This posting will be a bit technical and is mostly posted for the Deaf and HOH online community. I’ve been so blessed at many junctures by the great information and support I’ve received by other parents of Deaf and HOH kids and thus, in restarting this blog, I am also committing to occasionally blogging about Josh’s hearing journey so as to add to the collective knowledge and wisdom available for other parents who will face similar issues in the future. Because of its technical nature, family and friends–you sure don’t have to read this post. I don’t want you all to worry because I am sure we will get this all figured out eventually.
Anyway, I know that there are often disagreements in the medical community, but lately, I’ve been feeling like audiology is more of an art than a science. Since Josh was first diagnosed with congenital hearing loss at nine months of age, we’ve received a variety of advice about what hearing aids Josh should be using. In fact, Josh is already on his third set of hearing aids. Although his sedative ABR indicated a pure conductive loss, we were encouraged to first try air conduction (in-the-ear) hearing aids. Josh was fitted for ear molds and we officially received the aids just as Josh turned ten months old. In spite of an enormous amount of Otto-ease and KY Jelly, Josh’s left ear mold continually became unseated after about 5-10 minutes of use and Josh would begin to get lots of feedback, at which point, he’d remove both of his hearing aids. He was even able to manage this through Hanna Anderson pilot caps. After a few adjustments, our audiologist decided that Josh’s left ear canal might just be too narrow and basically abnormal (confirmed by an MRI) to allow a good fit with ear molds. In the meantime, I had done some of my own research online and realized that many audiologists recommend bone conduction aids (typically BAHAs by Cochlear although now Oticon has a model as well) for Josh’s type of hearing loss. We talked to our audiologist about this and she felt it might be worth a try, although on the phone, her younger colleague repeatedly insisted to me that air conduction aids were far superior if we could at all manage it. Our audiologist was able to return our already-purchased aids to Oticon if we were willing to try their new bone conduction aid on a loaner basis. Our audiologist definitely felt that one aid was better than two (binaural) for Josh’s loss. During the exchange, we had about three weeks in August where we were without aids all together. In September, we received Josh’s Oticon bone conduction aid. Josh was just about to turn one and decided that he very much liked to take the aid off almost immediately after we had put it on. During the few times where we’d get him to wear it for an hour straight, we would notice a slight uptick in Josh’s babbling, but that is about it.
At this same time, Josh began receiving home bi-monthly visits from our school district’s Deaf and HOH teacher. She is great and really modeled for me how to use both ASL and spoken language to try to encourage Josh’s language development. This teacher knew that I was frustrated with how difficult it was to keep Josh’s aids on him. In late-October, she brought the school district’s audiologist with her to a visit. I had been planning on sticking with our private audiologist until Josh was in school, as we definitely were happy with her. The school district’s audiologist must have an alternative energy source because she lights up a room (and not always in a good way), I think she even raises the temperature of the room when she enters. She entered my living room in October, looked at Josh and told me that the school district was going to supply Josh’s hearing aids from now on. She told me that she was going to fit him for a new headband and was going to supply him with two BAHA (binaural aids) with FM capabilities. In fact, the school district wanted me to begin wearing an FM system around the house ASAP. I quickly agreed to this plan because: 1) We had not yet purchased the Oticon bone conduction aid and it was going to cost us $6,000-$7,000; and, 2) I understand that most people struggle to get the school district to supply a FM system, and here we were going to get it without even a battle. The audiologist assured me that we would get Josh speaking with the binaural aids in place.
Josh received his new aids on the last day of November. I was disappointed to see that they were very-used aids (as much as I like recycled clothing, not so much recycled hearing aids). I was also disappointed by how much less advanced they seemed than our Oticon aid, which had the capability of much more fine-tuning (much like our Oticon air-conduction aid). The BAHAs seemed to have just 3 settings; low, medium, and high. The head band was also much tighter than Josh’s Oticon band and far less adjustable. I do think, however, that we probably hadn’t been wearing the Oticon tight enough on Josh most of the time, thus likely decreasing it’s effectiveness. By this time, I had finished my ASL beginner course and Josh and I were signing with each other. Josh probably had about 15 signs at the end of November and 25 signs by his 15-month check up (mid-December). He also had occasional words that would show up for a day or a week and then disappear. We’ve seen Stop (Sta), Please (lees), Yes (Yahsss), Dog (dah), Down (da-ww), Star (ers), Horse (erse–very similar to star) and Nigh, Nigh (nursing) all come and go, occasionally resurfacing here and there but nothing consistent. He has consistently used “Dis” for “this” since about mid-October. Everything is “dis.”
Enter December–Josh got his top molars and 3/4 eye teeth throughout the month. He really stopped sleeping and awoke about 4-7 times per night (seems like it might have had to do with his teeth and his very off-centered bite). He also had a very bad cold and pretty much stopped eating. Finally, he developed extreme separation anxiety. So, we had plenty of battles and the hearing aid battle just fell of the radar. After one day where he took it off about 20 x in 30 minutes, I just put it aside and decided we had too many other challenges. Even our genetics doctor, who we saw on Dec. 23rd, told us to first solve the eating issues, than the sleeping, then go back to working on the hearing aid. She also referred us into the cranial-facial clinic because of concerns with Josh’s bite and jaw development. There was a cancellation for the January clinic, so we were able to get in to the clinic last week, which was a nice surprise.
We did re-start our efforts with the BAHA band in January, before our appointment. We discovered that one of the hearing aids wasn’t even working. Our Deaf and HOH teacher also confirmed this at school. Oh, I should mention that we changed Josh’s IEP at the end of December. Instead of the home visits, Josh was referred into the school district’s Deaf and HOH classroom (just 4 other kids spanning ages 16 mos. to 35 mos., of which Josh is the youngest), which meets two mornings/week for two hours at a time. Parents stay in the classroom with the kids, so there is no separation. The speech therapist also joins us in the classroom for about one hour each week and so far, that is the only access Josh has had to speech therapy. Josh always keeps his hearing aids on in the classroom so he is getting at least 4 hours of amplification each week, which is better than nothing. Anyway, we sent in one of his hearing aids to get fixed and they sent it back saying it was working. A few days later (in spite of trying a few different batteries), it was once again dead. At this point, we met with the facial cranial team. Josh saw yet another audiologist as a part of this team. This audiologist told us we needed to get Josh back in air-conduction aids because they were far superior to the BAHAs. She also told us we needed to get our own aids and should not be relying on the school district’s equipment. Later in the afternoon, the lead ENT also told us we needed to go back to our old audiologist because she was so good and we needed to have our own equipment. He told us, however, that Josh should be wearing the bone conduction aid, but just one, not binaural. He was very insistent that one was better than two. We didn’t leave the meeting with much more info on Josh’s ultimate diagnosis, other than that all parties seem to think his hearing loss is syndromic but no one knows what kind of syndrome it is. Thus, the head ENT doctor named it “Joshua’s syndrome.” We learned that Josh’s off-centered bite is mostly caused by nerve damage on one side of his face (??? more mysteries here for me than answered questions–hopefully the forthcoming medical report will explain this more) and nothing can be done to correct this condition (inoperable). Josh does have a small surgical repair he’ll require below his lip in a few years (a surgery to clip his low-hanging/attached frenum) and of course, he’ll need boat loads of orthodontics, but that is all in the future so I’m not even worrying about it now. Needless to say, I left the meeting more confused than ever. Still, I immediately booked an appointment with Josh’s original audiologist and we see her on Monday.
I am currently in the middle of John Tracy Clinic’s free correspondence course for Infants. Recently, I sent in a report and in the note section, I relayed my confusion about what hearing aids Josh should be wearing. I trust the folks at John Tracy for accurate guidance in this area. We will definitely be signing Josh up for a summer pre-school session when he is old enough and I cannot wait for Josh to see their audiology team directly. Anyway, John Tracy’s team just got back to me via email and the jury is in. They said bone-conduction aid is best, and just one (binaural just causes confusion). Also, they told me there was really no need for us to use an FM system with Josh at home right now. They told me to hold off on that until he was in school. I am relieved to hear this because the two times I tried to use the FM system, it completely terrified Josh. It made his whole head vibrate like crazy. I did call our old audiologist about this and she said she wouldn’t use an FM system with the BAHA at all. She told us to just not use it. We happily took that advice.
In the end, looks like we are going to be purchasing our own bone conduction aid. Not sure whether we’ll go back to the Oticon or go for a BAHA. In the meantime, the school audiologist took ear molds last Friday because she decided on her own that we should try air conduction aids again. I didn’t tell her about my “conflicting-opinions” drama. I just smiled and said we’d be happy to try the air conduction aids again. I figure, why not try the school district’s aids free of charge (they pay for the ear molds too). If for some reason Josh keeps these on better than the bone conduction aid and his ear canals will now keep these in, then I guess hearing aids that Josh actually wears are better than an aid that he pulls off constantly. We’ll have to see. I don’t have high hopes for the air-conduction aids.
One thing I can say for the lead ENT on the facial-cranial team was that he motivated me (and guilted me, which is okay). Actually, he verbally chided me about the hearing aid use. He said, “You wouldn’t let Josh go outside without a jacket on at this time of year–so to his hearing aids. He wears them every day just like he wears his shirt. No questions, no options.” Yep, he’s right. I am the parent. And so, I am happy to report that Josh’s use of the hearing aid has crept up from 2 hours per day, to 5 late last week to probably 8 hours yesterday and today. And Josh, who is up to about 50 signs, is now actually babbling quite a bit more, just in the last week. Denial is no longer an option for me. Josh benefits from amplification and it is my job, as his parent, to figure out how best to get him to the point of being amplified during all of his waking hours.
Whew! That’s quite a post.