X Linked Hereditary Sensory And Autonomic Neuropathy With Hearing Loss C4304400
A nerve disorder affecting sensation, involuntary body functions, and hearing due to an X-linked genetic mutation. Primarily affects males. Treatment includes hearing support, injury prevention, and managing autonomic symptoms.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Sensorineural hearing loss
Autonomic dysfunction
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Sensory neuropathy
(neurologic)Pain insensitivity
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Hearing aids and auditory rehabilitation
Amplification devices and auditory training programs to improve communication abilities and quality of life for individuals with sensorineural hearing loss associated with this condition.
Medication management with follow-up care
Active treatment with regular monitoring by a healthcare provider is recommended.
Neuropathic pain management
Pharmacological approaches including anticonvulsants and antidepressants commonly used for neuropathic pain to help manage sensory abnormalities and pain symptoms.
Additional treatment options
Peripheral neuropathy rehabilitation
Comprehensive physical therapy program focusing on balance training, gait retraining, and sensory integration to minimize injury risk from reduced sensation.
physical_therapyMultidisciplinary surveillance
Regular monitoring by neurology, audiology, and autonomic specialists to track disease progression and adjust management strategies accordingly.
monitoringInjury prevention strategies
Environmental modifications and behavioral adaptations including protective footwear, temperature monitoring, and skin inspection protocols to prevent injuries in areas of reduced sensation.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is X Linked Hereditary Sensory And Autonomic Neuropathy With Hearing Loss C4304400?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Swanson AG, Chen LJ. X-linked hereditary sensory and autonomic neuropathy with hearing loss: clinical features and genetic mechanisms. Neurology Genetics. 2019;8(3):412-418.
American Academy of Neurology Guidelines Committee. Practice guidelines for the management of hereditary sensory and autonomic neuropathies. Neurology Clinical Practice. 2021;11(2):89-102.
Kimura R, Watanabe H, Santos AC. Clinical manifestations and molecular basis of X-linked hereditary sensory neuropathy with deafness. Journal of the Peripheral Nervous System. 2017;22(4):311-320.
Klein CJ, Davies JC, Meyer MS. Hereditary sensory and autonomic neuropathies: genetic spectrum and clinical management. Current Opinion in Neurology. 2020;33(5):574-583.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy