Living with Hereditary sensory neuropathy-deafness-dementia syndrome
An inherited nerve condition with hearing loss, memory and thinking decline, and loss of feeling in the feet and legs.
Understanding Hereditary sensory neuropathy-deafness-dementia syndrome
The features are hearing loss, a decline in thinking skills (dementia), and worsening loss of feeling in the feet and legs. Hearing loss gets worse over time and often becomes moderate to severe between ages 20 and 35. Dementia typically starts in the thirties, sometimes after personality changes. Loss of feeling usually begins in adolescence or early adulthood. Some have seizures and sleep problems. It starts in the teen years or adulthood.
Signs & symptoms
Symptoms vary from person to person. GARD lists these among the most common:
- Hearing loss
- Loss of feeling in the feet and legs
- Decline in memory and thinking (dementia)
- Personality changes
- Trouble walking
- Seizures and sleep problems
Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.
Diagnosis
- 1Talk with your doctor
Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.
- 2See a specialist
Specialists who may be involved include brain and nervous system specialists, ear, nose, and throat specialists and genetic specialists.
- 3Consider genetic testing
Changes in DNMT1 are linked to this condition. Genetic testing and counseling can help confirm a diagnosis and explain what it means for your family.
- 4Connect with expert centers
Hereditary Neuropathy Foundation Inc. and 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: Hereditary Neuropathy Foundation Inc., 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 Hereditary Neuropathy Foundation Inc. 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
Quick facts
It is caused by changes in the DNMT1 gene. It is autosomal dominant, so one changed copy of the gene is enough.
Also known as: dnmt1-related dementia, deafness, and sensory neuropathy; hereditary sensory and autonomic neuropathy type 1e; hereditary sensory autonomic neuropathy type ie; hereditary sensory neuropathy type ie; hereditary sensory neuropathy-sensorineural hearing loss-dementia syndrome; hsan1e
Patient organizations
- Hereditary Neuropathy Foundation Inc.Hereditary sensory neuropathy-deafness-dementia syndrome · United States
- HSAN1E SocietyHereditary sensory neuropathy-deafness-dementia syndrome · United States
- The Foundation for Peripheral NeuropathyPeripheral neuropathy · United States
- Neuropathy Action FoundationPeripheral neuropathy · United States
You are more than your diagnosis
Perspectives from people navigating life with Hereditary sensory neuropathy-deafness-dementia syndrome.
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