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Living with Neuropathy with hearing impairment

An inherited condition that combines nerve damage in the arms and legs with hearing loss, starting in adulthood.

Understanding Neuropathy with hearing impairment

Neuropathy with hearing impairment is a condition that combines hearing loss from the inner ear with peripheral neuropathy (damage to nerves outside the brain and spinal cord).

GARD lists onset in adulthood. It is autosomal dominant.

Signs & symptoms

GARD doesn't list specific symptoms for this condition yet. The summary describes hearing loss and nerve damage.

  • Hearing loss (sensorineural)
  • Nerve damage outside the brain and spinal cord (peripheral neuropathy)

Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.

Diagnosis

  1. Talk with your doctor

    Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.

  2. See a specialist

    Specialists who may be involved include brain and nervous system specialists, ear, nose, and throat specialists and genetic specialists.

  3. Consider genetic testing

    Changes in GJB3 are linked to this condition. Genetic testing and counseling can help confirm a diagnosis and explain what it means for your family.

  4. Connect with expert centers

    The Foundation for Peripheral Neuropathy may keep lists of experts for this condition.

Care & treatment

Care team
Brain and nervous system specialists (neurologists); Ear, nose, and throat specialists (otolaryngologists); Genetic specialists (geneticists)
Expert care
Expert lists: The Foundation for Peripheral Neuropathy
Treatment
GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
Research
Clinical trials and observational studies may be options. See the research section below.

General information, not medical advice. Your care team makes the decisions.

Everyday life

Living with a rare condition is easier with support.

  • Connect with The Foundation for Peripheral Neuropathy and other patient organizations
  • Keep notes on symptoms, changes and questions for appointments
  • Ask about patient registries that help research
  • Look into financial aid and travel resources for care visits
Bring these questions to your next doctor visit
Should we have genetic testing for the GJB3 gene?
How often should hearing and nerve function be checked?
Which aids can help with hearing loss?

Quick facts

Symptoms usually startAdult
InheritanceAutosomal Dominant
GenesGJB3

GARD lists changes in the GJB3 gene. The condition is autosomal dominant, which means one changed copy of the gene can be enough to cause it.

GeneticNeurological

Also known as: peripheral neuropathy with sensorineural hearing impairment syndrome

Patient organizations

Source: NIH GARD · Neuropathy with hearing impairmentGARD ID 19919 · GARD page updated September 2026 · Plain-language summary by Know Rare; editorial review pending. General information, not medical advice.

You are more than your diagnosis

Perspectives from people navigating life with Neuropathy with hearing impairment.

Explore the Neuropathy with hearing impairment blog

Explore research for Neuropathy with hearing impairment

Explore actively recruiting Neuropathy with hearing impairment studies with HealthSpark.

HealthSpark

Find actively recruiting studies for Neuropathy with hearing impairment with HealthSpark. Share your city to explore research near you.

  • Recruiting studies from ClinicalTrials.gov
  • Share your city to look for research near you
  • Eligibility is confirmed by the study team

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Powered by HealthSpark, our AI chat helper · Study information from ClinicalTrials.gov · Eligibility is confirmed by the study team.

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