Personal Stories

The Struggle Of Dual Neurological Conditions

Superficial Siderosis and Hydrocephalus

Having superficial siderosis is one thing, but sometimes like me, it’s possible to have other disabling neurological conditions as well. In late 2001, three years after the removal of a benign brain tumour, I was diagnosed with Hydrocephalus, which translates from Greek to English as water and head. 

Hydrocephalus is a condition usually caused by the obstruction of the flow of CSF within the central nervous system. A ventriculoperitoneal shunt is the most common way to treat this. A VP shunt is a mechanical device that drains excess CSF from the head into an area in the abdomen (the peritoneal), reducing the abnormally raised pressure in the head. A valve on the shunt opens when the pressure gets too high and drains CSF through a plastic tube (catheter) into the peritoneal area to be absorbed.

the struggle of dual neurological conditions

I had a VP shunt fitted to drain excess cerebrospinal fluid (CSF) from my head. The reason for this was because I was suffering increased pressure within my head, which if left untreated, could have been fatal. Prior to having a VP shunt fitted, the pressure in my head would build to such high levels it brought symptoms such as dizziness, vomiting, lethargy, blurred vision and often caused me to lose consciousness. At only eleven years old, I did not understand what was going on and was worried that I had another brain tumour because the symptoms were strikingly similar.

The operation to fit the VP shunt was a success and gave me instant relief from the raised pressure. It took a few weeks to recover from the procedure, but I was back to school shortly afterwards. The shunt, however, didn’t come without its problems; it was very prone to blocking, and each time caused an onset of the same symptoms that I had before having it fitted. Every episode of shunt blockage required another brain operation to relieve the pressure as well as the symptoms. In 2002 I underwent four shunt revision operations, so in total, I had five brain operations within the space of a year; this was a rough time for me.

Raised protein levels in the CSF can cause a VP shunt to block. Having blood in the CSF is a known cause of increased protein; however, it is not known whether the bleed at the back of my head contributed to the blocking of my VP Shunt.

In 2015 at aged twenty-five, I was in hospital with hearing and balance issues when my shunt suddenly blocked for the first time in over ten years. Little did I know this was going to lead on to my Superficial Siderosis diagnosis. The shunt blockage came out of nowhere, and suddenly I was unconscious. I never expected it, and neither did the doctors looking after me. The next thing I remember is waking up in another hospital after having had surgery to unblock the shunt. The hearing in my right ear was nearly completely gone; my left ear hadn’t changed at all. I began to experience a popping sensation in my right ear soon after discharge, as well as the dizziness and balance issues caused by the Superficial Siderosis. The popping in my right ear felt like I was 20,000 feet in the air on a plane, but I remained helpless to stop it from happening.

ICP monitoring- ICP Bolt

In October 2016, I was diagnosed with superficial siderosis, continuing to get popping in my right ear. My shunt blocked again around May 2017 after I had been exercising on the treadmill. Further brain surgery unblocked my shunt, and the surgeon told me he would call me back in a few weeks later to monitor the pressure in my head. This monitoring required a minor operation to insert an ICP (Intracranial Pressure) ICP bolt at the front of my head which was connected a lead from the ICP bolt to a machine for recording the pressure. It wasn’t a painful procedure as such, just uncomfortable. 

The monitoring results revealed the pressure in my head was too low (intracranial hypotension). My surgeon said he would first look to rule out my VP shunt as the cause of my problem. It had been in place for many years and could be draining too much CSF. The plan was to replace the shunt valve with a newer programable type that could be adjusted with a special magnet. The magnet adjustments could change the resistance of the shunt valve, so CSF only drained when the pressure got to a certain level. The shunt valve also had an antisiphon device in it to prevent a syphoning effect which could have been causing the low pressure. Once the new valve was fitted, I was back and forth for weeks having it adjusted up and down but with no real benefit. 

The doctors and surgeons were finding it difficult to distinguish between the Superficial Siderosis symptoms and the VP shunt issues. A Superficial Siderosis impairment of my Neurological Reserve contributed to needing a much longer time to recover from the anaesthetic of the operations to unblock/revise the shunt. Both the intracranial hypotension and Superficial Siderosis played a part in my hearing loss.

In August 2018 a CT cisternogram (a brain scan in which contrast dye is inserted directly into the CSF via a lumbar puncture) showed that I leaked CSF at the back of my head which was presumed to be causing the low pressure along with its symptoms. My surgeon inserted a PEEK implant (a type of medical plastic implant used to repair defects) into the cavity at the back of my head to seal the leak. Following this, another cisternogram showed there was no longer a leak. This leak was thought to be the site of the blood infiltration into my spinal fluid which caused my Superficial Siderosis.

the struggle of dual neurological conditions

EVT – a front right sided burr hole was made to gain access to the 3rd ventricle in order to carry out the procedure


ETV – Endoscopic third ventriculostomy – where a hole is made in the third ventricle of the brain so that CSF can drain to relieve the pressure

Unfortunately, I now suffered relentless popping in both of my ears and developed instability of the pressure in my head. In early 2019, further neurosurgery was carried out, and my shunt moved from the right-hand side and inserted into the left-hand side of my head along with a new programmable valve. These changes did not help; pressure monitoring showed my pressure was still low. My surgeon consulted an expert in low-pressure Hydrocephalus. The expert recommended removing the shunt and carrying out an Endoscopic third ventriculostomy (ETV). 

surgery site
January 2019, The shunt was removed from the right-hand side of the head and put into the left-hand side

Today the pressure appears to have gone in the opposite direction, and I now have the feeling of high pressure in my head The ETV is keeping the pressure down, but it doesn’t seem to offer as much pressure relief as I need. Whenever I’m doing anything physical such as my balance exercises, I feel raised pressure in my head and behind my eyes. Each time this happens, I need to stop what I’m doing. Sometimes it can be so bad that I feel like I’m losing consciousness. I’m also dealing with slight swelling and pain adjacent to my 2018 operation scar. 

My surgeon decided to perform a CFS Infusion test so they could get an idea of the tolerance and pressure threshold in my head. After my ETV operation, the surgeons had left a valve in my head in case the pressure got too high and they needed to drain some off in an emergency. The surgeon carrying out the procedure found it interesting when I stood up, the pressure in my head appeared to increase when it is supposed to have the opposite effect and decrease.

CSF Infusion Test: fluid is inserted through a valve into the head to test pressure tolerance.

Currently, I’m waiting for a follow-up appointment with the consultant neurosurgeon to discuss my results and possibly adding a shunt on top of the ETV to sufficiency manage the pressure in my head. 

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Rhys Holmes

Rhys Holmes is a feature writer at Living with Superficial Siderosis and the U.K Director of the Superficial Siderosis Research Alliance. Rhys is a former musician, data analyst, shop assistant, and childhood footballer. In his spare time, he enjoys watching his favorite football team Liverpool FC. Follow Rhys on Twitter @RhysHolmes

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  1. Hi, is there anything what helps you to reduce the pressure or have you noticed anything what makes the fluid build up quicker

    1. I initially had a VP shunt which recued the pressure, I now have an ETV (endoscopic third ventriculostomy), which is where a hole is made in the third ventricle so the brain fluid can drain to relieve the pressure.

      If you have raised pressure, lying flat would exacerbate it, as would straining of any sort.

  2. Well God Bless you Rhys, you’ve been through way more then me , and for a longer period of time. I was 67 when I got my diagnosis, you , much younger.
    So I will keep you in my prayers ! The way I look at it, now with covid, everyone is now living our life !

    1. Thanks Maggie, I completely agree. To me covid hasn’t really changed much at all in terms of getting out, the only difference is it’s been more difficult to get medical appointments.

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