When Eye Exams Miss the Real Problem in EDS with Dr. Eric Singman

[01:01] Dr. Linda Bluestein: Welcome back, Bendy Bodies, to the Bendy Bodies Podcast. I'm your host, Dr. Linda Bluestein, the Hypermobility MD, a Mayo Clinic-trained expert in Ehlers-Danlos syndromes dedicated to helping you navigate joint hypermobility and live your best life. I am so excited today to be speaking with Dr. Eric Singman, neuro-ophthalmologist. As you may know from listening to previous episodes of the show, I have had lots and lots of problems with my eyes, so I'm super excited to be able to talk to someone who is so familiar with EDS, dysautonomia, mast cell activation, and all the conditions that can come along with Ehlers-Danlos syndromes and other connective tissue disorders.
[01:37] Dr. Singman is a neuro-ophthalmologist who lives at the intersection of the eyes, the brain, and symptoms that so many of our listeners struggle to get taken seriously. He's a professor at the University of Maryland School of Medicine and has spent decades working with patients whose vision problems don't show up on standard eye exams. Dr. Singman has held leadership roles at Johns Hopkins Wilmer Eye Institute, advised the Department of Defense and Social Security Administration, and helped shape national guidelines around disability, brain injury, and connective tissue disorders. His work focuses on visual dysfunction after brain injuries and the visual impacts of hypermobile Ehlers-Danlos syndrome, which makes this conversation especially relevant for the Bendy Bodies community.
[02:21] As always, this information is for educational purposes only and is not a substitute for personalized medical advice. I am super excited about this conversation because in my clinical practice, I see so many people with convergence problems, eye pain, visual snow, and so many other eye-related conditions. Stick around until the very end so you don't miss any of our special hypermobility hacks. Here we go.
[03:22] Okay, I am so excited to be here today with Dr. Singman. Thank you so much for joining me today on the Bendy Bodies Podcast.

[03:29] Dr. Eric Singman: Thank you for having me.

[03:31] Dr. Linda Bluestein: Yes, absolutely. I want to start out by talking about what a neuro-ophthalmologist is, because I would imagine a lot of people are not familiar with that term. So can you explain exactly what a neuro-ophthalmologist is?

[03:46] Dr. Eric Singman: After someone becomes an ophthalmologist — after they go to medical school and then do a residency in ophthalmology — there are a number of different post-residency training opportunities called fellowships. This allows an ophthalmologist to subspecialize. Neuro-ophthalmology is that subspecialty. It's a year-long fellowship in which someone studies intensively about the visual pathways, not just of the eye itself, but of the visual pathways of the brain. So a neuro-ophthalmologist really acts as a liaison, if you will, between the neurology community and the ophthalmology community. The brain is about 50% vision one way or another, so it lends itself to this sort of specialty.
[04:36] There are not a lot of neuro-ophthalmologists in the country. There are maybe fewer than 600 or so. Some states don't have one. About 97% of the counties in the United States don't have one. But what we do is we try to help guide patients. I like to speak of neuro-ophthalmology as — and for those people who are older, they might recognize this — the phone operator. When you used to call the phone operator, you don't want to talk to the operator. Very lovely person, I'm sure. But you want to say, hey operator, can you get me here? Or can you get me there? Neuro-ophthalmologists often aid in getting the patient to the right place they're supposed to be by helping guide a diagnosis.
[05:26] A perfect example might be with a neurologic condition that affects the whole body, but often presents with the visual system first. Multiple sclerosis is classic for this. About 40% of patients with multiple sclerosis present for the very first time — their disease shows itself and manifests itself — with the inflammation of the optic nerve called optic neuritis. And so they come to an eye doctor saying, hey, I can't see right. The eye doctor might be a neuro-ophthalmologist, usually isn't. The patient is then sent to a neuro-ophthalmologist. The neuro-ophthalmologist then helps define the optic neuritis, does some testing, recognizes that there may be a diagnosis of multiple sclerosis, and then hooks into the right specialties, usually neurology.
[06:14] Another example might be a patient who comes in with peripheral vision loss. They're sent to a neuro-ophthalmologist. The neuro-ophthalmologist recognizes that this might be from a pituitary tumor, does the appropriate tests, confirms the diagnosis, and sends them on to a neurosurgeon, for example. And there are other examples like this: Lyme disease, rheumatoid arthritis, sarcoid disease, myasthenia gravis. There's a whole list of conditions that neuro-ophthalmologists often are the frontline for, and help the patient get to where they need to go.

[06:51] Dr. Linda Bluestein: Okay. And today we're going to be talking about your specialty, neuro-ophthalmology, as it pertains to EDS, HSD, and comorbidities like dysautonomia and mast cell activation syndrome. How did you get interested in EDS in the first place?

[07:09] Dr. Eric Singman: It found me. When I was working as a neuro-ophthalmologist at Johns Hopkins and had founded the Vision After Brain Injury Clinic for Johns Hopkins Wilmer Eye Institute, there were some patients who just didn't seem to be doing as well. One of them turned out to have Chiari malformation, and on further questioning, they turned out to be hypermobile. Another patient turned out to have Ehlers-Danlos. And so I started asking my patients about that. I started making one of my official questions that I asked my new patients: are you hypermobile?
[07:57] By the time I came over to University of Maryland — I was recruited there after 10 years of being at Hopkins — we had found 50 patients who did not know they were Ehlers-Danlos patients. I did not make that diagnosis. I started to suspect it. I always sent them to geneticists to confirm, and the geneticists confirmed these patients had hypermobile Ehlers-Danlos or some other variant like classic Ehlers-Danlos. And so I just started to want to learn more about this condition. Patients were a wonderful resource helping me learn about it.
[08:41] We ended up surveying the members of the Ehlers-Danlos Society and got back 5,000 responses to our survey about their experience with eye surgery. We also found, sure enough, in a case series, that Ehlers-Danlos patients don't seem to do as well after traumatic brain injury — their symptoms seem to last longer and they don't get as full a recovery. We ended up finding other things too. For example, there was some information in the medical literature that was untrue. Decades ago at Johns Hopkins, there was a family with Ehlers-Danlos who also had another condition, and they had a retinal finding called angioid streaks. I looked at all my Ehlers-Danlos patients — by that time we had hundreds — and none of them had angioid streaks. So we actually published this. And then the Moorfields Clinic in Britain, a very famous world-renowned eye clinic, came with a second paper saying, yeah, we looked also, thanks to you, and we didn't find any either.
[09:54] It turned out that if you look at all the medical literature about what Ehlers-Danlos patients have, there was even a mnemonic made called PEPSI for patients with angioid streaks: Paget's disease of bone, Ehlers-Danlos syndrome, sickle cell disease, and pseudoxanthoma elasticum. It turns out the E in PEPSI didn't belong there, or may not belong there. We ruined the mnemonic, unfortunately. But we learned a lot — at least for the hypermobile type. When we looked back at the literature, all the people who had published that Ehlers-Danlos patients should have angioid streaks had never actually looked at Ehlers-Danlos patients. They just published it as a matter of fact because you have to give references. And when I traced that one reference back to Johns Hopkins, it was a family who also had pseudoxanthoma elasticum. So it was not true. But once something's in the literature, it seems to last.
[11:01] That's how it fell into my lap. Then I started meeting people in the Ehlers-Danlos community who were very prominent, like Clair Francomano and such. We developed relationships, became friends, and I started seeing more and more patients. In a sense, it kind of fell on me. I didn't pursue it.

[11:24] Dr. Linda Bluestein: Yeah, I think that's how it happens for most of us. Your story is quite similar to a lot of people's. So, okay — what kind of symptoms make you think this is not just an eye problem, but a brain-eye connection issue?

[11:40] Dr. Eric Singman: Neuro-ophthalmologists are sent two kinds of patients. One is: I don't know what this is. They're complaining about something either with their vision or related to their eye, and I'm sending it to neuro-ophthalmology — maybe they can figure it out. The other kind of patient is: I know what this is, but I'm a busy cornea specialist who sees 50 patients a day. I don't have time to deal with MRIs, CAT scans, and blood tests. I'm sending them to neuro-ophthalmology.
[12:15] When it comes to real stereotypic neuro-ophthalmology, the patient usually either doesn't see much, and neither does the doctor. The eye doctor looks in the eye and says, Mrs. Jones, your eye looks fine. I can't explain why you're having reduced vision, or accentuated vision, or glare sensitivity, or you're seeing things, or you're having pain in and around your eyes. Sorry, it's not my specialty. Why don't you see this person here and see what happens? That's usually the common story.

[12:55] Dr. Linda Bluestein: And so an EDS patient should see a neuro-ophthalmologist rather than an ophthalmologist or optometrist if they — well, maybe they should see those people first sometimes to rule out some other kind of problem. When should an EDS patient see a neuro-ophthalmologist?

[13:16] Dr. Eric Singman: Almost never. I love them when they come — they're wonderful people — but I feel bad because many of them are sent to me from wonderful, caring providers who said, hey, you have a pair of eyes and you have EDS, you better get your eyes checked. Unfortunately, or fortunately, it's really not like that.
[13:37] The fact about EDS is that depending on the type, there are pretty stereotypic problems patients are going to get, which usually can be handled. For example, if they have brittle cornea syndrome, they need to see a cornea specialist, not a neuro-ophthalmologist. If they have concussion symptoms, that's something I often see. If they have migraines, they usually would be best with a neurologist. I do some migraine work, but complex migraine patients I often send to neurology.
[14:25] I've actually put my own cell phone number on the EDS Society's website, because a lot of patients wanted to come from far away to see me. I tell them, why don't you call me first and see if there's something I can do to try to help? I don't charge for that. I'm on the phone frequently during the week simply because I want to save someone the trouble of coming in.
[15:01] There are times I think I can help. A patient with EDS who develops new onset double vision — that could be a medical emergency. I tell patients: if it's some new, clear neuro-ophthalmic problem — they've lost vision, they have double vision, they've lost peripheral vision — get that checked out immediately locally, as it could be an emergency. Once the dust has settled, if there are questions, we can certainly approach them.
[15:36] Other patients who just want a good eye exam by someone who's EDS literate — what we found in EDS patients is they get pretty much the same eye problems as everyone else. Some they get more often, but ultimately it comes down to a final common pathway. They may get more keratoconus, which is a laxity of the corneal tissues causing astigmatism. They may be more often very nearsighted, and they get problems from being very, very nearsighted. They may have more retinal detachments or retinal tears. Those patients need a retinal doctor. Patients with keratoconus need a cornea doctor. In my experience, for the hundreds of EDS patients who come to see me, the majority didn't have a particular neuro-ophthalmic problem, at least not one that we could help.
[17:08] A classic example is glare sensitivity. A lot of EDS patients notice that glare is very uncomfortable, perhaps more uncomfortable than other people experience it. Unfortunately, we don't know how to measure glare objectively, and we don't know how to treat glare sensitivity. I encourage patients to try whatever they want — though they have to be careful about snake oil. Unfortunately, we don't know how to deal with that.
[17:44] Another example is visual snow. I happen to have visual snow. I was born with it, and it's just something I've lived with — I thought it was normal until I found out it wasn't. I wish we could help patients with visual snow. Now, if someone develops visual snow new onset, that could be a different problem worth looking into. Any kind of new onset change — lost vision, peripheral vision changes, color vision changes, double vision — is something often sent to a neuro-ophthalmologist.
[18:28] EDS patients also seem to have Chiari malformation more often. That can be associated with elevated brain pressure, and we're often sent those patients to look for swollen optic nerves. Most decent eye doctors can determine if an optic nerve is swollen or not, but a lot don't want to be the final arbiter of that, and I understand — sometimes it can be subtle.
What patients want from someone who's EDS literate is someone who includes in their differential diagnosis some of the extra things EDS patients might get more often. A perfect example: an EDS patient I had recently went for chiropractic manipulation. The chiropractor did rapid manipulation of the neck, and the patient developed a vertebral artery dissection. That is a documented concern with rapid neck manipulation, and it may be more common in EDS patients simply because their blood vessels are more fragile.
[19:59] Another patient was having very bad headaches. They were sent to me to check for papilledema — maybe it was elevated brain pressure. When I work with residents, I tell them we're trying to avoid veterinary ophthalmology, so let's talk to the patient. When I talked to this patient — a very intelligent man who people weren't completely listening to — I found out that his headaches were much worse when upright and much better when supine. That's exactly the opposite of elevated brain pressure headaches. That's the kind of headache you get with a CSF leak. It turned out he had a CSF leak, and EDS patients are more prone to spontaneous CSF leaks. He did very well after they patched the leak at one of the specialty centers — I believe it was either Duke or Hopkins. There's another world-renowned specialist in California as well.
[21:30] Sometimes it's a matter of just asking the right questions. And the fact that the referring physician sent him to me because they worried about elevated brain pressure showed they had some background. Some of these can be tricky.

[22:03] Dr. Linda Bluestein: Yeah. I've had my share of eye problems — fortunately not visual snow, so that's good. But I've had very significant dry eye. I have bilateral punctal plugs. I have hypermobile EDS, and I've had plenty of problems like light sensitivity and eye pain. I can feel every little eyelash that gets in my eye. My husband teases me because he never seems to feel anything, and I'm constantly looking in the mirror — and usually I can find something if there's an acute onset of pain in my eye. I've had blepharoconjunctivitis, I've had a corneal ulcer, I've had a lot of different problems with my eyes. I don't know how many of them are related to EDS, or, like you said, they are more traditional eye problems that people with EDS are at higher risk for, and a regular ophthalmologist can treat them. Can an eye doctor who doesn't know a lot about EDS still manage those problems? It seems like a lot of my patients are struggling to get help with their eyes.

[23:23] Dr. Eric Singman: In your particular case, dry eye — that's a huge problem. First of all, it's more common in women, it's more common in women as they approach the perimenopausal period, and it's also more common in patients with mast cell. And EDS patients have more mast cell activation.
[23:42] When I have EDS patients with dry eye, I like to try topical cromolyn sodium for them, because they often have itchy eyes too. Most eye drops are mostly water or some carrier vehicle that lubricates the eye, but they have an active chemical. The active chemical in topical cromolyn sodium is a mast cell degranulation inhibitor. So I like to see if those help.
[24:17] I take a very stepwise approach to dry eye, and I'm very careful about it because my fear is that EDS patients are often very sensitive to medications — whether that's the mast cell or something else — so you have to really tread lightly. You don't want the side effects to be as bad as the disease.

[24:42] Dr. Linda Bluestein: Right.

[24:43] Dr. Eric Singman: So I usually start with preservative-free artificial tears just to see how that goes. I make sure there's good lid hygiene. Then I try cromolyn sodium, et cetera. And then I can step up to things like cyclosporine or Xiidra or these other medications.
[24:59] I like punctal plugs, but I only like to see them put in after the tear film is made healthier, because the tear film in dry eye is pretty much a witch's brew of inflammatory products. If you don't clean up the tear film first, then putting in the punctal plugs to increase tear film volume and prevent drainage into the nasolacrimal system basically just bathes the eye in that inflammatory brew. That's why plugs are important, but they're a little later in the game for me.
[25:44] If an EDS patient has dry eye, I refer them to a dry eye clinic. Most dry eye clinics are pretty good with this, especially ophthalmologists who specialize in dry eye. When I was at Hopkins, Dr. Esen Akpek was a world-renowned dry eye specialist, and she took this approach where she didn't want to make things worse — she was careful about what medicine she might apply.
[26:27] What I'm excited about is seeing whether hormone therapies are going to be helpful for dry eye. We know dry eye is more common in women. We know it's more common in the perimenopausal period. We know there are a tremendous number of estrogen receptors in the lacrimal system. So hormone therapy is going to have to be an area of research, and it is actually an area of research and an exciting one. I think the next breakthrough in dry eye is probably going to come from that sector.

[27:11] Dr. Linda Bluestein: I really appreciate what you just said about dry eye, because I feel like it's one of those things that is probably underappreciated if you don't have it. Eyes are very easy to take for granted until you have problems with them. And dry eye is so common — it's easy to say, oh, well, you have dry eye, it's not a big deal. But it can be a really big deal for a lot of people.

[27:39] Dr. Eric Singman: It is a really big deal, and it's starting to get the recognition it deserves. The research in dry eye has been exponential, and that's reassuring. Some of the new medications have come out that are willing to push the envelope in terms of what therapies they use. In the last 5 years there have been 4 or 5 novel therapies with different mechanisms of action, which is exciting.
[28:15] It's because enough people have said, you know something, you shouldn't have to know your eyes are there. And EDS patients have enough pain. When I do a review of systems on EDS patients, I used to ask, what hurts? And I would have a list of things 40 miles long. I just now ask, what doesn't hurt? It's much quicker to go that way.

[28:38] Dr. Linda Bluestein: It sounds like you really have seen a lot of EDS patients if you've changed the way you ask that question. Yes, that is very accurate for so many people. So true. What about more unusual treatments for dry eye like PRP? Is that something that you've ever prescribed or know anything about?

[28:58] Dr. Eric Singman: I don't. Whether it's for serum tears or PRP or these other novel therapies, I'm a neuro-ophthalmologist — I don't want to step on toes and I don't want to get into an area that's not my specialty. It's my habit and custom to send them to a dry eye clinic that knows what they're doing. A lot of people say they're dry eye clinics, but you have to be careful.

[29:22] Dr. Linda Bluestein: I honestly didn't even know there was such a thing as a dry eye clinic. That's really interesting. And it's blepharoconjunctivitis that I had. Can you expand a little on lid hygiene before we take a break? I feel like some of these basic things can probably help a tremendous number of people.

[29:43] Dr. Eric Singman: Lid hygiene is a twofold process. It's keeping bad things away from your face and eyelids, and it's getting rid of bad things from your face and eyes that might already be there.
[30:00] The basic lid hygiene for someone who has a little bit of debris at their eyelashes — we call it scurf — is when they take their daily shower, they might take some baby shampoo on a washcloth, close their eyes, and just scrub that gunk away from their eyelids. That gunk is food. It's food for Demodex mites, which live in many people's eyelids. And if you ever see one under a high-power microscope, they are frightening as all get out.

[30:33] Dr. Linda Bluestein: I saw one for the first time yesterday and I freaked out.

[30:36] Dr. Eric Singman: It's horrible.

[30:36] Dr. Linda Bluestein: It's horrible.

[30:43] Dr. Eric Singman: When I did parasitology in medical school, I lost about 9 pounds because I was refusing to eat or drink anything. So, that's just basic cleaning of the area. Obviously we love patients to stay away from things like cigarette smoke, smoke from any cause, fires, chemicals. If they work with chemicals — chemical vapors — all of these things are just not going to be good for your eye and your conjunctiva. So avoidance of that is valuable.
[31:20] Many people have service jobs where they do a lot of reading on computers. We know that lowers the blink rate, and the blink rate is what keeps your eye moisturized and clean. I have a feeling that some of the reason we're seeing a lot more dry eye and blepharitis is simply because people are having indoor desk jobs rather than outdoor jobs.
[31:47] Another thing is that certain illnesses are going to make the tear film less healthy. Rosacea is the classic one — that's when someone looks like they've been drinking even though they haven't, because they have a red nose and red cheeks. Certain medications — many medications, actually, including anticholinergics — cause dry eye. The list of medications that cause dry eye is humongous, and a lot of EDS folks are on medications of many types.
[32:30] Tea tree oil wipes have been popularized and have helped a lot of people — they kill Demodex, for example. It's amazing when people use those and kill the Demodex, how many patients with rosacea seem to improve, suggesting that maybe the Demodex was helping stimulate the rosacea. Tea tree wipes have been shown to be valuable.
[33:00] Keeping the eyes moist with artificial tears is important. If someone has mast cell activation, that's going to cause an inflammatory response in the tear film as well. If they have bad mast cell activation systemically, oral gastrochrome may help. If it's just topical, nasal cromolyn sodium or topical cromolyn sodium in their eyes.
[33:27] Some things we can't change. If a woman is going through menopause and has fluctuations in her hormones, that can be much more difficult to address. If she's put on hormone replacement therapy, there have been studies suggesting that can be valuable for more than just hot flashes — it might be valuable for protecting from bone loss, for example, and for dry eye.
There are also some patients who take hormones for other reasons — for example, those who want to transition, which often requires a hormonal intervention. That intervention, particularly when it's testosterone, has been associated with side effects. Besides neurologic problems like elevated brain pressure, it can also cause dry eye and other problems.
[34:46] Obviously, if someone uses makeup, certain makeups might be allergenic — especially makeups that come very close to the eyelid margin. Some might actually be protective. If you look at pictures from thousands of years in Egypt, people used something called kohl — K-O-H-L. It has antiseptic properties.

[35:22] Dr. Linda Bluestein: Mm-hmm.

[35:23] Dr. Eric Singman: So there may be things that are good to put on the eyes to protect them. But unfortunately, a lot of things put near or at the eye can be pretty bad for the eyes.

[35:35] Dr. Linda Bluestein: So there are some people who can get away with not doing these lid hygiene things because they're not prone to having Demodex, but there are other people who have to do these things because they have dry eye and they're prone to these problems. Is that correct?

[35:50] Dr. Eric Singman: Right. And a lot of people — probably the majority — have Demodex, and many of them are asymptomatic. But for those who have stubborn blepharoconjunctivitis or blepharitis, it certainly is reasonable to consider approaching that issue.

[36:14] Dr. Linda Bluestein: Great. We are going to take a quick break. And when we come back, we are going to talk about problems with double vision, convergence, and other things, and get more into the neuro-ophthalmology side of things that affect people with EDS. We will take a quick break and we'll be right back.
[38:25] Okay, we are back with Dr. Singman, talking about neuro-ophthalmology. We were just talking about some common eye problems in EDS that are not necessarily neuro-ophthalmology related, but I appreciate you letting me pick your brain about some of these things that I think so many people experience. They're often treated like they're not that big of a deal, but they can really impact your day-to-day life. So thank you so much for all those tips.
[38:56] Can you explain about things like double vision, convergence, and eye alignment? Are some of these things more common in people with EDS? You've already mentioned Chiari malformation. Are these things more common in people with connective tissue disorders? And if so, what are some of the reasons besides Chiari malformation? Of course, we should explain that Chiari malformation is when the foramen magnum — the bottom of the skull — the brain is kind of sagging through that hole, the brainstem is affected, and the visual centers at the back of the brain are affected. So can you explain to us why these problems might be more common in people with EDS, if they are?

[39:36] Dr. Eric Singman: As far as Chiari per se, my gut feeling is that because all the tissues are lax, it just seems more likely for things to settle because of gravity. I'm not sure we know 100% why Chiari is more common in EDS patients. We do know that when someone does have Chiari malformation, they're more prone to having things like convergence spasm, where their eyes can spasmodically cross. They're also more prone to having nystagmus, where their eyes jiggle, and when that happens, the world can jiggle — that's called oscillopsia.
[40:12] Other problems with EDS patients — and there may be a chicken-and-egg issue here — but EDS patients tend to have a tremendous amount of fatigue. When I go through my review of systems for an EDS patient, I ask a couple of questions, and I can't think of the last time I haven't gotten a yes. I ask them: do you have brain fog or some sort of slowing, do you feel cognitively you've been affected? Always, invariably, yes — I feel like I'm just not as sharp, especially after a trauma or something like that. I also ask about fatigue, and the answer invariably is yes.
[40:51] There are a couple of reasons for fatigue. When you hurt everywhere, it's hard to fall asleep. If you don't get good sleep, you're not going to have good energy. Also, EDS hurts — a lot — and that takes up a lot of bandwidth. My gut feeling is that if EDS patients could magically feel no more pain whatsoever, especially in the neck — and it's going to sound funny, I say this to my patients and they laugh at me — but the neck is the Achilles heel of EDS patients. If they could feel no pain, imagine what they could do instead of having their brain constantly interrupted by, hey, it hurts. Pain takes up bandwidth, and that's exhausting because they still have to go through their regular day.
[42:06] Convergence — bringing the eyes together for near work — takes a lot of work to initiate and maintain. More and more people have employment where they have to look at things closer than 20 feet. If you look at anything closer than 20 feet, your eyes have to cross some — that's called convergence. If they're insufficient at that, they have convergence insufficiency.
[42:42] I'm sent a bunch of patients where someone says, Eric, they have convergence insufficiency and that's why they can't read. Many of these patients have gone to people who say they do vision therapy and have spent a lot of money and a lot of time on that. I had 3 patients with EDS come to my clinic in Maryland yesterday, all new patients, and every one of them had the same complaint — they were having trouble reading and they were told it was their eyes. Now, it might very well be their eyes, but when I explain, I say reading is like a chain. It's a tool. Its job is to connect you with knowledge.
[43:23] Imagine a chain made of links connected to a big box called knowledge, and you want that box. So you pull that chain toward you. Supposing that chain has a broken link — in a dark room, you can't tell what it is. You pull that chain until you come to the first broken link. That first broken link might be that you need glasses for reading, or maybe you can't converge well. You fix that link. And then you keep pulling and you may find a secondary broken link — maybe cognitive issues that prevent you from having the executive function to remember what you're reading. Because reading is a chain made of different steps, you have to fix the first broken link you find until you get to the next.
[44:06] The reason I give that analogy is that many patients come to me being told a broken link has been identified, when in fact it was identified incorrectly. A patient came to me and said, I want you to give me exercises so I can converge better so I can read again. I said, well, why do you think you can't read? My physical therapist told me I can't read because I have this. And when they showed me how they measured it, they were having the patient look at fingers coming toward the face. That's not how you measure convergence — that's how you measure accommodation and convergence at the same time. Accommodation is focusing your lens. If you measure two things at once, you're measuring nothing.
[44:57] So I said, why don't we do a little trick? I put a patch on their eye — or covered one lens of their glasses. I made sure I covered the non-dominant eye, which I check for first. I said, please read for me. They were reading and said, this is terrible, I can't read like this. I said, well, is this how you feel when you try to read? They said yes — see, I have convergence deficiency. I said, convergence insufficiency is when your brain can't make your two eyes work together. I covered one eye. I relieved your brain of the need to work together. So how come you're still having a problem? Then they look at me and they get it.
[45:47] That's a simple test anyone could do in their office before sending someone for convergence problems. I had patients who'd had weeks and weeks of vision therapy, charging hundreds and hundreds of dollars to make their eyes work as a team — and yet when you cover one eye, things were just as bad. If you prevent eye teaming and reading is just as bad, it's not an eye teaming issue.
[46:24] Now, do patients with EDS get more convergence insufficiency? I find they do, probably because they're so fatigued. And if I can't fix the fatigue, I probably can't fix the convergence insufficiency. What I tell those folks is to read in smaller bits — frequent small bits rather than marathon sessions, little sprints rather than long marathons. That's sometimes the best we can do. If they really have bad convergence deficiency, of course I'll provide exercises, but I'll also sometimes say, look, there's nothing wrong as a stopgap with covering your non-dominant eye so you can get through the day. It's not going to give you a lazy eye or anything like that. That doesn't happen if you're past 6 or 7 years of age.
[47:12] I still want to help the patient find out why they can't read. I feel a responsibility, even if I show it's not a convergence problem, to still help them figure out what's going on. Oftentimes it might be a problem with abnormal pursuits — we can see that with Chiari as well. A lot of the time, it's an executive or cognitive problem where the EDS is simply not letting them do their job. The other problem I get is ergonomics.
[47:47] When all of us were taught to read as children, we were taught to read on those good cardboard books — you know, the Golden Books. And we would sit down and hold the book in our lap. That's horrible for EDS patients because it puts a strain on the neck. The last thing you ever want to do with an EDS patient is do anything mean to the neck. So the first thing I ask my patients when they're having trouble reading is, please tell me about the ergonomics of your reading situation. If they have a computer and it's not at eye level, I say, please put it at eye level. And oftentimes they already have it at eye level because EDS patients are pretty sharp. They know often more about their illness than many doctors do. But for those who might not have explored that, you get a really big bang for the buck.
[48:50] Sometimes we can't find why the reading is a problem. But at the very least, as a physician, I take a Hippocratic oath — do no harm. And that includes do no harm to the pocketbook. So at least the EDS patients who come in saying my doctor wants me to have 18 more weeks of vision therapy for my convergence — I can tell them, you could save the money and give a little to charity instead. Because how could it be helping if reading with one eye is just as bad as with two eyes open?
[49:27] Now, frank double vision — we can see that with EDS too, especially when they have elevated brain pressure. Elevated brain pressure not only can cause papilledema, it can cause a cranial neuropathy of any cranial nerve. I had an EDS patient — someone who didn't even know they had EDS — who came in with new onset papilledema with intracranial hypertension and a Bell's palsy. A workup had already been done for Lyme disease and herpetic disease, but no one had looked at the back of her eye. They sent her to me partly because of a tool I have as an ophthalmologist — external lid weights I can prescribe. They're made by a company called MedDev, M-E-D hyphen D-E-V. They're called Blinkies, B-L-I-N-K hyphen E-C-E. They're external lid weights that go on with double-stick tape and help the eye close for a person who can't close their eye. They're simple, not expensive. I prescribe them all the time.
[50:35] In the meantime, I did a full exam and found she had swollen optic nerves — papilledema — and it turned out she had elevated brain pressure, intracranial hypertension, presenting with the Bell's palsy. We lowered the brain pressure successfully with medication.
Then I started asking this patient some questions. The reason was her age said she was in her forties, but she looked like she was in her twenties. It's become a habit of mine when I see a patient who looks much younger than their stated age to ask them if they're hypermobile, because it seems very common with Ehlers-Danlos patients that they simply look younger than they are. She appreciated the compliment and thought I was just being nice. I did a Beighton score on her right in the clinic. She could bring her thumb to her wrist, her pinky so far back — she had actually been a gymnast, she was so flexible. Many of these patients say, yes, I was a gymnast, a cheerleader, in martial arts, a dancer, something like that.
[52:02] I started asking more questions and it turned out she had a history of uterine prolapse, which you can see with Ehlers-Danlos, and even a rectal prolapse, a history of hernias. She just thought she was unlucky. She said, yeah, my mom always had that too. So I spoke to her about Ehlers-Danlos, and sure enough, ultimately we diagnosed her.
Ehlers-Danlos folks have a funny way of having elevated brain pressure. They can sometimes blow a hole, going from elevated pressure to a spinal leak and back — high pressure, low pressure, high pressure, low pressure. I was sent a patient at the rehab hospital I worked at who had developed a spontaneous CSF leak from their nose. The doctor said, check for papilledema because I want to know if she's one of those cycling patients. The patient did not have papilledema, but she did have a spontaneous CSF leak. I asked about Ehlers-Danlos. The patient had never heard of the condition, but sure enough, she was hypermobile.
[53:29] I contacted the ear, nose, and throat doctor who was going to do the surgery. I said, this patient is hypermobile. I think we should check to see if she has Ehlers-Danlos. He said, well, why would that be important? I'm going to do the surgery regardless. I said it's important for a couple of reasons. In my experience, they tend to be more sensitive to medications, including anesthetics. They also tend to heal less well, more slowly. At the very least, if they do have Ehlers-Danlos, you want to give them reasonable informed consent — and you're actually protecting yourself by recognizing it. He agreed, we had the patient checked, and she did end up having Ehlers-Danlos syndrome.
[54:46] The one thing I've learned from surveying thousands of patients who had eye surgery is that Ehlers-Danlos patients seem to have the same complications as anyone else — not unusual or unique complications. They just seem to have them maybe more often. That's why it's important to be extra careful in listing all the possible complications to an Ehlers-Danlos patient, both to protect them and yourself as a physician.

[55:18] Dr. Linda Bluestein: I wrote down a whole bunch of notes as you were talking. I want to follow up on papilledema. Is that something that if there's high pressure in the brain, you will see right away, or is that a late sign? Can you have high pressure in the brain without papilledema?

[55:36] Dr. Eric Singman: Yes, and you can have it chronically. That's called pseudotumor cerebri sine — S-I-N-E — papilledema. It's Latin for "without papilledema." In fact, I had a whole series of patients when I was in private practice in Lancaster, Pennsylvania, with pseudotumor cerebri without papilledema. I had to work hard to make people believe this was even possible.
[56:03] Eventually I got validated when I sent a bunch of patients down to see Mike Williams, who at the time was a prominent neurologist specializing in cerebrospinal fluid hydrodynamics at Johns Hopkins, running their CSF clinic. He published a paper of 10 patients who had pseudotumor cerebri without papilledema, confirmed using an indwelling lumbar catheter — like a lumbar puncture, but it stays in place. Of the 10 patients, 4 were mine. He actually sent me a handwritten copy of the article. Very pleasant person. But the bottom line is: you can have pseudotumor cerebri without papilledema.
[56:55] Now, if papilledema is going to happen, it can happen quickly — certainly over weeks, if not days. The funny thing about papilledema is that a lot of it can also go away within weeks, perhaps days, but certainly weeks. In some patients it can take months to totally resolve.
One of the challenges is if I see a patient I've never met before and they have swollen optic nerves, I don't know what their normal baseline looks like. Once the nerves come down, I go through a number of different evaluations to confirm that we're at their baseline. I get a sonogram of the nerves. I might get a visual evoked potential to see if the nerves are unhealthy. I might get a fluorescein angiogram of the nerve — we inject a dye in the hand, it's a safe vegetable dye that glows green, and we take a picture of the back of the eye to see if the nerve leaks. I go through a lot before I tell a patient their papilledema is gone. And then I see them with serial OCT — pictures of the nerve — and visual fields. Once I'm comfortable that things have been stable for maybe a year, then I can tell them they can go back to their regular doctor.
There are also things that can make something look like papilledema that aren't, such as optic nerve drusen — little concretions of the nerve that can make a nerve look swollen when the nerve is actually fine.

[59:06] Dr. Linda Bluestein: Wow. First of all, I want to say that is incredibly generous that you have your phone number on the website. That's amazing. I'm so glad that you clarified about papilledema, because I know so many people who have signs and symptoms consistent with elevated intracranial pressure, but they go to an ophthalmologist and because there's no papilledema, they're told, you can't have elevated intracranial pressure. You just clarified that that is not necessarily the case. Yeah, it is sad.

[59:35] Dr. Eric Singman: And just as an aside, it's important to recognize that even if they don't have elevated brain pressure on a spinal tap, they could still have elevated brain pressure. Every patient's different. Dr. Williams showed that there are things called B waves, and CSF pressure fluctuates widely throughout the day. So let's say a patient gets a normal number on a spinal tap — that doesn't rule it out. If a patient has bad headaches, you do a spinal tap, and it relieves their headaches, and then the headaches slowly come back as the CSF pressure builds back up because you likely drained off some CSF — they have elevated brain pressure. I don't care what the number is. Nobody volunteers for a spinal tap unless it makes them feel better.

[1:00:29] Dr. Linda Bluestein: Yeah. That's super interesting. I also wanted to make a quick side note: yes, people with EDS are definitely more sensitive to medications — totally agree. There's also this phenomenon with hypermobile EDS and HSD of local anesthetic resistance. They tend to be less responsive to local anesthetics — they may need more, they may need a different local anesthetic like mepivacaine or articaine, they might need more frequent dosing or a longer onset time. I just want to put that out there.
[1:01:05] And then the other question I had was about spending so much time looking at a computer. I've definitely noticed that at the end of the day it can cause your eyes to hurt and your head to hurt. Is it a good idea to step away from the computer and look off in the distance — and not just go look at a phone, because that's not going to help — but are there certain habits we should try to get into?

[1:01:38] Dr. Eric Singman: There's something called the 20-20-20 rule: every 20 minutes, you take a 20-second break and look at least 20 feet away. I think that's a cute rule, and for most regular people it's probably okay. For my EDS patients, I tell them, look, you've got to get some fresh air and sunshine. I tell all my patients that, frankly.
[1:02:07] I tell EDS patients that for two reasons. One is that EDS patients, especially younger patients, probably have a higher risk of becoming high myopes. There's pretty good data to suggest that you need fresh air and sunshine to slow down myopic progression, and that looking at screens all day is going to make things worse. Being in front of screens all day is not good for you. We're forced to do it — our service industry is like that nowadays. I mean, I type all my own notes, and I might spend 3 hours going over my residents' notes in an evening.

[1:02:56] Dr. Linda Bluestein: Yeah.

[1:03:01] Dr. Eric Singman: Using screens for work — if you have to, you have to. But using them for entertainment, I would urge people to avoid that. Or if they have to use them for entertainment, do something where the screen is much further away than your hand or your desktop.

[1:03:17] Dr. Linda Bluestein: Your point about getting outside is so important. It's so easy nowadays, especially for those of us who work from home, to become — I was literally just talking to somebody about this — it gets harder and harder to actually get out of the house. I'm so glad you mentioned that, because it can be psychologically beneficial as well, and then we're looking off into the distance. Thank you for mentioning that.
[1:03:50] I also wanted to ask — and I'm cognizant of the time, we're going to have to wrap up before too long, which may mean a part 2 — you mentioned charlatans and people being talked into 18 sessions of vision therapy or more. How can people best reduce their chances of being taken advantage of that way? EDS has become like a gold rush, and there are lots of people putting it on their website and claiming they have vision therapy for EDS patients. How do people make sure they're not missing out on something that could genuinely help them, but at the same time they're not wasting their money?

[1:04:40] Dr. Eric Singman: First thing is, if somebody's offering some sort of therapeutic benefit, I would tell a patient — because my EDS patients are invariably very bright and very aware — go onto pubmed.gov and look at the research in that area. Find a Cochrane review or some review of research to see whether the therapy being offered has a sufficiently large scientific background to make it legitimate.
[1:05:18] The other thing is, patients have to be willing to ask other providers for their opinion, not in the same field. If someone says, I'm going to do all this wellness stuff for you, that's fine. But if they say, I'm the only one who can do it, you have to buy your stuff from me, and it's expensive — you have to wonder. I'm always concerned when the person who tells me I need something is the person who's going to get financial benefit from that need. It's the same as when I go to a mechanic — I know nothing about cars, and I'm very hesitant. Or when I go to a dentist: you tell me I need this, but what's going to happen if I don't do that?
[1:06:23] The second question to ask is: what's going to happen if you don't take the therapy being provided? Is it going to kill you? Is it going to make your life miserable? Are you going to get worse?
[1:06:36] Sometimes it's a matter of trust. If the only provider for a particular service is in your small town, that can be hard. But there are other references. One of the things I admire about the EDS community is that they're really excellent at social networking and social media. If you throw a question out there, you're bound to get pro and con opinions. You can ask those people what they think and why, and get a more balanced set of perspectives from people who've been there. That could be really handy.

[1:07:28] Dr. Linda Bluestein: Because there are a lot of people now who are after the pocketbook of people with EDS. A lot of people with EDS are on disability or have really limited funds, and yet they can be talked into doing things that may not be beneficial.

[1:07:46] Dr. Eric Singman: A perfect example — I apologize, but — I buy my glasses online and I'm a -3 myope, somewhat nearsighted. I buy single vision glasses without any fancy coatings or anything, and they cost me $9. And patients come to me saying, I spent $300 for my glasses. I say, why? Well, the doctor prescribed them and I could buy them right there in his shop. And they had a special tint, a special coating, microprisms that do all these different things. And I'm saying to myself, I wish you would've talked to me first.
[1:08:44] My practice has an optical shop — I'm not going to lie. If a patient says, can I have my pupillary distance, which is the one measurement you need to order glasses online, I measure it and give it to them. I don't steer them away from the optical shop. If they saw a pair of frames they love, go enjoy. But if they say, doc, where did you get your glasses? I tell them where I got them — one of the online sites. And if they ask how, I tell them you need your prescription and your pupillary distance, and I'll measure the latter and give it to them. Do no harm — that includes the pocketbook.
[1:09:31] I don't think opticians are charlatans, but glasses are marked up so enormously by thousands of percentage points that it can make someone uncomfortable.

[1:09:45] Dr. Linda Bluestein: Yeah. I got my glasses at Costco recently. Not online, but I'm sure they were a fraction of what I would've paid otherwise, and I'm very happy with them.

[1:09:56] Dr. Eric Singman: I'm a Costco member myself.

[1:09:58] Dr. Linda Bluestein: Oh, are you? I love Costco. They did a feature — May of 2024, I believe — about EDS. I was interviewed, and so were some other people.

[1:10:10] Dr. Eric Singman: They got you in their magazine. I saw it.

[1:10:13] Dr. Linda Bluestein: You saw it? Amazing. Okay, so before we wrap up, I would love to know if there are other things we should be aware of when it comes to conditions like POTS — postural orthostatic tachycardia syndrome — as they pertain to vision. And if there's anything related to mast cell activation syndrome, cervical instability, inflammation — I know this is a massive question, but if there's anything you feel we should mention. And like I said, we're probably going to have to come back and do a second part if you're willing. But if you have some parting thoughts before we wrap up.

[1:10:14] Dr. Eric Singman: With POTS, the big thing I deal with is that in many things with EDS, the visual system is a bystander — an innocent bystander that gets affected. It's collateral damage. POTS doesn't specifically damage the visual system, but when someone has a sudden drop in blood pressure to their brain, one of the things they're going to notice is less blood flow to their occipital cortex, and they may notice that the world gets dark and kind of cones in. When they recover, they may notice visual symptoms and phenomena. That's nothing I can do about directly. A patient came to me who didn't know they had POTS and described those symptoms. I said, I'm wondering if you have POTS. We sent them for evaluation and it turned out they did. They knew they had EDS, but POTS wasn't on their radar. It was their visual changes that gave us the hint.
[1:12:17] Most of the other things too — the mast cell, for example — the collateral damage might be dry eye. That's something we try to help with.
For CCI — the instability — I almost feel like my hands are tied if an EDS patient's neck is in bad shape. Because when an EDS patient's neck gets better, I've seen it time and time again that everything else seems to fall into place. It's almost magical.
[1:13:02] I had an EDS patient, and I suggested, just for the heck of it, try wearing a brace. Just see what happens. They wore the brace and loved it. I said, okay, just understand the brace is not the cure — it's a temporizing measure. But the fact that the brace helped suggested they could experience that kind of benefit from more permanent interventions. And that reminded me: when the neck is improved, not even cured, so many other things with EDS seem to fall into place. The reading improved, the headaches improved, the brain fog improved. The POTS might even improve. It's just remarkable.
[1:14:00] That's why I say the neck is the Achilles heel of EDS patients. When they come to me after traumatic brain injury or whatever they come to me for, and they say, doc, I feel like crap, and my vision is like this, and my reading is terrible — many times the best I can do is hold their hand and say, what have we done so far to try to get the neck better? Because by getting the neck better, without me, they're going to have more success. They need the neck doctor to make them feel better. Unfortunately, those can be hard to find.

[1:14:48] Dr. Linda Bluestein: It's so great that you have such vast knowledge about EDS and the comorbidities. Are you willing to do a second part so we can address some of the questions we didn't get to?

[1:15:03] Dr. Eric Singman: Sure, I think we could find time for that. Although I have a feeling we covered a lot. If there are particular questions you have, or a series of them, I'd be happy to address them. If you want to post them on your website, that would make it easier too.

[1:15:20] Dr. Linda Bluestein: Sure, we could do something like that. The reason I wanted to ask now is because if someone's listening and they think, oh my gosh, I wish she had asked X — it could have been something on my list. So if you're listening right now and you have a pressing question, you can send it to me at bendybodiesspodcast.com. Dr. Singman and I will figure out some way to get more questions answered. This has been such a great conversation. Do you have a hypermobility hack for us?

[1:15:52] Dr. Eric Singman: Don't hit your head, please.

[1:15:55] Dr. Linda Bluestein: Yeah, we didn't really talk about concussion.

[1:15:58] Dr. Eric Singman: No contact sports, please. Swim. Anything else — be careful.

[1:16:06] Dr. Linda Bluestein: Sounds good. Where can people learn more about you?

[1:16:09] Dr. Eric Singman: I have a lot of articles published on PubMed, including some about EDS, so that's a good place to start.

[1:16:18] Dr. Linda Bluestein: Wonderful. And you're obviously on the EDS Society website, and we'll include that link. I'm so grateful to you for taking the time to chat with me today. I know that you're extremely busy, and I really appreciate you sharing so much knowledge and wisdom with me and with the Bendy Bodies listeners.

[1:16:37] Dr. Eric Singman: Thank you.

[1:17:33] Dr. Linda Bluestein: Well, I really enjoyed that conversation with Dr. Singman, and I hope that you did as well. The field of neuro-ophthalmology is so interesting. He also generously shared general ophthalmology information for people with EDS, POTS, and mast cell activation syndrome. Thank you so much for listening to this week's episode of the Bendy Bodies Podcast with the Hypermobility MD.
[1:17:58] I have lots of other resources, including my newsletter, the Bendy Bulletin. You can check that out on Substack at hypermobilitymd.substack.com. You can help us spread the word about connective tissue disorders by leaving a review and sharing the podcast — this really helps raise awareness about these complex and multi-dimensional conditions.
[1:18:18] Did you know that I also offer one-on-one support for both clients and healthcare professionals? Whether you're living with a connective tissue disorder or caring for someone who is, I've got your back. Check out my coaching and mentorship options on the services page at hypermobilitymd.com.
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[1:18:50] As you know, we love bringing on guests with unique perspectives to share. However, these unscripted discussions do not reflect the views or opinions held by me or the Bendy Bodies team. Although we may share healthcare perspectives on the podcast, no statements shared on Bendy Bodies should be considered medical advice. Please always consult a qualified healthcare provider for your own care.
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