Overview
Acute Zonal Occult Outer Retinopathy, commonly known as AZOOR, is a rare eye condition that affects the outer layer of the retina — the light-sensitive tissue at the back of the eye. In AZOOR, one or more zones of the outer retina stop working properly, leading to blind spots and other vision changes. The condition most often affects young to middle-aged women, and it typically starts in one eye, though it can eventually involve both eyes. Patients usually notice the sudden appearance of blind spots (scotomas) in their field of vision, along with flashing lights (photopsia). Some people also experience a general dimming of vision or changes in how they see colors. The condition can be alarming because symptoms often come on quickly. In many cases, the visual loss stabilizes over time, but some patients experience progressive worsening. The exact cause of AZOOR is not fully understood. It is thought to involve an autoimmune or inflammatory process that damages the photoreceptor cells in the outer retina. There is currently no proven cure or standard treatment for AZOOR. Some doctors have tried corticosteroids or other immunosuppressive medications to reduce inflammation, but results have been mixed. Management focuses on monitoring vision changes and providing supportive care. Research is ongoing to better understand the disease and develop effective therapies.
Also known as:
Key symptoms:
Sudden blind spots in the field of visionFlashing lights or shimmering in vision (photopsia)Blurred or dimmed visionLoss of peripheral visionChanges in color visionSensitivity to lightWhite or pale spots on the retinaRapid onset of visual symptoms, often in one eye firstEnlarged blind spot on visual field testingGradual involvement of the other eye in some cases
Clinical phenotype terms (24)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
1 eventRandy Kardon — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Acute zonal occult outer retinopathy.
1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Acute zonal occult outer retinopathy.
Community
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Start the conversation →Latest news about Acute zonal occult outer retinopathy
Disease timeline:
New recruiting trial: New Non-invasive Modalities for Assessing Retinal Structure and Function
A new clinical trial is recruiting patients for Acute zonal occult outer retinopathy
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.How much of my retina is currently affected, and is the disease active or stable?,What tests will you use to monitor my condition over time, and how often should I be seen?,Are there any treatments you would recommend trying in my specific case?,What are the chances that my other eye will also be affected?,Should I be referred to a low vision rehabilitation specialist?,Are there any clinical trials or research studies I could participate in?,What symptoms should prompt me to seek urgent care between appointments?
Common questions about Acute zonal occult outer retinopathy
What is Acute zonal occult outer retinopathy?
Acute Zonal Occult Outer Retinopathy, commonly known as AZOOR, is a rare eye condition that affects the outer layer of the retina — the light-sensitive tissue at the back of the eye. In AZOOR, one or more zones of the outer retina stop working properly, leading to blind spots and other vision changes. The condition most often affects young to middle-aged women, and it typically starts in one eye, though it can eventually involve both eyes. Patients usually notice the sudden appearance of blind spots (scotomas) in their field of vision, along with flashing lights (photopsia). Some people also
How is Acute zonal occult outer retinopathy inherited?
Acute zonal occult outer retinopathy follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Acute zonal occult outer retinopathy typically begin?
Typical onset of Acute zonal occult outer retinopathy is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Acute zonal occult outer retinopathy?
Yes — 1 recruiting clinical trial is currently listed for Acute zonal occult outer retinopathy on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Acute zonal occult outer retinopathy?
1 specialists and care centers treating Acute zonal occult outer retinopathy are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.