Below is the information we provided to our district.
Andrew was born with profound hearing loss in both ears. The cause of the hearing loss is genetic defect in the Connexin 26 gene, which disrupts potassium flow in the inner ear that prevents the hair cells’ stimulation of the auditory nerve. The hearing loss was diagnosed at birth, and was later found to be at least 90 decibels in each ear.
Drew obtained bilateral hearing aids in December 2006 at 8 weeks old. With the amplification, audiological testing showed that Drew was only able to hear at 70 dbs for the lower frequencies. This level of hearing would include an airplane overhead, but no speech sounds or other typical environmental sounds, such as a doorbell, or dog barking. Parents never saw any reaction to sounds when Drew was using his hearing aids.
Drew’s parents were aggressive in their quest for Drew to hear. After traveling to St. Louis , Cincinnati , and Cleveland to consult with experts in the field of newborn hearing loss, cochlear implants, and oral-deaf education, they decided to pursue simultaneous bilateral cochlear implants, which were almost unheard of at that time. Not only was it rare for persons to have two cochlear implants, but it was even more rare for a child to receive two during the same surgery.
After interviewing and comparing three surgeons for this new and relatively untested surgical procedure, they finally found a surgeon in Columbus who would implant Drew simultaneously at a young age. They then fought the FDA guideline which suggested children be at least 12 months old before receiving a cochlear implant, arguing that implanting him at 8 months of age would give him a better chance at having normal speech.
Andrew continued wearing his hearing aids until he received simultaneous cochlear implants, which were first activated at age 9 months. These implants were activated fully by age one. Activation involves slowly turning the implants on so that he gradually became accustomed to hearing. The cochlear implant is an array of 22 electrodes inserted in the cochlea of each ear, with an accompanying magnetic receiver which is implanted in his skull. Andrew wears external processors on each ear, which are programmed by a skilled pediatric audiologist. The external pieces consist of microphones which pick up sound, the processor which “codes” the sound, a magnetic receiver (referred to as a coil) which transmits the electronic stimulus to the internal parts. His mother described his hearing as “electronic” and not natural, often referred to as acoustic. Drew will never hear as well as a hearing person does, and he will never hear more than a slice of the frequencies others do. Fortunately, his implants are programmed to pick up most of the speech frequencies.
Immediately following activation, Andrew began aggressive auditory training with professionals specifically trained in teaching deaf children how to listen and speak. Drew has attended therapy a minimum of two hours a week in a formal, clinical setting. These therapists have been instrumental in ensuring the processors are mapped appropriately, that Drew is meeting listening and language milestones and for his overall language and communication progress. This therapy has been essential in the development of spoken language for Drew.Potential Problems
1) Equipment Maintenance
His mother noted that equipment issues are very common and impact Andrew’s ability to hear properly. Faulty baby worn wires, coils, dirty microphone covers, loose batteries and cracked controllers can cause the speech processor to turn off. Dirty microphone covers can muffle the sound being coded by the speech processor. Some potential areas that could affect microphone clarity include dirt, sand and moisture. Also, if anything is placed over Drew’s ears, such as a hat or hood, ability to hear is compromised.
2) Internal Failure
An additional area of concern is breakage of the internal parts. A fall or bump at the “right” spot on the head can cause the magnetic receiver or electrode array to break. This would require surgery, and the process of reprogramming (referred to as mapping) would be extensive.
3) Static Electricity
Static also presents a problem. A static charge can trigger the processor to turn off, or in the case of a strong static charge, can erase the map on the processor, which would require an appointment with the audiologist. Areas to avoid include plastic play equipment and fleece clothing.
4) Ear Infections
Ear infections can cause the equipment to work inefficiently, and have the potential to cause an infection around the implant which could require its removal. Fortunately, he has had very few ear infections, and all ear infections are treated aggressively by Drew’s ENT/Surgeon.
5) Moisture
The speech processors (“ears”, as they are referred to by Andrew and his family) are not allowed to get wet, so he can not wear them when swimming or bathing. Andrew is now able to take his “ears” off by himself. He is also now able to sometimes let his parents know when they are not working.
6) Hearing In Noise and Sound Localization
Lastly, noisy situations, including classrooms, restaurants and the outdoors, can be difficult for Andrew, as sound localization and quality is affected.
Educational ImplicationsHis parents judge Andrew in need of continued auditory training/listening therapy, in order to develop his communication skills and ultimately literacy skills. They would like a preschool teacher, specifically one for the deaf, who would reinforce what he learns in an auditory training program, which will fine tune his ability to listen within his environment and will benefit him significantly when he moves to the mainstream.
As cochlear implant maps change over time, his parents daily check his hearing using the ling six sounds. Drew’s teacher will also be expected to review Drew’s ling sounds each morning as he arrives at school. Lings are six sounds (/oo/, /e/, /m/, /sh/, /ah/, and /s/) that predict well his ability to hear all other English phonemes. This check needs to be conducted in a variety of conditions, such as with noise, at a distance of 6, 9 or 12 feet and when the speaker is using a listening hoop. An equipment check is required at the beginning of each day to make sure the processors are on and working.
Another concern is literacy. Knowing that adult deaf people have an average reading level of fourth grade, the parents are concerned about Drew’s ability to learn to read. While speech alone is a huge concern, reading and greater literacy is a task that will be learned over many years. Drew’s Mom reports having struggled with literacy and knows how frustrating those struggles will be.
Drew needs to have an intensive literacy program, with teachers that are trained in teaching the deaf to read to provide a foundation that can be built upon during the school age years. Without this foundation, the parents are very concerned that Drew will fall behind and require significant intervention throughout his entire school career. With appropriate preschool education, they are hopeful that his needs will be minimal once he is of school age. The primary goal is literacy, with speech being only one part of being literate in society at large.
Other concerns include, but are not limited to, articulation, consonant deletion, consonant production through listening only (using a listening hoop where the speakers mouth is concealed) and grammar. Drew has also developed an issue with speech production, where he will “stutter” as he searches for the word(s) he needs to answer a question or to convey his need(s).
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