Visual Perceptual Effects of Long-Standing Vision Loss

  • Ava K. Bittner
  • Janet S. Sunness


This chapter focuses on the changes in vision experienced by patients with RP and AMD. The specific aspects of vision that are reviewed include progressive changes in central acuity, contrast sensitivity, visual field, color vision, night vision, glare, and light and dark adaptation. Emphasis is on patients’ perspectives, including the impact on functioning and performance of activities of daily living, as well as rates, patterns of vision loss, and day-to-day visual fluctuations experienced by those with retinal degenerative diseases. Several types of visual phenomena are presented, including Charles Bonnet Syndrome hallucinations in AMD, perceptual completion or filling-in of scotomas in AMD, remapping visual cortex in AMD, the preferred retinal locus in AMD, and photopsias or light show type flashes in RP. The proposed implications of these visual changes and phenomena as they apply to retinal prosthetic vision are discussed.


Visual Hallucination Visual Field Loss Punctate Inner Choroidopathy Charles Bonnet Syndrome Acute Zonal Occult Outer Retinopathy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



Acute idiopathic blind spot enlargement


Age-related macular degeneration


Acute zonal occult outer retinopathy


Charles Bonnet syndrome


Functional magnetic resonance imaging


Geographic atrophy


Multiple evanescent white dot syndrome


Punctate inner choroidopathy


Preferred retinal locus


Retinitis pigmentosa


Vascular endothelial growth factor


  1. 1.
    Abbott EJ, Connor GB, Artes PH, Abadi RV (2007), Visual loss and visual hallucinations in patients with age-related macular degeneration (Charles Bonnet syndrome). Invest Ophthalmol Vis Sci, 48(3): p. 1416–23.CrossRefGoogle Scholar
  2. 2.
    Alexander KR, Fishman GA, Derlacki DJ (1996), Intraocular light scatter in patients with retinitis pigmentosa. Vision Res, 36(22): p. 3703–9.CrossRefGoogle Scholar
  3. 3.
    Baker CI, Dilks DD, et al. (2008), Reorganization of visual processing in macular degeneration: replication and clues about the role of foveal loss. Vision Res, 48(18): p. 1910–9.CrossRefGoogle Scholar
  4. 4.
    Baker CI, Peli E, Knouf N, Kanwisher NG (2005), Reorganization of visual processing in macular degeneration. J Neurosci, 25(3): p. 614–8.CrossRefGoogle Scholar
  5. 5.
    Berson EL, Sandberg MA, Rosner B, et al. (1985), Natural course of retinitis pigmentosa over a three-year period. Am J Ophthalmol, 99: p. 24–51.Google Scholar
  6. 6.
    Bittner AK, Diener-West M, Dagnelie G (2009), A survey of photopsias in self-reported retinitis pigmentosa: location of photopsias is related to disease severity. Retina, 29(10): p. 1513–21.CrossRefGoogle Scholar
  7. 7.
    Bressler NM, Bressler SB, Fine SL (1988), Age-related macular degeneration. Surv Ophthalmol, 32: p. 375–413.CrossRefGoogle Scholar
  8. 8.
    Brown GC, Murphy RP (1992), Visual symptoms associated with choroidal neovascularization. Photopsias and the Charles Bonnet syndrome. Arch Ophthalmol, 110(9): p. 1251–6.Google Scholar
  9. 9.
    Burke W (2002), The neural basis of Charles Bonnet hallucinations: a hypothesis. J Neurol Neurosurg Psychiatry, 73: p. 535–41.CrossRefGoogle Scholar
  10. 10.
    Danks JJ, Harrad RA (1998), Flashing lights in thyroid eye disease: a new symptom described and (possibly) explained. Br J Ophthalmol, 82(11): p. 1309–11.CrossRefGoogle Scholar
  11. 11.
    Davis FA, Bergen D, Schauf C, et al. (1976), Movement phosphenes in optic neuritis: a new clinical sign. Neurology, 26: p. 1100–4.Google Scholar
  12. 12.
    Engel S, Glover G, Wandell BA, et al. (1997), Retinotopic organization in human visual cortex and the spatial precision of functional MRI. Cereb Cortex, 7: p. 181–92.CrossRefGoogle Scholar
  13. 13.
    Eperjesi F, Akbarali N (2004), Rehabilitation in Charles Bonnet syndrome: a review of treatment options. Clin Exp Optom, 87(3): p. 149–52.CrossRefGoogle Scholar
  14. 14.
    Fine AM, Elman MJ, Ebert JE, et al. (1986), Earliest symptoms caused by neovascular membranes in the macula. Arch Ophthalmol, 104: p. 513–4.Google Scholar
  15. 15.
    Fishman GA, Young RS, Vasquez V, Lourenço P (1981), Color vision defects in retinitis pigmentosa. Ann Ophthalmol, 13(5): p. 609–18.Google Scholar
  16. 16.
    Fletcher WA, Imes RK, Goodman D, Hoyt WF (1988), Acute idiopathic blind spot enlargement: a big blind spot syndrome without optic disc edema. Arch Ophthalmol, 106: p. 44–9.Google Scholar
  17. 17.
    Flor H, Elbert T, Knecht S, et al. (1995), Phantom-limb pain as a perceptual correlate of cortical reorganization following arm amputation. Nature, 375: p. 482–4.CrossRefGoogle Scholar
  18. 18.
    Gawande AA, Donovan WJ, Ginsburg AP, Marmor MF (1989), Photoaversion in retinitis pigmentosa. Br J Ophthalmol, 73(2): p. 115–20.CrossRefGoogle Scholar
  19. 19.
    Gerstenblith AT, Thorne JE, Sobrin L, et al. (2007), Punctate inner choroidopathy: a survey analysis of 77 persons. Ophthalmology, 114(6): p. 1201–4.CrossRefGoogle Scholar
  20. 20.
    Grover S, Fishman GA, Anderson RJ, et al. (1997), Rate of visual field loss in retinitis pigmentosa. Ophthalmology, 104(3): p. 460–5.Google Scholar
  21. 21.
    Grover S, Fishman GA, Brown J (1998), Patterns of visual field progression in patients with retinitis pigmentosa. Ophthalmology, 105(6): p. 1069–75.CrossRefGoogle Scholar
  22. 22.
    Hartong DT, Berson EL, Dryja TP (2006), Retinitis pigmentosa. Lancet, 368(9549):p. 1795–809.CrossRefGoogle Scholar
  23. 23.
    Heckenlively JR, Ferreyra HA (2008), Autoimmune retinopathy: a review and summary. Semin Immunopathol, 30(2): p. 127–34.CrossRefGoogle Scholar
  24. 24.
    Heckenlively JR, Yoser SL, Friedman LH, Oversier JJ (1988), Clinical findings and common symptoms in retinitis pigmentosa. Am J Ophthalmol, 105: p. 504–11.Google Scholar
  25. 25.
    Heinen SJ, Skavenski AA (1991), Recovery of visual responses in foveal V1 neurons following bilateral foveal lesions in adult monkey. Exp Brain Res, 83: p. 670–4.CrossRefGoogle Scholar
  26. 26.
    Herse P (2005), Retinitis pigmentosa: visual function and multidisciplinary management. Clin Exp Optom, 88(5): p. 335–50.CrossRefGoogle Scholar
  27. 27.
    Holopigian K, Greenstein V, Seiple W, Carr R (1996), Rates of change differ among measures of visual function in patients with retinitis pigmentosa. Ophthalmology, 103: p. 398–405.Google Scholar
  28. 28.
    Horton J, Hoyt W (1991), The representation of the visual field in human striate cortex. Arch Ophthalmol, 109: p. 816–24.Google Scholar
  29. 29.
    Jampol LM, Sieving PA, Pugh D, et al. (1984), Multiple evanescent white dot syndrome: I. Clinical findings. Arch Ophthalmol, 102: p. 671–4.Google Scholar
  30. 30.
    Khan JC, Shahid H, Thurlby DA, et al. (2008), Charles Bonnet syndrome in age-related macular degeneration: the nature and frequency of images in subjects with end-stage disease. Ophthalmic Epidemiol, 15(3): p. 202–8.CrossRefGoogle Scholar
  31. 31.
    Kiser AK, Mladenovich D, Eshraghi F, et al. (2005), Reliability and consistency of visual acuity and contrast sensitivity measures in advanced eye disease. Optom Vis Sci, 82(11): p. 946–54.CrossRefGoogle Scholar
  32. 32.
    Kiser AK, Pronovost PJ (2009), Management of diseases without current treatment options: something can be done. JAMA, 301(16): p. 1708–9.CrossRefGoogle Scholar
  33. 33.
    McDonald WI, Barnes D (1992), The ocular manifestations of multiple sclerosis. 1. Abnormalities of the afferent visual system. J Neurol Neurosurg Psychiatry, 55: p. 747–52.CrossRefGoogle Scholar
  34. 34.
    Massof RW, Dagnelie G, Benzschawel T, et al. (1990), First order dynamics of visual field loss in retinitis pigmentosa. Clin Vis Sci, 5: p. 1–26.Google Scholar
  35. 35.
    Massof RW, Finkelstein D (1981), Two forms of autosomal dominant primary retinitis pigmentosa. Doc Ophthalmol, 51(4): p. 289–346.CrossRefGoogle Scholar
  36. 36.
    Masuda Y, Dumoulin SO, Nakadomari S, Wandell BA (2008), V1 projection zone signals in human macular degeneration depend on task, not stimulus. Cereb Cortex, 18(11): p. 2483–93.CrossRefGoogle Scholar
  37. 37.
    Menon GJ, Rahman I, Menon SJ, Dutton GN (2003), Complex visual hallucinations in the visually impaired: the Charles Bonnet syndrome. Surv Ophthalmol, 48(1): p. 58–72.CrossRefGoogle Scholar
  38. 38.
    Oishi A, Miyamoto K, Kashii S, Yoshimura N (2006), Photopsia as a manifestation of digitalis toxicity. Can J Ophthalmol, 41(5): p. 603–4.Google Scholar
  39. 39.
    Pagon RA (1988), Retinitis pigmentosa. Surv Ophthalmol, 33(3): p. 137–77.CrossRefMathSciNetGoogle Scholar
  40. 40.
    Plummer C, Kleinitz A, Vroomen P, Watts R (2007), Of Roman chariots and goats in overcoats: the syndrome of Charles Bonnet. J Clin Neurosci, 14(8): p. 709–14.CrossRefGoogle Scholar
  41. 41.
    Ramachandran VS, Gregory RL (1991), Perceptual filling in of artificially induced scotomas in human vision. Nature, 350: p. 699–702.CrossRefGoogle Scholar
  42. 42.
    Rosenfeld PJ, Brown DM, Heier JS, et al. (2006), Ranibizumab for neovascular age-related macular degeneration. N Engl J Med, 355(14): p. 1419–31.CrossRefGoogle Scholar
  43. 43.
    Ross J, Rahman I (2005), Charles Bonnet syndrome following enucleation. Eye, 19(7): p. 811–2.CrossRefGoogle Scholar
  44. 44.
    Rovner BW (2006), The Charles Bonnet syndrome: a review of recent research. Curr Opin Ophthalmol, 17(3): p. 275–7.CrossRefGoogle Scholar
  45. 45.
    Sarks JP, Sarks SH, Killingsworth MC (1988), Evolution of geographic atrophy of the retinal pigment epithelium. Eye, 2: p. 552–77.Google Scholar
  46. 46.
    Schuchard RA (1993), Validity and interpretation of Amsler grid reports. Arch Ophthalmol, 111: p. 776–80.Google Scholar
  47. 47.
    Schumacher EH, Jacko JA, Primo SA, et al. (2008), Reorganization of visual processing is related to eccentric viewing in patients with macular degeneration. Restor Neurol Neurosci, 26(4–5): p. 391–402.Google Scholar
  48. 48.
    Slotnick S, Klein S, Carney T, et al. (2001), Electrophysiological estimate of human cortical magnification. Clin Neurophysiol, 112: p. 1349–56.CrossRefGoogle Scholar
  49. 49.
    Sunness JS (2008), The use of face fields for determining fixation location in eyes with central scotomas from macular disease. J Vis Impair Blind, 102: p. 679–89.Google Scholar
  50. 50.
    Sunness JS, Applegate CA, Gonzalez-Baron J (2000), Improvement of visual acuity over time in patients with bilateral geographic atrophy from age-related macular degeneration. Retina, 20: p. 162–9.Google Scholar
  51. 51.
    Sunness JS, Liu T, Yantis S (2004), Retinotopic mapping of visual cortex using fMRI in a patient with central scotomas from atrophic macular degeneration. Ophthalmology, 111: p. 1595–8.CrossRefGoogle Scholar
  52. 52.
    Sunness JS, Rubin GS, Applegate CA, et al. (1997), Visual function abnormalities and prognosis in eyes with age-related geographic atrophy of the macula and good acuity. Ophthalmology, 104: p. 1677–91.Google Scholar
  53. 53.
    Sunness JS, Rubin GS, Broman A, et al. (2008), Low luminance visual dysfunction as a predictor of subsequent visual acuity loss in age-related geographic atrophy of the macula. Ophthalmology, 115: p. 1480–8.CrossRefGoogle Scholar
  54. 54.
    Teunisse RJ, Cruysberg JR, Hoefnagels WH, et al. (1996), Visual hallucinations in psychologically normal people: Charles Bonnet’s syndrome. Lancet, 347: p. 794–7.CrossRefGoogle Scholar
  55. 55.
    Teunisse RJ, Cruysberg JR, Hoefnagels WH, et al. (1999), Social and psychological characteristics of elderly visually handicapped patients with the Charles Bonnet syndrome. Compr Psychiatry, 40(4): p. 315–9.CrossRefGoogle Scholar
  56. 56.
    Tsujikawa M, Wada Y, Sukegawa M, et al. (2008), Age at onset curves of retinitis pigmentosa. Arch Ophthalmol, 126(3): p. 337–40.CrossRefGoogle Scholar
  57. 57.
    Turano KA, Geruschat DR, Baker FH, et al. (2001), Direction of gaze while walking a simple route: persons with normal vision and persons with retinitis pigmentosa. Optom Vis Sci, 78(9): p. 667–75.CrossRefGoogle Scholar
  58. 58.
    Weiss NJ (1991), Low vision management of retinitis pigmentosa. J Am Optom Assoc, 62(1): p. 42–52.Google Scholar
  59. 59.
    White JM, Bedell HE (1990), The oculomotor reference in human with bilateral macular disease. Invest Ophthalmol Vis Sci, 31: p. 1149–61.Google Scholar
  60. 60.
    Young RS, Fishman GA (1980), Color matches of patients with retinitis pigmentosa. Invest Ophthalmol Vis Sci, 19(8): p. 967–72.Google Scholar
  61. 61.
    Zibrandtsen N, Munch IC, Klemp K, et al. (2008), Photoreceptor atrophy in acute zonal occult outer retinopathy. Acta Ophthalmol, 86(8): p. 913–6.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Lions Vision Research & Rehabilitation Center, Wilmer Eye InstituteJohns Hopkins University School of MedicineBaltimoreUSA

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