Audiology · Autosomal Dominant Optic Atrophy Plus

When Does Hearing Loss Start in ADOA-Plus Syndrome?

At a Glance

Hearing loss in ADOA-Plus typically begins in late childhood, adolescence, or early adulthood, though it can sometimes start later in life. It presents as auditory neuropathy, meaning patients can hear volume but struggle to understand speech, especially in noisy environments.

While the vision changes associated with classic Autosomal Dominant Optic Atrophy (ADOA) typically begin in early childhood, the hearing loss associated with ADOA-Plus usually develops later ^[1]. For most patients who develop hearing issues, the onset occurs in late childhood, adolescence, or early adulthood ^[1]. In some cases, neurological and hearing symptoms may not become apparent until the fifth or sixth decades of life ^[2].

Understanding Hearing Loss in ADOA-Plus

Hearing loss is the most common “plus” symptom (extra-ocular feature) in ADOA-Plus syndrome ^[3]. It happens most often in patients who have specific types of mutations in the OPA1 gene, particularly missense mutations (a genetic change where a single building block of the gene is altered) ^[3]^[1]. You can check your genetic testing report to see your specific mutation type, and your doctor can help you understand what it means for your individual risk.

The hearing loss in ADOA-Plus is specifically a type of sensorineural hearing loss known as auditory neuropathy ^[4]^[3]. This means the inner ear (cochlea) may still function normally, but the auditory nerve that carries sound signals to the brain is affected ^[3]. Patients with auditory neuropathy often have specific difficulties with speech perception—meaning they can hear sounds and volume, but have trouble understanding spoken words, especially in noisy environments ^[3].

Learning that you may face hearing challenges in addition to vision loss can be emotionally overwhelming. Knowing how this hearing loss works and how to effectively screen for it is an important step in maintaining your connection to the world and advocating for your care.

The Importance of Hearing Screening

Because the exact age of onset can vary widely from person to person, proactive monitoring is essential ^[1].

Baseline Screening: Every patient diagnosed with an OPA1 mutation linked to ADOA-Plus should have a baseline audiometric evaluation ^[1]. This provides a clear picture of current hearing function to compare against in the future.
Annual Follow-ups: Regular, annual hearing tests are strongly recommended to catch any early signs of auditory neuropathy ^[1].
Specialized Testing: Because ADOA-Plus affects the auditory nerve, standard beep tests (which only check volume) might not be enough. Screenings should include two specific, non-invasive evaluations: otoacoustic emissions (OAEs), which confirm that the physical inner ear is working, and an auditory brainstem response (ABR) test, which measures the actual electrical nerve response to sound ^[3].

Managing Changes in Hearing

If you notice new or worsening changes in your hearing, increased ringing in your ears (tinnitus), or increasing difficulty understanding conversations in crowded rooms, contact an audiologist or an Ear, Nose, and Throat (ENT) specialist.

Because the problem in ADOA-Plus lies in the nerve’s ability to clearly transmit speech signals, standard hearing aids often provide limited benefit—they amplify volume, but do not make garbled speech clearer ^[3]. However, for severe cases of auditory neuropathy in ADOA-Plus, surgical interventions like cochlear implants have been shown to improve speech perception by bypassing the damaged connections in the inner ear to directly stimulate the auditory nerve fibers ^[3].

Common questions in this guide

At what age does hearing loss typically begin in ADOA-Plus?

Hearing loss in ADOA-Plus usually develops in late childhood, adolescence, or early adulthood. However, the exact timeline varies, and in some individuals, auditory symptoms may not appear until their 40s or 50s.

What type of hearing loss is associated with ADOA-Plus?

Patients with ADOA-Plus typically develop a type of sensorineural hearing loss called auditory neuropathy. This occurs when the inner ear functions normally, but the auditory nerve struggles to clearly transmit sound signals to the brain.

Will standard hearing aids work for ADOA-Plus hearing loss?

Standard hearing aids often provide limited benefit because they amplify volume rather than clarifying garbled speech. For severe cases of auditory neuropathy, surgical interventions like cochlear implants may be recommended to improve speech perception.

How should I be screened for hearing loss if I have an OPA1 mutation?

Patients should have a baseline audiometric evaluation followed by annual check-ups. Because the condition affects the auditory nerve, screenings must include otoacoustic emissions (OAEs) and auditory brainstem response (ABR) testing.

Curated prompts to bring to your next appointment.

1.Based on my specific OPA1 genetic report, am I at higher risk for developing ADOA-Plus symptoms like hearing loss?
2.Do you have experience screening for auditory neuropathy, or should I be referred to a specialized neuro-otologist?
3.Can you ensure that both OAE and ABR testing are included in my baseline and annual hearing evaluations?
4.At what age should we begin the annual hearing screening process for my child with an OPA1 mutation?

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References (4)

1
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
2
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
3
OPA1-related auditory neuropathy: site of lesion and outcome of cochlear implantation.
Santarelli R, Rossi R, Scimemi P, et al.
Brain : a journal of neurology 2015; (138(Pt 3)):563-76 doi:10.1093/brain/awu378.
PMID: 25564500
4
Distinct genetic patterns and natural history of OPA1-related auditory neuropathy in Chinese population.
Wang H, Shi T, Wang W, et al.
Orphanet journal of rare diseases 2025; (20(1)):520 doi:10.1186/s13023-025-04040-4.
PMID: 41107984

This page provides educational information about hearing loss timelines and screening in ADOA-Plus. It is not a substitute for professional medical advice, diagnosis, or treatment from your audiologist, geneticist, or ENT specialist.

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