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PubMed This is a summary of 20 peer-reviewed journal articles Updated
Ophthalmology · Autosomal Dominant Optic Atrophy and Leber Hereditary Optic Neuropathy

ADOA vs. LHON: What Are the Key Differences?

At a Glance

ADOA and LHON are both inherited optic nerve diseases, but they progress very differently. ADOA typically begins in early childhood and causes slow, gradual vision changes over decades. In contrast, LHON usually strikes young adults and causes rapid, severe central vision loss.

In this answer

3 sections

01

Speed, Severity, and Onset of Vision Loss

02

Age of Diagnosis and Systemic Symptoms

03

Genetic Causes: Nuclear vs. Mitochondrial DNA

If you have Autosomal Dominant Optic Atrophy (ADOA), it is completely understandable to feel anxious when reading about Leber Hereditary Optic Neuropathy (LHON). Both are inherited diseases that affect the optic nerve (the cable that sends visual information from your eye to your brain), but they are fundamentally different in how they progress, when they begin, and their underlying genetics [1]. The most important difference is the speed of vision changes: LHON typically causes rapid, severe vision loss [2], whereas ADOA usually begins in early childhood and progresses slowly over a lifetime [3][4].

Speed, Severity, and Onset of Vision Loss

The way these two conditions impact vision on a day-to-day basis is very different.

  • LHON (Leber Hereditary Optic Neuropathy): This condition is characterized by a rapid, often painless decline in central visual acuity (the sharp, straight-ahead vision needed for reading and recognizing faces) [2][5]. The vision loss in LHON usually happens very quickly over a period of weeks or months before stabilizing [6]. Another distinct feature of LHON is that it frequently presents unilaterally (affecting one eye first), with the second eye typically losing vision weeks or months later [7]. LHON frequently leaves individuals with severe impairment in their central vision [8].
  • ADOA (Autosomal Dominant Optic Atrophy): In contrast, the natural history of ADOA involves a much slower, gradual thinning of the optic nerve fibers over decades [9][4]. Unlike LHON, ADOA usually presents bilaterally (affecting both eyes simultaneously and symmetrically) [10]. While ADOA can cause a significant decline in visual acuity and may lead to legal blindness, the severity is highly variable [4]. Importantly, it typically does not cause the sudden, dramatic loss of central vision seen in LHON [2].

Age of Diagnosis and Systemic Symptoms

When symptoms first appear is another major dividing line between the two conditions.

  • ADOA: This condition generally first appears in early childhood, often presenting as childhood-onset color vision deficits and reduced visual acuity [3][10]. However, because the disease severity is highly variable, some individuals experience such mild symptoms that they adapt well and are not officially diagnosed with ADOA until adulthood [3][11]. Furthermore, while typical ADOA primarily affects vision, about 20% of individuals can develop ADOA plus syndromes (meaning the optic nerve condition is accompanied by other symptoms like hearing loss or muscle weakness) [12][13].
  • LHON: This condition most frequently strikes in young adulthood, typically affecting individuals in their late teens to twenties, though it can occasionally happen at other ages [14].

Genetic Causes: Nuclear vs. Mitochondrial DNA

Although both diseases involve the mitochondria (the energy-producing powerhouses of the cell), the way they are passed down through families is entirely different [1].

  • Inherited Nuclear DNA (ADOA): ADOA is primarily caused by mutations in the OPA1 gene, which is located in your nuclear DNA (the main DNA inherited equally from both parents) [15]. It follows an autosomal dominant inheritance pattern, meaning that a child needs to inherit only one copy of the mutated gene from either parent to have the condition [16].
  • Maternal Mitochondrial DNA (LHON): LHON is caused by mutations in your mitochondrial DNA (mtDNA) [17]. Mitochondrial DNA is inherited strictly from the mother [18]. Therefore, a father with LHON cannot pass the genetic mutation on to his children, whereas a mother with the LHON mutation will pass it to all her children [18].

Understanding these key differences helps clarify that ADOA follows its own specific—and much slower—path. Your medical team will monitor your condition based on the expected progression of ADOA. This monitoring is not simply “watching and waiting”; it involves tracking your visual fields and optic nerve thickness to provide appropriate low-vision aids, optimize your remaining vision, and track your eligibility for future clinical trials [19][20].

Common questions in this guide

Does ADOA cause rapid vision loss like LHON?
No, ADOA usually causes a slow, gradual thinning of the optic nerve fibers over decades. In contrast, LHON typically causes rapid, severe vision loss over a period of weeks or months before stabilizing.
At what age do ADOA and LHON usually start?
ADOA generally first appears in early childhood, often presenting as mild color vision deficits. LHON most frequently strikes in young adulthood, often affecting individuals in their late teens or twenties.
How are ADOA and LHON inherited differently?
ADOA is passed down through nuclear DNA, meaning a child only needs one mutated gene from either parent to inherit the condition. LHON is caused by mutations in mitochondrial DNA, which is inherited strictly from the mother.
Can ADOA cause symptoms besides vision loss?
Yes, while typical ADOA primarily affects vision, about 20% of individuals develop 'plus syndromes.' This means the vision loss is accompanied by other systemic symptoms, such as hearing loss or muscle weakness.
Will both of my eyes be affected by ADOA?
ADOA usually affects both eyes simultaneously and symmetrically. This is different from LHON, which frequently affects one eye first, with the second eye losing vision weeks or months later.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Does my specific genetic mutation put me at risk for 'ADOA plus' symptoms like hearing loss, and should I be actively screened for them?
  2. 2.What low-vision specialists or rehabilitation services should I be referred to in order to maximize my usable vision?
  3. 3.How frequently should we be tracking my optic nerve thickness and visual fields, and what specific changes are we looking for?
  4. 4.Should my siblings or children consider genetic testing or clinical eye evaluations, even if they are only experiencing very mild vision issues?

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

Do Supplements Like CoQ10 or NAD+ Work for ADOA?Can Autosomal Dominant Optic Atrophy Skip a Generation?Can You Drive With Autosomal Dominant Optic Atrophy?Do Hormones Worsen ADOA Vision Loss in Teenage Girls?Do Prematurity and Birth Weight Affect ADOA Symptoms?How Does Blue-Yellow Color Blindness Present in ADOA?How Does Smoking Affect Vision Loss in ADOA Patients?How is ADOA Differentiated from Normal Tension Glaucoma?Why is ADOA Misdiagnosed as a Lazy Eye in Children?What Foods to Avoid With Autosomal Dominant Optic Atrophy?What Do ADOA Central Blind Spots Look Like? | Inciteful MedWhat Does an OCT Scan Show in ADOA? | Inciteful MedWhat If OPA1 Genetic Testing Is Negative For ADOA?What is the Risk of Total Blindness with ADOA?What is the STK-002 Clinical Trial for ADOA?When Does Hearing Loss Start in ADOA-Plus Syndrome?What Medications to Avoid with ADOA?What Support Groups and Registries Exist for ADOA?What Should I Ask a Neuro-Ophthalmologist About ADOA?

References

References (20)
  1. 1

    Mitochondrial Gymnastics in Retinal Cells: A Resilience Mechanism Against Oxidative Stress and Neurodegeneration.

    Mirra S, Marfany G

    Advances in experimental medicine and biology 2019; (1185()):513-517 doi:10.1007/978-3-030-27378-1_84.

    PMID: 31884663
  2. 2

    Gene Therapies for the Treatment of Leber Hereditary Optic Neuropathy.

    Sahel JA, Newman NJ, Yu-Wai-Man P, et al.

    International ophthalmology clinics 2021; (61(4)):195-208 doi:10.1097/IIO.0000000000000364.

    PMID: 34584057
  3. 3

    ATPase Domain AFG3L2 Mutations Alter OPA1 Processing and Cause Optic Neuropathy.

    Caporali L, Magri S, Legati A, et al.

    Annals of neurology 2020; (88(1)):18-32 doi:10.1002/ana.25723.

    PMID: 32219868
  4. 4

    Prevalence of Macular Microcystoid Lacunae in Autosomal Dominant Optic Atrophy Assessed With Adaptive Optics.

    Eckmann-Hansen C, Bek T, Sander B, et al.

    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society 2022; (42(3)):328-333 doi:10.1097/WNO.0000000000001592.

    PMID: 35439206
  5. 5

    Cross-Sectional Analysis of Baseline Visual Parameters in Subjects Recruited Into the RESCUE and REVERSE ND4-LHON Gene Therapy Studies.

    Moster ML, Sergott RC, Newman NJ, et al.

    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society 2021; (41(3)):298-308 doi:10.1097/WNO.0000000000001316.

    PMID: 34310464
  6. 6

    What are the characteristics and progression of visual field defects in patients with Leber hereditary optic neuropathy: a prospective single-centre study in China.

    Liu HL, Yuan JJ, Tian Z, et al.

    BMJ open 2019; (9(3)):e025307 doi:10.1136/bmjopen-2018-025307.

    PMID: 30878986
  7. 7

    Nuclear DNA Mutation Causing a Phenotypic Leber Hereditary Optic Neuropathy Plus.

    Mansukhani SA, Mehta DG, Renaud DL, et al.

    Ophthalmology 2021; (128(4)):628-631 doi:10.1016/j.ophtha.2020.09.011.

    PMID: 32918965
  8. 8

    Intravitreal Gene Therapy vs. Natural History in Patients With Leber Hereditary Optic Neuropathy Carrying the m.11778G>A ND4 Mutation: Systematic Review and Indirect Comparison.

    Newman NJ, Yu-Wai-Man P, Carelli V, et al.

    Frontiers in neurology 2021; (12()):662838 doi:10.3389/fneur.2021.662838.

    PMID: 34108929
  9. 9

    Genotype-phenotype heterogeneity of ganglion cell and inner plexiform layer deficit in autosomal-dominant optic atrophy.

    Rönnbäck C, Nissen C, Almind GJ, et al.

    Acta ophthalmologica 2015; (93(8)):762-6 doi:10.1111/aos.12835.

    PMID: 26385429
  10. 10

    Clinical and Genetic Findings in an Autosomal Dominant Optic Atrophy-Compatible Phenotype Harboring an OPA1 Variant: A Case Report.

    Murati Calderon RA, Landestoy G, Izquierdo N

    Cureus 2025; (17(10)):e95622 doi:10.7759/cureus.95622.

    PMID: 41322916
  11. 11

    [Differential diagnosis of juvenile normal pressure glaucoma].

    Geidel K, Wiedemann P, Unterlauft JD

    Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft 2017; (114(9)):828-831 doi:10.1007/s00347-016-0407-5.

    PMID: 27921132
  12. 12

    Autosomal dominant optic atrophy plus due to the novel OPA1 variant c.1463G>C.

    Finsterer J, Laccone F

    Metabolic brain disease 2019; (34(4)):1023-1027 doi:10.1007/s11011-019-00425-0.

    PMID: 31152339
  13. 13

    Meta-analysis of genotype-phenotype analysis of OPA1 mutations in autosomal dominant optic atrophy.

    Ham M, Han J, Osann K, et al.

    Mitochondrion 2019; (46()):262-269 doi:10.1016/j.mito.2018.07.006.

    PMID: 30165240
  14. 14

    A novel AFG3L2 mutation close to AAA domain leads to aberrant OMA1 and OPA1 processing in a family with optic atrophy.

    Baderna V, Schultz J, Kearns LS, et al.

    Acta neuropathologica communications 2020; (8(1)):93 doi:10.1186/s40478-020-00975-w.

    PMID: 32600459
  15. 15

    Mitochondrial Retinopathies.

    Zeviani M, Carelli V

    International journal of molecular sciences 2021; (23(1)) doi:10.3390/ijms23010210.

    PMID: 35008635
  16. 16

    Mutation Screening of mtDNA Combined Targeted Exon Sequencing in a Cohort With Suspected Hereditary Optic Neuropathy.

    Li JK, Li W, Gao FJ, et al.

    Translational vision science & technology 2020; (9(8)):11 doi:10.1167/tvst.9.8.11.

    PMID: 32855858
  17. 17

    Emerging Mitochondrial Therapeutic Targets in Optic Neuropathies.

    Lopez Sanchez MI, Crowston JG, Mackey DA, Trounce IA

    Pharmacology & therapeutics 2016; (165()):132-52.

    PMID: 27288727
  18. 18

    New avenues for therapy in mitochondrial optic neuropathies.

    Ng WSV, Trigano M, Freeman T, et al.

    Therapeutic advances in rare disease 2021; (2()):26330040211029037 doi:10.1177/26330040211029037.

    PMID: 37181108
  19. 19

    Clinical and Structural Parameters in Autosomal Dominant Optic Atrophy Patients: A Cross-Sectional Study Using Optical Coherence Tomography.

    Camós-Carreras A, Figueras-Roca M, Albà-Arbalat S, et al.

    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society 2024; (45(3)):273-277 doi:10.1097/WNO.0000000000002294.

    PMID: 39805076
  20. 20

    Correlation between quality of vision and clinical and structural parameters in patients with Autosomal Dominant Optic Atrophy.

    Camós-Carreras A, Figueras-Roca M, Albà-Arbalat S, et al.

    Eye (London, England) 2025; (39(9)):1837-1842 doi:10.1038/s41433-025-03762-w.

    PMID: 40140688

This page explains the differences between ADOA and LHON for educational purposes only. Always consult your ophthalmologist or genetic counselor for an accurate diagnosis, symptom monitoring, and personalized care plan.

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