Dr. Levy Clears Things Up

Hi Ms. Huguley,

The spinal fluid is made deep in the brain and circulates around the brain and spinal cord, as you said correctly, and then ultimately gets resorbed into the veins at the top of the skull. The total volume of spinal fluid is turned over 3 times every day so you can imagine the circulation is fairly brisk. That's why a bleed anywhere in the spinal fluid leads to exposure of the whole nervous system to the blood. As the blood breaks down, the blood iron products precipitate out and eventually settle down by gravity to the back of the head while sleeping and to the bottom of the spinal cord while awake. The reason hearing is affected is because the nerve that goes from the ear to the brain spends the majority of its time in the spinal fluid. All other nerves that come directly off the brain have < 1 mm of surface exposed to the spinal fluid. So the nerve to the ear is vulnerable to blood products circulating in the spinal fluid. Since that nerve has a large surface area relative to the volume of the nerve, a large percentage of the nerve is exposed to the iron. That's why hearing is usually the first symptom of superficial siderosis. 

Michael Levy, MD, PhD
Assistant Professor, Johns Hopkins University
Director, Neuromyelitis Optica Clinic
1800 E. Orleans St.
Pathology 509
Baltimore, MD 21287
443-287-4412 phone
888-523-4168 fax

What is Superficial Siderosis? And a Letter to Dr. Jordan & Dr. Levy

This information is located earlier in this blog, I'm adding it again for those of you just visiting this page. It's a real quick overview of what Z has and what possible side effects he will endure in the future. 

What is Superficial Siderosis

Superficial siderosis (SS) of the central nervous system is a rare brain disorder resulting from chronic, and repeated, slow haemorrhaging into the subarachnoid space, and if accurate international figures were obtainable, would most certainly prove to be evenly spread amongst all nations around the world – the small country of New Zealand does appear to have more than its fair share of diagnosed survivors however. People diagnosed with SS range in age from just a couple of years old, right through to their 80s, though the most common age bracket is the 50s and 60s.

Despite being known for over 100 years, it is only in the past few years that SS has been widely acknowledged and studied. Previously known by several titles it was only in 1985 a definite title was applied to the disease. The disease is unusual in that it carries so many side-effects that a sufferer may experience. The symptoms are still being discovered, but currently number over 30, only some of which are common to all, or most, known survivors of the disease.

Prior to the introduction of MRI machines, diagnosis of the disease was carried out on the autopsy table.

Side effects and symptoms

• The most common side effect is the lack of hearing, with 95% of those affected. What hearing may be retained, if any, is generally of no value. The hearing does deteriorate extremely quickly with SS and hearing aids provide only short term assistance. Cochlear implants currently appear to be the only permanent life buoy for this side effect.
• Ataxia (lack of balance) and loss of co-ordination are the next most common and frustrating side-effects for those diagnosed. Around 85% of all cases are affected with these two debilitating side effects. Mobility aids, such as walking poles or canes, wheeled walking frames, and wheelchairs provide valuable assistance. Many with the ailment advance to a stage where they are bedridden.
• Bowel and bladder complications are also commonly accepted complaints.
• Side effects vary right down to only two known cases of the 8th cranial nerve being covered in hemosiderin, stopping the survivor from feeling any pain in their teeth.

Known SS symptoms

• lack of hearing
• imbalance
• poor co-ordination
• early dementia
• bladder complications
• bowel complications
• headaches
• migraines
• severe tiredness
• short term memory loss
• loss of smell
• loss of taste
• muscle cramps
• double vision and other vision complications
• cranial nerve palsies
• imaginary pains, smells, tastes and sounds
• permanently bed bound
• failure of the senses
• one pupil larger than the other
• double sciatica
• mini strokes
• seizures
• speech difficulty
• nystagmus
• numbness and pain in parts of the body, especially feet
• swallowing difficulties
• failure to feel pain in the teeth
• ‘Parkinsons’ type leg and hand wobbles
• inability to control emotions – anger, sorrow, etc.
• intolerance and uncertainty to heat and cold; may feel roasting hot, but are actually cold
• failure of sexual organs – inability to arouse an erection, or no feeling of satisfaction for females

Understanding How SS Affects the Hearing

I wanted to be able to understand more about how SS affects hearing so I wrote a letter to Dr. Jordan & Dr. Levy to get their expertise on the subject. 

Dr. Jordan and Dr. Levy

I’m sorry to be such a burden. I just want to be sure I'm understanding the CNS and the CSF flow correctly. I’m seriously trying my best to understand all of this. Just when I think I’ve got it, I don’t.
I understand that iron is being deposited on Zachary’s spinal cord. What I don’t understand is how does that affect his hearing? I guess I’m confused by the details of Superficial Siderosis and it's affects on the hearing. How does the iron affect his hearing? I want to know exactly how SS affects the hearing. Does it destroy the inner ear somehow? I don’t understand this part of it and because this seems to be the reason for Z’s hearing loss I’d really like to know more about how SS wreaks it’s havoc on this part of the body.
I would be most grateful if you could help me to understand. I’m all about researching and if there are reliable places for me to do this that you can send me to, I’d be greatly appreciative. I would really like to be as educated, as a mother who has a child with SS can be.

I'm trying to understand the flow of the Cerebrospinal Fluid ... Is it true that it is produced in the center of the brain and then travels to the ventricles then down the spinal cord and back? I just want to be sure I understand that it does go down the central canal of the spinal cord and then back up again to the brain, which means it must pick up some of that deposited blood/iron at the base of Z's spine and it drags it back to the brain, right?

Thank you so much for your time
Michelle Huguley