distance
• Ella is susceptible to ear infections on her ‘good side’ when she has a cold or ear infection, her ability to hear at all is significantly reduced, winter is not a great season for Ella. Ella has trialed bone conduction hearing devices and FM systems within the classroom at school. Although they were initially helpful at home, Ella found after some time, in noisy and group situations the aids caused interference, and she could not filter the noise and focus on what the teacher was saying, therefore she has opted to attend school unaided as she finds it easier. There are days where Ella struggles, and quite often suffers from headaches and to some degree exhaustion from focusing and trying her hardest to concentrate to hear what is being said, while for others this comes naturally. Our aim is for Ella to have the necessary surgery to allow her to have bilateral hearing.
