BACKGROUND: Cone-Rod dystrophy is characterized by reduced central visual acuity, dyschromatopsia, and photophobia. Acute Zonal Occult Outer Retinopathy (AZOOR) is associated with photopsia and later atrophic retinal changes. Both conditions show electroretinographic (ERG) abnormalities and in advanced stages, peripheral field constriction. Patients with vision loss or scotomata will in most cases benefit from referral to low vision services.
CASE REPORT(S). A 52-year-old white female presented for an initial low vision exam with the differential diagnoses of cone-rod dystrophy verses AZOOR. Her chief complaints were decreased central vision and photophobia. Her primary goals were to read newspapers and bank statements. Other objectives included reducing glare and writing checks. She first reported visual changes at the age of 44 with symptoms of photopsia, hazy vision, color desaturation, and difficulty focusing from light to dark illuminations. ERG showed reduced A and B waves in the cone and rod amplitudes in both eyes leading to a potential diagnosis of cone-rod dystrophy. Further evaluation suggested atypical AZOOR. She was told there was no treatment available and elected to travel to Cuba to undergo ‘revitalization’ surgery, with no improvement. Clinical evaluation revealed reduced acuities, color vision, and contrast sensitivity OU. The patient demonstrated enthusiasm for device use and responded favorably to devices for glare control, contrast enhancement and magnification, which included light amber NOIR spectacles, yellow acetate filter paper, typoscope and a Zeiss head-worn loupe L 1.25X(4D) loupe and lens.
CONCLUSIONS. Cone-Rod dystrophy and AZOOR are disorders in optometric care that are encountered infrequently. However, as more physicians become familiar with their signs and symptoms, these patients may benefit from earlier diagnoses and referrals for low vision rehabilitation services.