Sunday, June 24, 2007

Nichole's Cochlear Implant Surgery 6/22/2007

June 22, 2007 - The Implantation Surgery

We checked into Children's Hospital, Boston around 10:40am. About 1:15pm, they moved us to the Pre-Surgical holding area, where Nichole changed into a Johnny and had an IV started. We met with her surgeon, Dr. Licameli and Dr. Scott. Nichole received a small amount of medication to start feeling sleepy. About 1:50pm I went with Nichole to the operating room. I helped move her to the OR table and talked with her until they put her under. I then took her hearing aids out.

The waiting area at Children's is very nice. They have two liaison nurses who check with the OR every hour and give parents updates. At 5:00pm, the Liaison nurse said they are finishing up and that it should be another 45 minutes. 5:45pm came and went. 6:00pm. 6:15pm. Then at 6:30pm, we were getting nervous. We decided to go check with Nurses on why no update. Dr. Licameli came out as we were heading to the nurse's office. He said “Nichole is doing well, and the implant is perfect. Now let me tell you what went wrong”.

The insertion of the first implant had an issue. Dr. Licameli had completed the implantation. They closed the incision and then our Audiologist (Marilyn) performed the post-implant testing. The signal integrity results were terrible. An X-ray showed that the implant was folded up. The tip, which is very soft and flexible, had folded over on itself when it hit a deformity within Nichole’s cochlea (Mondini Syndrome – see notes at bottom). Dr. Licameli decided that rather than send Nichole on her way and see “how she does” (which Marilyn indicated would be terrible), he opted to remove the first implant and use the backup. They re-opened the incision (had to cut all the stitches), removed the first implant and installed the second one. Dr. Licameli said that during the first implantation, he had removed the guide wire while inserting the electrode array. For the second implant, he left the guide wire in for the whole insertion process, and then removed the guide afterwards. An X-ray of the second implant showed it was well placed, and post-implant testing results were perfect. Nichole will get optimal use from this implant.

At 6:45pm, we joined Nichole in the recovery room. She was still sleepy, and appeared to be in a lot of discomfort. They have her IV morphine for the pain. Around 8:00pm, they released Nichole form recovery and moved her up to 8 West. Nichole started vomiting small amounts of "old" blood (not a great feeling seeing that!). This continued for the next five hours or so. The nurses (who were wonderful) gave Nichole medicine for nausea, but I think she just needed to get rid of all that drainage which had run from her ear to her stomach via the eustachian tube.

Around 6:00am, a resident came in and removed the bandage. Everything looked fine. Two other doctors came in at 8:00am to check on Nichole. They made sure her facial nerves were not effected by having her make a bunch of different faces. Whew. Then Nichole mentioned her tongue was numb.
They said that the numbness in Nichole’s tongue would fade over the course of a few weeks. There is a major nerve near the implant site that forks into two branches. One branch is the main facial nerve, while the other is the taste/sensation nerve to the back of the tongue. They needed to drill a hole in the skull between these two branches. They try to err on the side of the tongue nerve so that the facial nerve is not damaged. All testing of Nichole’s face muscles show no impact to the face nerve. Nichole mentioned her neck was sore on the right side (the implant side). The doctor indicated it was just stiff from being in one position during the surgery and would ease with time.

Nichole was discharged around 1:30pm. The pain med they prescribed is quite strong, making her a bit wobbly, and sleepy. We headed home (with a stop in the North End for some Regina's Pizza to bring home for Nichole's sister and brother. While Nichole is supposed to be on a "soft food" diet, we caved to her desire for a slice. Nichole basically rested the balance of the weekend.

Thursday, June 21, 2007

The straw that broke the camels back

Some people have asked what caused us to finally have Nichole implanted. In the fall of her Freshman year in high school, Nichole came home with an assignment to listen to three recorded interviews on NPR radio on-line, and critique the interviewers. I set her up in the computer room (quiet location), with her facing the screen with the speakers 2 feet from her face. Perfect conditions. We increased the volume, logged into NPR and started the first story playing. After about 30 seconds, Nichole turned to me and said "Dad, I know they are talking, but I can't understand anything they are saying". As a Dad, this made me want to cry. I ended up sitting across from her at the kitchen table, with headphones on, listening to the story on my laptop, pausing it, and repeating each sentence I heard so that Nichole could hear me and read my lips. This got her through that assignment, but I knew I couldn't always be there to help in the future.

My wife had been commenting in the weeks before this event that Nichole was not hearing too well. This inability to understand the electronically reproduced spoken word drove home the point to me; the proverbial final straw. I called her audiologist the next day and made an appointment for a hearing test. The results showed her down around 100dB to 110dB, worse at some frequencies. Her aided results were between 40dB and 60dB. Her word discrimination was in the low-mid 30% range. Clearly she was not hearing a lot of speech.

With no real options with respect to new, more powerful hearing aids, the Cochlear Implant offered us the only real hope for Nichole. So we contacted Marilyn Neault at Children's Hospital in Boston and brought Nichole in for a battery of tests. The results showed that Nichole was a good candidate. A CAT scan showed Nichole was a mild case of Mondini Syndrome, where the cochlea doesn't spiral the full two and a half to two and two thirds turns. Nichole's turned only about one and three quarters times. Even still, her surgeon, Dr. Greg Licameli indicated he thought she would still do very well with an implant.

Nichole was in every meeting and participated in all the discussions about the CI. Given the anticipated amount of work involved with hearing rehabilitation, we wanted her to be 110% committed to this, otherwise we would not proceed. She decided it was necessary, and that she would work to learn how to hear with it, so we decided to go ahead with the surgery.


Welcome. This blog chronicles my daughter's journey through the process of getting a Cochlear Implant. Nichole is 15 years old and was diagnosed with a moderate to severe bilateral sernsorineural hearing imparement (~70dB) at 18 months old. She started wearing hearing aids bilaterally at 20 months. For those not up on all the lingo, this just means that she couldn't hear normal conversations as a baby, so she wore hearing aids in both ears (see how simple that can be).

We decided on the Auditory Verbal Therapy approach to help Nichole develope her speech. This worked very well. Nichole has very natural speech. Most people don't know that she has a hearing imparement unless they are told. Nichole has been mainstreamed in public school her whole life.

Over the years, Nichole's hearing loss slowly progressed (got worse), until this past year, while in the 9th grade, Nichole's hearing got noticiably worse. It had drifted down from her original 70dB loss, down to 100/105 dB. For those who don't know the numbers, that means she could stand beside heavy machinery (like jet engines) and just detect a little sound. Her hearing aides allowed her to only hear 45/60dB sounds. Her word discrimination in a sound booth was about 30%. That means, that in the best conditions, she could only understand 30% of the words she heard.

Nichole is a smart young woman though. She developed a good lipreading skill which helped her fill in the blanks. She always used an FM system in school, to help her hear her teachers during classes. But still, she was only understanding a small fraction of the words being said in each class. Even with all that, she managed A's and B's (with one C) during her Freshman year in high school.