Autosomal Dominant Non-syndromic Hearing Loss and Deafness Linked to MYO6 (DFNA22)

Autosomal Dominant Non-syndromic Hearing Loss and Deafness Linked to MYO6 (DFNA22) is an inherited, progressive, sensorineural hearing loss that runs in families in an autosomal-dominant pattern. It’s caused by harmful changes (variants) in a gene called MYO6 (myosin VI) on chromosome 6 (region 6q13–q14.1). Myosin VI is a tiny motor protein inside inner-ear hair cells; when it doesn’t work properly, those cells can’t move and transmit sound signals normally, and hearing slowly worsens over time. Many people start with high-frequency hearing loss after learning to speak (post-lingual) and later lose hearing across all pitches; balance problems may occur in some families. Karger Publishers+3PMC+3OUP Academic+3 MYO6 helps hair cells in the cochlea move cargo along actin “tracks” and maintain stereocilia (the fine “bristles” that detect sound). Pathogenic MYO6 variants can disrupt this motor action, harm stereocilia structure and inner-hair-cell synapses, and lead to progressive sensorineural hearing loss. Both dominant (DFNA22) and recessive (DFNB37) MYO6-related deafness are described in people and in animal models, underscoring its essential role. PMC+1

DFNA22 is a kind of inherited hearing loss that runs in families in a dominant way. A single harmful change (variant) in one copy of the MYO6 gene can cause it. MYO6 makes myosin VI, a tiny motor protein that helps hair cells in the inner ear move things inside the cell and keep their stiff “hairs” (stereocilia) healthy. When MYO6 does not work properly, the hair cells slowly stop working and hearing goes down over time. NCBI+2PMC+2

Most people with DFNA22 develop progressive, sensorineural hearing loss that often starts in the high frequencies and appears after speech has developed (post-lingual). With time, it can involve more frequencies and get worse. Some families also report balance symptoms, but this is not constant. PreventionGenetics+1

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Other names

• Deafness, autosomal dominant 22

• DFNA22

• Autosomal dominant non-syndromic hearing loss 22

• MYO6-related autosomal dominant non-syndromic hearing loss National Organization for Rare Disorders+1

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Types

Although the genetic cause is the same gene (MYO6), doctors may group DFNA22 by how it looks on hearing tests and when it starts:

1. By age of onset

• Childhood–early teen onset: first changes noticed around school years; slow worsening over decades. PreventionGenetics

• Adult onset: normal hearing in youth, then gradual decline in high tones in young adulthood or later. Karger Publishers

2. By audiogram shape

• High-frequency sloping loss (most common description). PreventionGenetics

• Flat or mid-frequency involvement reported in some families. Karger Publishers

3. By speed of progression

• Slowly progressive (typical).

• Moderately progressive (faster shift at 4–8 kHz in some families). Karger Publishers

These patterns reflect how different MYO6 variants disturb stereocilia and intracellular trafficking in hair cells. PMC

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Causes

Important note: The root cause of DFNA22 is always a harmful variant in one MYO6 gene copy. The items below explain how different variant types or biologic factors can lead to the condition or modify what you experience. NCBI+1

1. Pathogenic MYO6 variant (core cause). A single damaging change in MYO6 disrupts myosin VI in cochlear hair cells and triggers progressive sensorineural hearing loss. PubMed

2. Missense variants (amino-acid change). Some families have a single letter change that alters myosin VI structure or motion, reducing hair-cell function. PubMed+1

3. Nonsense or frameshift variants. Premature stop signals can shorten the protein, leading to loss of function. PMC

4. Splice-site variants. Changes at intron–exon borders can mis-assemble MYO6 messenger RNA and yield faulty protein. PMC

5. Haploinsufficiency. Having only one working copy may produce too little myosin VI, which is not enough for stable stereocilia over time. (Shown in animal models.) ScienceDirect

6. Dominant-negative effect. Some altered myosin VI proteins may interfere with normal protein, worsening dysfunction inside hair cells. PMC

7. Defects in actin-attachment. Myosin VI needs to bind actin to move cargo; disturbed binding can impair hair-bundle maintenance. Frontiers

8. Defects in cargo trafficking. Myosin VI moves materials in and out of membrane areas; trafficking failure stresses the hair cell. PMC

9. Stereocilia maintenance failure. Over years, tiny hair-bundle structures degenerate, especially at high-frequency regions of the cochlea. Frontiers

10. Cochlear base vulnerability. High-frequency zones at the base are more fragile; MYO6 problems show there first. PreventionGenetics

11. Modifier genes. Other hearing genes may soften or worsen the phenotype in different families. (General genetic-hearing-loss concept.) Frontiers

12. Age-related stress. Aging adds cellular stress and can speed progression in genetically affected hair cells. ScienceDirect

13. Noise exposure (modifier). Loud sounds do not cause DFNA22, but they can accelerate loss in vulnerable hair cells. (General HL principle.) MDPI

14. Ototoxic drugs (modifier). Certain medicines (e.g., aminoglycosides) may worsen hearing in people with genetic risks. (General HL principle.) MDPI

15. Mitochondrial energy stress (modifier). Hair cells require high energy; stress may accentuate MYO6-related dysfunction. (General concept in genetic HL.) Frontiers

16. Cellular recycling (endocytosis) defects. Myosin VI supports membrane turnover; faults here strain stereocilia. PMC

17. Cytoskeletal instability. Weak actin networks make hair bundles less resilient to daily micro-movements. Frontiers

18. Variant location within MYO6 domains. Changes in motor or cargo-binding regions can produce different severities and audiogram shapes. PMC

19. Allelic heterogeneity. Many different MYO6 variants across families explain variable ages of onset and patterns. PubMed

20. Recessive MYO6 variants cause a different disorder (DFNB37). This helps doctors confirm that the dominant pattern in your family fits DFNA22, not DFNB37. PMC

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Symptoms

1. Trouble hearing high-pitched sounds first. Birds, beeps, or consonants may become unclear. PreventionGenetics

2. Difficulty understanding speech in noise. Voices blur in busy rooms even when it seems “loud enough.” MDPI

3. Gradual worsening over years. The change is slow but measurable on repeated hearing tests. Karger Publishers

4. Normal ear exam. The eardrum usually looks healthy because the problem is in the inner ear. MedlinePlus

5. Bilateral involvement. Both ears are typically affected to a similar degree. MDPI

6. Post-lingual onset. Speech has already developed before hearing loss begins. PreventionGenetics

7. Tinnitus (ringing). Some people notice a ringing or hissing tone. MDPI

8. Sound distortion. Sounds may seem sharp or tinny. MDPI

9. Recruitment. Loudness seems to jump quickly from soft to too loud. MDPI

10. Need for higher TV/phone volume. Everyday listening needs more amplification. MDPI

11. Trouble with phone calls. High-frequency loss makes speech on phones harder to follow. MDPI

12. Listening fatigue. Concentrating to hear causes tiredness and headache. MDPI

13. Balance symptoms in some families. A few reports note mild vestibular problems, but this is not universal. PreventionGenetics

14. Progression to broader-frequency loss. With age, mid and low frequencies can join the deficit. Karger Publishers

15. Family history. Many relatives across generations show similar patterns, fitting dominant inheritance. PubMed

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Diagnostic tests

A) Physical exam (ear-nose-throat focused)

1. Otoscopy. Doctor looks at eardrum; in DFNA22 it is usually normal, which points to an inner-ear (sensorineural) problem. MedlinePlus

2. Head and neck exam. Checks for syndromic signs; DFNA22 is non-syndromic, so other organ problems are not expected. MedlinePlus

3. Family pedigree review. Maps who is affected across generations to confirm autosomal dominant pattern. MDPI

4. Basic neurologic/vestibular screen. Looks for imbalance or nystagmus in families where balance symptoms occur. PreventionGenetics

B) Manual bedside tests

5. Rinne test (tuning fork). Compares air vs bone conduction; sensorineural loss keeps Rinne positive. Helps rule out conductive loss. MedlinePlus

6. Weber test (tuning fork). Sound lateralizes to the better ear in sensorineural loss; suggests inner-ear origin. MedlinePlus

7. Whisper/voice tests. Quick screen that may show reduced clarity, prompting full audiology work-up. MedlinePlus

C) Lab & pathological / genetic diagnostics

8. Targeted MYO6 sequencing. Confirms a pathogenic variant and clinches the DFNA22 diagnosis. PubMed

9. Copy-number analysis (deletions/duplications). Looks for larger gene changes if sequencing is negative but suspicion is high. ScienceDirect

10. Comprehensive deafness gene panel. Screens many hearing-loss genes while focusing on MYO6; useful when family history is unclear. ScienceDirect

11. Exome/genome sequencing. Broader test when panels are unrevealing or to study novel variants and domain effects. ScienceDirect

12. Segregation testing in relatives. Checks whether the same MYO6 variant tracks with hearing loss across the family. PubMed

13. Clinical-lab variant classification. Uses ACMG/AMP rules and functional data to label variants (pathogenic/likely). (General genetics workflow.) ScienceDirect

14. Genetic counseling session. Explains dominant inheritance (50% risk to children) and testing options. MDPI

D) Electrodiagnostic & audiology

15. Pure-tone audiometry. Measures thresholds; DFNA22 often shows high-frequency loss first, with progression over time. PreventionGenetics+1

16. Speech audiometry (word recognition). Assesses clarity; may be reduced, especially in noise. MDPI

17. Tympanometry. Middle-ear pressure and mobility are usually normal, supporting a sensorineural cause. MedlinePlus

18. Otoacoustic emissions (OAEs). Reduced or absent OAEs reflect outer hair-cell dysfunction from MYO6 impairment. Frontiers

19. Auditory brainstem response (ABR). Helps document sensorineural pathway function and rule out retrocochlear disease. MDPI

E) Imaging

20. High-resolution temporal-bone CT or inner-ear MRI. Usually normal in DFNA22, but imaging rules out other structural causes and helps pre-implant planning if needed. MDPI

Non-Pharmacological Treatments (Therapies & Others)

Each item explains what it is (≈150 words), the purpose, and the mechanism in simple terms.

1. Modern digital hearing aids (bilateral when indicated)
   What/How: Behind-the-ear or in-ear devices that pick up sound with microphones, process it digitally, and send amplified sound into the ear canal. Features like directional microphones, adaptive noise reduction, and feedback control make speech clearer. Purpose: Improve day-to-day hearing and speech understanding, especially in quiet or mild noise. Mechanism: They increase the loudness and clarity of incoming sounds at frequencies you can’t hear well, letting remaining hair cells and neural pathways work better. Evidence: Hearing aids are a first-line option for sensorineural loss, improving speech comprehension and participation. NIDCD+1

2. Real-ear-verified hearing-aid fitting and fine-tuning
   What/How: Audiologists program devices using probe-microphone measures in your ear to match scientifically derived amplification targets across frequencies. Purpose: Ensure amplification is safe, comfortable, and actually matches your audiogram. Mechanism: Precise verification prevents under- or over-amplification, improving benefit and reducing listening fatigue. Evidence: Verification and follow-up are core elements of adult hearing care. NIDCD

3. Remote microphone (RM/FM) systems
   What/How: A speaker wears a tiny mic; their voice streams wirelessly to your hearing aids/receivers, boosting the “signal” over classroom or meeting noise. Purpose: Improve speech understanding in noise, at distance, and in reverberant rooms (schools, meetings, clinics). Mechanism: Raises the talker’s voice level at your ear while reducing the relative background noise. Evidence: Guidelines and studies show RM systems significantly improve speech recognition in noise versus hearing aids alone. American Academy of Audiology+2PubMed+2

4. Cochlear implant (CI) candidacy evaluation
   What/How: If hearing worsens to severe/profound or aids give limited benefit, a CI team assesses speech scores, imaging, and medical fitness. Purpose: Decide if electrical stimulation of the auditory nerve will help more than acoustic amplification. Mechanism: A CI bypasses damaged hair cells and directly stimulates the nerve with coded electrical signals. Evidence: CIs are beneficial for many with severe/profound sensorineural loss; early implantation in children supports speech and language. NIDCD+1

5. Cochlear implant surgery and mapping (if indicated)
   What/How: Outpatient surgery places an electrode array in the cochlea; external processors are then “mapped” (programmed) over time. Purpose: Restore access to speech sounds when hearing aids no longer help. Mechanism: Micro-electrodes deliver patterns of current that the brain learns to interpret as sound. Evidence: FDA-approved CI systems with large real-world benefits for eligible children and adults. FDA Access Data+1

6. Electric-Acoustic Stimulation (EAS/Hybrid CI)
   What/How: Combines a short-electrode cochlear implant for high-frequency regions with low-frequency acoustic amplification to preserve residual hearing. Purpose: Maximize speech clarity and music perception when you still have low-frequency hearing. Mechanism: Acoustic amplification handles bass sounds; electrical stimulation covers treble sounds that are no longer accessible. Evidence: EAS is useful for progressive losses with preserved lows. PMC

7. Aural rehabilitation & auditory training
   What/How: Structured listening practice, communication strategies, and device coaching (often home-based computer programs + therapist sessions). Purpose: Improve brain-level processing of amplified/electrically coded sound. Mechanism: Neural plasticity—repeated, focused practice strengthens auditory pathways and top-down listening strategies. Evidence: Standard part of post-CI and hearing-aid care. NIDCD

8. Speech-language therapy (for children, and adults as needed)
   What/How: Therapy focused on articulation, receptive/expressive language, and auditory-verbal skills, especially after CI. Purpose: Support age-appropriate speech and language development and classroom participation. Mechanism: Intensive practice uses improved Hearing-in-noise to build language networks. Evidence: Early therapy after CI is associated with better outcomes. NIDCD

9. Classroom/Workplace accommodations
   What/How: Preferential seating, captioning, RM systems, quiet meeting rooms, written summaries, and teleconferencing captions. Purpose: Reduce listening effort and prevent missed information. Mechanism: Environmental and technological supports increase the signal-to-noise ratio and provide visual access to speech. Evidence: Widely recommended in audiology/education guidance for hearing loss. American Academy of Audiology

10. Captioning (live/remote) and communication apps
    What/How: Real-time captions for meetings, lectures, calls, and media; smartphone apps for transcription and sound-level monitoring. Purpose: Ensure access when noise or accents make listening hard. Mechanism: Visual text complements auditory input, reducing cognitive load. Evidence: Common best-practice adjunct in adult hearing care. NIDCD

11. Tinnitus counseling and cognitive-behavioral strategies (if tinnitus)
    What/How: Education, sound therapy, and CBT-style coping skills to reduce distress. Purpose: Lower the impact of tinnitus that often accompanies sensorineural loss. Mechanism: Reframes attention and stress pathways; external sound reduces perceived contrast with internal noise. Evidence: Standard supportive care for tinnitus with hearing loss. NIDCD

12. Vestibular (balance) assessment and therapy when indicated
    What/How: If dizziness occurs, formal vestibular testing and targeted physical therapy. Purpose: Improve balance, reduce fall risk. Mechanism: Exercises recalibrate visual, proprioceptive, and vestibular inputs. Evidence: Some DFNA22 families have vestibular issues; vestibular PT is evidence-based for balance disorders. PreventionGenetics

13. Noise exposure reduction / safe-listening habits
    What/How: Limit loud sound, use hearing protection, follow safe headphone levels. Purpose: Protect remaining hair cells and slow additional damage. Mechanism: Prevents noise-induced oxidative and mechanical injury on top of genetic vulnerability. Evidence: Core public-health guidance for hearing preservation. NIDCD

14. Avoidance/minimization of ototoxic medications and chemicals when possible
    What/How: Review meds with clinicians; flag aminoglycosides, cisplatin, loop diuretics, high-dose salicylates, and certain solvents. Purpose: Avoid additive ear damage. Mechanism: Many drugs/chemicals directly injure hair cells or synapses; risk is cumulative with noise. Evidence: Ototoxicity from several drug classes is well-documented; workplace chemicals can potentiate noise injury. Medscape+2CDC+2

15. Family genetic counseling and cascade testing
    What/How: Explain inheritance, test at-risk relatives, discuss family planning (e.g., prenatal/preimplantation options). Purpose: Early identification enables earlier hearing support and monitoring. Mechanism: Targeted testing finds the causal variant in relatives before major disability occurs. Evidence: Standard of care in hereditary hearing loss. Hereditary Hearing Loss

16. Routine audiologic follow-up (lifelong)
    What/How: Regular audiograms and device checks, with timely upgrades. Purpose: Match changing thresholds with appropriate technology. Mechanism: Progressive losses need periodic adjustments to maintain speech audibility. Evidence: Fundamental best practice. NIDCD

17. Tele-audiology support
    What/How: Remote programming, troubleshooting, and counseling. Purpose: Maintain access and adherence, especially for those distant from clinics. Mechanism: Secure remote connections let clinicians fine-tune gains and settings. Evidence: Increasingly adopted in adult hearing care. NIDCD

18. Auditory brainstem implant (ABI) evaluation in rare situations
    What/How: If the cochlea or auditory nerve cannot be used (very uncommon in DFNA22), an ABI stimulates brainstem nuclei directly. Purpose: Provide sound awareness when CI is not feasible. Mechanism: Electrodes placed on the cochlear nucleus bypass the cochlea entirely. Evidence: Reserved indications; part of the implantable-hearing-device continuum. UCHealth

19. Community & peer support / hearing-loss education
    What/How: Support groups, credible patient organizations, and family training. Purpose: Improve coping, device use, and communication in real life. Mechanism: Practical tips plus psychosocial support improve outcomes and adherence. Evidence: Embedded in adult hearing-health-care approaches. NIDCD

20. Early childhood intervention services (for affected children)
    What/How: Newborn screening follow-up, early fitting, therapy, and classroom supports. Purpose: Optimize language, literacy, and social development. Mechanism: Early auditory access and therapy during brain plasticity windows. Evidence: Early CI and intensive therapy improve language outcomes. NIDCD

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Drug Treatments

Crucial truth up front: There are no FDA-approved drugs that treat or reverse DFNA22 (MYO6-related) genetic hearing loss. Current medical literature and federal sources agree: for sensorineural hearing loss—including genetic forms—pharmacotherapies are not approved to restore hearing; management relies on devices and rehabilitation. Please avoid lists that imply approvals that do not exist. PubMed+1

What is FDA-approved in the hearing-loss space?
The FDA has approved sodium thiosulfate (Pedmark®) to reduce the risk of cisplatin-associated ototoxicity in pediatric cancer patients. This does not treat DFNA22; it prevents chemo-induced damage in a different scenario. It’s included here only because you asked for FDA-sourced drug evidence relevant to hearing outcomes. U.S. Food and Drug Administration+1

Below, for completeness, are illustrative drug summaries anchored to FDA or clinical-trial evidence relevant to hearing outcomes, with clear statements that none are approved for DFNA22:

1. Sodium thiosulfate (Pedmark®; FDA-approved for pediatric cisplatin ototoxicity risk reduction, not DFNA22)
   Class: Sulfur-donor/thiosulfate. Dose/Timing (per label): Weight-based IV dosing given 6 hours after cisplatin; see label for specifics and modifications. Purpose: Reduce risk of chemo-related inner-ear injury. Mechanism: Binds cisplatin (detoxifies platinum species) and reduces oxidative injury in cochlear hair cells. Key safety: Nausea, vomiting, electrolyte changes; not interchangeable with other STS products. Evidence source: FDA approval, labeling, and reviews. U.S. Food and Drug Administration+2FDA Access Data+2

2. Intratympanic sodium thiosulfate (DB-020; investigational)
   Class: Local otoprotectant (hyaluronate gel + STS). Dose/Timing: Intratympanic before cisplatin; research protocols only. Purpose: Prevent cisplatin ototoxicity by delivering high cochlear concentrations locally. Mechanism: Local detoxification of cisplatin in the inner ear. Safety: Study-monitored; transient ear discomfort possible. Evidence: Phase 1/1b studies; not FDA-approved. PubMed+2PMC+2

No other FDA-approved drugs exist to treat genetic DFNA22. Any additional “drug list” would be misleading. If you want, I can compile clinical-trial candidates (gene therapy/editing, neurotrophins, synaptopathy agents), clearly labeled as experimental.

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Dietary Molecular Supplements

Straightforward reality: No dietary supplement has high-quality evidence to treat or reverse DFNA22. Supplements sometimes show effects in noise or drug-induced models, but translation to human genetic hearing loss is unproven. Use caution and discuss with clinicians—some products interact with medicines. PubMed

If you still want a neutral, safety-first overview, I can outline commonly discussed nutraceuticals (e.g., antioxidants, omega-3s) with mechanisms (oxidative stress reduction, membrane support) and explicit statements that evidence does not support DFNA22 treatment. (Say the word and I’ll add them, with citations to human data where available.)

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Immunity-booster / Regenerative / Stem-cell Drugs

I cannot provide such a list because it does not exist. There are no FDA-approved immune-boosting, regenerative, or stem-cell drugs for restoring hearing in DFNA22—or any hereditary sensorineural hearing loss. Mislabeling products this way would be unsafe. What does exist: early research in gene/cell therapy and neurotrophic strategies; none are approved for MYO6-related loss today. Frontiers+1

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Surgeries

1. Cochlear implant (CI) surgery
   Procedure: Implant an electrode array in the cochlea via mastoid/round-window or cochleostomy approach; place internal receiver; close incision; activate later. Why: For severe–profound sensorineural loss when hearing aids no longer help, to deliver electrical hearing directly to the auditory nerve. Regulatory context: Multiple FDA-approved CI systems. FDA Access Data+1

2. Bilateral CI (staged or simultaneous)
   Procedure: CI in both ears (same day or separate). Why: Improve sound localization and hearing in noise when both ears meet criteria. Note: Center-specific protocols. Mayo Clinic

3. Hybrid (EAS) CI surgery
   Procedure: Short-electrode CI aimed at preserving low-frequency acoustic hearing while providing electrical highs. Why: Progressive high-frequency loss with residual lows (common pattern in DFNA22). PMC

4. Revision/upgrade CI surgery
   Procedure: Replace malfunctioning or outdated internal hardware. Why: Device failure, infection, or to access newer arrays when appropriate. Regulatory context: Ongoing PMA supplements for device iterations. FDA Access Data

5. Auditory brainstem implant (ABI)
   Procedure: Place an electrode pad on the cochlear nucleus in the brainstem. Why: Rarely, if the cochlea/auditory nerve cannot be used (not typical for DFNA22). UCHealth

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Preventions

1. Protect your ears from loud sound (music, tools, traffic). Gene-related loss can be worsened by noise. Use plugs/muffs; follow safe-listening rules. NIDCD

2. Limit ototoxic drug exposure when alternatives exist. Discuss risks (aminoglycosides, cisplatin, loop diuretics, high-dose salicylates) with your clinicians. Medscape

3. Manage chemical exposures at work. Some solvents/chemicals increase ear damage, especially with noise. Use PPE and ventilation. CDC

4. Get routine audiology follow-ups. Adjust technology as thresholds change; earlier action helps. NIDCD

5. Use hearing tech consistently. Daily wear improves brain adaptation and outcomes. NIDCD

6. Consider vaccination per CI guidance. If you receive a CI, follow pneumococcal vaccination recommendations to lower meningitis risk (center-specific protocols). NIDCD

7. Adopt safe-communication habits. Favor face-to-face, good lighting, quiet spots; use captioning when needed. NIDCD

8. Support cardiovascular health. While not DFNA22-specific, general vascular health supports cochlear function; follow standard preventive care. NIDCD

9. Early intervention for children. Prompt fitting/therapy after diagnosis maximizes language development. NIDCD

10. Family genetic counseling. Identify at-risk relatives early for monitoring and timely support. Hereditary Hearing Loss

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When to See Doctors (red flags)

• Rapid hearing drop or sudden deafness—urgent ENT/audiology evaluation. Sudden sensorineural loss is a medical urgency and may be managed differently from genetic progression. NIDCD

• New dizziness, unsteadiness, or falls, especially with hearing changes—seek vestibular assessment. PreventionGenetics

• Hearing aids no longer helping—ask about CI candidacy. NIDCD

• Children with suspected delay—request early hearing evaluation, technology fitting, and language therapy without delay. NIDCD

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What to Eat and What to Avoid

What to eat: A heart-healthy pattern (fruits, vegetables, whole grains, lean proteins, omega-3-rich fish) supports overall vascular and neural health; hydrate well and maintain good sleep for listening stamina. These are general wellness measures—not specific DFNA22 treatments. NIDCD

What to avoid/limit: Excessive alcohol; tobacco; unnecessary exposure to ototoxic medicines or workplace chemicals; and loud sound without protection. Always discuss medication changes with your clinician—some necessary drugs have ear-risk that can be managed with monitoring and dose strategies. Medscape+1

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Frequently Asked Questions

1. Is DFNA22 curable with medicine today?
   No. There’s no approved drug that reverses genetic hearing loss; care relies on devices and rehab. PubMed

2. Does DFNA22 always get worse?
   Usually it’s progressive, starting in high pitches and extending over time, but speed varies by family and variant. Karger Publishers

3. Can hearing aids help?
   Yes—especially in mild-to-moderate stages. They amplify the sounds you can’t hear well and improve speech audibility. NIDCD

4. When do people consider a cochlear implant?
   When hearing aids no longer give enough benefit and speech scores stay low, a CI can restore access to speech cues. NIDCD

5. Do cochlear implants work for genetic losses like MYO6?
   Many people with genetic sensorineural loss benefit from CIs because they bypass hair-cell damage and stimulate the nerve directly. PMC

6. Will a CI restore “normal” hearing?
   No, but it can allow understanding of speech, conversation in many settings, and phone use for many recipients after rehab. NIDCD

7. Are there medicines to protect against drug-induced hearing loss?
   Yes—sodium thiosulfate (Pedmark®) is FDA-approved to reduce cisplatin ototoxicity risk in pediatric oncology. This does not treat DFNA22. U.S. Food and Drug Administration

8. What about gene therapy?
   Promising in animals and early human programs (e.g., OTOF), but not approved for MYO6-related loss yet. NIDCD

9. Could supplements fix DFNA22?
   No reliable human evidence shows supplements reverse inherited sensorineural loss; discuss any product with your clinician. PubMed

10. Why avoid loud sound if the problem is genetic?
    Noise adds extra damage to already-vulnerable hair cells, speeding decline. NIDCD

11. Which medicines can harm hearing?
    Classes include aminoglycoside antibiotics, cisplatin, some loop diuretics, and high-dose salicylates; always review risk/benefit with your doctor. Medscape

12. Do remote microphones really help?
    Yes—solid evidence shows better speech understanding in noise compared with hearing aids alone. PubMed

13. Is DFNA22 the same as DFNB37?
    No. Both involve MYO6, but DFNA22 is dominant (often progressive, post-lingual) while DFNB37 is recessive (often congenital/profound). PMC

14. Should my family get tested?
    Genetic counseling and cascade testing help relatives plan early hearing support and life decisions. Hereditary Hearing Loss

15. How common are implants?
    Over a million CIs worldwide; in the U.S., ~118,100 adults and ~65,000 children have devices (2024 NIDCD stat). NIDCD

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: October 03, 2025.

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