A Little History

Sydney was born on July 22, 2004. She was full term and was born via repeat C-section. Soon after she was born we found out she had Treacher Collins Syndrome. The first few months were a roller coaster of emotions as we tried to learn all we could about TCS and the choices we would have to make for her future. After hours in front of the computer I stumbled across 2 yahoo groups that have been very valuable to Alan and me--the Treacher Collins Group and the Atresia Microtia group.

HEARING
Initially we didn't know if Sydney could, or would be able to hear. In September of 2004 we went to St. Louis Children's Hospital for an ABR where we found that Sydney only had a conductive hearing loss-- occurs when sound is not conducted efficiently through the outer ear canal to the eardrum and the tiny bones of the middle ear. I cried when the audiologist said they had a bone conducting hearing aid we could take home and use until we got her BAHA (Bone Anchored Hearing Aid) softband. Her eyes lit up when it when the loaner hearing aid was turned on for the first time. In February of 2005 she received her first BAHA thanks to the hard work of our case worker from Early Intervention and the audiologists at the Central Institute for the Deaf in St. Louis. In May of 2007 we purchased a second BAHA to have so she would never have to be without her "ears" as we call it. With her BAHA she has near perfect hearing. Alan always says that she can "hear a mouse fart with that thing on" :-)

On the Atresia/Microtia group I learned about atresia(absence of an external ear canal) repair. However, after a CT scan in January of this year we found that Sydney was not a candidate for this type of repair. Her facial nerve runs to close to where the canal would need to go. The risk was not worth it for us. Therefore she will always need her BAHA.

MICROTIA
Microtia is the term for an ear that does not form completely. Sydney has bilateral atresia and microtia. It was in the Atresia Microtia group that I learned about the 2 main methods of ear reconstruction--rib graft and Medpor. In rib graft reconstruction the child's own rib is used to make the framework for the new ear. We found Medpor reconstruction with Dr. John Reinisch to be the right choice for Sydney. Medpor ear reconstruction is done in 2 stages. Since Sydney needs bi-lateral ear reconstruction we will do stage 1 on the first ear on September 8, 2008, stage 1 on the second ear in January 2009, and stage 2 on both ears in May of 2009. It has been over a year since we scheduled this surgery, and we are very excited and nervous about finally starting this process.