Fungus Among Us

The Rite Aid managed to find the right anti-fungal medication for Nolan's ear. Just in case anyone else ends up in the same predicament and has the pharmacist try to hand them a certain feminine anti-yeast cream, here is a picture of what the medication should look like.

This is quite a big difference from the *ahem* first dispensing method they tried to offer us. I placed the first drops in his ears last night. So far, his ear is still quite infected. Hopefully things will improve over the next few days!

While solutions of vinegar and/or rubbing alcohol are commonly used for fungal outer ear infections, this is not advisable for any child with patent middle ear ventilation tubes. Since any medication dispensed into Nolan's ear canal will end up in the middle ear space, everything from the eustachian tube to the membrane-covered oval window is exposed to the solution. The only safe medication to treat the fungus at this point is a non-ototoxic medication, which has a fairly neutral pH to prevent irritation and pain. The reason the Ciprodex drops were so painful was the low pH in the medication, which burned when in contact with the raw skin in Nolan's ear canal. So far, he seems to be handling the anti-fungal drops fairly well.

In other news, we are preparing for preschool. The 3 year old class requires a potty-trained child, so we have jumped in with both feet and have candy, a potty (chosen by Nolan), and lots of training underwear. The score so far- puddles: 2, potty: 1.



Wish us luck: preschool starts on September 14!

Of Dinosaur Birthday Parties and the Hunt for Lotrimin

The Physician's Assistant told me I might get some odd looks from the pharmacy, since he was ordering a liquid suspension of Lotrimin that is typically used for Athlete's Foot for Nolan's ear. I went to the pharmacy on Friday afternoon, and the pharmacy technician said the order hadn't been filled yet. Sidenote: killing fifteen minutes in a Rite Aid with two small boys should earn any mother an award.

I returned to the counter and the tech had a big smile on her face. "Oh, no! this isn't a prescription product- it's over the counter!" Even better- I could get the stuff at low cost, and it didn't even need a prescription. I wondered why the PA had warned me about the difficulty of getting the Lotrimin suspension.

Then the tech handed me some Monistat.

"Erm... I know the main ingredient is the same, but we need this in a liquid suspension. It has to go in his ear."

I picked up the box and pointed to the applicator and cream mixture, so that she could see this was a tad different than what our ENT office had ordered. The tech looked flustered and said she needed to confer with the pharmacist.

Another fifteen minutes later, the boys had taken their blood pressure at the free stand about 50 times and the pharmacist came out from behind the counter to troll the aisles of Rite Aid with me. Apparently, the only over-the-counter formulations of Lotrimin are creams. After I convinced him that the creams will not penetrate Nolan's PE tubes to get to the source of the fungal infection in his middle ear, the pharmacist left to consult his Big Book of Medications.

He found the suspension the PA had ordered, and they have it in stock at their warehouse. This is the good news. The bad news? The suspension won't be in the store until Monday afternoon. Sigh...

Fortunately, the infection doesn't seem to be bothering Nolan very much. We had his dinosaur birthday party on Saturday, complete with Funfetti cupcakes, dinosaur pinata, and dinosaur adoption events. Everyone had a blast, and Nolan is finally convinced that he is no longer two years old. Happy third birthday, Nolan!

The dinosaur adoption center

Opening presents

Nolan vs. Tyrannosaurus

Three

Three years ago, the amazing Nolan was born. I walked into the hospital at 10:30pm, and just after midnight he was in our arms (yes, less than two hours' worth of labor: don't hate me). He is rather cuddly because of the crisp morning, so he is enjoying watching Mickey Mouse Clubhouse and relaxing on the couch. His birthday party is tomorrow, and he is extremely excited to play with his friends and decorate dinosaur cupcakes.

His ear infection has not gone away. The other night, I gave him the Ciprodex and Tobramycin drops, and he screamed for over an hour. It was horrible: he sweated and writhed in agony, and I couldn't help him.

He begged me to take him to the doctor, "Go doctor. Doctor put fists (gloves) on and take eardrops out!"

When he begged me to take him to the ENT to have his ear suctioned out, I knew he was in tremendous pain. The next morning, I did not give him his drops. I called the ENT's office to talk to the physician's assistant. He didn't want to stop the drops until we had the culture results back: if it was a resistant infection, at least we would be slowing the growth until we knew what would work. His solution was to stop one of the drops. We were allowed to stop the Ciprodex- it hasn't been working for six weeks, so there seemed to be no reason to continue it. We had to press on with the Tobramycin, however, which created a rather unhappy morning for all of us.

Fortunately, with only one set of drops, the agony seemed to last for a relatively brief fifteen minutes. I gave him the Tobramycin again last night, then rocked him for about an hour. With a sippy cup, blanket, and Mommy, he slowly quieted and was past the pain.

I haven't given him the Tobramycin yet this morning, and I am glad I held off. The ENT's office called with the preliminary culture results. This is not the full report, but it gives us a pretty good indication of what is going on with the little guy. The culture grew out fungus rather than bacteria. The antibiotic drops are only making his infection worse, not better. I am so, so grateful they cultured the ear and waited before prescribing some systemic, heavy-duty antibiotic. We are to stop the Tobramycin completely, since it will only wipe out the natural flora of the ear and allow the fungus free reign in Nolan's ear.

While fungal infections of the outer ear are fairly common (swimmer's ear), fungal infections of the middle ear are quite uncommon. The best guess is that he had a bacterial infection, which was treated with antibiotic drops. The drops wiped out the natural flora of his ear via the PE tubes, which allowed the fungus to start growing in the middle ear. His earmolds weren't helping the situation, trapping moisture and creating a perfect environment for the fungus to grow.

The next step is to pick up an anti-fungal suspension from the pharmacy. Lotrimin isn't typically used for the ears, so we were told to get some funny looks from the pharmacist. We were warned about two things:

1. The culture results are preliminary. The full report could show fungal and bacterial growth, so we may have to start the Tobramycin in addition to the anti-fungal. We'll have the full report on Monday, but with the preliminary results, treatment for the fungus can begin immediately.

2. Fungal infections in the ear can be very difficult to treat. The Lotrimin drops alone may not clear the infection. If the infection persists, a more "mechanical" method will be required. This involves suctioning everything out and painting the inside of the ear with gentian violet. Gentian violet is ototoxic, so I really, really hope the Lotrimin takes care of the infection.

I truly hope the Lotrimin takes effect before his party tomorrow!

Yippie!

Nolan's favorite song recently has been "Celebration" by Kool and The Gang. Whenever he hears the song on the radio ("celebrate good times-come on!") he'll shout the "COME ON!" part at the top of his lungs. We should turn on the song now, because we have something to celebrate.

I received an email from the school district that Nolan's FM system has been ordered from Phonak. Within minutes, I received a second email stating that the FM system is here. We were pretty sure the FM system wouldn't be ordered in time for preschool, and they managed to get it ordered and in-house within a couple of weeks. I am so glad we live in our particular school district- for all the worry about getting the services and accommodations Nolan needs, they have made everything happen in a very timely manner.

I never thought I'd be so happy over a piece of equipment. Without that little machine, Nolan would only hear a lot of mumbling during circle time. With that little machine, he will hear his teacher read the story, ask questions, and give directions. He'll be able to hear his teacher, even when she's outside of his 5' hearing bubble.

He'll be able to hear the music the other kids are dancing to.

Here's to a whole new world of sound- in the classroom!

Recalcitrant Infection

When an infection lingers for six weeks, is resistant to amoxicillin and Ciprodex, and begins to threaten a child's residual hearing, something has to be done. The ENT's office stated we couldn't be seen until September 13, and I was reluctant to go back to our pediatric "random doctor of the day" clinic.

Instead, I called the ENT's office and plead our case: a child with pre-existing hearing loss, persistent infection, and antibiotics that aren't doing any good. While our ENT is out of town for an extended period of time, they could get me in today to visit with a physician's assistant (PA). I leaped at the opportunity. Could we be there by one o'clock? Sure!

I looked at my watch: 10:50am. We have to leave by 11:00 to make it by 1:00- I threw the kids in the car, added a few Capri Suns and a diaper bag, and headed out. Of course, this sudden journey meant that lunch was catch-as-catch-can. I'm pretty sure I won't be winning "Mother of the Year" for their lunch today (some breakfast bars, 'Nilla Wafers, and string cheese). Oh, well. We'll make up for it with an especially nutritious dinner.

The PA took a look at Nolan's ear, and decided to culture the nastiness within. Nolan was quite good and sat patiently while the PA looked for "Spiderman." Thank you, John Tracy: he never cringes from the otoscope because of the hope of a lurking Spiderman. That little trick has served us well over the past few weeks! The culture was quick and painless, and we should get the results from that test in a few days. It will be interesting to see if Nolan is infected with an antibiotic-resistant strain of bacteria.

In the meantime, the PA decided to suction out the gunk from Nolan's ear. The ear vacuum is Nolan's least favorite invention on the face of the planet Earth. We tried to do it without papoosing him, but we resorted to the full body restraint and a nurse assist. He proved to be tiny but mighty- but we got the job done. The little guy hates the Big Scary Ear Sucker, and I don't blame him. It's loud, it is poking into an infected and tender area, and he has to be held down against his will. It is also (unfortunately) necessary and the antibiotic ear drops will be able to reach his middle ear now.

We are to continue the Ciprodex, in addition to a new antibiotic ear drop (Tobramycin). Aminoglycosides always make me nervous because of the whole ototoxicity thing, but the persistent infection isn't great for his hearing, either. Technically, Tobramycin is an eye drop, but the germs in his ear don't know that (it hits gram-negative bacteria that are resistant to Ciprodex). Nolan hates ear drops, and now he gets two different kinds every day. If the infection doesn't clear up after this round of medication, we have to come back.

We still have our appointment on the 13th to discuss the whole shifting hearing levels thing, so any remaining infection can be dealt with by our trusty ENT. I really, really hope the infection is gone by then!

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!

Sleep Study Results: Round 2

The wait for the results of Nolan's most recent sleep study has been excruciating. Since breathing seems to be rather vital for growth and survival, it is an issue that frequently weighs on my mind. The results are a bit of a mixed bag- I'll start with the good news.

Central Apnea:

Nolan's central apnea has improved by quite a bit. In the first study, he had 60 central events with his oxygen plummeting to 79%. The apneas were about 10 seconds in length, which was troubling considering his apneas were so short.

In the most recent study, Nolan only had 10 central apneas. TEN. The lowest oxygen saturation was 90% (for 12 seconds), which is a considerable improvement! The length of the apneas was anywhere from 10-15 seconds.

And now the "bad" news:

Obstructive Apnea:

Nolan's obstructive apnea in the first study consisted of 3 obstructive events and 41 hypopneas.

The current study shows that his obstructive apnea has actually gotten worse, post-tonsillectomy. He had 69 obstructive hypopneas. A normal Respiratory Disturbance Index (RDI) is <1.5 for children. Nolan's RDI is 11.5.

This is considered "moderate" obstructive sleep apnea for the pediatric age range, and is generally treated with C-PAP. Unfortunately, the neurologist noted that he has mild frontal bossing and a decent amount of midface hypoplasia. This means she's not very keen on the idea of a C-PAP mask on a still-developing face, when there is already an underdeveloped midface. The pressure of the mask could cause further problems with his still-growing bones.

Our treatment options are as follows:

Option 1: C-PAP. We could start C-PAP now and just watch Nolan's face carefully for any worsening of his hypoplastic mid-face area. The benefit is that it should solve the obstructive apnea and aid in any central apneas. The negative is the obvious issue with Nolan's facial structure.

Option 2: Medication. We could start Nolan on two different drugs (essentially steroids) to see if they help clear up the obstructive apnea. This is usually reserved for milder cases of obstructive apnea, but it remains an option. I'm not keen on this plan, for two reasons.

One is that all medications have side effects, and I don't know if the benefits outweigh the risks. The medications may not help, and the effects of the medications on a growing child concern me. The other reason I'm not keen on this plan is that we don't know why Nolan has obstructive apnea. He's not overweight (actually slightly underweight) and his tonsils were clearly not the cause of the apnea. Throwing him on more medications in the "hope" that it "might" help seems a tad rash to me.

Option 3: Watch and wait. We'll do nothing now, and follow-up with a sleep study in 6-12 months to see where things stand. Based on the information at that point, we'll make treatment decisions.

This is the option we chose. We'll do another sleep study in May, nine months from now. If the apneas (of either type) get any worse, we'll initiate C-PAP or BiPAP. If they stay the same, the likely solution is C-PAP or BiPAP. If they get much better, then I'm throwing a party and you're all invited!

We'll see what the ENT thinks of the latest round of information. Overall, the sleep study results are much better than the baseline study. We'll remain cautiously optimistic until we get the results from the May 2011 study!

Getting the Most Out of Summer

Nolan in the Chautauqua Gorge

Our regular computer is down again, this time for the count. In the meantime, I will be posting from the laptop, which is rather temperamental. If my posts in the coming days are rather sporadic, bear with me. The technical difficulties should be resolved soon!

Nolan's ear seems to be drying out, which is good news. He'll be on "pink medicine" (Amoxicillin) for another 5 days, along with the dreaded ear drops. I've been leaving his hearing aids out to allow his ear canals access to fresh air. This helps with the moisture issues, but also results in lots of shouting and jumping around to get Nolan's attention (we sign to him in these situations, but we have to get his attention first). Hopefully we'll be back to full-time aid wearing very shortly.

With only two (TWO!) weeks left of summer before school starts, we have been visiting local hiking trails and pools. This has resulted in several injuries on my part (did you know slate is very slippery when wet?) and two very happy kids. Nolan is quite pleased to go anywhere there is water, though we are being very careful about his head/ears getting wet. Luckily he is not a fan of getting water up his nose, which means that his head stays very dry.

I'll update again when we get any news (namely, a certain sleep study report), but until then, we're off playing in the summer sunshine!

Drying Out

Playing in Box City

We headed out to the pediatrician's office yesterday, to get a gander at Nolan's leaking ear. The random-pediatrician-of-the-moment peeked with the otoscope and declared a rather severe infection. He can't even see the eardrum, because the infection is so thick it is completely occluding his ear canal. The solution? Ten days of Amoxicillin and Ciprodex ear drops. If the infection doesn't clear, we are to return to try something stronger. Hopefully the Amoxicillin will wipe the infection out.

Nolan hates ear drops. He'd rather chug oral medications by the truckload than deal with the sensation of liquid sliding down his ear canal. You can imagine the fun we're having pinning him down several times per day for the infernal drops.

Hopefully, in ten days, the infection will be gone and we won't see any more leaky ears for the rest of the year. A girl can hope, can't she?

Leaky

Perhaps Nolan's little art demonstration yesterday was really his attempt to write us a letter:

"Dear Mom: Just as an FYI, my right ear is going to start leaking again tonight."

Remember the ear infection he developed in California? We treated him with Ciprodex for the recommended period of time, things seemed to dry up, and we were relieved. Until last night, that is. Nolan started chucking his right hearing aid across the room yesterday, which usually indicates a dead battery or an ear infection. Sure enough, there is sticky fluid in the ear canal.

Beautiful.

We're off to see the pediatrician at 4:00 today. We're in for more Ciprodex and possibly some oral antibiotics. Ugh.

Wordless Wednesday: The Littlest Tagger

Mommy Summer School

We have been having a very crafty week, doing different activities with a picnic theme. Both of our boys have weak fine motor skills, so we have been doing a lot of cutting and coloring lately.

First, we wove placemats. Matt did his by himself, cutting out the strips and weaving them. I did supply the small cut-out shapes, upon his request. I did an example alongside his version, so he could see what to do next. Nolan glued the little hearts and stars on the sample - in the meantime, he was cutting freehand from a supply of construction paper. Nolan isn't quite ready to cut on a line yet, so we just give him a pair of scissors and let him cut paper into bits.

Next, we made picnic baskets. Matt cut his out by himself, and I stapled on the handle. I'm working on the short vowel sounds with Matt right now, so he cut out an apple, egg, insect, olive, and umbrella- almost entirely by himself! I cut out the umbrella's handle, since that was a little more difficult for him, but he cut out the triangle portion of the shape. His ability to cut on a line has really improved, and cutting on a line is an important pre-writing skill.

I cut Nolan's shapes out for him while he cut more "shapes" out of construction paper. Both boys colored their picnic baskets and enjoyed pretending to go on a picnic. I'm not sure what our project will be tomorrow, but it might include gluing little tiny seeds on a paper watermelon (more fine motor skill work)!

Sleep(less) Study #2

It started raining on our drive up to Buffalo Children's yesterday evening. Sleeping in one's clothes is never a comfortable business, but sleeping in damp clothes is rather unpleasant. Ugh.

We checked in at 8:00pm and were old pros at getting hooked up- Nolan got into his jammies and we started applying the 6 quatrillion leads and wires to his body. Two leads on each leg, a few on his chest, several on his head (including under his eyes and on his chin), two belts around his chest, and a nasal cannula for monitoring CO2 on his face. And the pulse-ox, too. We can't forget about the pulse-ox.

Nolan was generally a good sport, though he did get anxious from time to time and declared, "These stickers hurt me!" They didn't really hurt, of course, but Nolan was worried the would start to hurt, or were harbingers of worse to come. The pulse-ox brought more tears, and we gave him the option of wearing it on his toe or his finger. Through sobs, he announced, "ON MY FINGER!" The nasal cannula was the coup de grâce, causing general shrieks and outrage. He settled down fairly easily, however, and was soon only whimpering about getting the "tubie" off.

I promised him that if he went to sleep, that we would take everything off in the morning. He looked at me and said, "Sun come up, take tubie off. Take stickers off." This seemed to calm him down greatly, though he was still anxious. The pulse-ox on his finger was of considerable concern. He decided he'd rather have it on his toe, or better: he'd rather have it taken off entirely.

A flash of brilliance entered my mommy brain. "It's a light saber. You're a Jedi." Adding in some detailed accounts of how jealous his brother would be to have a light-saber-finger sealed the deal. Nolan started battling "light sabers" with his fingers, and fell asleep in short order.

I stayed awake for a while to watch his CO2 monitor (his respiration rate and CO2 concentration are the only monitors in the sleep study room). I'm pretty sure he had some apneas, but we won't know for sure until we get the report back from the neurology center on August 19th. In any case, he slept better than he did for the last sleep study, giving us a good deal of sleep time to monitor his breathing.

I happened to wake up at 5:00am, which was good because the Sleep Study center comes in to wake everyone at this time. The technician came in and we unhooked Nolan from his myriad tubes and wires. The EEG goo left his hair looking rather disgusting, so I washed some of it out in the sink. I just got his temples, but it was enough to allow him to wear his hearing aids again! It was a relief to get to the parking garage, because we looked like zombies- covered in splotches of goop with a bad case of bed-head.

70 miles later, Nolan fell asleep again. This was rather convenient, because I placed him on the couch and brewed the Biggest Pot of Coffee That Ever Existed.

I'm going to end this post now, because the coffee is calling. Here's to a good report, with no future sleep studies required!

Creepiest Experience Book Ever

I thought I'd make an experience book for Nolan's sleep study tonight. It is a useful way to discuss the test and help him understand what will happen (and also, a way to reassure him that nothing will hurt during the test).

Since I don't have any photographs of Nolan's last sleep study experience, I had to draw the pictures. This is where the creepy comes in- I do not have any artistic talent. So my attempt to illustrate the process of putting the "stickers" on (EEG leads) is rather frightening:

Not only does Nolan lack hair, but it appears that his head has been taken over by a many-tentacled monster. Also, the technician looks a little too happy to place the leads on Nolan's noggin. I probably should have used clip-art, but there is no clip-art for sleep-study pictures. My horrifying rendition of the sequence of events is all I have, so I thought I would ditch the book before Nolan could see the seriously freaky images I drew.

Unfortunately, Nolan found it before I could toss it. And guess what? He loves it. He wanted me to read it about four times in a row, even with the creepy pictures. In my defense, not all of the pictures are creepy- some are even cute. Who can resist a gauze-headed cartoon Nolan?

I think the next experience book will feature photographs, though. Otherwise, we'll have to start investing for his future therapy bills!

Of Birthdays, Preschool, and Sleep Studies

Yesterday, I received Nolan's preschool registration papers in the mail. On one hand I can't believe my baby is ready to head out with a tiny backpack and join a class of other children-the time from his birth to now seems far too short to produce a three year old child. On the other hand, Nolan is incredibly ready for the extra enrichment that a preschool program provides, and I am thrilled that he will be in a class with many of his friends.

His birthday is at the end of this month, and I want to throw a small birthday party for him. One with only a few children, of course- owing to his age and to his general dislike of noisy situations. I don't have anything put together yet, but it will consist of dinosaurs (Nolan's favorite animal), cake, and possibly a pinata.

Before preschool and birthday parties can commence, however, we have one last medical hurdle to clear. Nolan's sleep study is on Monday night, and I am praying with every fervent bone in my body that the central sleep apnea will disappear. If it disappears, then it means all of the apnea was caused by his acid reflux (reflux inflames the tonsils, which then causes obstructive apnea, and long-standing obstructive apnea can induce central apnea spells). If it doesn't, then he has a separate neurological issue causing the apnea, and we will have to begin bi-pap trials.

Here's to a successful sleep study, completed birthday party planning, and a great year in his three-year-old preschool class!

On Conductive Hearing Losses

In many cases, conductive hearing losses are caused by earwax or fluid in the ears. This condition is both temporary and easily remedied (with wax removal or pressure-equalization tubes to allow the fluid to drain).

Since Nolan was discovered to have a large conductive component to his hearing loss (the frequencies from 250Hz-1000Hz have a large air-bone gap), I have been trying to figure out what could cause this component. It is not caused by fluid, infection, or any other temporary situation. It has been there since birth, and has not improved with the insertion of PE tubes. In fact, it has only gotten worse or fluctuated over time. In addition, he has had several hearing tests with type A tympanograms (no fluid), which demonstrate a hearing loss with a clear middle ear space.

With some research via Dr. Google, I have found a few causes for air-bone gaps (conductive hearing losses) in children. They include:

• Ossicular malformations (the middle ear bones are malformed). Nolan's CT scan at the age of six months states: "Bony ossicles are well formed. Middle ear cavities and mastoid air cells are clear." The CT scan rules out any malformation of the middle ear bones.
• Atresia. This means the external ear canal is not formed and prevents the transmission of sound to the eardrum. Nolan does not have atresia.
• Congenital cholesteatoma. The MRI and the CT scan did not indicate any cholesteatomas present.
• Fixed stapes or malleus (middle ear bones). Usually identified on CT scan- Nolan's CT scan was unremarkable.
  

Of course, Nolan doesn't just have a conductive loss. He has a moderate sensorineural hearing loss from 2000-4000Hz. So, what could cause a congenital hearing loss consisting of both sensorineural and conductive components, which fluctuates over time?

• Enlarged vestibular aqueducts: there is often a low frequency air-bone gap in children with EVA. Nolan's CT scan report states, however: "Vestibular aqueducts are unremarkable." So it is probably not that.
• X-Linked Stapes with Perilymphatic Gusher. This occurs only in boys and causes a fixed stapes, which cannot be repaired because a "stapes gusher" will occur and the ear will lose all hearing. This is a mixed hearing loss profile, but is typically severe-profound (Nolan is moderately severe).
• Otosclerosis. This is rarely encountered in children, and is extremely rare in congenital form. This is also visible on CT scan, and Nolan's CT scan was normal.
  

I can't really find anything else that would cause Nolan's hearing loss profile. In any case, we either have an idiopathic conductive hearing loss and an idiopathic sensorineural hearing loss, or the two are related and Nolan's CT scan or MRI failed to show the problem.

I plotted his thresholds over time, excluding any tests with middle ear pathology (all scores below are with either type A tympanograms or patent PE tubes). Anyhow, you can see the fluctuation in his air conduction scores over time- something our ENT and audiologist have never really paid attention to:

Left Ear

Right Ear

IEP Meeting: A Complete Success

Team Nolan

We had Nolan's Individualized Educational Plan (IEP) meeting this morning with the Committee for Preschool Special Education (CPSE). It was a resounding success!

We went over Nolan's history and current hearing situation, under-amplification, and desired goals/accommodations. We had placed a picture of Nolan on the front of the report to help make the meeting more personal. I couldn't find childcare for Nolan this morning, so the picture was a moot point since he was sitting at the table. Still, it put a face to all of the nameless data in the reports.

A draft of his psychological testing was present, and he is functioning above age level with regard to cognition. This is great news: while it is always wonderful to hear you have a bright kid, it also means that any delays Nolan is experiencing are a direct result of his hearing loss and not any other cognitive issue.

It was decided that Nolan's speech and language services would continue, with a mixture of services provided in the home and at the preschool. In addition, his need for an FM system was written into the IEP under "assistive technology."

We did hit a roadblock when we mentioned the FM system, with regard to funding. We managed to bring the conversation back to the point by reminding everyone that we simply need to place the fact that he needs an FM on the IEP now. We're worried about the "what," not the "how." The school district and county can determine the funding for the FM system at a later date.

We also obtained consultations services from a Teacher of the Deaf (TOD), for one hour per month. This is primarily to monitor Nolan's progress in both the social and language arenas.

We did discuss Nolan's potential sensory integration issues and problems dealing with large groups and noise. One fortunate aspect of his attendance at the meeting was that he had a meltdown from the noise/people- demonstrating behaviors we see when he is overwhelmed. Seeing as how there were only six adults in the room and not the sixteen children he will have in his preschool class, it was easy to see why we are concerned.

After watching his behavior and inconsolable nature, then mentioning that he has (very) mild hypotonia in his hands/arms, the team thought an evaluation by an occupational therapist (OT) would be a good idea. I doubt Nolan really needs services in this area, but an evaluation can't hurt. We added the OT evaluation into his IEP at the end of the meeting.

Everything is signed, and Nolan will have all of the services he needs to excel.

Today is a good day!

IEP Booklets

When a parent goes to an IEP meeting, it is good to have all of the proverbial ducks in a row. Know what the necessary goals and accommodations are, and have the data supporting the requested goals/accommodations at hand.

To accomplish this, we made informational booklets about Nolan's particular situation and his needs in an educational setting. The books consisted of:

1. Nolan's name and picture, to personalize the report.
2. Future Planning Statement.
3. History.
   
4. Audiology: all reports from birth to present.
5. FM Systems: letter of support from our audiologist and research on personal FM systems
6. Language testing: Testing from John Tracy and from our home Speech Language Pathologist.
7. IEP References: this included the IEP checklist, deaf student policy guidance, essential aspects of an IEP, and Did You Know?

We made a booklet for each person present at the meeting, so everyone could have their own reference during certain discussions. These were extremely valuable during our IEP meeting- a big thank you goes out to the John Tracy Clinic and to Drew's Mom.

Still Preparing for the IEP

Nolan dearly misses the John Tracy Clinic

Once completed, I will post a complete description of the booklets we are preparing for Nolan's IEP meeting. The information is nearly complete, thanks to help from Drew's blog and from the John Tracy Clinic.

The reason we are providing this information to the IEP team is twofold: one is to educate the team on the needs of children with hearing loss (the needs vary by the child, but Nolan's particular concerns deal with social difficulties, language holes, access to auditory information, and articulation). The other reason is to provide the necessary data to back up our requests for services and accommodations. At our pre-CPSE meeting, we were told that personal FM systems and TOD services are not typically offered until Kindergarten. With Nolan's under-amplification, fluctuant hearing loss, and articulation delay, the FM system is necessary. Every professional (from our own audiologist to the audiologist, TOD, and speech language teacher at John Tracy) has stated that Nolan's access to sound is compromised without the use of a personal FM system. Please note that ear-level FM systems are not universally required among deaf or hard-of-hearing preschoolers (they can even be detrimental to toddlers with cochlear implants, who are unable to report on the functionality of the device). For Nolan's specific hearing situation, however, the personal FM system is vital.

Nolan had his cognitive testing this morning, and did well. He did manage to look like a prodigy on the receptive language portion of the test, pointing to the correct pictures for "horizontal," "parallel," and "equivalent." He doesn't actually know these words (testing officials don't do any evidence gaining- if the child happens to point to the correct picture, they get the point). He's age appropriate (or a little above age level) for his receptive language, but he's not a savant! Still, the test will show that he does not have a cognitive disability and any speech or language gaps are due specifically to his hearing loss.

He did miss the question, "Point to the doll," consistently pointing to the ball. His compromised hearing definitely comes into play, even in a quiet environment. We really need to get his hearing situation figured out and get his amplification set to the correct levels!

We will be running to Office Max tonight to make copies of our little booklets- full of research articles on FM systems, Nolan's history, and audiological/language testing results. Our IEP meeting is at 11:20am tomorrow- wish us luck!

Speaking of preschool, I mentioned the start of the school year to both of our boys. They LOVE school, and the conversation went something like this:

Matthew: "I can't wait to go to Preschool."

Nolan: "You go preschool. Nolan go JOHN TRACY!"

Every conversation ends with Nolan insisting that he is attending John Tracy in a few weeks. I'm not quite sure how to get the concept through to him, though I suspect a few experience books will help him through the transition. He is still in love with his John Tracy teachers and misses his friends dearly.

IEP Part 2: Future Planning Statement

A future planning statement will be included in Nolan's IEP booklet, to share our vision of Nolan's successful future:

We see the first five years of Nolan’s life as a critical window where he needs intensive intervention to establish his communication and listening skills that he will need in all future educational and life experiences.

In the short term, we see Nolan attending First Covenant Preschool in Jamestown, New York. This is a mainstream program, designed for three year old children. As Nolan will be attending a mainstream preschool, a personal FM System is vital to allow him equal access to the curriculum. Weekly sessions by a licensed Speech Language Pathologist are required to maintain Nolan's current language levels, and to address his difficulties with articulation. Regular monitoring by a certified Teacher of the Deaf (TOD) are necessary to verify that Nolan is functioning socially in the classroom, in addition to monitoring language, equipment (FM System) usage, and Nolan’s ability to hear and communicate in a mainstream classroom setting. The goal for these preschool years is to establish an appropriate foundation of communication, language, listening and literacy that will hopefully allow him to integrate into a mainstream Kindergarten class in Southwestern School District with minimal or no speech and language delays.

If Nolan receives the appropriate accommodations and services, we see Nolan graduating from High School and attending college or post-secondary education, should he choose to do so. We see Nolan as a positive, contributing member of society, who will be successful in life, without letting his deafness define him.

IEP Booklet, Part I: History

I am putting together a packet of information for those on Nolan's IEP team. The first section will be his history, and this is what I've written (yes, I plagiarized Drew's Mom's report, changing it where necessary):

Nolan was born with mild/moderate hearing loss in both ears. Nolan’s hearing loss is now moderate/moderately severe with a history of fluctuation and progression. Recent tests have demonstrated an air-bone gap, indicative of a conductive component to his loss. As tympanometry, patent pressure-equalization tubes (PE tubes), and normal CT scan indicate no cause for a conductive low-frequency hearing loss, the loss is permanent. In addition to the conductive low-frequency hearing loss, a sensorineural mild/moderate high frequency hearing loss is present. Hearing loss characteristics are currently under investigation due to conflicting bone conduction test results from two audiological testing centers. His air conduction thresholds remain in the moderate/moderately severe category.

Nolan obtained bilateral hearing aids in January 2008 at 4 months old. With amplification, Nolan is demonstrating a moderate loss in the low frequencies, normal hearing levels in the mid frequencies, and a mild/moderate loss in the high frequencies. While Nolan responds to speech sounds when amplified, he is not obtaining all of the sounds of speech with his hearing aids at the current levels. His current aided speech awareness thresholds are 30dB, which is consistent with a mild hearing loss.

Nolan started speech therapy through the Early Intervention system at the age of five months. Through intensive rehabilitation and therapy, Nolan has learned how to discriminate the sounds of speech with the use of his hearing aids. Nolan is meeting listening and language milestones for his overall language and communication progress. Unfortunately, due to fluctuating hearing loss characteristics and underamplification, Nolan is testing one chronological year behind his hearing peers with regard to articulation (Goldman-Fristoe: See testing reports).

Nolan has also suffered from chronic otitis media with effusion, which adds to his permanent, pre-existing hearing loss. Thresholds as high as 80dB have been observed in the presence of fluid. Nolan has had three sets of PE tubes placed, in addition to a tonsillectomy and adenoidectomy in an effort to eliminate the effects of chronic ear infection. He has had two ear infections since the placement of his third set of PE tubes in April 2010.

Nolan also has a significant medical history consisting of the discovery of posterior urethral valves, which were ablated at 18 months of age. Severe gastro-esophageal reflux disease (GERD) with mild delayed gastric emptying was discovered when Nolan was two years old, in conjunction with failure-to-thrive. He was placed on a special diet and started taking Nexium (Esomeprazole), which has allowed him to gain sufficient weight to place on the growth charts. In January 2010, a sleep study was performed and found the presence of severe obstructive and central sleep apnea. A tonsillectomy was performed to help eliminate any potential cause for the obstructive apnea. The central sleep apnea is neurological in origin and is still under investigation. A second sleep study will be performed in August 2010 to determine if the central sleep apnea is still present.

Potential Problems

1) Equipment Maintenance

Equipment problems can crop up at any time, and will significantly impact Nolan’s ability to hear. Dead batteries, dirty microphone covers, moisture, earwax in the earmold, and intrinsic mechanical failure of the devices will prevent the hearing aids from functioning appropriately. Some potential areas that could affect microphone clarity include dirt, sand and moisture. Also, if anything is placed over Nolan’s ears, such as a hat or hood, ability to hear is compromised.

2) Ear Infections

Ear infections have a deleterious effect on Nolan’s hearing levels. His permanent loss is currently moderate/moderately severe, and will increase to a severe level of approximately 80dB when fluid or infection is present. Nolan has a history of chronic ear infections that are partially alleviated by the presence of patent PE tubes.

3) Moisture

The hearing aids (“ears”, as they are referred to by Nolan and his family) are not allowed to get wet, so he can not wear them when swimming or bathing. Nolan cannot replace his hearing aids without help, and assistance is needed when he is entering a wet or moist environment. He is also now able to sometimes let his parents know when his hearing aids are not working.

4) Hearing In Noise and Sound Localization

Noisy situations, including classrooms, restaurants and the outdoors, can be difficult for Nolan, as sound localization and quality are affected.

Educational Implications
His parents judge Nolan in need of continued auditory training/listening therapy, in order to develop his communication skills and ultimately literacy skills. They would like a teacher, specifically one for the deaf, who would reinforce what he learns in an auditory training program, which will fine tune his ability to listen within his environment and will benefit him significantly when he moves to the mainstream.

As Nolan’s hearing changes over time, his parents check his hearing daily using the ling six sounds. Nolan’s teacher will also be expected to review Nolan’s ling sounds each morning as he arrives at school. Lings are six sounds (/ah/, /oo/, /e/, /sh/, /s/, and /m/) that predict well his ability to hear all other English phonemes. This check needs to be conducted in a variety of conditions, such as with noise, at a distance of 6, 9 or 12 feet and when the speaker is using a listening hoop. Currently, Nolan is able to correctly repeat the Ling sounds at a distance of 3-5 feet. An equipment check is required at the beginning of each day to make sure his hearing aids and FM System are on and working.

Other concerns include, but are not limited to, literacy, socialization, articulation, consonant deletion, consonant production through listening only (using a listening hoop where the speakers mouth is concealed) and grammar.

Appointments Made

Nolan's preschool class at John Tracy

I miss the John Tracy Clinic so much! They have set us on a path to figuring out Nolan's hearing loss, and for this I am forever grateful.

I called our audiology center today and made an appointment for another bone conduction test. We're going to run bone conduction first, and see what we get. Our audiologist said that sometimes there is a conductive component in children (or adults) with no identifiable etiology- in other words, we may not find a reason he has a large air-bone gap. The biggest concern is that his air-bone gap is larger than we've ever seen, indicating normal cochlear function in the low frequencies. This is completely different than any other test we've seen, so we definitely want to see if we can replicate the results. We'll see what happens on August 23rd.

I also called our ENT to make an appointment for Nolan. He has an appointment in October, and they said they could not squeeze him in any sooner than that. They did have one appointment on September 20, at 7:45am. This is definitely not ideal, since Matthew would have to miss school and we would have to leave the house at 5:45am. I told them I couldn't take that appointment- I'll find a new ENT before we have to deal with that situation. When I mentioned that I'd just go talk to his pediatrician or find another ENT because we think he's been misdiagnosed for three years, the office person changed course and said that perhaps the nurse practitioner could squeeze him in sooner. We'll see. If they don't call back tomorrow with an afternoon appointment sometime before the start of fall, then I am seeking another ENT.

Meanwhile, I am preparing Nolan's IEP information, including an entire section on FM Systems. I'm stealing ideas from the material provided at John Tracy, and from Drew's mom- who is definitely a superstar in the world of advocate moms.

Audiograms

Here is Nolan's aided audiogram from John Tracy, transposed onto an audiogram with a speech banana. We sort of wondered where /f/ went- I suppose it disappeared because he can't hear it. The magic box shows that the aids aren't meeting DSL targets, either.

Here is his unaided audiogram, with bone conduction scores. Currently, his right ear is testing better than his left. The shaded yellow area represents the air-bone gap, indicating a conductive component to his loss. The frequencies above 2000Hz do not show an air-bone gap, indicating the loss in that region is purely sensorineural.

Home Flooded Home

We landed in Buffalo last night, happy to be off the planes and close to home. The kids, of course, fell asleep about 5 minutes before we landed, so we had some fun carrying Noodle Boy and Cranky Boy (I'll let you sort out who was who). By the time we got off the plane, all the luggage was stacked up by the Southwest Airlines office. All the luggage, except for Nolan's car seat.

We gathered our suitcases and I sat with the two tired boys while Dennis filled out the necessary missing item paperwork. Fortunately, Southwest Airlines has a loaner program, so they loaned us a car seat until ours could be found. The loaner car seat is slightly smaller than Nolan's car seat, and this was a good thing because the two tired boys had no intention of walking. With three large suitcases, three backpacks/carryons, a booster seat, and a car seat, and a stroller, we needed the boys to walk. There was a brief and intense temper tantrum by Matthew, who wanted to go in a different direction than the parking lot shuttle.

We did manage to get to our car, with all children and luggage intact. Dennis tried unlocking the car with the remote, and nothing happened. For a minute, we had the sickening thought that the battery had died (this happened to us in Cleveland a couple of years ago). Fortunately, it was just the remote that was dead, and we loaded the kids up. We came home to a house that was well tended by our neighbors while we were away.

Sadly, our little flooding situation didn't just disappear, and our family room is quite sodden. We currently have a fan going to help dry things out a bit. Then the computer went on the fritz- we are having difficulty connecting to the internet, and then I get the "blue screen of death" periodically. Rebooting sometimes restores the connection, so if I don't blog for a while, it is because our computer has rebelled and gone on strike.

I have fielded about a thousand phone calls this morning, mostly involving Nolan's IEP. I have scheduled his psychological (IQ) testing for Wednesday morning, and we still have his IEP on schedule for Thursday. I do have to take a trip down to the pupil services office to change the date on his referral form, because otherwise the school can't get the testing and meeting done in the allowed time frame. I still haven't called the ENT or audiologist, though I will probably call the pediatrician soon to take a look at that right ear (still slightly infected, but not as bad as it once was).

We have a lot of ducks to get in a row!