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State-of-the-Art

Rehabilitation for Visual Disorders


Susanne Trauzettel-Klosinski, MD
Downloaded from https://journals.lww.com/jneuro-ophthalmology by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3Bvi4U12RiFssoJm908BKlrfkpxfVq8p+FH/chJO7Pt9Ssh2DDACqeQ== on 05/21/2019

Abstract: Rehabilitation for visual disorders demands and the optimal prescription of glasses come first. Visual
thorough assessment of many components of vision and fields must also be determined accurately, because defects
a tailored strategy of maximizing residual function.
Magnification with optical or electronic aids and the involving the central field limit the size of the reading
use of eccentric fixation and specific reading training visual field (,5° from fixation). Visual field defects in
exercises are helpful techniques in patients with central the periphery can lead to orientation difficulties. To detect
scotomas. Visual exploration training is beneficial in small defects, one must use a dense grid or a thorough
patients with homonymous hemianopias. manual strategy. If perimetry cannot be performed with
Journal of Neuro-Ophthalmology 2010;30:73–84 a standardized instrument, bedside confrontation fields are
doi: 10.1097/WNO.0b013e3181ce7e8f useful to detect large field defects, especially hemianopias.
Ó 2010 by North American Neuro-Ophthalmology Society Tangent screen campimetry may also be used.
Contrast sensitivity testing is also critical. Contrast can
be improved by optimal illumination or by marking the

M any diseases of the eyes and visual pathways are


associated with persisting visual deficits that require
rehabilitation. There is an increasing demand for rehabil-
environment with special high-contrast landmarks.

itation for these disorders (1,2), particularly in view of READING


increased survival rates and prolonged life expectancy (3–5). In modern society, adults spend approximately 2.5 hours
A precondition for successful rehabilitation is an exact per day reading, especially during work activities. Approx-
assessment of visual impairments. The World Health imately 90% of all jobs require dealing with written material
Organization general classification of impairments, dis- (8). To read newspaper print at a distance of 25 cm, a visual
abilities, and handicaps (ICIDH) (6), later modified to the acuity of at least 20/50 (0.4) is necessary. Whereas visual
International Classification of Functioning, Disability and acuity testing depends on recognizing only 1 optotype at
Health (7) can be well adapted to the visual system (Fig. 1). a time, reading demands a simultaneous overview of a group
It considers 3 fields: 1) impairment, which assesses the of letters. The minimum reading visual field (9) is an area
pathologic condition and the function on the basis of the of approximately 2° to the right and left of fixation and
involved organ(s); 2) disability or activity limitation, which corresponds approximately to the ‘‘visual span’’ or ‘‘word
indicates the difficulties caused by the impairment; and 3) recognition span’’ (10,11). Within this area, letters are seen
handicap or participation restriction, which stands for the clearly. Figure 2A shows the functional and morphologic
resulting problems in the patient’s environment. data related to a fundus image. The ‘‘minimum reading
visual field’’ (turquoise oval) corresponds more or less to the
DIAGNOSTIC PROCEDURES IN area of the fovea (green oval).
VISUAL REHABILITATION Parafoveal information processing can extend the total
‘‘perceptual span’’ (‘‘reading visual field’’) during 1 fixation
Determination of visual acuity for distance and near
in the reading direction up to 15 letters (11,12) (Fig. 2B,
viewing, refractive error, and accommodative amplitude
red oval). This extended perceptual span provides in-
formation about word length, capitalization, and word
Low Vision Clinic and Research Laboratory, Center for Ophthal- shape, and offers a preview benefit, which is useful in
mology, University of Tübingen, Tübingen, Germany. guiding the next saccade to the appropriate landing
Address correspondence to Susanne Trauzettel-Klosinski, MD, Low position. For fluent reading, a total perceptual span of 5°
Vision Clinic and Research Laboratory, Center for Ophthalmology,
University of Tübingen, Schleichstrasse 12-16, D-72076 Tübingen, (15 letters) to the right and 1.3–2° (4–6 letters) to the left of
Germany; E-mail: susanne.trauzettel-klosinski@uni-tuebingen.de fixation is necessary, as shown in window experiments in

Trauzettel-Klosinski: J Neuro-Ophthalmol 2010; 30: 73-84 73


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FIG. 1. World Health Organization (WHO) classification of impairments, disabilities, and handicaps (ICIDH) (6) and of
functioning, disability and health (ICF) (7) adapted to visual rehabilitation. (Modified from Reference 45.)

normal subjects (12) and in patients with homonymous though there is a reciprocal mathematical relationship
hemianopias (13). The perceptual span is a dynamic between visual acuity and magnification need, in reality
parameter that is also influenced by top-down mechanisms, there is often a discrepancy.
such as visual attention.
During reading, the holding positions of the eyes Reading Speed
between saccades have a mean duration of 250 ms (14). Reading speed should be determined by having the patient
In normal subjects, eye movement recordings show a typical read a paragraph of text aloud. A whole paragraph of text
staircase pattern, a sequence of saccades and holding is preferable to a single sentence for more accurate speed
positions (Fig. 8A). Information processing occurs during measurement and judgment of fluency and mistakes. For
the holding positions. The retinal area used for reading this test, a newly developed set of equivalent texts in dif-
comprises only a few square millimeters but is highly ferent languages is available (18, http://www.amd-read.net).
magnified in the visual cortex (15). The central 10°
diameter of the visual field, which accounts for approxi- Fixation Behavior
mately 2% of the total visual field, is mapped onto nearly
Knowledge of fixation behavior is helpful if discrepancies
50% of the primary visual cortex (16,17).
between good visual acuity and impaired reading perfor-
mance arise (as in ring scotomas, see below). Clinical
ASSESSING READING ABILITY IN LOW methods of judging fixation are 1) determination of the blind
VISION PATIENTS spot in perimetry (Fig. 3C); 2) sighting the position of the
corneal reflexes when the patient looks at the examiner;
Refractive Error and 3) noting the fixation locus and motion of the eye
Exact determination of refractive error is necessary in low during direct ophthalmoscopy. Further methods are
vision patients. If visual acuity is #20/200, the measure- fixation photography and fixation behavior determined
ment should be performed by ETDRS charts because with the scanning laser ophthalmoscope (SLO) (Fig. 4).
they allow more steps in the low vision range by reducing
the distance. Measuring near visual acuity and range of Parafoveal Contrast Sensitivity Testing
accommodation are also important here. This testing provides valuable information on parafoveal
deficits (19,20), which can precede central visual loss (20).
Magnification
Assessing whether magnification would be helpful is Eye Movements
an important step. The smallest print size that can be read Recording of eye movements during reading is a valuable
fluently corresponds to the magnification need. Even method of showing ocular motor behavior during reading.

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FIG. 2. A. Reading-relevant morphologic and functional data on a fundus image. Visual acuity (yellow) decreases rapidly
with increasing eccentricity, as does cone density (dark blue). The proportions of the foveola (1°, green circle) and the
fovea (5° diameter, green oval) determine the minimum reading visual field (turquoise oval) of 2° to the right and left of
fixation and 1° above and below fixation. B. The data in A are related to a reading text. Because of the visual acuity curve
(yellow), only in the minimum reading visual field (turquoise oval) can the text be perceived clearly. The total perceptual
span (red oval) can be extended up to 5° (or 15 letters) in the reading direction by parafoveal information processing.
(Modified from References 22 and 47).

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FIG. 3. The minimum reading visual field (central square) related to the 30° visual field (upper panel) and a reading text
(lower panel) in a normal subject (A) and in a patient with a central scotoma (B–C). B. When the patient has an absolute
central scotoma and uses central fixation, the reading visual field is covered by the scotoma, so that reading is
impossible. C. When the patient has an absolute central scotoma and uses eccentric fixation, the scotoma is shifted
(here upwards) together with the blind spot, the reference scotoma (upper panel). The insufficient retinal resolution of
this eccentric retinal area (middle panel) can be overcome with text magnification (bottom panel). (Modified from
Reference 27.)

REHABILITATION FOR RETINAL AND OPTIC lower visual field remains free for spatial orientation on the
NERVE DISEASES page. However, not all patients show this favorable fixation
behavior. Some 20%–50% of patients shift the central
scotoma to the right or left of the normal fovea (23,27–29).
Central Scotomas It is hard to explain why such an unfavorable fixation locus
Patients who have central scotomas that cover the reading would be chosen. However, apart from the resolution at
visual field can learn to use eccentric fixation (21) in an a certain eccentricity, sustained focal attention, which
intact area of the visual field at the margin of the scotoma facilitates stimulus discrimination (19,30,31), influences the
(22–24). The new fixation locus becomes the new center of choice of a PRL location. Patients with good attentional
the visual field (25,26). This eccentric fixation locus is called capabilities in the lower visual field install their fixation
the ‘‘preferred retinal locus’’ (PRL), even though patients locus below the scotoma. If attentional capabilities are
often use more than 1 eccentric locus. The eccentric retinal reduced in the lower visual field, patients prefer a fixation
area used for reading does not have sufficient resolution to locus to the left or right of the scotoma (19,32,33).
read normal newspaper print, so that the ability to read can Patients who have established an eccentric fixation
be regained only by magnifying the text (Fig. 3). Eccentric locus can regain reading ability by text magnification.
fixation plus magnification of the text is the basis for the The spectrum of magnifying visual aids includes
effectiveness of magnifying visual aids in patients with handheld magnifiers, stand magnifiers, simple high-plus
a central scotoma (Fig. 4.). spectacles, and telescopic spectacles. Handheld and stand
The shift of the scotoma toward the upper visual field magnifiers have the advantage of a comfortable working
theoretically represents the most favorable situation for distance. When magnifying spectacles are used, the text
reading. The line of text becomes free for reading and the has to be moved much closer, especially when simple

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FIG. 4. Fixation of a target and of a line of text observed with the scanning laser ophthalmoscope in a normal subject
(A–B) and in a patient with a central scotoma due to Stargardt’s disease (C–D). (The text is seen as upside down only
for the examiner; it appears upright to the patient.) The patient can read the text with the eccentric retinal locus and
2.5-fold magnification.

high-plus spectacles are used. Telescopic magnifying and learning to use the optimal retinal locus. Concerning
spectacles allow a longer viewing distance, but they are this last component, several studies have reported posi-
cosmetically unfavorable. In patients with a magnification tive results (37,38), but there is considerable controversy
requirement of more than 8-fold, who have no experience about criteria and methods for choosing the optimal
with optical magnification, an electronic reading device, area (39).
such as a closed circuit television (CCTV) monitor,
should usually be chosen. It is important to provide Ring Scotomas
sufficient illumination without glare or ultraviolet or These defects may easily remain undetected because
infrared light (cold light source). It is helpful to be able visual acuity can be good. However, a discrepancy
to vary the brightness, which can be achieved by a simple between good visual acuity and impaired reading
dimmer switch. For far distance viewing, handheld performance often indicates a ring scotoma. The central
telescopes are useful. seeing island may be too small to include a sufficient
The success rate of magnifying visual aids for reading number of letters for fluent reading (Fig. 5, 5.2, 5.4, 5.8).
is high. In a cohort of 763 patients in our low vision If patients learn eccentric fixation for reading magnified
clinic, only 13% were able to read newspaper print before texts, they can regain reading ability.
consultation; 90% were able to do so afterwards. Patients
with a central scotoma (n = 293) showed a success rate Constricted Fields
of 94%. Those with age-related macular degeneration In degenerative retinal diseases and in the late stage of
(AMD) (n = 191) showed a success rate of 94% (34), glaucoma, visual fields are often constricted (Fig. 5, 5.3 and
confirmed in a recent study with 835 patients with 5.9). The central seeing island may be too small for reading,
AMD (35). and no peripheral visual field area is available. Reduction of
Training in reading involves proper handling of the letter size with contrast enhancement may be helpful. When
visual aids, reading exercises with the aim of enlarging the constriction limits the field to less than 30°, orientation
the perceptual span, optimizing eye movements (36), and mobility will be impaired.

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FIG. 5. The principal visual field defects in retinal, optic nerve, and bilateral occipital lobe lesions. The visual
rehabilitative approach is based on the functional effects of the field defect independent of its origin.

CHIASMAL DISORDERS Therefore, preexisting phorias easily develop into tropias. In


cases of preexisting esophoria or intermittent esotropia,
In patients with bitemporal hemianopia, the limited patients will experience a separation of the nasal hemifields,
temporal fields cause orientation problems. Depth percep- causing a blind area in the center of the field. Patients with
tion may be impaired, which leads to difficulties with preexisting exophoria or intermittent exotropia will have an
near distance tasks such as sewing, threading needles, or overlap of the 2 hemifields, and patients with preexisting
using precision instruments. In these cases, convergence hyperdeviations will experience a vertical separation of the
causes crossing of the 2 blind temporal hemifields, images crossing the vertical meridian (40). The hemifield
resulting in a completely blind triangular area posterior slide phenomenon can be especially disabling in reading
to fixation (40). long numbers in tables and bank statements.
Patients must be made aware of the hemifield slide
Hemifield Slide Phenomenon phenomenon to guard against misinterpretations of reading
Another problem is the hemifield slide phenomenon material. Monocular reading can be helpful in such cases.
(Fig. 6), which results from the lack of a normal overlap The use of a ruler can be a valuable aid to improve
of the nasal visual fields and which prevents fusion. navigation on the page.

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Retrochiasmal Disorders In ‘‘macular sparing,’’ the reading visual field may be


preserved and reading can be normal (Fig. 7B). Paracentral
In retrochiasmal lesions, the visual field defect is homon- homonymous scotomas typically cause severe problems
ymous, sometimes limited to a quadrant. It may spare or with reading because they obscure half of the reading visual
split the macular (fixational) region. When the lesion spares field (Fig. 7C). These small paracentral scotomas are often
the occipital pole, macular sparing of 2–5° in the blind overlooked in automated perimetry if the grid of the test
hemifield along the 0° meridian often occurs (16,17,41,42). program is not dense enough.
If the occipital pole is spared, the visual field will be The severity of the reading problem in homonymous
constricted with defects respecting the vertical midline and hemianopia is also influenced by the side of the defect in
showing a ‘‘step’’ at the vertical midline (Fig. 5, 5.11). An relation to the reading direction. In left-to-right readers,
isolated lesion of the occipital pole causes a small paracentral a right homonymous hemianopia is extremely impairing
homonymous hemianopic defect. Bilateral occipital pole because the patient cannot see the oncoming groups of
lesions may produce binocular central scotomas (Fig. 5.10). letters or words (Fig. 8C) (13). On the other hand, a patient
Visual rehabilitation is conducted along the guidelines with left homonymous hemianopia gets through the line
described earlier for central scotomas and constricted fields, quite easily but has difficulties finding the beginning of the
except that the problem may be even more challenging as next line (Fig. 8B).
the defects are always binocular. Eccentric fixation may help some patients with macular
Retrochiasmal lesions may be associated with traumatic splitting (Fig. 9). The use of eccentric fixation (Fig. 9A)
brain injury (TBI), an important cause of disability causes a little sacrifice of visual acuity but creates an
(43–45). TBI often includes perceptual disorders, which extended perceptual span that is crucial for fluent reading
are frequently overlooked (46). Their rehabilitation requires (Fig. 9B). Eccentric fixation causes a shift of the field defect
a multidisciplinary approach. toward the hemianopic side in conventional perimetry
(Fig. 10B), which can be misinterpreted as improvement
Reading With Homonymous Hemianopia of the visual field. This process indicates high cortical
When the field center is involved (‘‘macular splitting’’), plasticity, because the new eccentric fixation locus is used
homonymous hemianopia causes severe reading problems not only as the new center of the visual field, but also as the
because half of the reading visual field is missing (Fig. 7A). new center of the coordinates of the reading eye movements,
which means a shift of the sensory and motor reference (47).
Patients spontaneously develop unstable asymmetric fixa-
tional eye movements with saccades toward the hemianopic
side (Fig. 10A). This phenomenon also leads to a shift of the
vertical field border in conventional perimetry (Fig. 10B).

Rehabilitation of the Hemianopic


Reading Disorder
Orientation on the page can be improved by visual and
tactile tools such as a ruler or a forefinger, especially in
guiding patients with left homonymous hemianopias in
finding the beginning of the next line of text.
Another approach is turning the text to a vertical or
diagonal orientation, but this technique has never been
tested in a large patient group. Training in predictive
saccades can be beneficial in those with left homonymous
hemianopias. Such saccades improve their ability to find the
beginning of the next line of text. Scrolled text training
programs have been shown to be effective in this regard
(48–50).

Hemianopic Orientation Disorder


FIG. 6. Hemifield slide phenomenon in bitemporal Patients with homonymous hemianopias are severely
hemianopia. Exodeviation leads to an overlap, impaired in regard to spatial orientation. However, many
esodeviation to a gap, and hyperdeviation to a vertical
misregistry of the hemifields, causing severe reading spontaneously develop a beneficial compensation strategy:
disability, especially for long rows of digits. (Modified exploratory saccades toward the hemianopic side for better
from Reference 40.) usage of their field of gaze.

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FIG. 7. Right homonymous hemianopia related to the 30° visual field (upper panel) and to the reading text (lower panel).
A. In macular splitting, one half of the reading visual field is covered so that reading is impaired. B. In macular sparing,
the reading visual field is spared, and reading is normal, although the large field defect hinders orientation. C. In a lesion
of the occipital pole, a small paracentral homonymous field defect occurs, which causes severe reading disability.
(Modified from Reference 76.)

This spontaneous compensation strategy can be sup- the target (Fig. 8B) and later show overshoot as a predictive
ported and improved by saccadic training (see below). Fig. strategy (52).
10C shows the shift of the field defect by saccades in
a natural scene. In conventional perimetry, this behavior Optical Devices
shifts the field defect to the blind side (51), a pattern that Most patients are confused by the double images and
can be misinterpreted as an improvement in the visual field disturbances in spatial orientation caused by optical devices
(compare Fig. 10B–C). used in an attempt to improve vision in homonymous
In saccadic search paradigms, a development of hemianopia. Binocular sector prisms cause a relocation of
saccadic strategies can be observed. In early stages, the field or a shift of the position of the field loss (53).
patients often use a staircase pattern of saccades to find Although binocular prisms are not effective in treating

FIG. 8. Eye movements during reading 1 line of text. A. The normal subject needs 8 saccades per line and approximately
1.5 seconds. B. The patient with left homonymous hemianopia gets through the line quite easily but has difficulty finding
the beginning of the next line and makes several small regressions during the return sweep. C. The patient with right
homonymous hemianopia makes numerous forward and backward saccades to get through the line; reading speed is
prolonged 6-fold over normal.

80 Trauzettel-Klosinski: J Neuro-Ophthalmol 2010; 30: 73-84


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Training to Improve the Hemianopic


Orientation Disorder
In evaluating the many programs that allege success, the
following issues should be considered: 1) spontaneous
recovery must be ruled out, especially in the first weeks
after the neurologic insult (spontaneous recovery normally
does not occur after 6 months) (57,58); 2) a control
group is essential; 3) the methods used to measure improve-
ment must be reliable; and 4) the improvement must be
durable.
Spontaneous recovery may occur in 7%–53 % of
patients, depending on the definition of improvement and
the cohort of observed patients (57,59,60). Hier et al (61)
reported an improvement of 60%–80%, probably due to
compensatory strategies rather than a real change in the
visual fields.
The main problem with conventional perimetry in
assessing improvement is insufficient fixation control. The
vertical visual field border depends essentially on the quality
of fixation. If fixation is unstable or eccentric, the visual field
border is shifted toward the hemianopic side in conven-
tional perimetry, which can mimic an improvement of the
visual field defect (Figs. 9 and 10). This phenomenon has
been shown clearly with fundus-controlled perimetry by
SLO (41).

Restitution Training Versus


Compensation Training
There are 2 different approaches in visual rehabilitation
FIG. 9 Eccentric fixation in homonymous hemianopia.
A. Slight eccentric fixation causes a new functional
for homonymous hemianopia: restoration of lost visual
midline, which causes a new perceptual area. B. In field (‘‘restitution training’’) and compensation strategies
reading, this eccentric fixation creates a small reading for visual field loss ("compensation training"). In 2 studies
visual field on the side of the blind hemifield by shifting the of restitution training, the stimulation has been at the
field defect toward the blind side. (Modified from border of the hemianopic field defect (62,63). Here the risk
Reference 76.)
is stray light and especially eye movements toward the
stimulus. The authors of the first study (62) reported an
hemianopia, they have been shown to be beneficial in improvement of the visual field of up to 40°, but these
patients with hemispatial neglect (54). Monocular prisms results could not be confirmed by Balliet et al. (64). In
and mirrors have been used to shift the image of the blind a later study (63), ‘‘visual restitution therapy’’ (VRT) was
hemifield toward the normal hemifield (55). Unfortunately, performed by presenting perimetric targets above the
these devices usually cause diplopia and confusion. Creating threshold along the visual field border. The authors
confusion is intentional, because it induces an eye or head described an extension of the seeing hemifield by
movement toward the blind side. However, the diplopia in approximately 5°. Our laboratory performed a SLO study
the central visual field is described as very unpleasant (55). before and after VRT using fundus perimetry with
Even so, Hedges et al (51) reported a benefit in 20% of their simultaneous fixation control and a grid of 0.5° spatial
patients. Monocular sector prisms placed across the whole resolution horizontally and 1° vertically in the 10° visual
width of the lens, but only in the peripheral field, have been field (65). Neither that study nor a study with conventional
reported to be beneficial by expanding the field without perimetry (66) could show any improvement in the visual
causing central diplopia. In 1 study (56), 47% of the field.
patients were still wearing the peripheral prisms after 12 Stimulation of the visual field using flickering letters and
months. However, there was no control group and no other targets in a more peripheral area (at 10°) in the visual
control treatment in that study. Optical aids may be helpful field was reported to normalize contrast sensitivity in the
in a few patients, but these interventions cannot be generally blind field in 2 patients (67). To examine this effect, our
recommended. laboratory used a flickering letter stimulation at 22°

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FIG. 10. Spontaneous, unstable, asymmetric fixational eye movements toward the hemianopic side (A) shift the visual
field defect toward the blind hemifield (B), which can be misinterpreted as an improvement in the visual field. Scanning
eye movements toward the blind side shift the field defect to a larger extent and enlarge the field of gaze (C). This effect
can be enhanced by training. (Modified from Reference 68.)

eccentricity (peripheral to the blind spot), a location that Saccadic training is recommended for patients with hemi-
reduces the risk of eye movements toward the stimulus (68). anopias to improve the usage of their field of gaze and thereby
No changes of the visual fields were observed. their orientation, mobility, independence, and quality of life
The reported effects of the 3 restitution studies (68; http://www.uak.medizin.uni-tuebingen.de/sba/).
(62,63,67) should be distinguished from the ‘‘blindsight’’
phenomenon, which is an unconscious perception of visual
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