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These answers relate to hearing with regard to Superficial Siderosis and its associated symptoms. If you're looking for something specific, please use your internet browser's page search facility (usually in the top right hand corner of your screen) to search particular keywords or alternatively, browse down the page to learn more about how Superficial Siderosis can affect everyday life and what can be done to help. If you'd like to broaden your search, you may find what you're looking for on another part of the Silent Bleed site - please use the search box in the header above.

Part of our Q & A pages with Dr Levy


We're really grateful that the expert based at Massachusetts General has been working with us since 2015, and we've built up quite an archive over the past few years. With a little sifting you should find the answer you're looking for. However, we know we haven't covered everything, so if there's something else you need to know, please contact us - we'd be happy to forward your question. It's part of what we do.





Blood brain barrier questionÂ There are current studies being done on using ultrasound waves to get meds past the BBB to treat other diseases such as ALS, MS, etc. Is this new technology a future possibility for SS sufferers? Could Ferriprox possibly ever be injected straight into the brain, to more effectively target the bad iron in the brain as opposed to the good iron in the blood and body?


>> Ferriprox gets into the brain very readily. Within a few minutes of ingestion, Ferriprox is already detectable in the brain. Access to the brain is not an issue. (Injecting directly into the brain won’t help.)


Ferriprox is a very simple molecule that just binds iron. When iron binds ferritin and forms hemosiderosis in the spinal fluid, the product is insoluble and remains trapped outside of the circulation. When Ferriprox binds iron, it remains soluble and returns the iron to your circulation.


You can’t direct Ferriprox to bind bad iron only. That is a worthy goal for us researchers!!


Does hearing loss caused by SS follow the same course as age-related hearing loss? And, do hearing test results look the same? I have high frequency hearing loss in both ears (left more than right) but the ENT doctor dismissed my SS diagnosis and said the hearing test was classic for age-related (I'm 55). Is there a way to test for what type it is specifically? Or any way to tell the cause specifically?



High frequency hearing loss is the pattern of hearing loss for both age related hearing loss and superficial siderosis. They look the same to an audiologist. But age related hearing loss at 55 years is a little young unless you have a family history of hearing loss. If you balance problems too, then the most likely explanation is that the superficial siderosis is causing damage.

I live in PA one hour north of Baltimore and will be 63 in May. In Nov I went to get my hearing checked. It was abnormal so they sent me for an MRI. It came back with extensive SS.

I've now had CT and MRis of my spine, at the bottom of my spine the fluid was dark. My neurosurgeon wants to do a angiography carotid cerebral bilateral and lumber puncture. As of now he is the only Dr I am seeing. At my age am I a candidate for the medicine? How or where do I go next for help with SS? Is there an instruction book on what to do when one is diagnosed with SS?


>> You are doing the right thing by connecting with the Silent Bleed and learning about superficial siderosis. It sounds like your doctor is interested in your case and has a plan for you. If he would like, he can reach out to me for advice (email:

SS Headaches


I have been diagnosed with SS going on 3 years, lately I have been getting these head aches, i do physical therapy and exercise on a regular basis, but I still continue to get light headed along with the head aches , should I do anything different? I am 56 years old and i will not give up from fighting this rare disease I am open to suggestions?


>>>>> You might be exacerbating your bleeding by exercising. Most people with SS have bleeding coming from the spinal column, usually from the lining between the spinal

 cord and the vertebral bones. The lining may be prone to bleed when doing certain activities. If you find that your headaches and lightheadedness occur within 24 hours of a certain activity, don’t do that activity anymore. Alternatively, try to isolate where the bleeding is occurring and investigate possible fixes (blood patch, gluing, surgery).


Life expectancy



Does superficial siderosis shorten life expectancy?


>>> No, not that we know of. 




Q: who is the most suitable doctor to diagnose an active CSF bleed/leak and confirm/rule out SS?

For the last 10 years i have been experiencing many debilitating neurological symptoms and some diagnoses such as Craniocervical instability, occult tethered cord syndrome and propable arachnoiditis. I would like to ask who is the most suitable doctor to diagnose an active CSF bleed/leak and confirm/rule out SS. A neuroradiologist or a neurosurgeon.

A neurosurgeon is a good doctor for fixing surgical problems in the spinal column. A neuroradiologist is a good doctor for 1. Identifying leaks based on MRI and myelograms and 2. Injecting glue or blood patches to correct problems that are non-surgical. A neurologist is the doctor who would help coordinate the care, make the diagnosis and initiate chelation therapy.

can SS symptoms be transient/periodic?


I have an AVM - Grade 4, inoperable. It bled repeatedly twenty years ago (9 bleeds), but seemed to settle down after Proton Beam Radiation (which did not work, but strengthened the vessels enough that I was relatively well for twenty years). This past year, I've been dealing with increasing dizziness, tingly and painful feet and behaviour changes...felt as though I've been going insane. My first question (I'm sure there will be SS symptoms be transient/periodic? I have had a few episodes of severe dizziness that lasts three to four days, and then it'll back off to mild feelings of being off balanced or lightheaded, and then I'll have days where I feel well and steady. Same with my feet, some days good, some bad, some in-between. Can the symptoms come and go like that? Also...why would it only show up on MRI after twenty years? Or is it that, in all of the 20 years I've been getting scanned, this was the only time it was seen by someone who knew to look for it?

Superficial siderosis is a progressive condition. It progresses very slowly and while it causes damage to the brain tissue, your brain will try to compensate. It’s the compensation ability that fluctuates, not the siderosis. Compensation ability is bolstered by good sleep, regular exercise, physical therapy, young age. Compensation ability is hampered by heat, stress, poor sleep, illness and older age.

Superficial siderosis worsens radiologically over time. Depending on how much iron/blood there is, regular MRIs may miss it. Special MRI scans called T2*(star), gradient echo imaging, or susceptibility weighted imaging make the iron/blood much more visible and obvious. These scans are being done more routinely these days.

Q: Uni-lateral Hearing loss - only symptom.


My husband has recently been diagnosed with superficial siderosis via MRI as they were investigating the source of his unilateral hearing loss. He has no other symptoms that he is aware of. He did have a motor vehicle accident causing nerve root avulsion C6-T2, 19 years ago and as a result of that he has a paralysed arm and chronic pain. He's seen a neurosurgeon who said the signs on MRI are from an old bleed from his accident and said that some people do not have progressive SS symptoms. So he has been discharged essentially - and we feel we are just waiting for the onset of balance problems or something similar that may get some attention. Is there anyone else out there with a diagnosis and only hearing loss? Is it possible that he may only have hearing loss? We expect not so your honesty is appreciated. Thanks

A: There are two separate issues here: ongoing bleeding and progressive SS symptoms. 

Regarding the question of one-time bleed vs. ongoing bleed, if there is convincing evidence by MRI that the siderosis extent and pattern has not changed over the past 19 years, then I would support the idea that the bleeding occurred at the time of the accident, and not since. If no MRI was done before the recent one, it can be hard to ascertain. There are two ways to try. One way is to perform a lumbar puncture. If blood is found in the spinal fluid, that is evidence of ongoing bleeding. The second way is to watch and wait. If the MRI gets worse over time, the bleeding is ongoing – but this could take years to see.

If the bleeding is ongoing, it may be worth letting your neurosurgeon explore the area of injury and try to find and stop the bleeding. 

The other issue is progressive symptoms, which is unrelated to ongoing bleeding. There are a few people who were discovered to have siderosis by MRI and have no symptoms. These are non-progressors. We don’t know why they don’t have symptoms but it’s fairly rare. Most people have hearing loss at least. These non-progressor may have ongoing bleeding, but for some reason they don’t have symptoms. It could just be a matter of time, of course. Some people have very little iron deposits and a lot of symptoms, while others have a lot of iron and a little symptoms. We don’t understand the susceptibility of patients to superficial siderosis. 

If he progresses, the expectation is that he will continue to decline at the same rate he has been up to now – slowly.

Q: Recently diagnosed SS sufferer. Very interested to read all of the emails and especially to watch your broadcast. I was diagnosed last month having referred myself to a local Private Neurologist. For many years I have suffered with back pain, balance issues and headaches. Sadly this was treated by my GP surgery as being due to me being overweight!!! Having undergone many of the normal balance tests etc I was sent for two MRI scans. These highlighted a prolapsed disc at T8 and evidence of bleeding along the spine and around my brain. This was shown to me as if someone had taken a black highlighter pen and drawn around my spine and brain! I was asked about previous trauma to establish why the disc and why the bleed. My only knowledge is that I was diagnosed with viral encephalitis at age 5. I will be 50 later this year. As a result of the encephalitis I was left with total nerve deafness in my left ear which has never recovered. I was referred to a neuro surgeon who initially responded by stating he felt the risk of operating on T8 was too high for the potential rewards. The Neurologist who referred me to him stated that the risk of not operating was even higher. I am still, over 3 weeks later in a stalemate situation, awaiting a decision on the way forward .The issue it seems is whether the bleed/s will be found. Meanwhile my balance issues have worsened considerably and the constant back pain is becoming unbearable. Whilst I suffer also from fatigue and concentration issues as yet I haven't noticed any decline in my hearing, although I was left deaf in my left ear as a result of the encephalitis at age 5. I am slightly concerned of bowel and bladder issues having suffered a couple of problems in the last few months. Just before this issue I was advised that I also suffered with an under active thyroid however another GP said this was wrong. Has anyone also suffered SS and linked thyroid issues? I know I must stop reading about SS on Google and forums like this as it appears there is little of a positive nature to look forward to! Any help, guidance or encouragement will obviously be appreciated. Equally if there is anything about my diagnosis that help others now or in the future then please let me know.


A: I think the best reason to consider surgery is to treat the back pain. That is likely due to the disc and not the siderosis. Your balance issues may be due to both, but it’s hard to know how much each is contributing unless you get the surgery and see how things turn out. Thyroid issues are not related to siderosis but they are very common in the general population.

Q: Low Frequency Hearing Loss. I have severe and rapidly declining low frequency hearing loss and recently an MRI that indicated the potential of siderosis. Have you ever seen just low frequency hearing loss with SS or is it typically loss across frequencies? Thanks!


A: In SS, hearing loss is typically lost in the high frequencies first.

Q: My friend now has unilateral hearing loss currently under investigation. Might this be relevant?


A: Hearing loss in superficial siderosis usually starts out as unilateral (one sided) and then involves the other ear later.

Q 98: 5 years ago my husband attended an audiology clinic to be fitted with hearing aids. The Doctor said that he had an unusual form of hearing loss that was due to brain malfunction and that he should tell his neurologist (who duly dismissed it).  Recently he has had his hearing reassessed and the audiologist  confirmed that the hearing loss had a peculiar pattern.  Is this true of all Superficial Siderosis sufferers and if so could it be used as a tool for early detection?


A: The type of hearing loss in Superficial Siderosis is called sensorineural. Sensorineural hearing loss refers to the fact that the problem is in the nerve or brain, as opposed to the ear.  In contrast, age related hearing loss is due to problems in the ear. Some Superficial Siderosis patients who are older have both types of hearing loss!



Q: My wife has Superficial Siderosis. Recently she has had surgery which was verified to stop the bleed. She has balance & hearing loss as a result of her condition. Since her bleed has been stopped, will her condition continue to decline?

A: Yes. The iron that is still stuck on the surface of her brain and spinal cord will continue to cause nerve damage over time.



Q: Can you explain how SS effects the central nervous system? It seems as if some nerves don't work at all i.e. hearing, smelling, tasting and others work in overdrive, tingling in hands, sharp miscellaneous pains, feet. Are the nerves working or dying, or what?


A: SS causes only loss of function of nerves. The overdrive feeling is the brain's interpretation of the nerve dysfunction. As the disease progresses, the nerves may eventually die and then the overdrive feeling is lost too.



Q: I was diagnosed with SS one year ago. I am from Lithuania, maybe first patient with SS in my country. What are the statistics for SS. How many SS cases are out there per million people? I can't walk without crutches anymore because of poor coordination, also I can constantly "hear" rhythmic noises in my head. Is that some of the symptoms of SS?


A: SS is incredibly rare. The incidence is approximately 1 per million. Your symptoms of balance problems and tinnitus are classic for SS.



Q: Hi there I'm messaging from Sheffield, UK on behalf of my dad.  He's 73 and been diagnosed with SS around 2000.  He's in the advance stages with total hearing loss, nystagmus, loss of bladder and bowel control and in a wheelchair as he can no longer walk. I've heard about Ferriprox and wondered if it can be used in the advanced stages of SS. I've never heard his consultant mention this drug. 


A: Ferriprox is approved in the UK and should be accessible. There is no other iron chelator that gets in the brain so it's the only option for treatment at this time. No telling if your dad could see the benefit of Ferriprox since it will take several years to remove the iron.



Q: I've been taking Ferriprox for 2 years now, and my iron levels on the brain have almost been depleted. My symptoms are still uncomfortably strong, no hearing, some improvement in my balance, and very loud tinnitus. I've been taking OTC pill called Lipo-Flavonoid for about 5 months which is supposed to help with the tinnitus. It hasn't helped much with that but it has a lot of vitamin C. Should I give that up, as it is probably counteracting the Ferriprox. My next MRL should be in a couple of weeks, early June.


A: the only trouble with vitamin C is the same as iron (answer above). It binds Ferriprox in the gut and you’ll lose both. If your MRI is improving, hopefully the healing will begin and you will regain some neurological function.



Q: Currently my husband's GP does not seem to think that he needs any specialist care at all!  His hearing is deteriorating quite rapidly. Is there any value in him seeing an auditory consultant on this matter rather than just attending the hearing aid clinic?


A: Your husband may be a candidate for a cochlear implant to improve his hearing. Not all patients are good candidates so it's important to get an expert opinion.

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