Introduction
Sierra Domb, founder of the Visual Snow Initiative, is at the forefront of a historic and groundbreaking campaign to obtain an ICD code for Visual Snow Syndrome (VSS). She is advocating for the recognition of VSS in the latest edition of the International Classification of Diseases, or ICD-11, a system maintained by the World Health Organization (WHO). In support of this initiative, testimonies were submitted by Domb, VSS researchers, and medical experts. Their testimonies can be found below.
Background
The ICD is essential for global healthcare providers to classify medical conditions and is crucial for diagnosing, monitoring, and treating patients worldwide. An ICD code for VSS would cement its status as a distinct neurological disorder, characterized by visual and non-visual symptoms. This recognition is vital for those who have been marginalized, misdiagnosed, mistreated, and/or doubted due to the medical community’s historical misunderstanding of VSS. Including VSS in the ICD offers numerous new and significant benefits. This includes enhanced research opportunities, data collection, and eligibility for insurance coverage/financial support associated with VSS-related services, significantly impacting patients, researchers, and medical professionals globally. For a full list of benefits of securing an ICD code for VSS, please click here.
The process to designate an ICD code is intricate, involving expert proposals, supporting testimonies, thorough reviews, and several voting stages. The WHO, supported by contributions from member countries and health professionals, manages this process. The Medical Scientific Advisory Committee (MSAC) has favored the proposal for both Visual Snow Syndrome (the condition) and Visual Snow (the symptom of seeing snow-like static 24/7) to receive their own ICD codes. The Classification and Statistics Advisory Committee (CSAC) is currently reviewing the proposal, which is the final step in the approval process.
Despite challenges and initial doubts about the eligibility of VSS for an ICD code, Sierra Domb and the VSI team have tirelessly pursued this goal. They have funded scientific research and united global experts to support their cause, demonstrating unwavering commitment to legitimizing VSS.
Sierra Domb, unifying leader and initiator of this mission, Dr. Peter Goadsby, originator and submitter of the proposal, and Dr. Owen White, collaborator on the proposal’s development, have all provided testimonies to the WHO, alongside colleagues and medical experts.
The VSI team expresses heartfelt gratitude to all who have supported their initiative. For further details on the contributions of those involved in the efforts to secure an ICD code for Visual Snow Syndrome (VSS), please refer to their testimonies below:
Supporting Testimonies from the VSI Team & Experts
(In Chronological Order)
“Visual snow syndrome has recently been codified and proven to have an underlying pathophysiological basis. It is in the interest of all patients, and people who provide both clinical care and research in this area, to be able to codify it and search databases to establish incidence and frequent co-morbidities.
I collaborate in the development of this proposal put by Prof Peter Goadsby of King’s College London.”
Prof. & Dr. Owen White – Clinical Director Ocular Motor Laboratory, Monash University
“I agree with adding Visual Snow to ICD, it will add diagnostic clarity and assistance to these patients. This will assist in future research and management.”
A/Prof & Dr. Clare Fraser – Save Sight Institute, The University of Sydney
“Adding VS and VSS to the ICD will allow for better clinical characterization and clarity over the condition, ultimately leading to improved research and shared knowledge.
I would suggest adding the following reference to the phrase ‘There are other associated sensory processing disorders, occurring at greater than chance frequency that are not necessary for the diagnosis of the syndrome’ in the first paragraph:
Puledda F, Schankin C, Goadsby PJ. Visual snow syndrome: A clinical and phenotypical description of 1,100 cases. Neurology. 2020 Feb 11;94(6):e564-e574. doi: 10.1212/WNL.0000000000008909.
This study has measured the association between VS, tinnitus and migraine.”
Dr. Francesca Puledda – Neurologist, King’s College London
“I agree with Dr. Puledda that both visual snow (VS) and visual snow syndrome (VSS) should be added to the ICD. There are no current diagnostic options for these patients within the ICD, as they only loosely fit into “visual disturbance, unspecified.” Having these more specific diagnoses will help considerably both in clinic and research.
Importantly, there are cases of secondary visual snow that do not meet criteria for the full VSS. Rather than pollute the diagnoses by forcing clinicians to pick “VSS” even if the patient only has the visual snow and not other criteria, I would propose that both VS and VSS be added separately.
Mehta DG, Garza I, Robertson CE. Two hundred and forty-eight cases of visual snow: A review of potential inciting events and contributing comorbidities. Cephalalgia. 2021 Aug;41(9):1015-1026.”
A/Prof & Dr. Carrie “Beth” Robertson – Department of Neurology, Mayo Clinic
“We definitely need to have Visual Snow (VS) and Visual Snow syndrome (VSS)–This addition will assist researchers and clinicians to have a diagnosis that can be further studied and explained to patients that this is a real syndrome. Secondary visual snow is also important since there are secondary mimics. See:Patel RC, Vitale AT, Creel DJ, Digre KB. Not All That Flickers Is Snow. J Neuroophthalmol. 2021 Mar 1;41(1):e97-e99. doi: 10.1097/WNO.0000000000000935. PMID: 32235228.”
Prof. & Dr. Kathleen Digre – Neurology and Ophthalmology, University of Utah
“Thank you for the comments. Regarding adding visual snow (VS), we thought adding visual snow syndrome (VSS) as an entity was the most pressing issue. We would plan, after VSS is accepted, to add VS under “symptoms, signs or clinical findings of the visual system” in chapter 21, for use where cases are not VSS. It seems better to get the entity included first before the symptom. The symptom can then be coded particularly when it appears as a secondary manifestation.”
Prof. & Dr. Peter Goadsby – Director, National Institute for Health Research – Wellcome Trust King’s Clinical Research Facility, King’s College London, UCLA
“I agree with the proposal by Prof. Goadsby and Prof. White adding VSS to the ICD. Having an ICD-code is crucial for patients giving them a diagnosis, physicians making the diagnosis, insurance companies having a starting point for looking for information regarding their patients, and researchers having an option for searching databases and building registers.
VSS is a syndrome affecting the visual and non-visual perceptual as well as non-perceptual systems. Patients with VSS have a quite specific but complex clinical picture and potentially a common pathophysiology. VS itself, in contrast, is an unspecific symptom that can (i) be symptomatic(1), (ii) episodic(2), and (iii) a migraine aura phenomenon. I agree with Prof. Goadsby’s comment to focus on VSS for now and later think at adding VS.
I would suggest to even add a sentence similar to: “The additional symptoms as well as the duration of the syndrome, which can be stable over years (3), distinguish VSS from potential mimics (1,2) and support that it is a distinct disorder.
(1) Headache. 2023 Jan;63(1):173-176. doi: 10.1111/head.14445.
(2) JAMA Neurol. 2020 Mar 1;77(3):392-393. doi: 10.1001/jamaneurol.2019.4050.
(3) Brain Commun. 2022 Sep 9;4(5):fcac230. doi: 10.1093/braincomms/fcac230.”
Prof. & Dr. Christoph Schankin – Department of Neurology, Bern University Hospital
“I agree with the proposal of recognising Visual Snow Syndrome as a distinct entity in the ICD. I am a clinician (Neurologist and Neuro-Ophthalmologist) and researcher. Visual Snow Syndrome, as a new entity in the ICD’s coding, will facilitate recognition, treatment, and research in this field.”
Dr. Sui Wong – Consultant Neurologist and Neuro-ophthalmologist, Guy’s and St Thomas’ NHS Foundation Trust, Moorfields Private Eye Hospital
“I am a paediatrician and researcher in childhood dorsal visual stream impairment (our clinical database numbers around 400 children) and its attendant motor disorder which we view as originating from disruption to posterior parieto-cerebellar networks. Hypotonia sufficiently severe to merit exclusion of primary neuromuscular disorder is not uncommon in this group and exists with simultanagnosia and optic ataxia of variable severity.
I became aware of Visual Snow syndrome when one of my older patients asked me if he had it! Certainly the impairments described are often seen in children with dorsal stream visual impairment. I have never seen vestibular impairment in this group: hearing and balance is usually exceptionally good, presumably compensatory for the spatial vision deficit. Optic ataxia of the lower limbs can however be misinterpreted as vestibular impairment. We only test lower limb cerebellar function using heel- shin testing because of the confounding impact of impaired visually guided movement on tandem walking.
I assume Visual Snow syndrome is a subtype of cerebral visual impairment and am more than happy for any formal acknowledgement of the condition.”
Isobel Hay – NHS Dumfries and Galloway, Pediatrics
“In 2015, at 21 years old, my life changed in an instant. As I was driving to university, suddenly, my vision went black. Thankfully, I instinctively pulled my car over to avoid a crash and the darkness only lasted for a moment. But when my vision returned, the world did not look the same. My entire visual field was overwhelmed by a frightening overlay of flashing lights and moving particles, which resembled dense television static, or “white noise”. This static overlay was unceasing, persisting 24/7 whether my eyes were open or closed. I would later discover this visual phenomenon had a name: “Visual Snow”. In the days that followed, other visual and non-visual symptoms appeared, including palinopsia, photophobia, enhanced entoptic phenomenon, diplopia, photopsia, tinnitus, and derealization.
Due to the debilitating nature of my symptoms, I was forced to stop attending university. I could no longer see, work, drive, or function normally. Fearful I was going blind (or worse), my family and I sought help from dozens of doctors and specialists at many renowned medical institutions. But no one could offer an explanation, let alone a diagnosis. Over the next year and a half, I was subjected to intrusive, costly, and unnecessary medical tests, most of which focused on my eyes. My tests always came back normal. After my medical tests revealed nothing, some doctors simply told me to “get on with your life”, as though they no longer believed me. One doctor even referred me for a psychiatric evaluation. With my own doctors doubting me and no answers, I lived in constant fear and isolation.
I realized it was up to me to figure this out. I soon located an article published in a well-known medical journal that described my symptoms with astonishing precision. The article described a medical condition called Visual Snow Syndrome, which can manifest suddenly. Visual Snow Syndrome (VSS) is a neurological disorder, which explains why all the tests on my eyes came back normal. VSS can affect vision, hearing, cognition, sensory processing, and quality of life. There is no cure. However, I learned that VSS would not kill me, nor would it cause me to go blind.
Soon, it became clear that I was not a medical outlier. My story mirrors that of thousands of people around the world. People of all ages and backgrounds worldwide are affected by VSS. Many have embarked on medical odysseys much like my own. They are distraught from being marginalized, misdiagnosed, and/or not diagnosed. Many are left isolated and traumatized, some to the point of suicide due to the severity of their symptoms and the lack of acknowledgement of the reality and seriousness of their condition by many in the medical community.
I knew further awareness, education, research, and solutions for VSS were necessary. In 2018, I organized the first Visual Snow Conference at the University of California, San Francisco. Doctors, researchers, and scientists with knowledge of Visual Snow Syndrome flew in from Australia, Canada, England, and various cities in the United States. The Visual Snow Conference was free to the public. After remarks from myself and international VSS experts, the audience, made up primarily of VSS patients and their families, fell silent as they were validated and acknowledged for the first time in their lives.
Shortly after the conference, I founded the nonprofit organization, Visual Snow Initiative (VSI), which is dedicated to global awareness, education, resources, patient advocacy, treatment development, and research for VSS. Every day since then, my team and I have worked diligently to facilitate collaboration between physicians and academic institutions, develop solutions alongside researchers, make neuroscience more palatable for everyone regardless of age or health literacy, raise awareness for VSS via multimedia content, and help people with VSS worldwide.
Academics and researchers have identified measurable differences in the brains of those with VSS. Data has also revealed that VSS symptoms are not a manifestation of anxiety and depression. Instead, the debilitating nature of VSS symptoms coupled with the medical community’s historic marginalization of VSS patients can profoundly and negatively impact their mental health. VSS is a distinct neurological disorder that entails both visual and non-visual symptoms.
To date, my nonprofit has heard from people affected by VSS in 93 countries. With rising awareness and education for VSS, the number of people we have been able to reach grows daily. Inclusion of Visual Snow Syndrome in the ICD-11 will provide recognition, legitimacy, and a huge stimulus for this condition’s identification, amelioration, as well as the necessary research to diagnose and treat it. This will give a voice to a whole community of patients around the world who have tried in vain to be heard.”
Sierra Domb – Founder & CEO, Visual Snow Initiative
“The North American Neuro-Ophthalmology Society (NANOS) fully supports the establishment of an ICD-11 code for visual snow syndrome (VSS).
Patients with visual snow syndrome (VSS) have long been presenting to physicians but have been misdiagnosed as having either untreatable migraine or a psychological disorder. Having been first recognised in 1995 (Liu et al., 1995), VSS was further defined as a distinct disorder characterised by persistent visual snow for more than three months and two other symptoms out of photophobia, palinopsia, blue field entoptic phenomena and nyctalopia (Schankin et al., 2014). There are other associated sensory processing disorders, occurring at greater than chance frequency that are not necessary for the diagnosis of the syndrome (Puledda et al., 2022).
Psychophysical and electrophysiological studies have shown distinct abnormalities of visual perception in patients with VSS, differentiating them from migraine patients (McKendrick et al., 2017, Brooks et al., 2022, Eren et al., 2018). Studies of central processing dysfunction in the visuo-oculomotor system have also shown distinctive abnormalities characteristic of VSS (Foletta et al., 2021, Solly et al., 2021, Solly et al., 2023). Specialised imaging studies with 3T and 7T fMRI have shown disordered connectivity in the brains of patients with VSS compared to control participants (Puledda et al., 2021, Strik et al., 2022), as well as altered brain volume (Schankin et al., 2020) and activity (Puledda et al., 2020). It is notable that the disorder has been studied by multiple research groups in Australia, Europe, Iran, Turkey, United Kingdom and United States.
Prevalence studies are limited, but since identification of the syndrome, ophthalmologists, neuro-ophthalmologists and neurologists have seen these patients frequently and prevalence has been estimated as being approximately 2% in the adult population (Kondziella et al., 2020).
It is important that VSS be recognised as a separate ophthalmic disorder since it is mainly seen by ophthalmologists, which will facilitate collection of related health statistics, to enhance management of patients, and to facilitate future research. We seek secondary parenting in Other Disorders of the Nervous System since it is often seen by neurologists.”
Dr. Prem Subramanian – MD, PhD, NANOS President
Toma Kinville-Ossian – NANOS Executive Director
Further Updates
VSI will continue to share updates on the status of an ICD code for Visual Snow Syndrome (VSS) and related news on all of its platforms, including its website, newsletter, and social media channels.
You can also access additional information about our Founder’s mission to get an ICD code for VSS on her personal website, sierradomb.com, where she is documenting the process.
Thank you so much for your support!