Aug 2011 – Beep…Beep…Beep…

Madison was fitted to have molds made for her hearing aids (HAs) when she was 9 weeks old.  Green foam was inserted into her ear, almost like silly string, which hardens and takes an impression of her inner ear.  Two weeks later we went to Cornell to pick them up.  Our audiologist showed us how the hearing aids work and how to clean them.  Since Madison’s hearing loss is so severe, they don’t expect her to be able to hear sounds of conversation.  There is a chance that she may hear environmental sounds (fire truck, airplane, ferry boat, etc), but she may continue to hear nothing...  At the very least, the HAs will prime the brain for the CIs (pending she has an auditory nerve).  The HAs seem somewhat difficult – first, they seem hard to insert in her ear and second, they make a beeping noise when not sitting in her ear properly.  Hopefully both of these aspects will get easier with practice. 

A few hours after returning home from picking up the HAs, I realize something isn’t right.  “Beep… Beep… Beep…” The noise would not stop from her left HA.  I kept trying to reposition it – but quickly realized the HA was too small and not sitting in her ear properly.  I wish this was realized while we were still at Cornell.  What a headache… back we went the following day.  New impressions were made of both ears.  Since Madison is growing so quickly, this is completely normal.  It seems like she will need to have new impressions/molds made every few weeks for the first couple of months. 

Until this day, Madison’s hearing loss was always in disguise.  Since she will always be wearing HAs during waking hours – not only does it serve as a constant reminder of our situation, but I assume will stir immediate questions from others… friends/family and strangers.  This is something I haven’t quite prepared myself for… but than again, I haven’t quite prepared myself for any of this.  I keep telling myself that things will get better and easier – we just need to take one step at a time. 

Aug 11 - Uptown Girl

On August 11, we went to the Clarke School on 83/East End Ave for 3 things:

• Tour of the facility
• Meeting with our EI coordinator
• Begin our EI State Evaluation Process

The Clarke School provides services for speech therapy in addition to having pre-K for children 3, 4 and 5 years old.  Plus, the 5 year old kindergarten class is integrated - half of the students have no hearing loss.  We met numerous students as well as the teachers. Everyone was extremely nice and it was great to see all of these children, with either CI or HA, they were talking (back) to the teachers! It was very encouraging to see the 'finished' product of where we would like to be in a few years.  All of these kids were very happy

and smiling and learning in a great environment.

 

We had our initial meeting with our EI coordinator - Tamara.  As we've mentioned before, we feel lucky to be in NYC with so many options available to us.  The EI program is actually a state-run program that will provide Madison with services X times a week.  Because of Madison's profound hearing loss, we hope to qualify for at least 2 days, and possibly 3 days a week.  Tamara has been involved with the EI program for several years and we believe she'll be a great asset for us as we embark on this journey.  Tamara was able to offer us a lot of advice and insights - into what we should expect with the EI program, as well as her experiences in dealing with children and parents in our situation.

The initial state evaluation process took about an hour and was conducted by two therapists.  The therapists asked us a ton of questions, and the test would not end until we didn't pass 4 questions in a row.  It was upsetting having to go through parts of the test, as it's used for children with numerous types of disabilities.  So, they would ask us if she responds to sound(s) and we all knew what the answer would be...  Then, the next question would be - does your child try and imitate your voice after you speak with her...  Followed by - does your child turn their head when you talk to them.  It was very frustrating to have to deal with this.

We are eager to have our follow up meeting with the state and Clarke in September.

August 2011 - DIP

DIP = Deaf Infant Program

We went on a tour of the Center for Hearing and Communications in Lower Manhattan.  In addition to being a facility that provides Early Intervention (EI) services, they conduct a special program called DIP.  The DIP program provides speech therapy services for infants until they are 3 years of age.  Because of Madison's profound hearing loss she qualifies for DIP and EI.  DIP gives Madison 2 speech therapy appointments per week for 60 minutes.
What could an infant with no hearing actually get out of a speech therapy session?  (This was one of our questions).  Initially, the sessions are geared more for the parents than the infant - considering Madison slept through her initial session.  The therapist tries to educate the parents, as well as instruct them on how to interact with their child.  The main goal is for you TO INTERACT with your child, just as if there was no hearing loss.  They encourage you to mimic your child's noises - if they speak some jargon, try to imitate that back.  Plus, they want you to constantly talk to your child - almost like the narrator of a story.
The therapists have seen the success stories and know the first few years are pivotal in her growth.  It's going to be a lot of hard work, but with the right therapy and supporting cast we're hopeful Madison will be chatting up a storm shortly after receiving CI.

Aug 8 – Madison Is 2 Months Old

Madison is smiling every day and enjoying her morning bottle feed with her daddy.  She has already outgrown most of her newborn clothing.  She had her 2 month pediatrician visit and is doing great… She received 2 vaccines and, surprisingly, she did not scream! She grew almost 2 inches and weighs 10.2 lbs.  I am amazed how fast she is growing and how quickly she seems to be learning.  The one thing she hasn’t done yet was notice her older brother & soon-to-be best friend, Alan.  Alan is probably the most well behaved chocolate lab ever! Aside from trying to enter her jungle activity mat, AKA Costa Rica, he hasn’t bothered her one bit!  

Speaking of Alan, another thing Brian and I have discovered during our journey was about “hearing dogs”.  How would Madison know to wake up if there was a fire alarm? If she was napping, how would she know the door bell was ringing or someone was trying to call her? These are all questions going through our head.  Just like there are seeing eye dogs, there are also hearing ear dogs!  Well, dogs are trained to be able to work with people who have a hearing disability.  What a great job!  We plan on getting Madison a hearing dog (lab of course!) for her 5^th birthday.  Please don’t tell her!

Aug 5 – 20 Is the Magic number

We had a surgical consult with Dr Brown, pediatric surgical ENT surgeon at Cornell Presbyterian, to discuss the possibilities of a CI.  He began by telling us that when this procedure is done at such a young age, the chances for success (meaning one being able to learn to speak) are extremely high… and chances that she could be mainstreamed into regular school at a young age is very likely.  But, not everybody is a candidate for this procedure.  On paper, Madison is an ideal candidate… but a MRI needs to be conducted to determine if she has an auditory nerve.  We were told that there is 1 in 100 chance that she will NOT have a nerve…it is extremely rare.  In addition, this physician has never encountered such thing.  We try to remain positive… but than again, given our situation already being very rare, it is very difficult to keep our hopes up.  Considering she is a candidate for a CI, she can be implanted as young as 6 months… but she needs to weight 20lbs.  Although this is a minimally invasive procedure, anesthesia is still administered and blood is still lost… thus the weigh requirement. 

August 2011 – Early Intervention

One of the best things regarding our daughter’s hearing loss is the amount of resources and support that are available, especially here in NYC.  There is a state-run program called Early Intervention (EI) that provides services from birth to 3 years of age to those with hearing loss.  Since Madison’s hearing loss is so severe, she automatically qualifies to receive speech therapy sessions.  Speech therapy for a deaf child??  If Madison is a candidate for a cochlear implant, she will be able to hear… and eventually will be able to speak.  We met with a surgeon to discuss what this would entail, but basically she would not receive the implant until she is 1 years old.  Anyway, back to Early Intervention.  In addition to speech therapy, Early Intervention will provide hearing aids (HAs) for Madison.  Although we were told that the HAs won’t allow her to hear conversation, it will give her access to more sounds than she currently receives… and prepare the brain to process sounds via the cochlear implant.  We were assigned a service coordinator that walked us through the EI process.  Boy, a lot of information was thrown our way...  I guess it’s a good thing that there are so many resources out there but it was and is very overwhelming! 

Cochlear Implant (CI)

A hearing aid amplifies incoming sound but there must be functioning hair cells and an intact nerve to transmit that amplified signal to the central auditory pathways. A CI, on the other hand, attempts to replace a function lost by the cochlea, usually due to an absence of stimulable hair cells. In a normal hearing ear, the hair cells within the cochlea act as transducers of mechanical and hydraulic vibration of the tympanic membrane, ossicles of the middle ear and perilymph and endolymph of the inner ear to chemo-electric energy capable of stimulating the eighth nerve. The decrease in hair cells causing a sensory hearing loss results in the cochlea losing its ability to stimulate the eighth nerve. The cochlear implant replaces the function of the lost hair cells by converting mechanical energy (sound waves) into electrical energy capable of exciting the auditory nerve.  CIs are surgically placed within the inner ear, bypassing the hair cells of the cochlea and directly stimulating the endings of the auditory nerve. Although there have been many variations on the theme, the basic design of an implant system has remained relatively stable over the years. It consists of an external microphone, processor and transmitter and an internal receiver-stimulator and electrode array.

Feel free to visit this website to learn more about Cochlear Implants: http://www.cochlear.com/

August 2011 - Hope

Due to advancements in technology, there is a high probability that Madison will one day learn to hear and speak just like you and I.  Most people are surprised when I tell them this but basically there are two options regarding how to communicate/raise a deaf child – learn sign language or through spoken language.  There is a device called a cochlear implant (CI), which enables someone who is profoundly deaf to hear (see above).  We plan on learning as much as possible regarding cochlear implants and hope that this is an option for Madison.

July 2011 – A Trip to Holland

We received an email from my sister-in-law a few days after receiving this news… It was titled “Trip to Holland”

A Trip to Holland

By Emily Perl Kingsley

I am often asked to describe the experience of raising a child with a disability – to

try to help people who have not shared that unique experience to understand it,

to imagine how it would feel. It’s like this…

When you’re going to have a baby, it’s like planning a fabulous vacation trip to

Italy. You buy a bunch of guide books and make your wonderful plans…the

Coliseum, the Sistine Chapel, Gondolas. You may learn some handy phrases in

Italian. It’s all very exciting. After several months of eager anticipation, the day

finally arrives. You pack your bags and off you go.

Several hours later, the plane lands. The stewardess comes in and says,

“Welcome to Holland!” “Holland?” you say. “What do you mean, Holland? I

signed up for Italy. I’m supposed to be in Italy. All my life I’ve dreamed of going

to Italy.” But there’s been a change in the flight plan. They’ve landed in Holland

and there you must stay.

The important thing is that they haven’t taken you to a horrible, disgusting, filthy

place full of pestilence, famine, and disease. It’s just a different place.

So, you must go out and buy new guidebooks. And you must learn a whole new

language. And you will meet a whole new group of people you would never have

met. It’s just a different place. It’s slower paced than Italy, less flashy than Italy.

But after you’ve been there for a while and you catch your breath, you look

around. You begin to notice that Holland has windmills. Holland has tulips. And

Holland even has Rembrandts. But everyone you know is busy coming and

going from Italy, and they’re all bragging about what a wonderful time they had

there. And for the rest of your life you will say, “Yes, that’s where I was

supposed to go. That’s what I had planned.” And the pain of that experience will

never, ever, ever, go away. The loss of that dream is a very significant loss.

But if you spend your life mourning the fact that you didn’t get to Italy, you may

never be free to enjoy the very special, the very lovely things about Holland.

July 26 – The Sound of Silence

Today was the day Madison had the ABR Diagnostic hearing test at Cornell Presbyterian.  She must be sleeping for this test as well.  Since it is more involved, she needed to be sleeping for 2 hours.  If we weren’t able to complete the test naturally, the test would be repeated using sedation.  Of course, we wanted to avoid this at all costs.  Madison behaved and she slept the entire length of the test! Tiny electrodes were placed on her head again and connected to a computer system which measured her brain’s response to different clicking sounds.  These sounds were played at 3 different frequencies and ranged from 60 decibels to 120 decibels.  Normal hearing ranges from 0 – 20 decibels.  The audiologist allowed 3 hours for the test.  Since we started on time, she was able to analyze the results and share them with us in her office later that afternoon.  Normally she would have us return the following day.  We were very anxious to hear the results and glad we did not have to wait one more day.  My mother was with us for the day so she was able to join us for the appointment.  The audiologist brought the four of us into a room and sat us down at her table.  Before she was able to say anything tears started rolling down my cheeks and my heart sunk.  Sitting in front of me was a piece of paper with Madison’s name written on top and a red line was written in across the bottom graph with the word PROFOUND.  The results showed that Madison has profound hearing loss in both of her ears.  Basically, if she is sitting outside on a runway, and an airplane takes off, she MIGHT be able to hear it.  So many questions were running through my head… WHY, WHY, WHY? Was this really happening to us? The audiologist spoke with us for a good hour discussing what her hearing condition means for us.  I couldn’t tell you what she said because all sorts of thoughts were running through my mind.  All along we had been praying for miracle… than realized we had already been given one.

July 21 – Tests, Tests and More Tests

After discussing potential outcomes between my husband and I – we were expecting the doctor to say that the left ear was better than the right ear.  We were not prepared for the news we were about to year….  Instead, we were told, “Your daughter has at the very least moderate hearing loss in both ears.”  Shocked, we asked about the different (better) results for the left ear vs. the right ear.  The doctor said that because the readings were so low, that he was unable to assume that one ear was better than the other.  Tears, followed by more tears…followed by the doctor saying, “It’s good that we’re finding this out at such a young age.  If this is her only issue, chances are she’ll be able to live a great life…It could always be worse.”  Another hearing test was scheduled to determine the exact level of hearing loss, this was called a ABR diagnostic test.  This is similar to the ABR screen test but the test is longer and results are much more detailed.  Additional appointments were scheduled – one with an Opthalmologist to determine if Madison has any vision problems and another with a genetics specialist – to determine if there is a genetic link to her hearing loss.

July 19 – “Good… She passed”

We needed Madison to sleep while the audiologist conducted the test.  The test itself was about 30 minutes.  After numerous feeding sessions she finally cooperated.  Tiny electrodes were placed on her head – one on her forehead and one behind each ear.  On the computer screen during the test was a graph with an X and Y axis.  The y-axis was numbered from 1 through 5.  The x-axis was more of a timeline.  We needed the plot to get to the number 3.  It would rise from 0 to 1, then 1 to 1.5 – and for the left ear it made it all the way to a tad above 2, but then would drop back down.  Reality started setting in, but we thought that there had to be some hearing.  Why else would the line move up? The next test was for fluid.  Upon seeing the results, the audiologist said, “Good…. She passed.” – translation = she didn’t have any fluid in her ears.  This was a test we were hoping that she would fail.  Unfortunately, that was not the case…

Auditory Brainstem Response (ABR) Hearing Screen

• The normal auditory system generates small electrical currents when excited. These electrical responses are the measured response during ABR tests.
• To record an ABR, small surface electrodes are attached to the head at several places.  An earphone is place in the ear, brief sounds are played, and the electrical signals across the electrodes are recorded.
• These electrical signals, which include the ABR, are amplified and then fed to a computer, where they are averaged. Signal averaging is used to reduce the recorded level of other ongoing brain activity and to resolve the small electrical responses generated by the auditory system.
• If hearing is normal, these electrophysiological responses should be observed for low level stimuli.
• If hearing loss exists, the threshold of these responses will be elevated.
• ABRs are less dependent on the status of the middle ear, compared to OAE tests, because the response is recorded across surface electrodes and does not have to travel back out through the middle and external ear.

July 8 - Madison Is 1 Month Old

Madison received her first round of vaccinations at her 1 month pediatrician visit…. Where she screamed more than ever! She is growing and now weighs almost 8 lbs.  The medical assistant performed the OAE screen again.  Brian and I were hopeful that she would pass…. But she did not.  Our physician still told us that this happens often and not to worry… the cause is most likely fluid trapped in her ears.  She than referred us to an audiologist where Madison would have another, more accurate, hearing test conducted, ABR hearing screen (see below).  In addition, they would conduct a which is a test to determine the amount of fluid trapped in the eardrum.

June 2011 – 1 in 1,000

Over the past few weeks Brian and I noticed that Madison was not responding to sounds.  Pans would drop on the floor of the kitchen and she would remain sound asleep.  Fire trucks would pass down our block and she would not stir.  We found ourselves purposely making loud noises to see if Madison would flinch… She would not.  This is when our research began… We started reading about different reasons babies do not pass the newborn hearing screen.  Since the pregnancy was healthy and there were no direct links to hearing loss, we didn’t even suspect this was an issue.  We learned that for every 1,000 babies born, 1 has profound hearing loss.  We felt the reason she wasn’t passing the tests was due to fluid in her ears.  Worst case scenario was she would need to have tubes surgically placed to remove the fluid, if it didn’t resolve on it’s own. 

Otoacoustic Emissions (OAE) Hearing Screen

• The normal cochlea produces sound in response to external stimulation; this internally generated sound is the measured response during OAE tests.
• To measure OAEs, a small probe is placed in the infant’s ear canal and sound is presented by either one or two tiny loudspeakers. Any response generated by the ear is recorded with a small microphone housed inside the probe.
• If an infant’s cochlea is functioning normally, internally generated sounds will be recorded.
• If cochlear hearing loss exists, the cochlea either will not generate a response or it will generate a response that falls below the level that is expected from an ear with normal hearing.
• Normal external and middle ear function is important if OAE measurements are to be interpreted correctly as tests of cochlear function. Even if a cochlea is functioning normally and produces an OAE, the response may not be recorded if it is attenuated by fluid in the middle ear or any external ear canal anomalies.

June 13 - "Most Likely Fluid"

Madison had her first physician’s visit a few days after we got home from the hospital.  She was a healthy baby and would not receive any vaccines until her one month visit.  The medical technician performed the same “newborn hearing screen” that was conducted in the hospital.  This is known as the OAE hearing screen (see below).  Madison did not pass in either of her ears.  Our physician asked if we had any hearing loss issues in our families.  We told her that we did not.  Once again, we were reassured by our physician that things are okay and not to worry.  She stated that the cause is most likely fluid and that will drain on it’s own over time.  Brian and I didn’t really think much of it, but we did start to pay attention to Madison’s response to sounds…

June 9 - Newborn Hearing Screen

At the time, little did we know that this was going to be the first of many tests in Madison’s life.  An audiologist came into our recovery room saying she needed to conduct a “newborn hearing screen”.  She inserted tiny probes into Madison’s ears and measured the output of sound waves with a small hand-held device.  She didn’t pass… We were told not to worry, that most babies don’t pass at this age.  The likely cause is fluid in the ear from birth.  She reassured us that everything was going to be okay.  Brian and I thought nothing of it and continued enjoying the moments of Madison’s first day of life.

June 8, 2011 - Happy Birthday To You!

7:58pm Madison Elizabeth is welcomed into the world weighing 7lbs & 1 oz and 21 inches