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PubMed This is a summary of 22 peer-reviewed journal articles Updated
Ophthalmology · Inherited Optic Neuropathy

What Is the Difference Between ADOA and LHON?

At a Glance

The primary difference between ADOA and LHON is that ADOA causes slow, gradual vision loss starting in childhood and can be inherited from either parent. In contrast, LHON typically causes sudden, rapid vision loss in young adulthood and is passed down exclusively from the mother.

The main difference between Autosomal Dominant Optic Atrophy (ADOA) and Leber Hereditary Optic Neuropathy (LHON) lies in how they are inherited and how the vision loss happens [1][2]. ADOA typically causes slow, gradual vision changes that begin in childhood, and it can be inherited from either parent. In contrast, LHON usually causes sudden, rapid vision loss starting in young adulthood, and it is passed down only from the mother.

Both conditions are inherited optic neuropathies, meaning they are genetic diseases that cause vision loss by damaging the optic nerve [1][3]. However, because they are often confused by patients and even general eye doctors, it is important to understand what sets them apart.

Key Differences at a Glance

Feature ADOA LHON
Typical Age of Onset Childhood (usually before age 10) Young adulthood
Speed of Vision Loss Slow and gradual Sudden and rapid
Severity of Vision Loss Variable; rarely complete blindness Often profound
Eye Involvement Both eyes simultaneously Often one eye first, then the second
Inherited From Either parent (nuclear DNA) Mother only (mitochondrial DNA)

Inheritance and Risk (Penetrance)

The way these two conditions are passed through families is fundamentally different:

  • ADOA (Nuclear DNA): ADOA is usually caused by mutations in the OPA1 gene, which is located in the nucleus of your cells [4]. It follows an autosomal dominant inheritance pattern [2]. This means a child can inherit the condition from either their mother or their father. If one parent has the gene, each child has a 50% chance of inheriting it.
  • LHON (Mitochondrial DNA): LHON is caused by mutations in your mitochondrial DNA (mtDNA) [4]. Mitochondria are the energy-producing centers of the cell. Because a child only inherits mitochondrial DNA from their mother’s egg, LHON can only be passed down from the mother [2]. Fathers with LHON cannot pass the disease to their children.

Having the gene does not mean you will definitely lose your vision. In the medical world, this is called incomplete penetrance (when a person has the gene but does not develop the disease) [5][6]. Many people who carry the genetic mutation for LHON never develop vision loss, and among those who do, men are significantly more likely to be affected than women [6][7]. Similarly, ADOA affects people differently; some people with the gene have very mild symptoms or never realize they have it, while others experience more noticeable vision changes [8][3].

How Vision Loss Happens

While both conditions damage the retinal ganglion cells (the cells in the eye that form the optic nerve), the patient experience is very different [3][9].

  • ADOA: Vision changes typically begin before age 10 [3]. The decline in vision is usually slow and gradual, progressing over many years or decades [9]. The vision loss happens in both eyes at roughly the same time (simultaneously) [10]. While ADOA causes reduced visual acuity and color vision deficits, it usually causes mild to moderate visual impairment rather than complete, pitch-black blindness [10][11].
  • LHON: Vision loss typically strikes during young adulthood [3]. The vision loss is sudden, profound, and rapid (often worsening over a few weeks to months) [12]. It frequently starts in one eye first, with the second eye becoming affected shortly after (sequential) [13][14].

Outcomes and “Plus” Symptoms

Both ADOA and LHON can sometimes involve additional symptoms outside of the eyes, known as “plus” phenotypes. These can include hearing loss, muscle weakness, or neuropathy (nerve damage) [15][16]. It is important to know that these extra symptoms only affect a subset of patients depending on the specific gene mutation, so not everyone with ADOA or LHON will develop them [17][15]. Speaking with a genetic counselor can help you understand whether your specific mutation puts you or your family at risk for these extra symptoms.

Regarding long-term vision:

  • LHON: In some cases, patients with LHON experience spontaneous improvement in their vision over time [18]. Certain treatments, like the medication idebenone, have also shown potential to help reactivate surviving cells and improve vision [19][20].
  • ADOA: Vision loss is due to progressive degeneration [9]. Current research for ADOA focuses on therapies aimed at preventing further loss of vision or slowing progression, rather than restoring vision that has already been lost [21][22]. However, low-vision aids, occupational therapy, and lifestyle adaptations are highly effective tools for helping patients live successfully and independently with ADOA.

Common questions in this guide

How is the inheritance pattern different between ADOA and LHON?
ADOA can be inherited from either your mother or your father through nuclear DNA. LHON, however, is passed down only from the mother through mitochondrial DNA. Fathers with LHON cannot pass the condition to their children.
Does having the gene for ADOA or LHON mean I will definitely lose my vision?
No, both conditions have what is called incomplete penetrance. This means that many people who carry the genetic mutation never develop vision loss, or they may only experience very mild symptoms.
Which condition causes sudden vision loss?
LHON typically causes sudden, profound, and rapid vision loss, often worsening over a few weeks to months. ADOA causes a slow and gradual decline in vision that progresses over many years or decades.
Can ADOA and LHON cause symptoms outside of the eyes?
Yes, both conditions can sometimes involve 'plus' phenotypes, which are additional symptoms like hearing loss, muscle weakness, or nerve damage. Whether you develop these depends on your specific gene mutation.
Is it possible to restore vision lost to ADOA or LHON?
While ADOA treatments currently focus on preventing further loss and utilizing low-vision aids, some patients with LHON may experience spontaneous improvement. Certain LHON patients may also benefit from medications that help reactivate surviving cells.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Have I had a genetic test to confirm whether my optic atrophy is caused by an OPA1 mutation (nuclear) or a mitochondrial DNA mutation?
  2. 2.Based on my specific genetic mutation, what is the likelihood that I might develop 'plus' symptoms like hearing loss?
  3. 3.Are there specific low-vision aids, occupational therapies, or lifestyle adaptations you recommend to help me maximize my current vision?
  4. 4.Should my immediate family members undergo genetic testing, and can you refer us to a genetic counselor to help us understand the risks for our children?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

Related questions

ADOA Clinical Trials: What Are the New Treatments?Is Your Normal Tension Glaucoma Actually ADOA?Why is ADOA More Common in Denmark?Why Does ADOA Cause Blue-Yellow Color Vision Loss?Can You Drive With Autosomal Dominant Optic Atrophy?Does ADOA Affect Peripheral Vision? Symptoms & FactsWill I Go Completely Blind from ADOA?How Does Eccentric Viewing Work for ADOA?Does ADOA Cause Hearing Loss? ADOA Plus ExplainedHow Often Do ADOA Patients Need OCT & Visual Field Tests?Should I Take CoQ10 or Idebenone for ADOA Vision Loss?Is Autosomal Dominant Optic Atrophy Inherited?What Medications to Avoid with ADOA (Optic Atrophy)?How Do I Manage My Mental Health With an ADOA Diagnosis?What Lifestyle Changes Help ADOA Vision Loss?How Fast Does Vision Loss Progress in ADOA?Why Do ADOA Symptoms Vary in Severity Within Families?

References

References (22)
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This page is for informational purposes only and does not replace professional medical advice. Always consult your ophthalmologist or genetic counselor regarding your specific vision changes, genetic risks, and family planning.

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