What is Jervell and Lange-Nielsen syndrome?

Medical Advisory BoardAll articles are reviewed for accuracy by our Medical Advisory Board
Educational purpose only • Exercise caution as content is pending human review
Article Review Status
Submitted
Under Review
Approved

Last updated: February 22, 2025View editorial policy

Personalize

Help us tailor your experience

Which best describes you? Your choice helps us use language that's most understandable for you.

From the Research

Jervell and Lange-Nielsen syndrome is a rare genetic disorder characterized by a combination of congenital deafness, abnormal heart rhythms, and a high risk of sudden death. The key features of Jervell and Lange-Nielsen syndrome include:

  • Congenital deafness
  • Abnormal heart rhythms, particularly a prolonged QT interval
  • High risk of sudden death, often due to cardiac arrhythmias Unfortunately, none of the provided studies [ 1, 2, 3, 4, 5 ] directly address Jervell and Lange-Nielsen syndrome, and therefore, no specific study can be cited to support this answer. However, based on general medical knowledge, it is known that Jervell and Lange-Nielsen syndrome is a genetic disorder that affects the heart and hearing. Treatment for Jervell and Lange-Nielsen syndrome typically focuses on managing the cardiac symptoms and preventing sudden death, and may include:
  • Medications to regulate heart rhythm
  • Implantable cardioverter-defibrillators (ICDs) to prevent sudden death
  • Avoidance of certain medications and activities that may trigger cardiac arrhythmias Genetic testing can confirm the diagnosis, and patients and family members may benefit from genetic counseling, as the condition is inherited in an autosomal recessive pattern.

References

Research

Reducing mortality in near-hanging patients with a novel early management protocol.

The American journal of emergency medicine, 2018

Research

Symptomatic treatment, care, and support of CJD patients.

Handbook of clinical neurology, 2018

Research

When do symptoms become a disease?

Annals of internal medicine, 2001

Research

Chronic traumatic encephalopathy is not a real disease.

Archives of clinical neuropsychology : the official journal of the National Academy of Neuropsychologists, 2018

Research

Obesity: A preventable, treatable, but relapsing disease.

Nutrition (Burbank, Los Angeles County, Calif.), 2020

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

Have a follow-up question?

Our Medical A.I. is used by practicing medical doctors at top research institutions around the world. Ask any follow up question and get world-class guideline-backed answers instantly.