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(VSS): an Evolving Neuro-Optometric Clinical Perspective Kenneth J

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(VSS): an Evolving Neuro-Optometric Clinical Perspective Kenneth J Visual Snow Syndrome (VSS): An Evolving Neuro-Optometric Clinical Perspective Kenneth J. Ciuffreda, OD, PhD Barry Tannen, OD M.H. Esther Han, OD SUNY/College of Optometry, Brain Injury Clinical Research Unit ABSTRACT Visual snow syndrome (VSS) is a rela- tively new diagnosis in optometry and in medicine. VSS represents a constellation of visual and non-visual problems, with the hallmark symptom being the perception of visual snow (VS) appearing in a single plane in front of and throughout the visual field. PERSPECTIVE Individuals with VS describe seeing Figure 1a: Patient depictions of VS throughout the entire visual field. Case One looking at her cat. “snow”, dots, pixelated fuzz, bubbles, and “static” as found on a poorly-tuned television. In this paper, the evolving area of proposed, several cases and related information VSS is briefly reviewed, a VSS symptom survey is are presented, and current neuro-optometric therapeutic interventions are discussed. Correspondence regarding this article should be emailed to Kenneth J. Ciuffreda, OD, PhD, at kciuffreda@ sunyopt.edu. All state ments are the authors’ personal INTRODUCTION opinions and may not reflect the opinions of the One of the most interesting and intriguing, College of Optometrists in Vision Development, Vision as well as bothersome, visual abnormalities Development & Rehabilitation or any institution or organization to which the authors may be affiliated. is the presence of “visual snow” (VS), more Permission to use reprints of this article must be obtained specifically referred to as the “visual snow from the editor. Copyright 2019 College of Optometrists 1,2 in Vision Development. VDR is indexed in the Directory syndrome” (VSS). These individuals perceive of Open Access Journals. Online access is available at a somewhat pixelated visual world, sometimes covd.org. https://doi.org/10.31707/VDR2019.5.2.p75 fuzzy, with either black-and-white or chromatic Ciuffreda KJ, Tannen B, Han MHE. Visual Snow Syndrome dots appearing in a single plane in the (VSS): An evolving neuro-optometric clinical perspective. foreground superimposed over the entire visual Vision Dev & Rehab 2019;5(2):75-82. scene (Figure 1a). This is typically reported to be a constant, persistent phenomenon that Keywords: neural disinhibition, neuronal has been present for years. They frequently hyperexcitability, neuro-optometry, describe it as being akin to the “snow” that is sensory hypersensitivity, visual perception, present when the primary television signal is visual snow, visual snow syndrome, reduced, and the residual electronic “noise” VSS symptom survey predominates on the screen. 75 Vision Development & Rehabilitation Volume 5, Issue 2 • June 2019 Individuals with VSS typically manifest two In the present paper, several cases of or more of the following four visual perceptual VSS that the authors have examined will be phenomena: (1) palinopsia; (2) photosensitivity/ discussed, as well as related information. The photophobia; (3) “nyctalopia”; and (4) enhanced emphasis will be placed on the development entoptic imagery.1,3 They may also report of a comprehensive VSS patient diagnostic having other visual and non-visual symptoms.2 symptom survey, as well as discussion of the These will be described in more detail in the neuro-optometric treatment options that were next section. successfully prescribed. As interest and knowledge in this relatively new area (1995)4 increases and evolves, the topic VSS Symptom Survey of its treatment becomes important. Currently, We have developed a VSS diagnostic symp- there is a paucity of such information. Treatment tom survey, including demographics and VS in the past has included:2 (1) chromatic filters; history, primary VSS-related visual symptoms, (2) pharmacologic interventions; and (3) simple and secondary VSS-related visual and non- explanation of the condition to the patient. visual symptoms (Table 1). These questions were These too will be considered in more detail later. based on a summary of several reports in the Table 1. Visual Snow Syndrome (VSS) Symptom Survey VISUAL SNOW SYNDROME (VSS) SYMPTOM SURVEY Patient’s Name:____________________________________________________________ Date:_______________________________ DEMOGRAPHICS CURRENT AGE (IN YEARS) GENDER AGE FIRST NOTICED SNOW (IN YEARS) IS VISUAL SNOW CONSTANT OR TRANSIENT? IS VISUAL SNOW CHROMATIC OR MONOCHROMATIC? PROVOKING ENVIRONMENTS? POSSIBLE ETIOLOGY? POSSIBLE CO-MORBID CONDITIONS? TREATMENT(S)? MEDICATIONS? Please rate each symptom below as: 0=Symptom is not present 1=Symptom is transient 2=Symptom is constant PRIMARY VISUAL SYMPTOMS 0 1 2 COMMENTS PALINOPSIA ENTOPTIC IMAGERY PHOTOSENSITIVITY "NYCTALOPIA" (IMPAIRED NIGHT VISION) SECONDARY VISUAL/NON-VISUAL SYMPTOMS PHOTOPSIA MIGRAINE PHONOPHOBIA HYPERACUSIS CUTANEOUS ALLODYNIA TINNITUS BALANCE PROBLEMS TREMOR 76 Vision Development & Rehabilitation Volume 5, Issue 2 • June 2019 literature over the past two decades involving on the clinical testing and formal queries of several hundred individuals with VSS.1-11 The first section queries the basic demo- graphics, VS history, and possible related Figure 1b: Patient depictions of VS throughout the entire factors. There are also other areas that visual field. Another VSS patient looking at a pencil. the clinician may elect to probe based on historical aspects of the field that might yield avoid provoking environments, and secondly to additional insights:1-11 (1) Did headaches assure the patient that it is a relatively benign precede the perception of VS?; (2) Is the VS and not progressive condition, assuming that stable or progressive?; (3) Was the perception any possible abnormal neurological or ocular of VS of sudden onset?; (4) Are there any condition has been ruled out.2 In general, its family members with VS?; and (5) Has the presence appears not to have any adverse individual ever taken hallucinogenic drugs? consequences on their activities of daily living Early literature suggested a link. (ADLs), although some have reported difficulty The second section queries the presence driving at night due to VS interference. However, of the four primary VSS visual symptoms, this aspect has not been well documented. their provoking environments, and treatment The third section presents a list of several aspects. Based upon the latest clinical possible secondary visual and non-visual thinking,3 typically the patient must also symptoms that the patient with VSS may also report at least two of the following four visual experience. These include: (1) photopsia: per- symptoms/visual perceptual phenomena to be cep tion of flashes of light, usually very brief diagnosed with VSS: (1) palinopsia: persistence and intermittent; (2) phonophobia: unwarranted of an image, at times superimposed on the fear of/aversion to specific sounds (e.g., traffic new image, and frequently with trailing; noises, voices); (3) hyperacusis: increased (2) enhanced entoptic imagery: imagery per- sensitivity to certain sounds, at times specific sistence above what many others would frequencies; (4) cutaneous allodynia: pain sen- normally perceive; (3) photosensitivity/photo­ sa tion to the skin/scalp from innocuous stimuli phobia: visual discomfort, perhaps even actual (e.g., hair brush on the scalp, spectacles on pain, when exposed to light stimuli that are the nose/ears), (5) tinnitus: perception of not bothersome to others; and (4) “nyctalopia”: noise (e.g., hissing) or “ringing” in the ears; difficulty seeing well at night, likely due to VS (6) balance problems: difficulty maintaining body interference/overlay rather than to an actual position/stability; and (7) tremor: involun tary, retinal problem or retinal disease. In addition, unintentional, rhythmic movements of the body determining provoking environments may (e.g., hands). In addition, migraine is included help the patient emphasize and reinforce such as a possible comorbid but not a causative “avoidance”. Lastly, treatment is a rapidly condition that may exacerbate the perception evolving and exciting area, especially for of VS.8 Since some believe that VSS represents the neuro-optometrist. Treatment has fallen a general neurological “hypersensitivity” into three categories: (1) filters to reduce the phenomenon,7,12,13 then several of the above perception of the VS intensity (e.g., transmission conditions would be logical to be present (e.g., peaking in the yellow-blue spectrum);9 (2) drugs hyperacusis, cutaneous allodynia, tinnitus). to reduce the VS intensity (e.g., lamotrigine);7 The last two symptoms of balance problems and (3) simply discussing the condition with the and tremor above should be carefully probed, patient to explain that VS is a real phenomenon especially if there is no past history of head which some individuals experience, as well as to trauma or neurological disease. 77 Vision Development & Rehabilitation Volume 5, Issue 2 • June 2019 Table 2. Patient Demographics case histories. In addition, the group data Age tested: 11-35 years have been summarized in Tables 2-6. Age VS first noticed: 11-29 years Gender 7F/3M Case One: Transient Idiopathic VSS VS Constant (C) or Transient (T): 7C/3T This was a 31-year-old, female profes- Etiologies of VS sional student. Her comprehensive optometric • Concussion examination revealed an accommodative insuf- • Auto-immune disease ficiency and versional oculomotor dysfunction, • Brain cyst surgery for which she was prescribed conventional • Antibiotic usage oculomotor-based vision therapy. At a later • Finesteride
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