Introduction to Deaf-Blindness Part 2: Categorizing Learners Who Have Deaf-Blindness SLIDE 2 So what we want now, for the next several slides, we're going to look at, what causes the deaf-blindness? What possibly causes the deaf-blindness? How many categories? How do we try to get a handle on what we might expect from a particular learner with deaf-blindness? Kids might be at risk for having a combined vision and hearing losses due to some hereditary syndrome or hereditary disorder, some prenatal condition-- and I'm confident most everybody knows that means something that happened before the child was born-- some the post-natal condition-- something that happened after the child was born-- accident, illness, injury. Family history can sometimes be connected, head injury or direct trauma to the ear and/or eye, or simply being born way too prematurely can cause deaf-blindness as well. SLIDE 3 So the structure that I would like to share with you today about how I think it's most useful to categorize deaf-blindness is by these three different variables you see on your screen right now. The first is by the time of onset of the sensory loss. And we're going to go back through each of these. I want to give them, the three of them, to you. And then we'll investigate them in more detail. The first one is by the time of onset of the sensory loss. The second one is by the degree or the type of sensory loss. And third, both, by considering the time of onset and the degree of sensory loss. By far the best predictor of what an individual learner is going to be able to do, the best predictor of what an individual learner is going to need from a team, from a family, is if you use this third model of categorization where you consider when the child's vision and hearing were impacted and how and to what degree they were impacted. SLIDE 4 So let's start with the first one and see what we can do to see if we can make this make sense. We want to talk about time of onset of sensory loss. Most of you probably are familiar with these terms. Just in case somebody maybe doesn't know them, we'll use the word "congenital." That means the loss is the disability. The condition already is present at the time the child was born. I think that's a pretty common word. This next one may be a lot trickier to some of you. We use the word "adventitious." I mention that because you'll see it in the literature if you do any reading in this area. And an adventitious loss is wide open, to tell you the truth. It means that some time after the child was born there was illness, injury, accident. Something happened, and sometimes we don't even know what that something is. Something happened that impacted the child's vision and/or hearing. And I'm sure for family members if you don't have an answer to what that adventitious loss was caused by, it's incredibly frustrating. I can only imagine how much you would want to know why and what happened. But honestly, when you're working with your child's team, the most important thing to remember is to share with them the timing of the incident. And if it was an infection, if it was a head injury, a direct injury to the eye, whatever it was, if you can share with them when and what. Although I have no doubt you'd like to know why, the team doesn't necessarily have to know why to be able to figure out how to help your child learn to the best of their abilities. When you're talking about time of onset, this needs to be figured out in regard to both vision and hearing and not jointly. It needs to be figured out once in terms of when was the onset of the vision loss, when was the onset of the hearing loss. SLIDE 5 The time of onset is critically, critically important. Although you might think I'm talking out of both sides of my mouth and say, well, this is the most important, and this is the most important, because everything is important or the Kansas Deaf-Blind Project wouldn't have me sharing this with you today. But time of onset truly, truly is critical, probably the most critical. And as we look at this slide, I hope that it might trigger something in your own experience to help you understand why this is so important. Because if a child was born with a hearing, with some degree of hearing, she might have some recollection of sound, or she might have some recollection of the properties of sound, or she might have some basic foundation laid down in her brain-- in the neural networks of her brain-- for language and language interaction, or at least communication interaction. And that is what it's all about. We need those communications networks for learning. Same thing with vision. If a child was born with some vision, that child might have some residual visual imaging, meaning that child might have some visual memory, might have some visual memory of people or what people are generally like, or of boundaries in the world, or simply the fact that there's stuff-- if you allow me to say that-- that there's stuff outside their own body. And we don't know. I think that's the tricky part. SLIDE 6 This it's where I'm not necessarily in 100% agreement with the published literature. I will own that. And you're welcome to contact me if you disagree with what I'm saying. And I will try to explain that a little bit more. But my feeling is, though the medical profession and neuroscience has progressed by leaps and bounds-- unbelievable leaps and bounds-- we still don't have a sufficiently sophisticated knowledge base in neuroscience to rule out what possible benefits there are if a child had vision or if a child had hearing, or both, even if only for a relatively short time after they were born, after he or she was born. In regard to-- let me hold that thought till we go to the next slide. We just don't know. And I like to know if a child had vision or hearing for three months or five months or seven months or whatever it was because there's a possibility we've got some neural networks there that are providing some foundational information for the learner. SLIDE 7 Let's talk about the hearing side first, and then we'll talk about the vision side. I think I'm more consistent with the published literature in regard to hearing. The key thing in regard to the benefit of very early auditory input is whether or not the child had developed language prior to her loss of hearing ability or whether that child had developed language prior to a partial loss of hearing ability. If you look at the professional literature, you'll see this notion, this key point, discussed in terms of prelingual or post-lingual deafness. And what that means, prelingual deafness is that the child lost her hearing or lost a significant portion of her hearing prior to developing language-- pre-language, or prelingual. Inversely, post-lingual is the child lost her ability to hear or significantly lost a portion of her ability to hear post-lingually, or after she had developed language. And kids who are post-lingually deaf have a much better chance of achieving more proficiency, better long-term outcomes, or at least more rapidly achieving long-term communication language outcomes because they had a foundation for language before they lost their hearing and their auditory input. What I try to link this to in my own mind is kids' first true words. Those of you who've worked with very young children or families who may have very young children, you say that you, quote, "understand" what they're saying to you at six months of age or eight months of age because they babble and make noises that sound like inflected sentences. But in the literature, we learn that a child speaks his or her first real word, or in the literature it's called the first true word, anywhere from 10 months of age till 13 months of age. So again, that's not an absolute criterion that can be applied, but I like to use that as a mile marker for me. We can't say specifically if it's 10 or 13 because, as every one of you knows, every child achieves those developmental milestones at his or her own rate, whether there's a disability, whether there's deaf-blindness, whether there's no disability. So it's somewhere in that range. And if I know, when I'm working with a child, that she didn't lose her hearing until after 10 to 13 months of age, I would deem that child as post-lingually deaf. It wouldn't necessarily immediately change everything I did with her, but it is a consideration that I would encourage you all to try to track down and bear in mind. SLIDE 8 Now we're going to look at the same kind of key point in regard to vision, and then the speed of this should pick up a little bit. The key thing in regard to the benefit of early visual input is whether or not the child had developed visual memory prior to her loss of vision. This is where I take a little bit of a different position than a lot of stuff I've read. From the field of visual impairments, it's not uncommon to read that visual memory isn't fully developed until the child reaches approximately three years of age. And I don't mean to argue with that point. But what I think is really important, I'd say even had 18 months of age, we know that the sophistication and refinement of the sense of vision lags behind the sense of hearing. I mean, when babies are born, they really can't focus at all, even if there's no visual impairment. That development proceeds more slowly. But from my experience with kids with vision loss, my experience with kids with deaf-blindness, if they had vision to somewhere in the neighborhood of 16 months of age or 18 months of age, before they lost that vision or before that vision was severely impacted, I truly believe it makes a difference in how they learn. Because I think by 18 months of age, not 3 years but by 18 months of age, kids have mastered visual searching. They're aware there's a world outside their own body. They've been able to get vertical in most instances. You get vertical because of your spatial awareness and your spatial skills. And for kids who have a significant vision impairment or are blind from birth, one of the hardest, earliest milestones to achieve with them is to get them vertical, get them standing up. In kids with that spatial awareness, 16, 18 months of age, if they've gotten vertical, it's a huge benefit to them and their future learning. Also, we know so much-- babies learn so much visually from the facial expressions of their parents, when they're being fed, the bonding experiences from those early interactions that are largely based on vision. If a young child's vision was intact until 16 or 18 months of age, I think they have the benefit of those experiences. And I think it makes a difference. We don't have a set of terms pre something and post something, but I would encourage you to think of those thoughts about the milestones and whether we're going to call a child's vision loss congenital or adventitious. SLIDE 9 So hold those thoughts and we'll look at the second major way we categorize sensory loss. This one is a lot simpler. Partial? Yes. If a child has partial sensory loss and vision, some functional visual skills remain. If a child has partial loss in hearing, some functional auditory skills remain. Total-- the opposite. Essentially no functionality can be gained through that sensory channel. And once again, the degree of sensory loss needs to be considered in regard to both vision and hearing, but that needs to be done individually-- the degree of vision loss, the degree of hearing loss. I think you guys got that. SLIDE 10 Thirdly, and this is where we'll spend our next several minutes, if you're going to do a combination in considering onset and degree or types of loss, it results in these four major categories of deployment. We'll name them, and then we'll take a quick look at each of them. You can figure these out by yourself. I know you could. The first one is that both the vision loss and the hearing loss were present at birth. That's called congenital deafness and blindness. The second one is that both the deafness and the blindness, or the hearing loss and the vision loss, occurred at some point after birth. This is where you have to remember our discussions of pre-lingual/post-lingual deafness and what the child's particular age was and what visual skills the child might have developed. Just because a child might be six weeks old when something happens, it really would probably be best to consider that child's loss as congenital because it's right around the time of birth. Third category, the deafness or the hearing loss is present at birth, and the vision loss comes some time later or is adventitious. The final category, the flip of that one, the blindness or the vision loss is present at birth, and the hearing loss is adventitious or occurs some time later. I can't say that phrase. I apologize. So we're going to look at each of these four categories in this order. SLIDE 11 OK. As we do, even in this dual model, when we're thinking of degree of loss, type of loss, timing of loss, every little guy, every little gal, every teenage guy, every teenage gal with whom you work that has a label of deaf blindness, you really need to look at his or her vision individually. I can give you some guidelines, I can try to give you some questions to ask, maybe some things to expect, to check out, but I can't tell you for sure. SLIDE 12 Because-- here it is again-- remember, learners who have deaf/blindness are an incredibly heterogeneous group. SLIDE 13 Even within each of the four categories we're going to look at, they're still amazingly heterogeneous. Just because you know one learner, one child who might have one of these disabilities-- cytomegalovirus, CMV-- you don't have a good picture of what the next child you encounter with CMV is going to be able to do, is going to need to learn. I have a couple of slides. This is the first of two that oftentimes does result in a vision loss and hearing loss present at birth. Congenital deafness and blindness. Rubella, we thought we had that one kicked, but it's coming back. So please know there's a rubella vaccine. Ladies, get that for your children, for yourselves. Gentlemen, get that for your lady friends, because we can control congenital deafness and blindness through the administration of this rubella vaccine. Cytomegalovirus, or CMV, toxoplasmosis, unidentified birth trauma. Toxins-- these could be some toxin that the mom ingests during pregnancy, particularly during the first trimester of pregnancy. It could be something environmental. It could be a sexually transmitted disease-- herpes, syphilis. SLIDE 14 Other things that cause congenital deafness and blindness-- you might see in a record ROP. You go, I don't know what that is. ROP is used to refer to retinopathy of prematurity. Sometimes in kids' medical records, it simply says prematurity. That can cause congenital deafness and blindness. Sometimes cerebral palsy, sometimes Leber's syndrome, oftentimes CHARGE syndrome-- kids will have vision and hearing loss. So if you see one of these diagnoses in a learner's record, I think it's a heads up. That's how I would treat it. It's a heads up to say, wow, we better check out what this learner, this child, this adolescent, can do, can process in terms of vision, visual input, and in terms of auditory input. SLIDE 15 So let's go to the second category. That was category one-- hearing and vision loss both present at or near the time of birth. Now we're going to talk about the flipside-- adventitious deafness and blindness. I have one slide on this one. This is when the hearing loss and vision loss comes some time after birth. And this type of a label, this type of a descriptor, is most especially appropriate if that child had some language, had achieved that first word in language at 10 to 13 months of age, had achieved some degree of visual memory around 16, 15, 18, I don't know, but somewhere beyond 12 months of age. It can be some kind of trauma, accident, or injury, meningitis-- I'm sure many of you know-- encephalitis. Again, we have the toxins. This would be this time that the child would ingest the toxins or there would be environmental toxins. Maybe you've heard of Usher syndrome, particularly with type three-- there's one, two, and three- type three Usher syndrome. It's adventitious deafness and blindness-- Friedreich's ataxia, neurofibromatosis. Again, I'm not expecting that each of you is comfortable with each of these disability or condition categories. But if you see these, these can be red flags to say, I need to check this out. And more importantly, I need to check out this learner's vision and hearing skills and abilities and needs. SLIDE 16 Now we'll go to the last two categories. And I have one slide for each of these two categories. Congenital deafness, or the hearing loss present at birth, and the blindness or vision loss comes some time later. Shaken baby syndrome. Diabetic retinopathy. Here's that Usher syndrome again. This is type II of Usher syndrome, when the deafness or the hearing loss is pretty severe at birth and the vision loss comes later. Macular degeneration. I put this on here. I'm not sure that it necessarily will apply to many of the learners with whom you work. It usually impacts older adults, older populations. But if you're working with teenagers or kids as they're nearing their 20s, you may indeed encounter some of this. So I did put it on. SLIDE 17 In the final category, congenital blindness, adventitious deafness-- where the child's vision loss is present at birth and the deafness comes some time later. In particular, it comes when the child is post-lingually deaf. Trisomy 13, a chromosomal disorder, some type of trauma, some type of injury-- you all would expect these things. We're always all at risk for illness, injury, meningitis, encephalitis, these infections that can impact how well we hear.