SPECIAL FEATURE

medical literature has had very little to Vertical Heterophoria: New say about it since then.1 More than a century ago, G.T. Stevens claimed to have successfully treated vertical het- Discoveries Enable Treatment erophoria with surgical intervention (extraocular muscle tenotomy), but of Many Associated Disorders his results have not been reproducible. Mark S. Rosner, MD, and Debby Feinberg, OD VERTICAL HETEROPHORIA New work on vertical heterophoria has dramatically advanced { Causes both our understanding of the condition and our ability to treat it. — Congenital — Acquired brain injury { Classic Symptoms n 2006, Penelope, a fabrics engineer, Vertical Heterophoria: A Brief — Headache was critically injured in a car accident History — Neck ache in which she suffered a significant A form of binocular vision dysfunc- — Dizziness Itraumatic brain injury. One year into tion, vertical heterophoria is a visual — Anxiety her recovery, she continued to expe- condition in which the line of sight — Difficulty reading rience a myriad of symptoms, includ- from one eye is higher than the line of { Typical physical sign ing headache, dizziness, nausea, neck sight from the other eye when at physi- — Head tilt pain, eye strain, fatigue, and anxiety. ologic rest (an ocular posture created { Diagnostic testing She found it difficult to walk or drive; by disrupting fusion with a Maddox — VHSQ she also had trouble focusing, reading, Rod or prism). Due to this alignment — Prism challenge and writing. The worst, however, was challenge, patients with vertical hetero- { Treatment that the symptoms did not improve, phoria have marked difficulty main- — Vertical prismatic lenses even though Penelope received the full taining a binocular image. Two com- — Progressive prescription range of treatments typically given to pensatory mechanisms employed by patients with traumatic brain injury. these patients to facilitate fusion in- By the time Penelope was referred clude the overuse of the elevator and In the 1950s, Raymond Roy, OD, to us in 2008, she had been through depressor extraocular muscles and the successfully diagnosed and treated speech therapy, occupational ther- tilting of the head (Figure 1). The extra- vertical heterophoria using prolonged apy, physical therapy, and even vision ocular muscle overuse leads to strain monocular occlusion to delineate the therapy without gaining more than and fatigue, which causes many of their direction of vertical misalignment. He marginal relief from her symptoms. other symptoms. The head tilt leads to utilized much smaller than standard She received a comprehensive vision chronic neck pain. units of prism in his spectacle lens evaluation, was diagnosed with verti- Although vertical heterophoria was prescriptions.2 Since then, very little cal heterophoria, and was prescribed first described in the 19th century, the research has been published on verti- vertically realigning prismatic spec- cal heterophoria. tacle lenses. In a matter of days, her symptoms were markedly reduced. New Correlations Within one week, her occupational The most common form of vertical therapist and neuropsychologist noted heterophoria is congenital, and it is at- significant improvements. tributable to facial or orbital asymme- At this point, we have seen more try. To date our practice has seen over than 1,000 patients similar to Penelope. 4,000 of these patients. Symptoms can What these cases have in common is occur at any age, but the average age of a history of brain injury, a complex symptom onset is about 40, which is also group of symptoms, and minimal the time at which normal age-associ- improvement to multiple treatment ated skeletal muscle weakening begins. modalities. These cases also share one It is not widely recognized that ver- other thing: successful treatment with tical heterophoria can be precipitated vertically realigning prismatic lenses. by brain injury (including traumatic, All of these patients had vertical het- FIGURE 1 Head tilt characteristic of ischemic, or hypoxic) due to a cere- erophoria, which we now know can vertical heterophoria. (Photo courtesy of brovascular accident, near-drowning, the authors.) produce a wide range of symptoms. asphyxia, or near-exsanguination.

REFRACTIVE EYECARE® | FEBRUARY 2012 5 ©2012 Ethis Communications, Inc. Do Not Reproduce. We have recently described our ob- tically misaligns the visual axes of the vertical heterophoria. servation that vertical heterophoria two eyes.3 The specific locus or loci We confirm the diagnosis with and traumatic brain injury are cor- of the brain injury responsible for a technique we have named Prism related, our hypothesis being that these symptoms has yet to be identi- Challenge, in which the examiner brain injury leads to the generation fied, though. Vertical heterophoria is uses a trial frame to incrementally of an aberrant neural signal that ver- often unrecognized in these patients add small units of vertical prism to since the symptoms, as in Penelope’s the baseline prescription, until the pa- TABLE 1 Symptoms of vertical case, overlap significantly with those tient’s vertical heterophoria-associated heterophoria (by symptom category). of traumatic brain injury. symptoms are significantly reduced Blue lettering indicates traditional Up to now, vertical heterophoria (Figure 3). Patients usually recognize vertical heterophoria symptoms. has been associated almost exclusively a significant reduction of symptoms 20 PAIN SYMPTOMS with visual symptoms, although some to 30 minutes after applying the appro- ■ Headache would include headache on the list. priate prismatic lens prescription. We ■ Face ache / “sinus” pain Based on our experience, patients with Eye pain or pain with eye movements Vertical Heterophoria Symptom Questionnaire (VHSQ) ■ vertical heterophoria can have a wide Symptom Domains Headache: questions 1 and 2 Anxiety: questions 9, 10, and 12 Binocular vision: questions 14-18 Standard vision: questions 20-23 Dizziness: questions 4-8, 11 Neck ache / head tilt: questions 3 and 13 range of symptoms in seven major do- Reading: questions 19, 24, and 25 HEAD TILT SYMPTOMS Directions For each of the following questions, Always = Every day please check the answer that best describes your Frequently = At least 1 time / week situation. If you wear glasses or contact lenses, answer Occasionally = Less than 1 time / week the questions assuming that you are wearing them. Never = Never

■ Neck ache and upper back pain due to a mains (Table 1). The specific combi- ALWAYS FREQUENTLY OCCASIONALLY NEVER head tilt 1. Do you have headaches and/or facial pain? nation of symptoms, the severity, and Draw in location of discomfort (Scale 1-10: 1 = extremely mild, 10 = extremely severe) DIZZINESS SYMPTOMS the frequency can vary widely from ■ Dizziness patient to patient. FACE BACK OF HEAD ■ Lightheadedness 2. Do you have pain in your eyes with eye movement? 3. Do you experience neck or shoulder discomfort? 4. Do you have dizziness and/or lightheadedness? ■ Off-balanced feeling 5. Do you experience dizziness, light-headedness, or nausea while performing close-up activities (eg, Testing for Vertical Heterophoria computer work, reading, writing)? ■ Motion sickness (frequently the first 6. Do you experience dizziness, light-headedness, or nausea while performing far-distance activities (eg, driving, television, movies)? Traditional vertical heterophoria 7. Do you experience dizziness, light-headedness, or nausea when bending down and standing back up, or symptom of vertical heterophoria—can when getting up quickly from a seated position? occur very early in childhood) tests include both dissociated phoria 8. Do you feel unsteady with walking, or drift to one side while walking? 9. Do you feel overwhelmed or anxious while walking in a large department store (eg, Target, Wal-Mart)? ■ Nausea 10. Do you feel overwhelmed or anxious when in a crowd? tests (Von Graefe, Maddox rod, and 11. Does riding in a car make you feel dizzy or uncomfortable? ■ Poor depth perception 12. Do you experience anxiety or nervousness because of your dizziness? vertical vergence testing) and asso- 13. Do you ever find yourself with your head tilted to one side? 14. Do you experience poor depth perception or have difficulty estimating distances accurately? ■ Lack of coordination 15. Do you experience double / overlapping / shadowed vision at far distances? ciated phoria tests. Our unpublished 16. Do you experience double / overlapping / shadowed vision at near distances? ■ Unsteadiness or drifting to one side while 17. Do you experience glare or have sensitivity to bright lights? walking data indicates that the Von Graefe pho- 18. Do you close or cover one eye with near or far tasks? 19. Do you skip lines or lose your place while reading (do you use your finger or a ruler or other guides to maintain your position on the page)? ■ Difficulty walking down grocery aisle ria far, Von Graefe phoria near, and 20. Do you tire easily with close-up tasks (eg, computer work, reading, writing)? 21. Do you experience blurred vision with far-distance activities (eg, driving, television, movies, chalkboard ■ Disorientation at school)? vertical vergence tests are often inac- 22. Do you experience blurred vision with close-up activities (eg, computer work, reading, writing)? 23. Do you blink to “clear up” distant objects after working at a desk or working with close-up activities (eg, computer work, reading, writing)? READING SYMPTOMS curate and produce contradictory re- 24. Do you experience words running together with reading? 25. Do you experience difficulty with reading or reading comprehension? ■ Difficulty with concentration sults, making it difficult, or impossible, Scoring: Always = 3; Frequently = 2; Occasionally = 1; Never = 0. The total score is obtained by summing the individual scores. ■ Fatigue with reading In those 14 or older, a score of > 15 is suggestive of a diagnosis of vertical heterophoria. to determine the prism prescription. ©2004–2012 Vision Specialists of Michigan ■ Difficulty with reading and reading comprehension The patient’s physical appearance FIGURE 2 Vertical heterophoria symp- ■ Skipping lines while reading (head tilt, asymmetrical face, and uni- tom questionnaire (VHSQ). Find a full-sized, downloadable PDF of this questionnaire at ■ Using a line guide (finger, ruler, envelope) lateral furrowed brow) and history www.refractiveeyecare.com. to maintain one’s place while reading may suggest the presence of vertical ■ Words running together while reading heterophoria. To help identify key ele- ■ Losing one’s place while reading ments in the history, and to quantify a find that those who respond to Prism ROUTINE VISUAL SYMPTOMS patient’s symptoms, we have developed Challenge almost always do well at the ■ Blurred vision at near or far distances the 25-question Vertical Heterophoria end of the prism treatment process. ■ Difficulty with close-up vision (ie, reading or computer use) Symptom Questionnaire (VHSQ), a ■ Difficulty with night vision validated self-administered survey in- Differential Diagnoses ■ Eye strain strument. The VHSQ includes ques- The differential diagnosis of ver- ■ Sore eyes tions from all seven major symptom tical heterophoria is broad and in- BINOCULAR VISION SYMPTOMS domains (head and eye pain, dizziness, cludes conditions such as migraine ■ Double or overlapping vision anxiety, head tilt or neck pain, read- or muscle tension headaches, idio- ■ Shadowed vision ing difficulties, binocular vision symp- pathic or psychogenic dizziness, fre- ■ Light sensitivity ■ Difficulty with glare or reflection toms, and routine vision symptoms) quent non-purulent sinusitis, anxiety ■ Closing / covering one eye while reading and asks the patients to assess the fre- or panic disorder, ADD/ADHD, read- quency of their symptoms (Figure 2). ing or learning disability, chronic neck PSYCHOLOGICAL SYMPTOMS ■ Feeling overwhelmed or anxious in crowds Scoring is accomplished by summing pain and convergence insufficiency ■ Agoraphobia the values for all responses, with the (Table 2). Patients who have been ■ Feeling overwhelmed or anxious when maximum score possible 75 and the treated for these diagnoses with mini- in large contained spaces like malls or big minimum 0. For those 14 years and mal improvement may have vertical box stores older, a score of > 15 is suggestive of heterophoria.

6 FEBRUARY 2012 | REFRACTIVE EYECARE® ©2012 Ethis Communications, Inc. Do Not Reproduce. Treatment Looking Forward set of symptoms and the condition’s Although vision-care professionals Improved diagnosis and treat- relationship to brain injury. Similarly, are frequently advised to avoid or to ment of vertical heterophoria and patients must become familiar with be very sparing in their use of verti- its many associated symptoms will the symptoms of vertical heterophoria cal prism, we have found that, when come as clinicians (including both and learn to seek eye care when expe- used with proper technique, vertical eyecare and other healthcare profes- riencing those symptoms. To achieve prismatic lenses can be very effective sionals) gain familiarity with the full these goals, more research and educa- in reducing symptoms due to vertical tion will be critical. heterophoria. TABLE 2 Differential diagnosis of When treating vertical heteropho- vertical heterophoria (by symptom THE BOTTOM LINE category). ria with prism, patients are typically Vertical heterophoria, a condition unable to accept the entire required PAIN SYMPTOMS in which the two eyes are vertically Migraine headache prism in the first prescription; hence ■ misaligned, can be congenital or ■ Sinusitis we use a technique we have named precipitated by a brain injury and ■ TMJ ■ Chronic daily headache can manifest non-visual symptoms ■ TBI / Post concussion syndrome including headache, dizziness, neck ache, anxiety, and difficulty read- HEAD TILT SYMPTOMS ■ CN 4 lesion / SO palsy ing. Head tilt during normal upright ■ Scoliosis posture is a characteristic physical ■ Torticollis sign. Vertically realigning prismatic DIZZINESS SYMPTOMS lenses are an effective treatment for ■ Benign positional vertigo vertical heterophoria, with the prism ■ Menieres disease prescription slightly increased as the ■ Visual vertigo patient’s eye muscles progressively ■ Psychogenic dizziness relax. The VHSQ and Prism Chal- ■ Chronic subjective dizziness lenge technique can help establish CVA FIGURE 3 Patient wearing a trial frame, ■ the diagnosis and determine the in which small units of vertical prism ■ Neuromuscular weakness proper prismatic prescription. are incrementally added to her baseline ■ Brain tumor prescription. (Photo courtesy of the authors.) ■ TBI / Post concussion syndrome ■ Migraine associated vertigo Mark S. Rosner, MD, is adjunct Progressive Relaxation, which in- ■ Cervical vertigo clinical instructor in the ■ Superior semicircular canal dehiscence volves approximately three visits dur- Department of Emergency ing which the vertical prism prescrip- READING SYMPTOMS Medicine at the University of tion is increased in small steps as the ■ Reading or learning disabled Michigan Medical School and ■ ADD / ADHD patient’s eye muscles progressively re- Emergency Department staff physician at St. ■ Convergence insufficiency lax. The Progressive Relaxation process Joseph Mercy Hospital in Ann ■ Binocular vision abnormality Arbor, MI. Debby Feinberg, can take anywhere from 2 weeks to 3 ■ Astigmatism months to complete. Our experience ■ Hyperopia OD, specializes in diagnosis indicates that most vertical prism pre- ■ TBI / Post concussion syndrome and treatment of neurovisual conditions and is the owner of scriptions are about 1 D or less. ROUTINE VISUAL SYMPTOMS Patients on benzodiazepines, nar- ■ Myopia Vision Specialists of Michigan in Bloomfield Hills, cotics, muscle relaxants, or antidepres- ■ Hyperopia MI. They have no financial interests to disclose. sants may require additional time, as ■ Astigmatism Refractive Eyecare associate editor Ying Guo these medications can slow the prism BINOCULAR VISION SYMPTOMS assisted in the preparation of this manuscript. prescription process by interfering ■ CVA with either muscle strength or neuro- ■ Neuromuscular weakness References logical coordination of the two eyes. ■ Brain tumor 1. Stevens, GT. Functional Nervous Diseases. New York, NY:D. Appleton and Com- To obtain the full benefit of prismatic ■ TBI / Post concussion syndrome pany;1887,200-203. lenses, patients typically have to mark- PSYCHOLOGICAL SYMPTOMS 2. Roy RR. Ocular migraine and prolonged oc- edly reduce or completely eliminate ■ Anxiety clusion. Part 2. Optom Wkly.1953;44:1513-8. 3. Doble JE, Feinberg DL, Rosner MS, et al. their use of these medications. Partial ■ Psychogenic dizziness Identification of binocular vision dysfunction reduction of symptoms resulting from ■ Depression (vertical heterophoria) in traumatic brain the first prism prescription encourages ■ Agoraphobia injury patients and effects of individualized ■ Chronic subjective dizziness prismatic spectacle lenses in the treatment patients to take this step, in coopera- of postconcussive symptoms: a retrospective ■ TBI / Post concussion syndrome tion with their other doctors. analysis. PM R. 2010;2(4):244-53.

8 FEBRUARY 2012 | REFRACTIVE EYECARE® ©2012 Ethis Communications, Inc. Do Not Reproduce.