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SJOPT 530 No.

of Pages 5, Model NS

Saudi Journal of Ophthalmology (2017) xxx, xxx–xxx

2 Review article

5
4
6
Low vision rehabilitation: An update
7 Mark E. Wilkinson a,⇑; Khadija S. Shahid b

9 Abstract

10 This article provides information concerning issues related to the care of individuals who are visually impaired. Issues reviewed
11 include determining who should be referred for vision rehabilitation services, Charles Bonnet syndrome, visual acuity, contrast sen-
12 sitivity and visual field testing along with Useful Field of View testing. This article also discusses technology advances that can
13 enhance the visual functioning of individuals who are visually impaired, including how these advances can help drivers with visual
14 impairments to continue to safely operate motor vehicles, at least on a limited basis. Finally, resources that are available to both
15 encourage and motivate patients to take advantages of vision rehabilitation services are reviewed.
16

17 Keywords: Visual impairment, Low vision rehabilitation, Charles bonnet syndrome, Useful field of view
18

19 Ó 2017 Production and hosting by Elsevier B.V. on behalf of Saudi Ophthalmological Society, King Saud University. This is an open
20 access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
21 https://doi.org/10.1016/j.sjopt.2017.10.005
22

23 Introduction vision loss is not correctable by standard glasses, contact 40

lenses, medicine, or surgery. 41

24 In the past, low vision was defined by visual acuity of 20/70 Low vision rehabilitation should be considered part of the 42

25 (6/21) or less.1–3 The problem with this numeric definition is continuum of eye care that includes refractive, medical and 43

26 that it did not take into account the functional problems surgical eye care, which begins at birth and carries forward 44

27 many individuals with better than 20/70 vision have with con- throughout life. The goal of vision rehabilitation is to maxi- 45

28 ditions that cause glare and/or contrast loss that are not evi- mize an individual’s functional vision. In so doing, the individ- 46

29 dent during high contrast visual acuity testing routinely ual’s functional potential will be enhanced, resulting in 47

30 performed by eye care providers. As a result of this, the increase independence and improved quality of life. 48

31 National Eye Institute adopted a functional definition of low Vision rehabilitation often requires a team approach. The 49

32 vision.4 Based on this functional definition, low vision rehabil- vision rehabilitation team may include, but is not limited to, 50

33 itation care is more inclusive now then in the past, encom- medical, optometric, allied health (Occupational Therapist/ 51

34 passing the management of individuals of all ages, who Physical Therapist), social, educational/rehabilitative, mobil- 52

35 have a congenital or acquired impairment of visual acuity ity and psychological services. Potential additional team 53

36 and/or visual field and/or other functionally disabling factors, members may include psychologist, speech and hearing spe- 54

37 in the better seeing eye, in which the loss of vision interferes cialist, nurse/nurse educator and adaptive/technology con- 55

38 with the process of learning, vocational or avocational pur- sultant. The vision rehabilitation team is lead by the vision 56

39 suits, social interaction, or the activities of daily living. This rehabilitation doctor – an optometrist or ophthalmologist 57

Received 22 February 2016; received in revised form 17 October 2017; accepted 18 October 2017; available online xxxx.

a
University of Iowa Carver College of Medicine, Department of Ophthalmology & Visual Sciences, Director, Vision Rehabilitation Services, Wynn Institute
of Vision Research, 200 Hawkins Drive, Iowa City, IA 52242, United States
b
University of Iowa Carver College of Medicine, Department of Ophthalmology & Visual Sciences, 201 Hawkins Drive, Iowa City, IA 52242, United States

⇑ Corresponding author.
e-mail address: mark-wilkinson@uiowa.edu (M.E. Wilkinson).

Peer review under responsibility Access this article online:


of Saudi Ophthalmological Society, www.saudiophthaljournal.com
King Saud University Production and hosting by Elsevier www.sciencedirect.com

Please cite this article in press as: Wilkinson M.E., Shahid K.S. Low vision rehabilitation: An update. Saudi J Ophthalmol (2017), https://doi.org/10.1016/j.
sjopt.2017.10.005
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2 M.E. Wilkinson, K.S. Shahid

58 (OD, MD, DO) with special training and interest in the care of associated with more concerning neurological diseases such 117

59 individuals who are visually impaired. as Alzheimer’s, Parkinson’s, or psychosis which will have asso- 118

ciated sounds or smell.10 119

The cause of CBS is unknown. Zuckerman and Cohen11 120


60 Who needs vision rehabilitation services?
reported that 19% of normal individuals experienced visual 121

5 hallucinations during sensory deprivation experiments. Com- 122


61 Roy G. Cole, OD developed the following simply screen-
mon factors associated with CBS are sensory deprivation 123
62 ing protocol for determining who needs vision rehabilitation
(bilateral vision loss), social isolation, advanced age (mean 124
63 services. The following series of questions allows rapid
age 75.7 years) and the experience of a recent loss of 125
64 screening of individuals to determine if they would benefit
vision.12 126
65 from vision rehabilitation services.
It is important to know about CBS because it occurs in up 127

66  Do you have trouble doing what you want to do because to 38% of patients with age-related macular degeneration.13 128

A study done at the Henry Ford Health System Vision Reha- 129
67 of your vision? For example:
bilitation Research Center found that those experienced 130
68 – Reading your mail?
CBS images initially do not admit to them when ques- 131
69 – Watching television?
tioned.13 Yet, all patients welcomed validation of their expe- 132
70 – Recognizing people?
rience and the opportunity to describe their images when 133
71 – Paying your bills?
subsequently questioned. 134
72 – Signing your name?
Many patients choose to keep their experience of seeing 135
73
74 – Walking stairs, curbs, crossing the street or driving?
objects they know are not real concealed, for fear others 136
75  During the past month, have you often been bothered by:
would believe they were mentally compromised or develop- 137
76 – Feeling down, depressed or hopeless?
ing dementia. With this in mind, Menon suggested the use of 138
77 – Having little interest or pleasure in doing things?
indirect or direct questioning to detect CBS14:
78
139
79

80 These last two questions are 90% effective in detecting – (Indirect question) Apart from blurred vision, have you 140
81 depression.6 It is important to be aware that depression is noticed anything unusual about your vision? Have you 141
82 not uncommon among the elderly in general. Up to 3% expe- had any unusual visual experiences? 142
83 rience major depression, with another 8–16% experiencing – (Direct question) It is well known that some people with 143
84 clinical depressive symptoms.7 However, the risk of depres- blurred vision can sometimes see things that they know 144
85 sion in those with vision loss increases significantly, with some are not real. Have you experienced anything like this? 145
86 studies suggesting there is a 4-fold increase in developing 146
87 depression in those with vision loss.8 Reported visual hallucinations should not be disregarded 147
88 If the answer to any of the above 8 questions is ‘‘yes,’’ and altogether, because they can signal the presence of undiag- 148
89 these difficulties cannot be ameliorated refractively, medi- nosed organic pathology (tumor or lesion), untreated mental 149
90 cally and/or surgically, the patient should be referred for disorder and/or possible substance abuse. Finally, it is impor- 150
91 additional vision care and/or low vision rehabilitation services tant to know that a reduction in visual acuity alone cannot be 151
92 and/or counseling, education and/or problem-solving ther- the sole source of CBS because not all individuals who are 152
93 apy services. visually impaired have hallucinations.15 153

Currently, there is no effective treatment for CBS. For 154

94 History taking most, management that includes physician recognition, 155

empathy, reassurance and patient education are enough to 156

95 An often-unrecognized issue experiencing by individuals help the patient and form the cornerstone of treatment for 157

96 with vision loss is the phantom vision condition known as CBS. When patients are increasingly affected by CBS, a refer- 158

97 Charles Bonnet Syndrome (CBS), a condition that may repre- ral for psychological counsel can help as well as addressing 159

98 sent a type of release or deprivation phenomenon in those social factors since we know isolation can affect the occur- 160

99 with sudden, and, or severe, acquired vision loss. Bonnet first rence. Pharmaceutical agents are rarely effective.15 161

100 described CBS in 1760s when he noted the symptoms in his


101 visually impaired grandfather.9 Core features of CBS include
102 vivid and complex hallucinations that are usually recognized
103 as unreal by the patient and occur in the absence of any other Visual acuity testing (Distance and Near) 162

104 psychiatric syndrome. Images that have been described by


105 patients include dwarf people, animals, plants, buildings Accurately measuring visual acuity is important for deter- 163

106 and scenery. These images may be static or moving. The mining best-corrected acuity with refraction; monitoring the 164

107 images may have no personal meaning and last for a few sec- effect of treatment and/or progression of the disease, and 165

108 onds to most of a day and can occur for a few days to several to estimate the dioptric power of optical devices necessary 166

109 years. Often the images may change in frequency and com- for reading regular size print. Additionally, visual acuity test- 167

110 plexity. For some patients, the onset of visual hallucinations ing is used to verify eligibility for tasks such as driving and to 168

111 can be distressing without knowledge that this is a known verify eligibility as ‘‘legally blind.’’ Inaccurately measuring 169

112 association of vision loss. Therefore, direct questioning, edu- visual acuity underestimates ability. 170

113 cation, and reassurance are important when treating patients It is important to realize that when we test visual acuity, we 171

114 with vision loss at risk for CBS. Others describe the images as are only quantifying the degree of high contrast vision loss. 172

115 interesting. The images are exclusively visual, making no Visual acuity testing does not tell us about the individual’s 173

116 noise and causing no other sensations, unlike hallucinations quality of vision. A person’s quality of vision is determined 174

Please cite this article in press as: Wilkinson M.E., Shahid K.S. Low vision rehabilitation: An update. Saudi J Ophthalmol (2017), https://doi.org/10.1016/j.
sjopt.2017.10.005
SJOPT 530 No. of Pages 5, Model NS

Low vision rehabilitation 3

175 by contrast sensitivity testing, which will be discussed later in ing collisions and falls.19,20 Additionally, with improved light- 233

176 this article. ing, individuals with vision loss have been found to have 234

177 When measuring visual acuity, if you must use ‘‘counts fin- increased well-being.21 With this in mind, it is important to 235

178 gers’’, it is important to document what the testing distance discuss task lighting with all patients who are visually 236

179 was. Even better, if the patient can see fingers, they can read impaired. 237

180 the larger numbers or letters on a low vision eye chart. If your
181 office does not have an ETDRS (acuity testing to 20/800) or a Visual field testing 238
182 Feinbloom chart (acuity testing to 1/700 = 20/14,000) for
183 measuring lower levels of visual acuity, now is the time to It is important to use the right test when doing visual field 239
184 acquire one. testing on individuals with vision loss. Goldmann perimetry is 240
185 For near acuity testing, M-unit is the only letter size unit still considered the best testing strategy for individuals that 241
186 that is well defined.16 A 1 M letter subtends 50 of arc at 1 are visually impaired.22,23 However, Goldmann perimetry is 242
187 m, versus a 20/20 Snellen letter, which subtends 50 of arc at not readily available in most locations and requires trained 243
188 6 m (20 feet). Near acuities are recorded as M units at test technicians to perform. With this in mind, to quickly screen 244
189 distance (e.g. 1.25 M @ 40 cm or 1600 ). M-unit near acuity test- a person’s visual fields for unrecognized peripheral defects, 245
190 ing is useful for easily determining how much magnification is confrontation testing is still of value. It is also a useful educa- 246
191 needed for a patient to read a specific size print. For exam- tional tool for individuals with central loss, to demonstrate 247
192 ple, if a patient can read 4 M print at 40 cm, and they want that their periphery vision is still normal. 248
193 to read 1 M sized print, they will need to use a 4 magnifier Automated perimetry is the mainstream testing strategy 249
194 or hold the reading material 4 closer (10 cm). Remember, at now employed by most eye care providers. It offers standard- 250
195 10 cm, the accommodative demand will be 10 diopters, an ized testing protocols with llongitudinal databases. However, 251
196 important consideration for adults with reduced accom- the problem with standardized databases is that threshold 252
197 modative abilities including but not limited to individuals with related visual field-testing over-estimates visual field loss 253
198 presbyopia and pseudophakia. for individuals who are visually impaired. This happens 254

because the individual with a visual impairment is compared 255

199 Refraction to individuals with normal visual fields. To get a more accu- 256

rate assessment of the extent of your patient’s peripheral 257

200 The cornerstone and starting point for all vision rehabilita- visual fields, consider using the SSA Kinetic testing protocol 258

201 tion care is a careful, often trial frame based, refraction. The on the Humphrey Visual field analyzer or do kinetic testing 259

202 indications for prescribing spectacles for individuals with with an Octopus automated perimeter. 260

203 reduced vision include when the patient sees a qualitative


204 improvement in their vision with the RX; for intermediate Useful field of view testing 261

205 needs such as writing, sewing, using a video magnifier, a


206 computer or a tablet/smart phone; and/or to facilitate the The Useful Field of View (UFOV) test is a specialized visual 262

207 use of optical devices. field test used to determine how well an individual is able to 263

process both central and peripheral visual information and 264

can be specifically used to predict driving performance in 265


208 Contrast sensitivity testing patients who are visually impaired.24–29 It differs from other 266

tests of peripheral visual function by incorporating measures 267


209 Contrast sensitivity testing provides the clinician with a of reaction time, stimulus localization, simultaneous central 268
210 longitudinal measurement of visual function beyond visual and peripheral visual tasks (multitasking), target identifica- 269
211 acuity. Additionally, it provides the vision rehabilitation clini- tion, and complex decision making. The UFOV test provides 270
212 cian with diagnostic/functional information concerning which a means of evaluating a driver’s ability to perform multiple 271
213 eye has better functional vision, not just better high contrast tasks accurately and quickly as they relate to the task of safely 272
214 vision as is measured with standard acuity testing. Contrast driving. Studies have determined that UFOV testing corre- 273
215 sensitivity testing helps to establish binocular potential and lates well with driving performance.24–29 The authors find 274
216 will also show the effects of corneal opacities or cataracts UFOV testing very helpful when there are concerns about 275
217 on visual function. Finally, contrast sensitivity testing is an safe driving with cognitive decline. 276
218 excellent tool for patient/family education because it
219 explains so many of the patient’s visual difficulties/com-
220 plaints, which are not explainable by high contrast distance
Vision enhancement options 277

221 acuity testing alone.


Magnification is the main treatment option for enhancing 278
222 The Mars letter contrast sensitivity test is considered the
the visual functioning of individuals with vision loss. There 279
223 test of choice for measuring contrast sensitivity in individuals
are 4 types of magnification individuals with visual impair- 280
224 who are visually impaired.17,18 The Mars test consists of a set
ments employ to enhance their visual abilities.30 281
225 of 3 charts (OD/OS/OU) that are viewed at 50 cm (20 in.). On
226 these charts, each letter fades by 0.04 log units. Norms have
1. Relative distance magnification – by holding the materials 282
227 been established for different levels of contrast loss from pro-
closer to the eye, they appear bigger. Children with visual 283
228 found, to severe, moderate, and normal for those both above
impairments do this naturally. An adult will require the 284
229 and below 60 years of age.
appropriate powered reading correction or bifocal for 285
230 When a person’s contrast sensitivity function is reduced,
this to work efficiently, due to limited accommodative 286
231 they will require increased illumination for activities of daily
abilities. 287
232 living, as well as for reading, recognizing objects and avoid-
Please cite this article in press as: Wilkinson M.E., Shahid K.S. Low vision rehabilitation: An update. Saudi J Ophthalmol (2017), https://doi.org/10.1016/j.
sjopt.2017.10.005
SJOPT 530 No. of Pages 5, Model NS

4 M.E. Wilkinson, K.S. Shahid

288 2. Angular magnification – occurs when using a low vision deficits, while others will demonstrate significant perfor- 346

289 device, such as a hand-held magnifier or telescope. mance deficits when behind the wheel. Similarly, some dri- 347

290 3. Electronic magnification – is available in hand held, desk or vers may be able to drive safely under certain conditions 348

291 arm mounted electronic magnification devices, computer (e.g., driving locally to navigate their immediate neighbor- 349

292 software, as well as built in accessibility options on smart hood for shopping and/or to travel to medical and other 350

293 phones and tablets. Electronic magnification can make appointments) but may be hampered by other situations 351

294 the image both larger and with greater contrast. (e.g. dense urban traffic, unfamiliar environments, night driv- 352

295 4. Relative size magnification – makes the object larger, such ing, poor weather). The use of restricted driver’s licenses has 353

296 as with large print materials. The problem with large print been adopted in some areas as a solution to these situations 354

297 is that it is not readily available in the myriad of materials to aide in the maintenance of independent travel when safe 355

298 that individuals with a visual impairment need to read on a and possible.32,33 356

299 regular/daily basis (i.e. bank statements, bills, most other Now that cost-efficient, talking Global Positioning System 357

300 general mail, work related materials, etc.). (GPS) devices are available in the marketplace, consideration 358

301 should be given to recommending these devices to older dri- 359

302 Task lighting continues to be the single most important vers in general, and drivers with visual impairment in particu- 360

303 factor in enhancing visual functioning. A study done by Silver lar. Individuals using a talking GPS device are freed from the 361

304 found that more than 90% of individuals with vision loss distraction that takes place when a driver spends time look- 362

305 showed some improvement in near or distance visual acuity ing for/at road signs, particularly in more complicated driving 363

306 when the illumination was improved.31 environments. 364

Finally, with adaptive cruise control, lane alert warnings 365

and cars that will park themselves already available, it can 366
307 Technology
be expected that continued advances in automobile tech- 367

nologies will allow all drivers to be safer behind the wheel. 368
308 Technology advancements over the past decade have
Eye care providers have a moral and ethical obligation to 369
309 removed significant barriers for all individuals with vision loss,
report a patient who is at high risk for a motor vehicle acci- 370
310 allowing them to engage in activities that would have been
dent in order to preserve both patient and public safety. This 371
311 impossible in the past. An added advantage of these technol-
should remain standard even when working in areas where 372
312 ogy advances is that they are used by individuals with and
reporting such risk is not mandatory.34 Additionally, there is 373
313 without vision loss and so don’t stigmatize users who are visu-
the Duty to Warn,35 a legal rational intended to provide a 374
314 ally impaired. For example, despite their small screens and
means of protecting the patient from an unreasonable risk 375
315 keypads, several features built into smart phones and tablets
of harm. This rational indicates that failure to warn patients 376
316 make them easily accessible to users who are blind or visually
of conditions that create a risk of injury will be upheld as a 377
317 impaired. Leading the industry are Apple products that pro-
cause of action against eye care providers when it can be 378
318 vide easy accessibility to users with vision loss through their
shown that the failure to warn is the proximate cause of an 379
319 VoiceOver and Zoom programs.
injury.35 The patient can argue that they had insufficient 380
320 VoiceOver is a screen reader that uses text-to-speech to
warning of their impairment, and because of their impair- 381
321 read aloud what is onscreen, confirm selections, typed letters
ment, their operation of a motor vehicle or other machinery 382
322 and commands, and provide keyboard shortcuts to make
resulted in an injury. With this in mind, patients whose vision 383
323 application and web page navigation easier. The Zoom app
no longer legally qualifies them to operate a motor vehicle 384
324 magnifies everything onscreen from 1.2 to 15 times its origi-
should be warned not to drive and a notation to this effect 385
325 nal size, while maintaining their original clarity. Additional
should be entered into the patient’s record.32,33 386
326 options that increase accessibility are the ‘‘Large Text’’
The American Medical Association’s – Physician’s Guide to 387
327 option, that allows the user to select a larger font size (20–
Assessing and Counseling of Older Drivers (2nd Ed.)36 states 388
328 56 point) for any text appearing on their device.
that every physician, (the author would include all eye care 389
329 Many individuals with vision loss see better with the
providers in the category), should assess risk factors for their 390
330 reversed contrast setting of ‘‘White on Black’’. Reversing
older patients who drive. For those individuals at risk for 391
331 the contrast is often the only change needed to allow an indi-
unsafe driving, the practitioner should recommend a formal 392
332 vidual with a visual impairment to easily read on their phone
assessment of vision, cognition and motor skills and also refer 393
333 or tablet.
for a behind the wheel driving assessment when appropriate. 394
334 Finally, there are free and low-cost apps for smart phones
To appropriately advise patients with vision loss about 395
335 and tablets that can make them function like a hand-held
their driving status, it is important to know if your 396
336 video magnifier. Two of the authors’ favorites are the
patients are still driving. The following series of questions 397
337 Brighter and Bigger and Better Vision apps.
concerning driving are an easy way to determine patient 398

driving status. 399

338 Driving with a vision loss


– Do you drive an automobile? 400

339 There are many issues surrounding driving with a vision o If yes, what type of driving do you do? 401
402

340 loss. As our population continues to age, it is important to – Do problems with your sight cause you to be fearful when 403

341 note that there are large individual differences in the ability you drive? 404

342 to compensate for a visual impairment when driving. A num- – During the past six months, have you made any driving 405

343 ber of studies have demonstrated that similar visual impair- errors? 406

344 ments in groups will affect individuals different. Specifically, – Is your mobility affected by your vision? 407

345 some members will not manifest any driving performance


Please cite this article in press as: Wilkinson M.E., Shahid K.S. Low vision rehabilitation: An update. Saudi J Ophthalmol (2017), https://doi.org/10.1016/j.
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Please cite this article in press as: Wilkinson M.E., Shahid K.S. Low vision rehabilitation: An update. Saudi J Ophthalmol (2017), https://doi.org/10.1016/j.
sjopt.2017.10.005

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