Vast improvement in toddler's hearing at second opinion, but what does it all mean?

[Courtaney]

ABR test Dec. 2009--4 months, non-sedated, slept through test

Frequency Right Left
500 55 55
1000 60 50
2000 60 50
4000 65 55

Masked BC
Right DNT, but 55 at prior appointment
Left 40

OAE (from two months prior)--no hits on right ear; hits at just 2000-3000 on left.

This ABR was consistent with one performed 2 months prior at 2.5 months (2/3 only was completed). The results were within 5 dB at the various frequencies. Tympanograms were normal both times.

Hearing aid testing attempted twice, completed once in infancy. Two behavior tests were attempted at 17 and 19 months. The first one found fluid in the right ear and the left ear tested 60 to 70 dB but with no double hits...test very unreliable (to parents). The second of these tests was unable to be completed due to crying, but there was one clicker thrown out there during soundfield testing that got a response at 25 dB.


ABR test, parent requested, September 2011--25 months, under general anesthesia

Frequency Right Left
500 40 40
1000 35 35
2000 40 30
4000 45 40

Masked BC
Right 40
Left 30

Testing was completed after thorough ear exam by ENT during tonsillectomy; no fluid in ears.


Behavioral testing--February 2012--HA check (sans aids)--30 months (picture spondees, VRA)


Frequency Right Left
500 35 35
1000 35 30
2000 45 45
4000 45 50

srt
Right 35
Left 30

Soundfield sat 25
Soundfield srt 30

Parent remembers seeing 30 on testing paper somewhere for BC.

Normal tympanograms


Behavioral testing--May 2012--HA check (sans aids)--33 months (picture spondees, combined VRA/CPA)

Frequency Right Left
500 40 35
1000 35 30
2000 40 45
4000 50 45

srt
Right 30
Left 25

With hearing aids:

Frequency Soundfield
500 20
1000 15
2000 15
4000 20

Normal tympanograms


Behavior testing (second opinion, new hospital)--June 2012--complete hearing evaluation (sans aids)--34 months (picture spondees, CPA)


Frequency Right Left
500 CNT 5
1000 CNT 10
2000 CNT 10
4000 CNT 5

srt
Right 20
Left 5

Bone conduction--0 dB

OAE--normal in left ear at all frequencies tested. Right ear--normal at 2000 and 5000-8000; abnormal at 1500, 3000 and 4000.

Tympanograms--normal for left ear, abnormal for right ear suggesting a stiff, retracted tympanic membrane. Evidence of conductive loss.

*More frequencies were tested in the later booth tests (nothing that stands out), but I wanted to keep the records similar and just report on 500-4000.

I made a post on here months ago regarding my young son's unusual hearing loss history and had some good advice from Doc Audio among others. He was an extremely premature infant and the ages above were corrected for his prematurity. Obviously, we have some incredible gains between the ABRs in 2009 and 2011 as well in the behavioral testing from 2010 and 2012, and especially between May and June 2012. No, I haven't noticed gains in my son's hearing this past month. He's always heard really well without his hearing aids, at least since he hit the toddler years (18 to 24 months) and could respond back.

There was a progression of sorts, though, in noticing subtle noises, especially those coming into the home from outdoors, such as a faraway train, the chimes from a church down the road, a bird singing outside, etc. He did not appear to understand my whispers at close range, behind his back at 18 months but by 24 months he could hear them from a good distance, back turned (very good responses from 6-12 feet, the furthest I went). By 30 months I lengthened the distance on whisper testing and got very good results from 12-15 feet and pretty good/fair results up to 25 ft. I always suspected because of the whispers and the fact that he has never seemed to not hear or understand anyone's voices in normal conversation, across the room, and even wakes up unless my husband and I speak in hushed tones on the first floor (his room is on the second floor, up a winding stairway), that his hearing was at least in the minimal range. I also always suspected my son was not giving his all in the booth.

It was a combination of his functional hearing in his environment and my own questions about how he was tested in the booth that led to the second opinion testing this month, where they took him all the way on his spondees and tones and he responded. He played the games well, and I had a little talk with him that morning, asking him to turn to the tones. He tried really hard. To me, it's a combination of things, including toddler behavior that may have seemed cooperative, but was not, combined with an audiologist perhaps having a preset opinion about what the results would look like, and sticking close to that area in testing.

That still doesn't explain the ABRs, however. Would he have a normal one today? And if not, what would it mean? Obviously, something brain related involving his prematurity, most likely. But the only thing out there that has the ability for improvement, that I can find, is audiotory neuropathy, but he doesn't have any of the signs of it...not one. He didn't have a normal or near normal OAE. While his ABRs were abnormal, they weren't absent or severely abnormal as is common in ANSD. And, he doesn't have poor speech perception at all. In fact, it's always been considerably better than his tones in the booth testing (so much so his data results I calculated from the May test could point to pseudohypoacusis, I read--feigning a hearing loss!), except for this latest where it's right where it should be. His hearing also never appears worse than how it's tested; it's always appeared much better, including than the 2011 ABR and all booth tests prior to this one. Basically, his results, with the exception of that ABR which didn't fit his environmental hearing, have been, if anything, the opposite of what's typical in cases of ANSD.

Personally, I suspect it's something that may be related in someway to AN (or at the very least brain related like it is), but very different in outcome, where speech perception is not affected and the cochlear was, somehow. Every test has read SNHL. That's his diagnosis (was until the latest test anyway). But of course SNHL isn't supposed to get better and occasionally AN does. Hopefully when his conductive loss clears up, the right ear will all test normal, which I believe the audiologist pretty much expects. She, by the way, suspected AN after she tested him, upon seeing his prior ABR tests, and no OAE on those papers I handed her. But I remembered he had an OAE as a 2-month-old and later found those results. We also haven't had a chance to discuss his speech perception history, both in the booth and especially in his environment. Even with the rare cases of AN with normal hearing (in a booth test), the srt numbers are poor. Any expert opinions out there? An extremely rare case of AN that doesn't fit the mold at all? Something brain-related but yet undiscovered that perhaps is only found in babies born so early? Or a miracle in a little preemie who's had his share of miracles? I always wondered if at 23 weeks, his brain didn't put his hearing system on hold somehow so it could concentrate on living, and during his first years, it came on, with brain testing for some reason trailing behind his functionality. I just don't know, but wondered if anyone on here had an opinion what it could be.

Whatever it is, we are all thrilled...shocked in a way because I had been hoping for srts in the minimal loss range, since I figured the prior booth tests had to be somewhat reliable, but deep down I think part of me always knew his hearing was normal and I always felt a pang of disappointment when we'd exit the test booth and I'd see the results.

[Um bongo]

They are great results: it's likely that a large part of the initial loss was conductive: eustachian tube function in new-born babies is never fantastic.

As for the neo-natal screening audiometry - not all cochleas have the same kind of responses, even for the same stimulus tones, even if the signal is actually being heard. It's certainly an indication of a potential loss, but it's not positively conclusive.

SRT scoring for kids can be equally poor and more a sign of whether they are 'playing ball' or not: delayed speech acquisition can have a bearing too.

In practical terms, get a management strategy in place to deal with the current level of loss: if there's speech therapy available, it might be beneficial to get on a pre-school program.

The Doctors on here might be able to give you more advice on the best steps to take as things progress.

Best of luck with everything.

[Courtaney]

Thanks for your reply, um bongo. The thing about the original ABR testing was that I had corrected for his prematurity regarding age, meaning he was actually 8 months post-birth when the first ABR above was completed, 6.5 months when the initial one was given, where 2/3 was completed due to him awakening, so probably a lot less likely fluid still remained from birth (at least I am assuming). Plus he not only had normal tympanograms, but his ENT examined his ears to approve the hearing aids 3 weeks after his first ABR/3 weeks before the second and saw nothing (although I admit he's had some fast appearing and disappearing fluid in the past, but normally it's slight in nature).

He really tried hard on all the picture spondees in the booth tests (February, May and June)--in fact he appeared to be acing those words, greater than 90 percent on all of the spondee testing, which led me to ask how the srt was found, and the audiologist mentioned dropping down to 30 (or 25 or whatever, depending on the test), and him missing one, then getting another, so that was the 50 percent. It was done as part of a "hearing-aid" check, without the aids, so maybe that's why things were kept close to that ABR in terms of decibel levels attempted. I do realize SNHL isn't supposed to improve, so I understand where the audiologist was coming from in terms of testing. Though I had of course relayed everything we were seeing at home (I have done that since he was a young toddler) but never felt that I was being taken seriously. As far as the spondees in February, I can remember only two he didn't locate, but one of them he was given just 3 seconds to find and it was on the outer edge of the pictures--I had no clue at the time that a miss like that was given the weight it apparently was.

In May, I only heard two misses again, though I admit I didn't catch everything at the end of the testing...the only misses I noticed were for the same word, cowboy, which he appeared not to know (that time the words weren't reviewed ahead of time)...so it appears that lack of knowledge probably cost him there. More than anything, I wanted the second opinion audiologist to go as far as she could with the words because I knew he had a real chance to go lower, I just didn't realize how low! For the tones, I always thought he simply wasn't turning/reacting to the softest ones, or that he was reacting slowly and by the time he did turn, it was already louder. He does process things slowly (particularly the tones), the June audiology team noticed that right away, but they didn't let that keep them from searching for just how low he could go with things.

As for speech therapy, we have had that since he was 4 months corrected. His speech development has been anything but ordinary (no cooing stage, slightly behind but excellent, albeit incredibly short, repeated babbling stage, excellent early vocabulary (hit 30 word approximations before 14 months, 70 by 16 months, 100 if I include his signs, too, for other words), early first phrase (14 months), but late to have them finally get past the emerging stage (29 months, when his speech exploded with sentences), stretching in length each and every month after. Receptive language has always been great, including a score of 105 on the Bayley at 5 months, 4 weeks after he got his hearing aids (and supposedly had a moderately severe/near moderately severe loss). The testers couldn't believe it, and commented at the time, saying "Are you sure he just got his hearing aids 4 weeks ago?"

His receptive ability never fit that level of loss, and certainly not auditory neuropathy, looking back. At 12 months (all ages have been corrected) his language scores led the pack on the Bayley III, with scores of 115 in expressive and 110 in receptive. He reversed that to 110 and 115, respectively, at 18 months, but upon testing 7 months later, his expressive language had fallen a lot, in part due to lack of phrases (into the 80s, and likely would have been in the 70s by winter, if he had been tested then). Cognitive at the time, however, tested better than ever, at 125, up from 90 at the original Bayley at 5 months. Articulation was very poor until February (did not say second syllables until then, among many other things). His one ST estimated he was more than 3 standard deviations behind the mean in articulation for a long while, rating him at just 25 percent intelligibility at 24 months. That too has come on strong since and he's now age appropriate there (a gain of more than 2 standard deviations in 3-4 months). There are many things that could have had something to do with the articulation difficulties, however. The lack of cooing, extremely short babbling period, quiet nature in general as an infant/toddler and pure referential speaking once he started allowed for a serious lack of sound play. We had hearing loss, vocal cord damage, oral motor issues, and a family history of very poor articulation (birth mom was 60 percent intelligible at age 5, her brother at best 25 percent at that same age). And there's the extreme prematurity. We think a lot of it was brain related too. It was so interesting that once his utterances took off, so too did the syllables in words and articulation in general. Everything was shooting forward at an incredible pace, and still is.

My son qualified for preschool in articulation based on his testing in early March (expressive language was a 97 at the time for actual age while articulation was 65). A new SLP at his hearing clinic only 4 weeks after testing said she was surprised he qualified, pegging him then as at least 75 percent intelligible at the time. That's how fast things came on this past winter/spring. He's now probably 80 to 90 percent, depending on the person, and is age appropriate in his conventions and above age in his vocabulary. Sentence length is typically 4 to 7 words now, for most, but he's had sentences up to 10 words in length. But regardless of where things stand now, he will get speech/preschool next year, which I think will be great for him for many reasons, including the fact that we've had to keep him isolated from group situations/children in general for so long due to precautions with his lungs, but he's free to mix and mingle, finally, this summer. He also gets private speech therapy and has for two years (with his former, now retired EI SLP). She is an awesome resource for him with her history of work and ability to see the whole child. She said he doesn't fit any child's speech development (or hearing for that matter) she's ever seen but at the same time, she sees how fast he's coming on and is confident he's not going to need it when he enters kindergarten, based on his rate of gain. She always knew he was functionally appearing to hear much better than testing seemed to show, as did I. She is working on voice issues now with him, as she said everything else is coming along fast now, on its own. Her oral motor work that started in December, via feeding therapy, also had to be part of the reason why his articulation took off so fast.

Our plan is to see his ENT soon for wax cleaning (there was wax in both ears) and to check the fluid beneath the wax in the right ear, and go from there. He has a history of fluid in the right ear only (never in the left) but it's usually fleating, there one month and gone the next. When he had his tonsillectomy, the ENT was going to place a tube in that ear if he saw anything at all, but unfortunately it was dry at the time. Three weeks later, his pediatrician saw a little bit of fluid in that ear at his well check, but a month after that, at his post-tonsil check-up with the ENT, it was gone. As an infant, it would appear at times and disappear, just the same. He only ever had the abnormal tympanogram twice at testing, though, and that last time fluid was discovered when the NP removed the impacted wax. It appears it will probably be the same thing this time. Perhaps a tube will be recommended, or at the very least close observation of the ear. But I know a conductive loss doesn't explain his history. I am sure we will have his hearing followed for a while to make sure things are indeed as they appear. Thanks for your reply. I do hope to hear from some doctors too.