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)!

Hearing Tests

We took the boys up to Buffalo for hearing evaluations on Tuesday. Nolan didn't have a hearing test, but we did do another aided audiogram (new audiologist) and selected new hearing aids. We are still waiting on a price quote, but Nolan will be getting Phonak Bolero Q hearing aids. We're quite excited about this, as we feel it will let him hear better. Technology has improved over the past 6 years, so new hearing aids are a good thing!

Nolan did get new impressions, as he will receive new earmolds with the new hearing aids. He chose a new color, too - he loves changing his earmold colors!

Matthew had testing for central auditory processing disorder, which is called CAPD, CAP, or APD, depending on where you live. He does NOT have APD, which is good to rule out, as it has a similar symptom set as ADD and as sleep apnea. We already know Matt has sleep apnea, so the inattention/moodiness/zoning out are due to the apnea and nothing else.

We did, however, find that Matthew has developed some very minor hearing loss. It is nothing that can or should be aided at this point, but his audiogram looks something like this:

He used to test as 15dB across the board (normal for children). While the main area of the speech banana is not affected, he is now on a yearly monitoring schedule so that we can aid him if and when the loss enters the speech range.

I am waiting to receive his audiogram in the mail, as this might be slightly "off" from the actual one. The right ear is slightly better than the left ear. Basically, he drops to 50dB at 8,000 Hz (ultra high frequencies) and touches mild in the low frequencies. He is "normal" for an adult hearing level, but has a slight hearing loss for the pediatric age range (better than 15dB hearing level). This does explain why he mishears the S vs. SH sounds. He can 'hear,' but might have difficulty in noise and might have some trouble with ultra high frequency sounds.

This does explain some of the "ADD" type behaviors.. calling his name when he's watching TV and in a busy location - it is not the busy nature of the situation that is distracting him.. he is having difficulty hearing with clarity in noisy situations. In any case, this is something to keep a close eye on.

ENT Appointment, Part 1: Audiogram and Aided Testing

We had a trip to the boys' ENT on Monday, and discussed several issues. I am going to break the appointment up into three posts (by topic) to help keep things short and sweet.

First, I managed to get a copy of Nolan's actual audiogram.

The audiogram has Nolan's bone conduction scores (the brackets), his unaided responses to sound (the x's and o's), and his aided responses (the B's). He has had some fluctuation in his unaided test results, which is typical for Nolan.

The biggest concern is Nolan's aided levels, which are only achieving a mild-moderate level of hearing loss. We have friends with similar levels of loss who are obtaining 20-25 dB aided levels, so this is clearly unacceptable. Nolan is only able to detect the presence of a voice when it louder than 30 dB (with aids on).

In any case, our ENT was not happy with the lack of aided testing and real-ear verification. She also thinks he probably needs power aids, since he has a mixed loss and the conductive component requires more power than a purely sensorineural hearing loss.We have some leads on different audiology centers that may offer better aided testing. I have to make a few phone calls, but it will have to wait until after the boys' surgeries (see parts 2 and 3).

Hearing Test and Audiogram

Yesterday, Nolan had a hearing test at Buffalo Children's. His hearing has been hit-or-miss, and his speech has shown some regression. Nothing serious, mind you, but I still don't like the disappearance of our friends /s/, /j/, /ch/, and /z/,

I don't have his official audiogram in the mail right now, but his unaided test scores are fairly consistent with the October results. His left ear has dropped by about 20 dB at 2,000 Hz, in typical Nolan fashion.

For those who are unfamiliar with audiograms, Nolan cannot hear the sounds above the lines without his hearing aids. Basically, he can't hear any speech sounds at normal conversational volumes. If you shout at him or talk directly into his ear, he can hear enough to make out what you are saying. This is a moderately severe to severe hearing loss.

The reason for his "slushy" speech is more likely due to his aided hearing levels. Obtaining a pure-tone aided audiogram is a tricky business, because digital hearing aids suppress pure-tones as "background noise," which can make the audiogram a bit tricky to interpret. A child may show hearing thresholds of 30 dB across the board, for instance, but really be able to pick up voices at 20 dB.

With that said, Nolan's aided audiogram is terrible. The suppression effect should affect all frequencies equally, and Nolan's results are showing a significant drop-off in the high frequencies. His higher frequencies are not set appropriately for his hearing loss. His aided results are:

Hearing with both ears, Nolan can't really detect f, th, or s with his hearing aids. Z and V are also above the 35 dB line, which explains why he's having difficulty with those sounds. The audiologist noted he couldn't detect her voice until it was at 30 dB with his hearing aids on - essentially, Nolan has a mild-moderate hearing loss even with his hearing aids on.

I did ask about his bone conduction results and whether the BAHA could be an option for him (to eliminate some fluctuation and to give him better hearing). The audiologist stated that the sensorineural component of his hearing loss is too severe for a BAHA, so he is not a candidate for that technology.

His hearing loss isn't severe enough for a cochlear implant, so he doesn't qualify for that technology, either.

Since Buffalo Children's does not have a hearing aid program, they can't adjust Nolan's hearing aids. Since Nolan's current audiologist does not believe in aided testing, she won't adjust Nolan's hearing aids.

Buffalo Children's was very surprised that our current audiologist does not do real-ear measurements, as this may be part of the problem. The DSL program that sets hearing aid amplification levels is based on a set of averages, but is not specific to an individual child's ear canal size and hearing loss type. With a mixed loss, Nolan likely needs more amplification than a child with a purely sensorineural loss.

And then there is the problem of  recruitment and a narrow dynamic range. Recruitment is a problem for people with severe losses. All of the outer hair cells are damaged, so only the inner hair cells remain to transmit sound in the cochlea. There is no gradual increase in the perception of volume - a sound goes from being inaudible to being WAY TOO LOUD very quickly. This makes setting hearing aids particularly difficult, as the level of amplification needed to make a sound audible is very close to the level which causes pain.

So the hearing aid program must be compressed to fit into this narrow range of hearing - the range between audibility and pain. We may not be able to increase his amplification by much, because it would cause him discomfort with sounds that are too loud.

Rock, meet hard place.

Other than the slushy speech sounds, however, Nolan is doing well - so we'll just repeat ourselves more often, use the FM more consistently, and monitor his hearing to see if (when) he qualifies for different hearing technology.

Dentist Appointment and Scheduled Hearing Test

Nolan and Matthew both had dentist appointments the other day, for a routine cleaning and "tooth count." Matt's teeth look great, and he has two more loose teeth. At the age of seven, teeth start popping out like popcorn. Nolan's teeth are hanging in there, but reflux and tooth-grinding has done some damage. He had a sealant placed on a bad tooth last year in the hope of stemming any further damage. Unfortunately, the tooth is breaking down and he'll need to have a filling placed.

We're returning to the dentist on February 4 to have Nolan's' tooth fixed and to have sealants placed on Matt's permanent molars. I didn't take any pictures at the dentist, so I'll post a picture of Nolan having fun on a recent trip to our local ski hill:

Nolan's hearing has been questionable over the past few months. He does hear with his hearing aids, but he has to work to hear. His speech teacher and teacher of the deaf have noticed some regressions lately - particularly with the soft sounds like /s/ and /th/, in addition to the loss of the /ch/ sound (he pronounces "chair" as "share," for example).

I took a video of his recent hearing difficulties to show to our ENT (transcript follows):


 [Transcript]: 
Me: Nolan, who's winning?
(no response)
Me: Nolan, who's winning?
(no response)
Me: (loudly) Nolan, Nolan - Hey, buddy!
(Nolan looks at me)
Me: Who's winning?
(Smiles and has no idea I''m speaking)
Me: (louder): Who's winning?
(Still can't hear me and imitates me covering my mouth)
Me: (loudly) Who's winning?
Nolan: What?
Me: Who's winning?
Nolan: What was running?
Me: Who is winning? Who's winning the game?
Nolan: Ruining the game?
Me: Winning. Who's winning. Are you winning, or is Matthew winning?
Nolan: I'm winning.
Me: You're winning? Good job!

I was about 6-7 feet away while taking this video. There was some background noise with the TV, but the volume was low. He doesn't hear soft speech at all with his hearing aids. Our audiologist refuses to do aided audiograms, and I know this child is not amplified appropriately. It is like pulling teeth to get any aided testing performed at all for him. I would give my left arm to have Jane Madell or Carol Flexor work as his audiologists.

In any case, we are having another independent hearing test performed on February 13 at Buffalo Children's. This time, we're requesting aided testing and open-set speech recognition. The audiologists at Children's cannot adjust Nolan's hearing aids, but at least they can tell us if they're not set appropriately. Nolan's hearing in his right ear is solidly in the severe range, and his left ear is nearly there.

Hearing Test

Nolan had a hearing test yesterday. The audiologist called and bumped the test up to 1:00pm, which was fine by me: our original time was 4:00pm and we have a 2 hour drive each way. I like getting home before nightfall.

There was no assistant available for the test, so our audiologist told me to wait outside with Matthew while she took Nolan into the booth. He did the test on his own, and raised his hand for the tones. This is the first time he has done "adult" type testing, rather than play audiometry.

The testing went very quickly - it only took about 20 minutes and he was done. His left ear has actually fluctuated up a bit, and is more of a moderate loss rather than moderately severe. This always makes me anxious as to the accuracy of the test, since that ear has been sitting at 65dB for the past two years. Still, it is what it is - I think the audiologist turned down the volume of that hearing aid to match the day's test result. I'm not exactly sure, though.

The right ear looks like it did in July, with that drop down to 70dB in the high frequencies. He's sporting a 75dB loss across the rest of the lower frequencies, though the additional loss is probably due to his active ear infection. He's normally 65dB dropping to 70dB.

Unaided speech recognition was 80% at 100dB for the right ear and 96% at 80dB for the left ear.  I'll post his audiogram when we get it in the mail.

Since both boys were very good during the hearing test, I took them to Chuck E. Cheese after the hearing test. I had a coupon for 100 tokens for $20, and the boys were in heaven.

Nolan's right hearing aid won't be worn for a while. The ear is still leaking (despite antibiotics), so hopefully it will dry up soon. He has an appointment with his ENT on Monday the 15th, so we'll see what she thinks about the continuing ear infections. He's been averaging an ear infection every-other month, always in that right ear.

"Nolan-Sick"

Nolan started coughing last night, and it had that particular hacking characteristic that makes parents reach for a thermometer. As soon as I touched his forehead, I knew he was not well. He had a 103F fever, so I gave him some Motrin with his feed last night.

In the morning, he was still running a temp. I took Matt to school and called the pediatrician. Both whooping cough and the flu are going around at the moment. I was pretty sure it wasn't whooping cough, but it could definitely be influenza.

I was really bummed, as we had our annual Enchanted Forest event planned. This event is at our local Audubon Society, and the naturalists dress up as local animals and tell the kids about the animal they represent. It is extremely fun and educational, and I highly doubted we would be going this year.

The pediatrician immediately thought influenza. The cough, the high fever, the congestion and headaches.

All of the tests they ran (from influenza to strep) came back negative. Then they looked in his ears - he has a raging ear infection in the right ear, which is probably the source of the fever. His cough sounds awful, but he has a floppy airway: even a typical cough will sound like croup (or worse) with his malacia.

The final verdict was that he has a nasty ear infection coupled with a cold. Of course, he is much sicker than a typical child with a cold, but that is because of his pre-existing airway and sinus/ear problems. He wasn't really sick - just "Nolan Sick."

We could carry on with our normal activities, as he's not really contagious. A regular cold just causes him a lot more problems than it would for a typical child. We left with a script for Augmentin for his sinuses and ear infection, and we'll give him Ciprodex drops to clear up that ear, too.

We spent the next hour (yes, HOUR) in Rite Aid, waiting for the prescription to be filled. To prevent the kids from checking their blood pressure for the fortieth time, we went "shopping" in the seasonal aisles. There were really cute animal hats on sale for $8, and Matt really wanted one. He picked up a wolf hat and said, "Its name is Snowy. I named it. We can't leave it here!" I bought the hats. I'm a sucker.

Considering they played with the hats for a solid three hours tonight, I'm considering the hats a huge WIN.

With a little more Motrin under his belt, he was feeling pretty well this evening. We made it to the enchanted forest.

I'm glad my little guy doesn't have a really nasty virus - though I hope this ear infection clears before his hearing test on Monday!

Visit to Seneca Lake and a Hearing Test

Dennis's parents visited this week and we took a trip to Seneca Lake (one of the Finger Lakes). It was so beautiful, and the boys really loved playing on the playgrounds, hiking through Watkin's Glen, and driving the pontoon boat. Nolan's most favorite activity was driving around the lake.

We loved our vacation, though once it ended the spate of July medical appointments began. Nolan had a hearing test on Friday, July 6. He had been stable for a year, so I was hopeful we'd see the same audiogram we saw in December. If he was still stable, then we would be able to go to once-per-year hearing tests.

Unfortunately, his right ear decided to drop down to 70dB at 4,000Hz. This explains where his /s/ detection went! I can't remember his actual new audiogram, so the picture below is just an estimation. I'll post the "real" audiogram once I get it in the mail. His right ear used to be the "better" ear, but is now the "worse" ear. The black line is what he was able to hear in December, the red line is his approximate new hearing level:

This is a 15dB drop at that particular frequency, which is where f, s, th, and the softer sounds of speech are found. This is typical for Nolan, and usually the other ear drops to match the worse ear in a matter of months. His unaided speech discrimination dropped from 96% to 80% in that same ear, at a 90dB volume. His left ear is currently unchanged.

This was a bit of a bummer, but his hearing aids have been reprogrammed to match his current level of loss. I did manage to convince his audiologist to do aided speech discrimination. The current recommendation is to do aided discrimination at 55dB and at 35dB, as children won't "overhear" incidental language if they can't perceive soft speech. I didn't get any data at 35dB, but our audiologist did do aided discrimination at 55dB. With both hearing aids, Nolan has a wonderful 96% discrimination at 55dB (via sound field, using both hearing aids).

On the bright side, his "dead" hearing aid slowly came back to life. I simply kept running it through the Dry and Store, changing out dry bricks, and it began to give its feedback whistle again. For the first few days, it would cut out after an hour or two, but after about four days in the Dry and Store, it was "back to normal." Our audiologist checked it and it was right as rain, so that is a HUGE relief. We have been using Ear Gear ever since, just to protect them from humidity and sweat.

Since Nolan has decided to show some progression in his hearing level, another hearing test has been scheduled three months out from this one: he'll go back into the booth on October 8 to determine if his right ear is going to stay at 70dB and to watch his left ear - since it is likely to drop to the level of the right ear, too.

We also discussed new hearing aids. Nolan's Unitron Element 8's are still working, though we are having more frequent maintenance issues with them. They are also not as "up to date" as other hearing aids. I would really like to get Phonaks for him, because I am in love with his FM system. Phonak also makes water-resistant hearing aids that have sound-recover technology.

The hearing aids we're thinking of getting are the Phonak Nios S H2O, though I'm a bit nervous about selecting these because they have a maximum fitting range of 70-75dB hearing loss in the low frequencies. Since Nolan is already sitting at 65dB there, there is a great likelihood that he will "outgrow" the ability of these hearing aids to amplify his loss. When Nolan loses hearing, it tends to be 15-20dB in a single frequency, and the affected frequency is unpredictable. The Nios S can handle the high frequencies well (up to 90dB HL). I'm just a tad concerned about the low frequencies - I'd hate to get this hearing aid and then wind up needing a Naida (power aid) 2 years later. We'll see what his hearing does over the next few months and make our decision from there, I suppose!

The Phonak Nios S H2O 

Stable

No more pictures, Mom!

Nolan has been having a lot of trouble hearing lately, so we were concerned about his test on Friday. As it turns out, his hearing is stable - changes of 5dB here and there, but the audiologist was happy to report a similar audiogram to the one he had in July. 

I suppose his difficulty in hearing simply comes from the deep end of a moderately severe hearing loss - he still can't hear his father (even aided) unless Dennis repeats words over and over again. His distance hearing is abysmal - he can hear me if I'm within 6 feet (aided) but outside of that he is lost. Frustrating, but something we'll just have to live with. Thank goodness for the FM system, because he wouldn't hear a thing in school without it. 

I should get the report sometime next week. I didn't actually see the audiogram, so I want to see if the "5dB" was a 5dB loss across the board, or if there were 5dB variations up and down. It makes a difference, because Nolan has lost hearing very slowly: 5dB + 5dB + 5dB adds up over time. If the 5dB is up and down, then I won't be concerned - that's just normal variation.

He does have granulation tissue on his left eardrum - not that surprising considering the recent infection. Otherwise, his tubes are open. We took new impressions for earmolds, since the right earmold had a small rip. He hasn't actually outgrown them (the last set were made before we went to John Tracy, over a year-and-a-half ago). With all the infections, however, I wanted new earmolds. I don't know if the old ones are harboring any infection, but why take the chance? We'll pick up his new molds on January 6 - for the first time, we didn't get blue-and-white swirls. Nolan wanted something different!

We're off to the ENT tomorrow to discuss the "abnormal" sinus CT scan results. I also have to ask about additional medication for the reflux, since Nolan is frequently nauseous throughout the day and begs for relief. I'm going to ask if he can have children's Tums (on top of his high-dose Nexium and Zantac). 

In the meantime, we're happy for hearing stability. We take good news where we can get it!

Finally, Dry Ears

Watching the Grinch Who Stole Christmas

Nolan's ear dried up on Wednesday. His hearing still seemed "off" in that ear (he should still hear unaided in that ear if you are within three feet or so and speaking loudly). His hearing seems to have improved today, though, which is wonderful. He has a hearing test slated for tomorrow afternoon, and we really wanted clear ears and a return to his standard level of hearing. His hearing is pretty crummy anyway, but we'd rather see his normal moderately-severe level than the severe-profound levels that an infection incurs.

The Teacher of the Deaf (TOD) for the elementary school called and asked about budgetary requests for next year. Nolan already has his FM system, so I couldn't really think of anything to add to his IEP. She did put in some extra money for batteries, since the FM system drains batteries fairly quickly. The only "snag" that we might hit is if we change hearing aids and need different receivers for the FM system, but I am pretty sure the receivers we have will work with nearly all hearing aid brands. Plus, the next set of aids will probably be Phonak, which happens to be the same brand as his FM system - they should function seamlessly.

I did have a laugh when she said that Nolan could just use a speaker soundfield system if his ears are infected. The school's only other child with a hearing loss has a very mild loss in the high frequencies. If Nolan has an ear infection and is unaided, he can't hear anything. Period. He drops down past the 90dB range and is functionally deaf - there is nothing to help that situation! Even when his ears are clear, he can only understand words projected at a 90dB level (unaided) - his last test showed 88% accuracy with words at 90dB. Unless she's going to set up a speaker to blast out rock-concert levels of sound, it won't work. I have a feeling the school will need a bit of an education to deal with his level of loss - he has to use his personal FM system at all times in the classroom. Nothing else will allow him to hear in noise (and if he's unaided, he simply can't hear conversational speech at all).

In any case, hopefully Nolan's hearing test tomorrow will show stability. I'm worried about that right ear, which has been having difficulty for the past two months. It was really a problem when the little guy (temporarily) lost the rest of his hearing in the left ear - let's just say that we have been using a lot of sign language in the classroom over the past 2 weeks, because he simply couldn't hear a thing. His right ear was also the first to drop the high frequencies back in 2008 (the left ear fluctuated up and down and then finally followed suit in 2010). The right ear seems to precede the left ear when we do see changes in hearing, so I will be greatly relieved if that right ear is nice and stable!

Hearing Test, Redux

Nolan and Casey the Snuggle Puppy

Nolan had a confirmatory hearing test today. His results look identical to the test last month, so his audiologist increased the amplification in his aids to suit the new audiogram. This is actually a bit overdue, since we saw those high frequencies drop off in the left ear last November - but that test was performed at Buffalo Children's, and they can't adjust hearing aids. Nolan's own audiologist hadn't seen that drop before, so she wanted to confirm before increasing the amplification.

Nolan's right hearing aid, which had been totally dead, sprang to life as soon as we pulled into the audiologist's parking lot. It is a lot like taking a car to the mechanic, and having it sound perfectly normal as soon as the professional looks it over. We still sent it in for repairs, since it is eating through a battery per day (he normally only goes through one per week). We'll go back in two weeks to pick it up - hopefully we won't have any more problems with it!

After the hearing test, we took a side trip to Buffalo Children's hospital to see if Matthew had left his hand-held video game in radiology during Nolan's Upper GI scan. Sure enough, it was sitting behind the radiology desk. I was so glad it was there! I think I am going to start labeling all of the "travel" toys - it is not-so-fun to run up to Buffalo when something expensive gets left behind!

We're all tired after the Independence Day weekend, which we spent with friends. The boys swam in a pond, played in a pool, roasted marshmallows, played with sparklers, and watched fireworks. After all the fun activities, we're ready for a few quiet days at home. Fortunately, Nolan's C-Pap titration was rescheduled for the 21st of July, so we have an entire week of relaxation ahead of us.

Newest Audiogram

I received the report from Nolan's most recent hearing test in the mail today. The results look remarkably similar to the test results from Buffalo Children's last November, but they are still a change from the last hearing test at our normal audiologist's office (particularly for the left ear). The low frequencies are still a mixed loss, but everything above 1000Hz is sensorineural. His bone conduction scores have dropped by about the same amount that the air conduction scores have dropped, which indicates that the small new loss is sensorineural (inner ear loss) in nature.

For those who aren't familiar with audiograms, Nolan can't hear anything above the plotted lines when his hearing aids are off. Basically, he can't hear any speech at normal conversational levels with his aids off, though he can hear things like rock music, lawn mowers, and other loud things. With his hearing aids on, he gets most of what lies in the middle frequencies, but still misses out on the high and low frequencies (hearing aid adjustment issues).

Thankfully, the worst hearing is at 65dB (I thought I had seen a 70dB mark on the audiogram in the office, but he is still just moderately severe in the low frequencies).

His speech reception thresholds changed from 40dB in each ear to 45dB (right ear) and 50dB (left ear). His hearing aids are still set for a mild loss in the high frequencies, which explains why John Tracy was showing under-amplification in those frequencies. He has lost a bit in the low frequencies, too - he seems to lose a bit at one frequency (or in one ear), and the rest follow suit shortly thereafter. Fortunately, it is a very slow loss - it has taken almost four years to go from mild/moderate to moderately severe.

We will return on July 5 to confirm the level of loss and (finally) adjust his hearing aids to match the loss.

Audiology: Check (Sort Of)

Hanging out at the Buffalo Zoo

The drive to Buffalo is long, but fortunately we have invested in hand-held gaming systems reared travel-hardy children. Nolan was a little recalcitrant about going into the audiology booth, swinging his legs and beginning to throw a fit. I used blatant bribery (a trip to the zoo) to get him to cooperate. I'm not above zombifying my kids with electronic entertainment or offering bribes anymore. I think I have "arrived" as a mother.

He cooperated nicely. I think the last full hearing test done at his regular audiologist was over a year ago. Their last record still shows a rising configuration in his left ear, which made his loss moderately severe rising to mild. Our testing at Buffalo Children's last November showed a flat moderately-severe loss, so I wasn't surprised when our current audiologist showed the same thing. I did see one point hit the 70dB mark, which makes me a little anxious. That's a little close to the "S" word (severe) for my liking. Thankfully that was only one frequency.

Before increasing the amplification in his hearing aids, they want to confirm with another hearing test in a month.

Oh, goody.

The strange thing is that his air-bone gap has largely disappeared. This means that his loss is purely sensorineural again - either he's been throwing false air-bone gaps (it happens), or he has a truly fluctuating/progressive hearing loss that proceeds in a very weird fashion (the drops appear conductive at first, but solidify into a sensorineural loss). Weird.

I suppose time will tell. Hopefully he'll stabilize where he's at.

Tomorrow we are going to Erie (a slightly shorter drive, but still an hour each way) to see the ophthalmologist. To patch or not to patch, that is the question!

This is otterly amazing!

A Busy Week Ahead

Nolan's preschool field trip to the Fire Station

This week is a little busier than most:

Monday: Matt's preschool, Nolan's speech therapy, and Matt's second preschool.

Tuesday: Drive to Buffalo for a hearing test, and to adjust Nolan's FM system. Matt's first soccer practice.

Wednesday: Matt's preschool, then drive to Erie for an ophthalmologist appointment (we get to find out if Nolan gets to rock the fashion world by sporting a patch and going pirate-style). Then Nolan has a soccer practice, and Matt has swimming lessons.

Thursday: Nolan's last day of preschool, and his "birthday" during class. I have a mothers of preschoolers steering meeting, and Matt has preschool.

Friday: Matt has an end-of-year picnic for pre-K at a local amusement park, so we will be spending the day having a ridiculous amount of fun in the sun!

I love the summer months, but June does get a little insane with the end-of-school parties/events, and then we have a bazillion doctor appointments for Nolan. He sees urology on the 16th (this is routine, and we are hopeful he will be discharged from this specialist). The sleep neurologist is scheduled for the 21st, and the upper GI is scheduled for the 27th. At least one trip a week to Buffalo Children's is on tap for the remainder of June: if only the drive were shorter!

The good news is that preschool ends on the 24th of June (for Matt), so July should ease up a bit with the schedule. I plan on spending a lot of time at the local parks, municipal swimming pools, and on "field trips" to the beach!

Audiology Report from Buffalo Children's

I received the official audiology report from Buffalo Children's audiology department. I am impressed it arrived so quickly! It states that Nolan has a moderately severe mixed hearing loss, has patent PE tubes (i.e. his middle ear ventilation tubes are working very well), and that he is an absolutely amazing kiddo. But we already knew that.

Since my friends/family are probably addled by all the charts, I'm going to explain Nolan's results- so my hearing loss bloggy friends can stop reading now. I'm pretty sure all of you are all too familiar with audiograms! Also, this will be boring. Fair warning.

For those not as familiar with an audiogram, it is a graph displaying "frequency" across the top. Frequency is pitch- it is like a piano keyboard rolled out in front of you. Middle C is at 250Hz, which is the lowest useful frequency used for human speech. 8000Hz is very high pitched- think of a shrill whistle (or the higher pitched sounds like "s" and "th").

The volume is placed down the side of the audiogram. It is recorded in decibels (dB)- children with normal hearing can hear from 0-15dB (adults with normal hearing can detect sounds from 0-25dB). A whisper is at about 30dB, conversational speech is at 55-60dB, and jackhammers are around 100dB.

I have placed Nolan's hearing levels onto an audiogram which has the sounds of speech placed onto it. Nolan's right ear is red, and his left ear is blue. He cannot hear any sounds above the lines. In other words, he hears almost no speech sounds without his hearing aids. He can hear shouting, dogs barking, and other loud sounds.


Audiologists like to know what is causing the hearing loss, so they test both the inner ear (how well the delicate hair cells in the cochlea are working) and the middle ear (the mechanical portion of hearing). The middle ear bones vibrate and transmit sound to the inner ear, and the inner ear hair cells move and transmit the sound information to the auditory nerve. If either system is "broken," sound will not get through to the brain. Problems with the middle ear are generally correctable, if the problem is caused by middle ear fluid or a malformed middle ear bone. Problems with the inner ear are not correctable.

A problem with the mechanical, middle ear function is called a "conductive hearing loss." A problem with the inner ear is called a "sensorineural hearing loss." Conductive hearing losses are treated with surgery to correct the problem, or hearing aids if the problem is not correctable. Sensorineural hearing losses can only be treated with hearing aids (or cochlear implants for severe-profound levels of hearing loss).

They test the "middle ear system" by placing a vibrating sound system on the head, called a bone oscillator. This sends sound directly to the inner ear (cochlea) and bypasses the middle ear. If there is a problem in the middle ear, this type of testing will show the true hearing ability of the inner ear.

They tested Nolan's middle and inner ear systems, and found the following:

The black line shows that when they bypass Nolan's middle ear system, he hears better than when they play sounds through headphones. This means that a portion of his hearing loss is due to a mechanical problem with his middle ear bones. The other part is due to inner ear hair cell damage.


This type of hearing loss is termed a "mixed hearing loss." In Nolan's case, nothing can be done to fix the conductive portion, since we don't have a cause for it. His mechanical problems are not caused by fluid, since he has functional tubes in his eardrums. It is likely that his mechanical sound transmission problems are caused by stiffened middle ear bones- the result of chronic infection. This is why ENT's are very aggressive in treating middle ear infections in children with pre-existing hearing loss. Even though our ENT has been right on top of Nolan's hearing loss and ear infections, damage has been incurred. Hopefully the new, spiffy "permanent" tubes will prevent further damage to his middle ear system.

In the meantime, Nolan has yet another cold with a fever- hopefully this will disappear in time for his pH probe on Monday. I am ready for summer to arrive and these nasty germs to disappear!

And We Have a Consensus

With Nolan in a cooperative mood, we set out for Buffalo Children's this morning. With the sun shining, a good night's rest, and some blatant bribery, he made it through an entire hearing test. The audiologists at WCHOB were very impressed with his skills in the booth- I don't think they see a lot of kids who have an auditory-verbal background, since listening and hearing test skills are integrated into the therapy we do with Nolan. While we are not a true AVT family (there is no actual AVT center around here), we incorporate as many of the "learning to listen" skills as we can into our therapy.

The result of the testing indicates that Nolan does NOT have a purely conductive loss. His hearing levels are currently a mixed, flat moderately-severe loss. This is fairly consistent with the most recent audiogram obtained at Buffalo Hearing and Speech Center.

Guesstimate Audiogram-
I don't really have a photographic memory.

This is good news, because it means that the sensorineural portion of his hearing loss is stable. This also means that the hearing loss detected at birth was sensorineural or mixed in nature, and not conductive. A bone anchored hearing aid is not appropriate or advisable in Nolan's case, so we can wipe that option off the table.

Unfortunately, he has lost hearing since birth. As it stands now, he cannot hear sounds softer than 60dB. All of the additional loss, however, is of a conductive nature. This means that there is a maximal point of progression, since conductive losses can only add so much to a sensorineural hearing loss.

There is a good chance that Nolan's low frequencies were a mixed hearing loss at birth (he had a 50dB loss in this region, even before he had any ear infections). The current theory is that his loss has progressed due to tympanosclerosis (thickening of the ear drum) and some stiffened middle ear bones from chronic ear infections.

We do need to get a "tune up" on his hearing aids, since they are programmed for a 30dB loss in the high frequencies and he currently has a 60dB loss in this region. The conductive portion of his hearing loss is not "fixable," since it is not due to fluid build-up or other reversible causes. Hopefully the new permanent tubes will prevent further infection and damage to his middle ear system!

Bone Conduction Hearing Test

Nolan, with balloon-cat hat and kitty face.

For a quick recap, we have a hearing test that shows a rising slope audiogram via bone conduction (January 2010 at Buffalo-BHSC) and a hearing test that shows a traditional slope via bone conduction (July 2010 at John Tracy).

Since these two test results are the exact opposite of each other, we wanted to get a third test to clear up whatever conductive component might exist to Nolan's loss. The test yesterday showed (wait for it)... a third configuration! BHSC managed to get bone conduction with masking, which means they managed to get the bone conduction scores in each ear, individually.

While I can't remember the exact numbers, he is showing something that looks like a shallow cookie-bite configuration in both ears. His bone conduction score was 25/30dBdB at 500Hz and dropped to 50dB at 1000Hz, then came back up to 40dB by 4000Hz. He was extremely reliable, so now the question arises: does he have fluctuating bone conduction levels in addition to fluctuating sensorineural levels?

Our test in January showed a conductive component, but only in the high frequencies. The test in July showed a large conductive component from 250Hz-1000Hz, with normal cochlear function in that range. The test yesterday showed a conductive/mixed component at 500Hz, but sensorineural across the rest of the testing range.

*Insert hysterical laughter*

The only thing we can be sure of is that his loss is fluctuating across all frequencies. Our audiologist is suspicious that something was missed on his CT scan- we need to take another look at his vestibular aqueducts. This is the only thing that causes an air-bone gap in the low frequencies with fluctuating hearing thresholds (the fluctuations occur when there is no fluid or infection present, so these changes in hearing level have nothing to do with his ear infections- we omitted those results). The next step is to see the ENT, to review the data and determine what to do next. This might include a FOURTH hearing test at an independent location, where we're likely to get a fourth result.

We did not do air conduction thresholds yesterday, except for a quick Speech Reception Threshold (SRT). His SRT is 55dB in one ear and 65dB in the other (my guess is that his infected ear is the one with the worse air conduction threshold).

Speaking of the infected ear, it looks pretty bad. I have to call the ENT this morning to beg and plead to get in. The amoxicillin did nothing for it, he's had it since the middle of July, and is now at risk for complications from a long term ear infection (namely, mastoiditis: something we'd really like to avoid).

Once we get the infection cleared up and talk to the ENT about Nolan's fluctuating hearing loss and random air-bone gaps that seem to shift frequencies, we'll go back to Buffalo for the air conduction scores (these are what he really hears and are in the moderately severe range).

It's really hard to set hearing aids for constantly shifting targets- our only solution may be more frequent audiological testing.

Still Infected

Alas, the "pink medicine" is completely gone, and Nolan's right ear is still sticky. I took a peek in there with an otoscope, and it doesn't look good. Thankfully, I have oodles of Ciprodex. I am giving Nolan the Ciprodex twice per day in the hopes that it will stave off some of the infection! The pediatrician will be called to (hopefully) get in tomorrow for a different oral antibiotic. The amoxicillin didn't cut it this time! We can't get in to the ENT until September 13, so we're doing the best we can until that date rolls around on the calendar.

Nolan has his bone conduction hearing test* today, to try and replicate the results we obtained at the John Tracy Clinic. If we get the same results, then we know that Nolan's low and mid-frequency loss is largely conductive in nature. I have a feeling our September 13 appointment with the ENT will be a rather long one.

*For those who are worried about the ear infection affecting the results of the hearing test: we are running a bone conduction hearing test, which bypasses the middle ear and any possible infection or middle ear dysfunction. The infection won't have any effect on the hearing test results. It will have an effect on his air conduction scores in the right ear, of course!

Official Audiogram and Information for John Tracy

I received the packet of Nolan's hearing tests/hearing aid settings in the mail today. This is the last documentation required for John Tracy's Summer Session, so I am pleased to have it! I am going to make a photocopy on Monday (so that we have a copy in case the other one gets lost) and then mail it to the Clinic.

Nolan's audiogram is below (copied onto a larger graph for photographic purposes). The "teaching audiogram" used below has different categories of hearing loss than typically used, so disregard those levels. Nolan's hearing loss is not in the "severe" category, but "moderately severe rising to moderate." For reference, normal hearing in children is above the 15dB line. Nolan cannot hear any sound above the lines on the graph (unaided). Basically, with his hearing aids off, he can hear dogs barking and babies crying, but none of the sounds of speech.

Over time, Nolan's hearing loss has fluctuated a great deal. When he was born, he had a 40dB loss rising to borderline normal hearing (with present OAE in his right ear). Obviously, this is no longer the case. Still, we've had improvements and decreases in hearing over time- I created a table to show his hearing levels, with the total change from May 2008 (first booth test) through May 2010. The first two dates are estimated hearing levels from ABR testing.



There is a net loss at each frequency, going from a mild/moderate loss to a moderately severe rising to moderate loss. I am interested in obtaining more bone conduction testing to determine how much of Nolan's hearing is conductive vs. sensorineural. For amplification purposes, it doesn't matter: even if there is a conductive component, it is permanent and his hearing aids had to be increased. On the other hand, it would be nice to know if there is a conductive component to gauge how "progressive" his loss might be. John Tracy will perform a full battery of tests when Nolan is at the clinic, so I am hopeful that a bone conduction test will be included.

Pump Up The Volume

The boys at the Buffalo Zoo

We had a successful hearing test today, which was nothing short of a miracle considering Nolan's recent state of mind (hint: it is not "cooperative"). Many things that could have gone wrong DID go wrong on this trip, but we still pulled off a good hearing test.

I showed up late for the appointment- for some reason I had written down the time as 10:00am, when the appointment was actually for 9:30am. Well, at least I had the right day this time! Of course, this gave Nolan less time to adjust after a two hour car ride. We all know how much he loves cars.

I was "in solo parentis" today, so I had both boys when I escorted Nolan to the audiology booth. Our wonderful audiologist reached over to remove Nolan's hearing aids, and he howled in indignation. I might have forgotten to note that Nolan has become a wee bit "attached" to his aids. In that no-one is allowed to touch them, or he goes berserk. He lets me remove them, of course, but he freaks out if anyone else gets near them.

Luckily we used his earmolds as the inserts for the hearing test, so he was happy once they were back in place. He wouldn't touch the picture cards for the speech reception threshold test, though. For those who aren't familiar with hearing tests, they use a certain set of words (snowman, bathtub, toothbrush) and present them at various levels. Nolan wasn't going to perform this task, so we invited Matt to the table to see who could "win" the pointing game. With a little competition spicing things up, Nolan decided to cooperate!

We were afraid to move once Nolan was happy, lest we set off the tear-fest again. Matt and I sat on the floor near the play table and I tried to keep Matt absolutely quiet. Those of you who have four-year-old boys know what a difficult task this is- let's just say there were many whispers of, "If you want to go to the zoo this afternoon, stay quiet!"

The testing was completed after about half an hour. We didn't get bone conduction testing done, but his tubes are patent and functioning. His hearing levels are a touch better than the last time we had testing performed, but worse than his levels in June 2009. I can't remember all of the numbers exactly, but his audiogram looks something like this:

His SRT's were 45dB and 50dB, slightly better than the January test, but worse than the June 2009 levels. Essentially, we've eliminated a slight amount of conductive loss, but the permanent loss has still progressed a little in a year. His permanent loss is now categorized as moderately severe rising to moderate (his better ear used to be moderate rising to mild based on the last bone-conduction test).

His audiologist turned up his hearing aids, especially in the high frequencies. They were set for a mild level of loss in the 2000-4000Hz range, and had to be adjusted for a moderate/moderately severe loss in that range, depending on the ear. He does squeal (feedback) a lot more, especially if you get too close to his aids. Knowing that our little guy has more access to sound is wonderful- maybe we'll get those /f/ /p/ and /s/ sounds back!