ORIGINAL ARTICLE

Patient Awareness of Binocular Central Scotoma

in Age-Related Macular Degeneration
Donald C. Fletcher*, Ronald A. Schuchard

, and Laura W. Renninger

ABSTRACT
Purpose. To assess whether age-related macular degeneration (AMD) patients are aware of binocular central visual field defects.
Methods. One hundred fifty-three consecutive AMD patients in their initial low-vision rehabilitation evaluation were
immediately asked at the beginning of their visit (1) whether they were able to see any blind spots or defects in their field
of vision and (2) whether they had any evidence or experiences that led them to believe that they had defects in their field
of vision. They then had their vision assessed by binocular central visual field testing using the California Central Visual
Field Test, binocular reading performance evaluated using the Smith-Kettlewell Reading Test (SK Read) and MN Read charts,
and visual acuity measured using the ETDRS chart at 1 meter. Mean diameters of the scotomas with borders near fixation were
noted.
Results. Visual acuity median was 20/253 (range 20/40 to hand movements). Binocular scotomas were present in 88%
of patients (66% had dense scotoma). Of patients with binocular scotomas, 56% were totally unaware of their presence,
even with dense scotomas measuring up to 30 in diameter; 1.5% could fleetingly see a defect in their visual field on
waking; and 44% related experiences of things disappearing on them. The median and range of scotoma diameters for
those unaware vs. those with some awareness of their scotomas were comparable. There was no significant relationship
of awareness of the scotoma with age, acuity, scotoma size, density, or duration of onset. Awareness of scotoma was
associated with fewer errors on the SK Read (p 0.01).
Conclusions. Low vision clinicians cannot depend on patients to report the presence of significant scotomas; thus,
appropriate testing must be performed. Presence of scotomas decreased reading accuracy, but some awareness of the
scotomas had a tendency to improve accuracy. The value of rehabilitation programs aimed at increasing patient
awareness of their scotomas may be supported by this evidence.
(Optom Vis Sci 2012;89:13951398)
Key Words: scotoma, AMD, low vision, visual perception, reading
I
n the United States, the majority of patients referred for low-
vision rehabilitation have age-related macular degeneration
(AMD), and a well-documented feature of this disease is the
presence of scotomas in the central visual field. Dense scotomas are
delineated as areas in the field in which the brightest test stimuli go
undetected. Relative scotomas are found with dimmer stimuli and
can occur in the absence of dense scotomas. When scotomas occur
in the central field, they have a significant effect on reading perfor-
mance and detailed near visual activities.
1
Visual performance dif-
ficulties far exceed those expected from reduced visual function
alone (e.g., acuity and contrast sensitivity). Ninety percent of peo-
ple with low vision entering a vision rehabilitation service were
found to have a dense scotoma, and 80% of low-vision people had
a central scotoma partially or totally affecting foveal function.
2
When bilateral central scotomas affect the fovea, the development
of one or more eccentric preferred retinal loci (PRLs) occurs nat-
urally and reliably.
29
The term PRL is reserved for the retinal area
selected by the visual systemto act as a pseudofovea for visual tasks.
Overall, the PRL is immediately next to the scotoma in 86% of
low-vision eyes
2
; thus, the portion of visual stimuli that falls on the
scotoma side of the PRL may be obscured or distorted. Even indi-
viduals with good acuity often have a paracentral scotoma that
obscures text during reading, although the low-vision patient and
specialist are not aware that this is occurring. Thus, it is not sur-
prising that the ability to use the PRL to compensate for a central
scotoma is better correlated with performance in activities of daily
living than with standard visual function measures.
10
Further com-
plicating the issue, people with central scotomas can have multiple
PRLs, depending on the lighting environment
8
and visual
task.
11,12
The consequence of multiple PRL locations is that the
*MD

PhD
Department of Ophthalmology, California Pacific Medical Center, San Fran-
cisco, California (DCF), Smith-Kettlewell Eye Research Institute, San Francisco,
California (DCF, LRW), and VAPAHCS Rehabilitation R&D, Stanford Univer-
sity School of Medicine, Palo Alto, California (RAS).
1040-5488/12/8909-1395/0 VOL. 89, NO. 9, PP. 13951398
OPTOMETRY AND VISION SCIENCE
Copyright 2012 American Academy of Optometry
Optometry and Vision Science, Vol. 89, No. 9, September 2012
scotoma position and interference can also vary with task and
lighting conditions. Finally, the central scotomas are often asym-
metrical between the two eyes; thus, the patient will have binocular
scotoma characteristics that differ fromeither monocular scotoma.
As people with scotomas normally perform visual activities with
both eyes open, it is the binocular scotoma characteristics that
must be compensated for during visual tasks.
Rehabilitation efforts are generally directed toward developing
skills in compensatory eye movements around scotomas and often
include eccentric viewing training when foveal function is lost. In
this rehabilitation education process, clinicians have frequently
noted that patients are not aware of the presence of their central
scotomas.
13,14
The common belief has been that the process of
perceptual completion is occurring for patients just as happens
with the physiological blind spot for all individuals.
1517
The pro-
portion of patients aware of their scotomas is not accurately known
and is certainly relevant to rehabilitation service delivery.
PURPOSE
This study was undertaken to assess whether AMD patients are
aware of binocular central visual field defects as they present for
low-vision rehabilitation. The study measures what proportion of
patients can see their scotomas, what proportion cannot see but are
aware of the defects, and what proportion are totally unaware of
the presence of binocular scotomas.
METHODS
Over a 4-month period, 153 consecutive AMD patients in their
initial low-vision rehabilitation evaluation were immediately asked
the following questions at the beginning of their visit: (1) Are you
able to see any blind spots or defects in your field of vision? and (2)
Have you had any evidence or experiences that lead you to believe
that you have defects in your field of vision? The questions were
asked before any testing was performed or any instruction that
might raise patient awareness of scotomas. The patients functional
history was completed, and then the usual battery of visual func-
tion testing was performed in The Frank Stein and Paul S. May
Center for Low Vision Rehabilitation at the California Pacific
Medical Center in San Francisco, California. All patients signed
Protected Health Information and Health Insurance Portability
and Accountability Act forms on admit to the clinic. Patient files
were mined retrospectively, with institutional review board ap-
proval from the California Pacific Medical Center.
A patients typical visual perception with everyday life activities
occurs with both eyes open. Any perception or awareness of their
scotomas would be expected to be noticed in their binocular visual
field. Thus, binocular central visual field testing is desirable over
monocular testing. The California Central Visual Field Test was
performed to identify dense and relative scotomas.
18
The test stim-
uli were presented as short flashes to the field (binocular viewing)
using red laser pointers that give a spot of light approximately 1
mm in diameter. The brightest/dense stimulus was provided by an
unfiltered laser, while the weaker/relative stimulus had a neutral
density filter that decreased the intensity to 10% of the bright/
dense stimulus. The stimuli were presented on a piece of white
paper in normal room lighting at a 57-cm testing distance (or 28.5
cm for large scotomas). At 57-cm test distance, 1 cm is approxi-
mately equal to 1 of visual field. The size and brightness of the
stimuli were matched across observers; however, the position and
timing of stimuli were adapted to map out individual scotoma
borders. Undetected presentations of the bright and dim stimuli
within 2.5 of fixation were recorded as dense and relative scotoma
areas, respectively, and the mean diameter of the defect was measured.
Visual acuity with the patients habitual correction was assessed
using the ETDRS chart at 1 meter, and the letter-by-letter scoring
system was used.
19
Binocular reading performance was evaluated
using the MN Read
20
and Smith-Kettlewell Reading Test (SK
Read) charts.
18
Patients read blocks of text to their limit of resolu-
tion, and the total number of errors divided by number of blocks
read was recorded. The MN Read assesses reading of continuous
text/sentences, whereas the SK Read uses the same format but uses
non-continuous text/unrelated words and letters. MN Read pres-
ents three lines of text in a sentence block structure, whereas SK
Read presents three lines of text in a non-continuous block of text.
The MN Read test is valuable in measuring reading speed vs. text
size, whereas the SK Read test is valuable in measuring reading
speed and reading errors vs. text size. Thus, the added value of SK
Read is predicting scotoma interference with reading, especially for
reading tasks without contextual clues such as telephone numbers
and bank statements. Accuracy on the SK Read is recorded as the
average number of errors per block, that is, total errors divided by
blocks of text read.
RESULTS
Patients median age was 84 years, with a range of 61 to 98
years; 67% were woman. All patients had AMD, with 48% having
dry and 52% having wet AMD. Of the wet AMD patients, 76%
FIGURE 1. When dense scotoma are present, relative scotomas
also also present and are often larger than the dense scotoma.
Plotted is the relationship between dense and relative scotoma
sizes as measured using the California Central Visual Field Test.
1396 Patient Awareness of Binocular Central Scotoma in AMDFletcher et al.
Optometry and Vision Science, Vol. 89, No. 9, September 2012
had received antivascular endothelial growth factor injection
treatments. The mean duration of AMD visual symptoms was 7
years, with a range of 0.5 to 23 years. Median visual acuity was
20/253, with a range of 20/40 to hand movements.
All patients were able to understand and complete the binocular
central visual field testing; however, no formal cognitive testing
was performed. Only 12% of patients did not demonstrate a bin-
ocular scotoma border within 2.5 of fixation. Monocular and
peripheral scotomas were not measured because they are less likely
to interfere with central vision and thus reach the awareness of the
patient. Sixty-six percent of patients had a binocular dense and
relative scotoma, and 22% had only a relative scotoma. Relative
scotoma mean diameter was 12.1, with a range of 0 to 30. Dense
scotoma mean diameter was 8.6, with a range of 0 to 30. In
patients with dense and relative scotomas, the relative scotoma was
equal to or larger than the dense scotoma (Fig. 1).
Fifty-six percent (75/134) of patients with dense and/or relative
binocular scotomas were totally unaware of the blind spot presence,
evenwithdense scotomas measuring upto30 indiameter. They were
aware of bad vision but could see no hole in their vision and were
not able to recall situations where things would disappear.
Two patients (of 134, 1.5%) could fleetingly see a defect in their
visual field. Each of these patients noted it on waking from sleep.
One described being able to see a dark spot on the ceiling when first
opening eyes in the morning. As soon as an upright posture and
movement was initiated, the dark spot would no longer be apparent.
The other patient described being able to see a dark area in the center
of her vision when awakening during the night and trying to navigate
to the bathroom before turning on the light. As soon as the light was
turned on, the missing area of vision was no longer noticed.
Forty-four percent (59 of 134) were not able to see consistent
missing areas of their vision but did relate experiences of things
occasionally disappearing on them. The most common report
was of words and letters disappearing while attempting to read.
Letters or numbers would disappear and reappear often causing
great aggravation. One very observant patient reported that while
attempting to step on a cockroach, the insect ran into an area of her
vision where it momentarily disappeared. She could not resume
trying to step on it until it reappeared out of its hiding spot!
Scotoma size was not useful in predicting scotoma awareness
(dense scotoma size: r
2
0.01, p 0.05; relative scotoma size:
r
2
0.02; p 0.05) because there was no significant relationship
in the size of scotomas (dense or relative) between patients who
were totally unaware and those who had some awareness of their
scotomas (Fig. 2). In addition, there were no significant relation-
ships (p 0.05) between scotoma awareness and (1) the location
of the scotoma relative to fixation or PRL (right, left, and up/
down), (2) visual acuity, and (3) duration of vision loss. There was
a significant (p 0.01) relationship between the scotoma aware-
ness and the age of the patients, but the predictive ability was low
(r
2
0.05). The mean age of the patients who were aware of their
scotoma was 81.5 years (95% confidence interval: 79.883.2
years) and of those who were unaware of their scotoma was 84.5
years (95% confidence interval: 82.986.0 years), indicating a
significant, but very small, difference in the mean ages.
Presence of scotomas within 2.5 of fixation decreased reading
accuracy, but the location of the scotoma relative to the PRL was
inconsequential (p 0.05) to the overall accuracy measured using
the SKRead (Fig. 3). Some awareness of the scotomas significantly
(p 0.01) improved SK Read accuracy but not MN Read accu-
racy (p 0.05; Fig. 4). The SK Read mean error rate for the
unaware group was 5.4 (95% confidence interval: 4.46.5) errors
per block and for the aware group was 3.3 (95% confidence inter-
val: 2.14.4) errors per block. The SKRead mean error rate for the
no scotoma group was 1.0 error per block. Therefore, awareness of
FIGURE 2. There was no difference in scotoma sizes between
groups aware (Yes) and unaware (No) of their defect. RS,
relative scotoma; DS, dense scotoma.
FIGURE 3. Patients with no scotoma border within 2.5 of
fixation (N) had the lowest reading error rates. Patients with
dense and/or relative scotoma to the superior and inferior (V),
left (L), right (R), or surrounding fixation in a ring (O) all had
similar error rates.
Patient Awareness of Binocular Central Scotoma in AMDFletcher et al. 1397
Optometry and Vision Science, Vol. 89, No. 9, September 2012
scotoma was associated with fewer errors on the SK Read test,
although the relationship was not strong (r
2
0.06).
CONCLUSIONS
On initial evaluation, 88% of AMD patients referred for low-
vision rehabilitation demonstrated binocular scotomas near fixa-
tion, and more than one-half were totally unaware of their
presence. Increasing size of scotomas did not increase awareness as
might be expected, and it was noted that scotomas as large as 30 in
diameter could go undetected. Primary care ophthalmologists do not
typically measure central field defects and are thus unlikely to discuss
their functional implications with their patients. Thus, low vision
clinicians cannot depend on patients to report the presence of signif-
icant scotomas, and appropriate testing must be performed.
Presence of scotomas decreased reading accuracy independent
of acuity scores. Interestingly, some awareness of the scotomas had
a tendency to improve reading accuracy. The value of rehabilita-
tion programs aimed at increasing patient awareness of their sco-
tomas may be supported by this evidence.
ACKNOWLEDGMENTS
DCF developed the CCVFT and SK Read tests and receives a modest commis-
sion from its manufacturer, Mattingly Low Vision (mattinglylowvision.com).
This research was funded by the Pacific Vision Foundation (DCF), the Smith-
Kettlewell Eye Research Institute (DCF, LWR), and the Department of Vet-
erans Affairs Rehabilitation R&D Service (RAS).
This work was first presented as a 2011 ARVO conference abstract in Fort
Lauderdale, FL (Fletcher et al., Invest Ophthalmol Vis Sci 2011;52:E-
Abstract 4236).
Received January 10, 2012; accepted April 17, 2012.
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Laura Renninger
Smith-Kettlewell Eye Research Institute
2318 Fillmore St
San Francisco, California 94115
e-mail: laura@ski.org
FIGURE 4. Reading accuracy on the Smith-Kettlewell Reading
Test decreased for patients with scotoma awareness (p 0.01).
Yes, aware; No, not aware.
1398 Patient Awareness of Binocular Central Scotoma in AMDFletcher et al.
Optometry and Vision Science, Vol. 89, No. 9, September 2012