Wednesday, September 28, 2016

Surgery Date Set

Playing in Brooklyn

We returned to the Big Apple to see the surgeon with regard to Nolan's cochlear implant. The hearing tests already demonstrated he would be a good candidate for the implant, but this appointment was to determine if he was a good candidate medically. The staff at NYU is just amazing - truly, they are world-class. Dr. R. looked at Nolan's MRI and CT scan results from when he was a baby (normal anatomy). His anatomy is good, so there will be no issues with the device placement.

The one thing we were worried about is the gaping hole in Nolan's right eardrum. We have been told the hole is about 15% to 20% of his eardrum, and I had no idea what they were going to do about that. Would they need to do a second surgery to seal the eardrum? Would that preclude him from candidacy? I truly had no idea, and was hopeful it wouldn't be that big of a deal.

Fortunately, this is NYU and they are amazing, so there will just be one surgery and Nolan will have his tympanoplasty when the cochlear implant is placed. I am extremely grateful this will be a "one and done" surgery. This will also prevent more infections from happening to that ear, as most infections arise because he has a massive hole in his eardrum. We are very careful to keep that ear dry, but it will be a relief not to worry about water in the ear anymore.

Surgery is scheduled for November 11, which is fantastic as Nolan will miss less school (Veteran's Day Holiday). He will obviously miss some school the week after surgery, but we are trying to keep the missed days to a minimum.

NYU  Medical Center

He will be "switched on" December 6 and December 7. He will get his first MAP (program) on December 14.  Hearing with a cochlear implant is a process, and the programming has to be adjusted frequently in the initial stages. The sound will likely be strange for a while, but with time and therapy his brain will adjust and the sound will normalize.

We drove home immediately after the appointment and made it home by 1:00 am. Fortunately we won't return until November 10, so we have some downtime before the crazy travel schedule hits again!

Saturday, September 24, 2016

Goodbye, Hearing - Hello, NYU

Life is insanely busy and I don't often update Nolan's blog - but here is my yearly post (ha). Nolan is now 9 years old. We have had an insanely cool summer, visiting Quebec City, Tadoussac to see beluga whales in the wild, and hiking the via ferrata in Ausable Chasm.

Last December, we went to Cleveland Clinic to evaluate Nolan's hearing. At the time, there were some small changes in his left ear (his good ear), but the right ear was largely stable, ranging from 60-90 dB (moderate to severe). His aided thresholds were in the mild-moderate range.

His speech perception using live voice was 80% in the "bad" ear and 100% in the good ear. We knew we would need to upgrade his hearing aids from Bolero Q SP's if his hearing degenerated anymore, but also knew that if his right ear lost more, he might be in the range for a cochlear implant.

Over the summer, we were getting more "whats" and non-responses from Nolan. If he was looking at you, he could understand you. If he wasn't looking at you, he sometimes wouldn't detect that you had spoken. The left side still seemed to be getting sound, but the right ear appeared to have great difficulty. I did the Ling sounds for just the right ear, and the only sound he picked up was "ah."

I knew this meant he had lost additional hearing, and made the call to NYU's cochlear implant program. I wanted an evaluation to get him into the system just in case the right ear had dropped. If he didn't qualify, that would be fine - but I had suspicions that his right ear was losing residual hearing quickly.

They reviewed his audiogram from last December and said he wouldn't be a candidate with that audiogram, but considering his history we should bring him in for an evaluation. We had hotels booked and made the 7 hour drive to New York City (this drive takes longer than 7 hours with traffic, but is still well worth the trip).

NYU performed a full cochlear implant evaluation for Nolan. We first met with the LSLS (Listening and Spoken Language Specialist) and the audiologist who would be testing Nolan's hearing. We explained that we knew he was not a likely candidate, but preferred to err on the side of caution since he had been having difficulty lately and is in the fourth grade - we don't want his academics to suffer if the left ear decides to take a sudden dive and we lose his "good" ear.

The testing was broken up into three days. Each day, Nolan sat in the booth for 90 minutes and performed various listening tasks. Day 1 was the unaided audiogram. I don't have a copy of the audiogram yet, but my suspicions were confirmed. The left ear is largely where it was at Cleveland Clinic (though his speech reception has declined slightly), but the right ear is now profoundly deaf. His best threshold was at 90 dB in the low frequencies, dropping to over 100 dB in the highs (to no response at 8000 Hz). Nolan's speech reception was 48% in the good ear with speech at 85 dB, and was very poor in the right ear (24% at 95 dB).

Suddenly we were no longer "getting into the system," but entering true cochlear implant candidacy evaluations. Cochlear implant candidacy requires a lot more than unaided testing, so we would return the following day for testing in the aided condition.

Nolan really wanted to see the Statue of Liberty while we were in NYC, so we left the clinic to explore Manhattan.

 Taking a cruise to see Lady Liberty

At the World Trade Center on September 12 

We returned to the cochlear implant clinic the following day to repeat a lot of the previous testing, this time with Nolan's hearing aids on. The audiologist said words and sentences, but this time the volume was at 60 dB instead of 95 dB, since he had hearing aids on. His left (good) ear was fantastic - he was able to get a score of 84% in that ear. The right ear, unfortunately, only scored 36% for words and 27% for phonemes, even with a hearing aid on. His right ear, even in the perfect silence of the sound booth, just wasn't really hearing clear speech. His candidacy looked like a surer thing, but they wanted to try one more thing first: more  powerful hearing aids.

Nolan has super power aids which are supposed to accommodate a loss of up to 110 dB in the high frequencies, but he was at or beyond that level unaided. We needed to determine if the Naida UP (the most powerful hearing aid on the market) would bring his aided hearing into the desired range. 

We left the clinic to explore more of New York City, including the Natural History Museum. 


Checking out Times Square

The third day of testing would be the determining factor for whether or not Nolan would qualify for an implant. Nolan had language testing performed (his language skills are insanely high, due to very early intervention and a mother that never shuts up   extensive exposure in the home environment.

He was brought back into the booth, this time with the Naida UP's. The UP hearing aid brought his left ear into the mild range for hearing levels and he obtained 100% speech recognition with it. We were thrilled! The right ear, however, did not fare as well. He was aided to the mild/moderate range with the Naida UP in the right ear. He did improve his speech understanding with the Naida UP, but was still missing half of the speech signal, at 60 dB in quiet (live voice).

This is a tricky situation - as he might do well with the Naida UP hearing aids, but his hearing loss is progressive, the right ear is already profound, and if we continue to wait the left ear will likely drop - leaving him with very poor hearing while trying to navigate the fourth grade. It is also likely the right ear will continue to drop, so we would be spending a significant amount of money (near $8000) for a very brief period of time. There is no telling when the left ear will drop, but it always trails the right ear by a few months to a year. We decided that if he was a candidate, we would proceed with implantation of the right ear. This will give him a "stable" ear (once rehabilitated) in case the left ear decides to take a dive on him in the future.

It was decided that Nolan would be a candidate for a cochlear implant in the right ear, and we were able to look at the three major brands who manufacture the device. Each brand is slightly different, but all serve the same function and operate in the same manner. It is a bit like buying a car - all are reliable and will get you to your destination, but the features vary from model to model. We took home a ton of literature from that appointment and returned to our home in Western NY to figure out which brand we want our son to have - for the rest of his life. While we know they all work just fine (cochlear implant centers wouldn't use a brand that didn't work), we need to determine what the best brand would be for Nolan.

We are returning to NYU tomorrow to meet with Nolan's surgeon. We are bringing Nolan's CT scan and MRI from when he was a baby so we won't have to repeat those tests. The process is long and involved, and we have to get insurance approval, the surgery scheduled, turn on the device, program the device, and do a lot of auditory-verbal-therapy to help his brain learn to use the signal generated by the implant. While it will take some time for him to adjust to it, his hearing will be better with the cochlear implant than it will be with his hearing aid. 

Tuesday, November 3, 2015

It's Been a Long Time

I haven't updated Nolan's blog in a long time. Primarily because I now work outside the home (and have little time for writing), and also because things have been going "hunky-dory." There is some exciting news on the home front: Nolan is growing on his own and we will be able to remove his feeding tube later this month. This is a wonderful development and we are absolutely thrilled. He'll never be a giant, but he is growing and otherwise thriving.

He plays soccer and loves it. He plays the piano and is flying through his lesson books - he has a natural talent and his fingers fly over the keyboard. He does wonderfully in the academic arena and is excelling in his third grade class.

His hearing, however, continues to deteriorate. This past spring, we had his PE tubes (grommets) removed and paper patches applied to try to heal his eardrums. Unfortunately, the right ear is stubborn with its infections and he had an infection in the recovery period. The paper patch didn't "take" in that ear.

The ear infections continue in that ear. It is a catch-22: they won't do a tympanoplasty in that ear until it is "dry," but the infections are not likely to stop, particularly since he has such a large hole in that eardrum. The nurse practitioner estimated about 15%-20% of his eardrum is missing. We had a hearing test done in an infection-free time and found the following:

The right ear is now in the severe range from 1000 Hz-8000 Hz. He does hit moderate at 500 Hz in that ear, but all other frequencies are in the severe range. The left ear is doing better, at a moderate-moderately severe level across the board. The conductive component is still there. The ENT does not believe it is due to the hole in his eardrum, though we cannot be sure. Our current ENT has decided to ignore the right ear and simply focus on the better ear. His better ear (the left one) is aiding to 25-35 dB (the "As" on the audiogram above). Our current audiology practice does not test the aided ears separately, so the worse ear is not evaluated.

Unfortunately, we are seeing some signs of speech "slippage" from Nolan. His high frequency sounds are less clear. Words like "kits" are becoming "kiss." He currently has (yet another) ear infection in the right ear. Buffalo cultures it each time, delaying the treatment time and causing that ear to continue to degenerate.

Since the audiology practices locally don't test the aided ears individually, it is hard to tell what, if anything, the right ear is perceiving. When we do the Ling 6 test with the right ear aided (taking out the left hearing aid), he only detects the "AH" sound.

I am concerned about the right ear "dropping off the map," so I contacted Cleveland Clinic for a second opinion. The audiology practices in Buffalo are not practicing according to the current best practice guidelines for pediatric audiology, and Nolan is not aided as well as he should be.

Cleveland Clinic is amazingly responsive and have a plan in place. We have an appointment for December 21 with audiology and the ENT surgeon there: we will see if there is anything that can be done for the right ear (since there is a perforation in the eardrum) and we will see what can be done with his aided hearing results in the sound booth. Cleveland does real-ear testing (Nolan has never had real-ear testing) and will test each aided ear individually. There are several options available to us, and the ultimate goal is to allow Nolan to hear better. It is possible his hearing aids can be cranked up to allow him to access more sound, or we may need a technology change.

In any case, there is our update (hey, it's only been nearly two years)!

Sunday, January 19, 2014

New Glasses

The optometrist's shop must have rushed Nolan's new eyeglasses frames after they ordered the wrong ones. Within 24 hours, we received a call stating they were ready to be picked up!

He is so excited to have his new glasses. He picked out an orange case and was thrilled to wear his new specs to school. These are definitely the right frames, and Nolan is pleased that the arms match his ski helmet (both are green).

We will pick up his new hearing aids on Wednesday. I am looking forward to it, because every other word is "what?" at the moment. I think his old aids are on their last legs!

Wednesday, January 15, 2014

New Glasses and New Hearing Aids: FAIL

On Tuesday, we were supposed to drive up to Williamsville to get Nolan's new hearing aids. The audiology center called and confirmed the appointment, I took the day off work, and sent Nolan to school on Tuesday morning in a state of glee - he was very excited about his new "ears."

The phone rang an hour before I was supposed to leave. The audiologist called, and said that the hearing aids didn't have the correct FM receivers, so there was no point in driving up to Williamsville, since we'd just have to return a few days later to have the FM receivers installed.

Apparently, the audiologist had noticed the wrong FM receivers were shipped from Phonak when they arrived in December, and had her staff member place an order for the correct ones. The proper receivers never arrived, and she checked on the status the day we were to drive up to collect the hearing aids. Phonak had no record of Nolan's name and no record of the order. The new FM receivers have been ordered and are on a "rush" status, but we won't be able to collect them until Wednesday the 22nd. Nolan was beyond disappointed, but what can you do?

On the bright side, at least they managed to call BEFORE we had driven for 2 hours to the hearing center.

Today, we were supposed to pick up Nolan's new glasses. I took another day off work, and Nolan missed his morning classwork. We arrived at Spectrum and the technician pulled out a set of frames. Blue and brown ones.

Nolan said, "They're supposed to be green!"

Nolan with his old glasses at the optometrist. This was supposed to be the "before" picture.

There was some argument - the tech insisted these were the correct frames, until she checked the serial number we had actually ordered - but we had ordered the frames with the green arms to match Nolan's ski helmet. Once the records were checked, she admitted the wrong frames had been ordered.

So Nolan doesn't have new glasses, either.


Now I get to take 2 more days off work - though we're not sure when the new glasses will be in. They have to order new lenses and get the proper frame sent to the store.

Hopefully sometime by the end of January, Nolan will have new hearing aids and glasses. Heavens knows that we pay enough for these necessary items - if only the medical staff were competent enough to provide them!

Friday, January 10, 2014

Happy 6th Hearing Birthday

Nolan's sixth hearing birthday was yesterday. I would have posted a blog then.. but, truth be told, I completely forgot. I didn't remember until the audiologist called to confirm his appointment on the 14th, when I suddenly realized - hey, it is January 10th! Yesterday was the anniversary of when he first heard sound!

He has grown a lot since those first moments with his hearing aids. I'll never forget the day before he received his first hearing aids. It was a sunny day in January, and I was so anxious about the future. How would we communicate? Would he lose more hearing? Was anything else wrong with our precious baby? Would he talk? What would his life be like?

The next day, his ears were born. He could hear something without hearing aids at this point in his life. He had a moderate hearing loss, but all speech sounded like Charlie Brown's teachers. Hearing aids would bring those blurry sounds into focus - allowing him to hear all the sounds of speech.

My bobble-headed baby boy loved sound, and was a mere four months of age when we turned on his ears. Over time, answers came to my questions.

Would he lose more hearing? Yes. Nolan now has a moderately severe to severe hearing loss. He is likely to continue to lose hearing (albeit slowly). With technology, however, this isn't as much of a worry - he will always have access to sound, even if we have to change the technology down the road.

As for how we would communicate - well, we often have to tell Nolan to take a five minute break from talking. He is the most verbal person in our family. His receptive and expressive language is off the charts. He talks. He sings. He plays the piano. And sometimes the pipe organ.

He is one of the top readers in his class. I used to worry that he would need help with his reading - as it turns out, he is pulled out for a special reading group. This reading group, however, is for the advanced readers in the first grade.

Was anything else wrong with him? That question unfolded over the years. For several terrifying years, we agonized over what was going on with our little boy. The inability to grow, breathing difficulties, constant ear and sinus infections, amblyopia... every new symptom caused a wave of panic. Now that we have some answers, we are able to help him and avoid the vomiting attacks and other problems that come with dysautonomia (one of the unfortunate conditions that often comes along with Ehlers Danlos Syndrome). He is growing. His vision is now correcting to 20/30, and we have stopped the sinus and ear infections. Some things aren't "fixable," but he is stable and we know how to help him. I finally feel like we have a handle on things.

And as for what his life would be like.. well, it is simply regular. He goes to a regular first grade class. He plays on a soccer team in the summer. He is a bit of a daredevil and rides roller coasters that I will never, ever dare to ride. He calls himself the "green bullet" when he darts down the ski hill. He is a confident, articulate, rambunctious six year old boy.

Life is good.

Wednesday, January 8, 2014

New Hearing Aids and Glasses

We haven't picked them up yet, but Nolan's new hearing aids are on order. We will drive up to Buffalo on January 14th to pick them up - we chose the Bolero Q70 SP model. These hearing aids have 16 channels (as opposed to the eight channels Nolan currently has) and are water-resistant. They also have an integrated FM receiver.

The integrated FM is a huge plus. Nolan's personal FM system has not been working properly since he started school in September. He has a Phonak Inspiro, and it has had multiple issues. First, the transmitter was sent to Phonak and they did some basic maintenance... but the transmitter would not work once it was sent back to the school. Phonak replaced the transmitter when it was sent in for the second time, as there was a faulty circuit board. Then it was sent in for a third time because it wouldn't work. Now, one hearing aid won't register the FM signal - but this time, we think it is the hearing aid and not the FM system. Hopefully getting Phonak aids (instead of the Unitron aids he currently has) will help solve our FM problems. While his teachers are so happy to state that he "compensates well," lip reading and filling-in-the-blanks is not the same as actual hearing. He misses a lot.

He is also getting new glasses, which will be picked up the day after getting his new hearing aids. I am excited for his soon-to-be improved vision and hearing!

In other news, the windchill has caused our school to cancel for two days in a row (-30 Fahrenheit with the windchill, -12F as the "regular" temperature). It is warming up tonight, and will actually rain on Saturday. The kids are getting a bit antsy, as the weather has been too cold to play outside and there is a lot of pent-up energy here!