Hearing loss: Rachel's story: the technological wonder of cochlear implants.

Rachel Kathryn Skergan was born to David and Natalie Skergan on September 17, 2003 at Duke University Medical Center in Durham, NC. She was the product of a healthy pregnancy and uncomplicated delivery and had APGAR (a test given just after birth to quickly evaluate a newborn's physical condition after delivery) scores of 9 and 9 at birth. A few hours after she was born, she was given the routine newborn hearing screening. She did not pass the newborn hearing screening in either ear. She was healthy so everyone told us that she probably had fluid in her ears that was causing her to fail the hearing screening and that it would resolve with time. Rachel was retested two weeks later and failed the screening again. Two weeks after that, on October 14, 2003, Rachel had no response in either ear on her diagnostic auditory brainstem response (ABR) test and was officially diagnosed with bilateral severe to profound sensorineural hearing loss. After more testing, the cause of Rachel's hearing loss was determined to be due to the 35delG mutation of the Connexin 26 gene. This is an autosomal recessive condition that causes hearing loss only. Rachel was fitted for hearing aids and began wearing her hearing aids on December 17, 2003 when she was three-months-old.

Rachel began auditory-verbal therapy (AVT) when she was three-months-old; however, she was not making the progress that she should have made with the development of spoken language with her hearing aids. We decided to pursue a cochlear implant (CI) for her. My husband and I researched all three CI manufacturers and chose MED-EL, a worldwide leader in hearing implant technology, because we felt it was the best device.

Rachel had surgery at UNC Hospital in Chapel Hill, NC and received a MEDEL Combi 40+ CI in her left ear on July 20, 2004 when she was 10-months-old. She did well with her CI, and by one year post-implant, her receptive and expressive language skills were on target with her normally hearing peers of the same chronological age. Since Rachel did so well with her first CI, we decided to pursue a second CI for her. We wanted to give her the opportunity for the best hearing possible. (We had actually inquired about simultaneous bilateral cochlear implantation when she received her first CI, but it was not recommended to us at that time.) Rachel had surgery again at UNC Hospital on May 23, 2006 and received a MED-EL Pulsar CI in her right ear (her sequential bilateral CI) when she was two-years- and eight-months-old. It was our hope that with two cochlear implants, her hearing in noise would improve (this would help social and academic situations) and that she would develop the ability to localize sound.

Rachel has done very well with her bilateral CIs. Her incidental hearing seems to have improved, and her use of natural language has also significantly improved since she became a bilateral CI user.

Rachel had formal language evaluations in August 2006 as part of her annual testing and also part of her transition from the early intervention program to preschool services. Her test results revealed that her expressive and receptive language is on target and ahead of her peers. Rachel was 2.10 years of age at the time of testing, and her scores were comparative to a child who is 3.6 years of age. We were pleased with her results. Rachel is currently attending a mainstream preschool two mornings a week. She has an itinerant teacher of the hearing impaired who comes to our house once a week for an hour of AVT. Rachel is doing so well that she almost did not "qualify" for IEP services; however, we all agreed that she has done so well because of the excellent services/AVT that she has received up to this point.

Rachel is currently wearing her MED-EL Tempo speech processors as BTEs with the angled battery pack configuration. We love her MED-EL CIs! She only broke one cable the entire first year that she had her CI. She is very gentle with her devices. She has been wearing her speech processors as BTEs since she was two-years-and five-months-old. Prior to that, she wore them pinned to her shirt in the Baby BTE configuration, and this worked well for her during that time.

Our family was devastated when Rachel was diagnosed with hearing loss, and we really didn't know what was possible for her at that time. We quickly learned all that we could and gathered all of the information necessary to find the best treatment option available for her and were excited by the realistic possibility of her learning spoken language. We are so fortunate to be in a time when medical technology, such as the cochlear implant, works so well. Hearing loss is not life threatening, but it is quality of life threatening, and we wanted to give our daughter the best quality of life possible. We also wanted to raise her to be independent and to be able to thrive in this world and, most importantly, to be happy. Rachel's CI performance has far exceeded our expectations. She has excellent speech and language, and most people do not even realize that she is hearing impaired. Many times we also forget this because she hears and talks so well! She is very happy and well adjusted, is very confident, loves preschool, and has many friends. She loves to sing, listen to music, tell stories, and talks all the time!

We are so thankful to everyone at MED-EL! The gift of sound and freedom that Rachel's bilateral CIs have given her is simply immeasurable. Her life is so different because of this awesome technology.

For more information on MED-EL and BRIDGE to Better Communication, a program especially for hearing loss professionals, schools, cochlear implant users and parents to help close the gap in the development of improved listening skills and spoken communication for children with hearing loss, please visit http://www.medel.com.