Jervell And Lange Nielsen Syndrome 2 C2676723
A genetic subtype of Jervell and Lange Nielsen syndrome linked to KCNE1 gene changes. Presents with congenital hearing loss and cardiac rhythm disturbances.
Key facts at a glance
- ICD-10
- Q24.9
- ICD-11
- LA00.0
- Body system
- cardiovascular
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Ventricular tachycardia
Syncope
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hearing loss
(cardiovascular)Palpitations
(cardiovascular)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
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Beta-blocker therapy
First-line pharmacological treatment using non-selective beta-blockers to reduce sympathetic stimulation and prevent potentially fatal cardiac arrhythmias.
Implantable cardioverter-defibrillator (ICD)
Surgical implantation of an ICD for patients at high risk of ventricular arrhythmias or those who have survived cardiac arrest despite beta-blocker therapy.
Additional treatment options
Activity restriction and trigger avoidance
Lifestyle modifications including avoidance of strenuous exercise, competitive sports, and known QT-prolonging triggers such as certain medications and electrolyte disturbances.
lifestyleCochlear implantation
Surgical placement of a cochlear implant to restore auditory function in patients with congenital sensorineural deafness associated with the syndrome.
complementaryRegular cardiac monitoring and genetic screening
Lifelong ECG monitoring, periodic Holter monitoring, and regular cardiology follow-up along with cascade genetic testing for family members.
monitoringWhen 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 Jervell And Lange Nielsen Syndrome 2 C2676723?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Jervell And Lange Nielsen Syndrome 2 C2676723. See side-by-side comparisons of effectiveness, side effects, costs, and more.
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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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy