When Your Cells’ Batteries Fail: How Mitochondrial Diseases Affect Vision

Imagine if the batteries inside your cells started to fail. That’s essentially what happens in mitochondrial diseases—a group of disorders that impair the body’s ability to produce energy. For the eye, one of the most energy-hungry organs in the body, the impact can be devastating.

At South Bay Retina, Dr. Keshav Narain and his team use a comprehensive, science-driven approach to detect and manage the ocular effects of these complex conditions—helping patients preserve vision for as long as possible.

Close-up of a richly detailed brown eye, highlighting the intricate patterns of the iris and the reflection of light on the surface.

What Are Mitochondrial Diseases?

Inside each of our cells are mitochondria—tiny, high-powered structures often called the cell’s powerhouses. Their job is to produce ATP, the molecule that fuels nearly every process in the body.

When mitochondria fail to work properly, cells in high-energy-demand tissues—like the brain, muscles, and eyes—are especially vulnerable.

These diseases are genetic and can be inherited in different ways:

• From the mother through mitochondrial DNA

• From both parents through nuclear DNA mutations
  

How Mitochondrial Disease Affects the Eye

The retina and optic nerve require a constant supply of energy to function. When mitochondrial dysfunction occurs, the results can be vision-threatening. Common symptoms include:

• Blurred or reduced vision

• Missing areas in vision (visual field defects)

• Sudden or progressive vision loss

• Optic nerve pallor or atrophy

• Light sensitivity (photophobia)

Some well-known mitochondrial-related eye disorders include:

• Leber’s Hereditary Optic Neuropathy (LHON)

• Dominant Optic Atrophy (DOA)

• Chronic Progressive External Ophthalmoplegia (CPEO)
  

How We Diagnose Mitochondrial Eye Disease

Because symptoms can be subtle—or overlap with other conditions—early and precise detection is critical. At South Bay Retina, our diagnostic process may include:

• Detailed patient and genetic history

• OCT (Optical Coherence Tomography) to measure retinal nerve fiber thickness

• Visual field testing to detect blind spots or peripheral vision loss

• VEP (Visual Evoked Potential) to assess optic nerve function

• Blood and genetic testing if systemic disease is suspected

Our aim: Catch mitochondrial-related eye changes before significant vision loss occurs.

Treatment: Preserving Vision Through Early Intervention

There’s no definitive cure for mitochondrial diseases—but with targeted treatment, we can slow progression, protect vision, and improve quality of life.

Our strategies include:

• Nutritional support (CoQ10, alpha-lipoic acid, B vitamins)

• Lifestyle counseling to minimize mitochondrial stress (avoiding smoking, alcohol, certain medications)

• Visual aids and rehabilitation for functional support
  

Subthreshold 532nm Focal Grid Laser Therapy

In cases where mitochondrial dysfunction leads to retinal fluid or leakage—sometimes as a secondary effect of diabetes—Dr. Narain may use subthreshold 532nm laser therapy. This non-scarring, tissue-preserving laser treatment can help stabilize vision while protecting delicate retinal structures.

Our Clinical Protocol at South Bay Retina

When treating mitochondrial-related eye disease, we follow a structured, personalized approach:

1. Comprehensive baseline exam with OCT, fundus photography, and electrophysiology testing

2. Genetic and metabolic consultation when needed

3. Custom treatment planning based on severity, symptoms, and disease progression

4. Use of subthreshold laser for retinal leakage or edema

5. Ongoing monitoring with visual field, OCT, VEP, and ERG scans

6. Patient education and support so you can take an active role in protecting your vision

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