Jervell and Lange-Nielsen syndrome (JLNS) is a rare condition that can lead to life-threatening arrhythmias. Symptoms include fainting and hearing loss in infancy and childhood. Healthcare providers prescribe medicine first, but children may also need medical devices for their heart and ears.
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Jervell and Lange-Nielsen syndrome (JLNS) is a condition your child can have at birth that causes an abnormal heart rhythm and hearing loss. It’s a rare type of long QT syndrome. With this issue, their heart takes longer than usual to get ready for the next beat.
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JLNS is a genetic condition that can cause serious complications like cardiac arrest, which can be fatal. Early treatment can help prevent these life-threatening complications.
Some people don’t have symptoms. Others may have mild or severe symptoms that start in early childhood and improve in adulthood. Jervell and Lange-Nielsen syndrome symptoms include:
Variations in the KCNE1 and KCNQ1 genes cause this condition. To get this condition, both of a child’s biological parents have to be carriers of the variant. Some carriers of the variant may have heart issues but no hearing problems.
Any biological child of two carriers is at risk of inheriting this condition. But it’s more likely that they won’t. Carriers pass this variation down in an autosomal recessive pattern. With this pattern, children are more likely to be carriers of the variation than to get JLNS.
Torsades de Pointes can progress to ventricular fibrillation. This abnormal heart rhythm can lead to cardiac arrest. Going into cardiac arrest can be fatal, which is why it’s important to get treatment for this syndrome.
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People with this condition may also have iron-deficiency anemia, which their healthcare provider can treat.
A healthcare provider can see an unusual pattern on an electrocardiogram (EKG) if your child has JLNS. They’ll see a long QT interval (contraction phase) on the EKG. This means your child’s heart takes longer than usual to prepare for its next heartbeat.
A blood test can check for the genetic variation that causes Jervell and Lange-Nielsen syndrome. You may speak with a genetic counselor about the results.
Your child’s provider may give them a physical exam and ask about their biological family’s medical history. An audiologist (hearing expert) may check your child’s hearing.
Treatment for JLNS may include medicine, medical devices and procedures, like:
Your child needs medical attention if they faint or can’t hear. Even if these symptoms are from other causes, a provider should treat these problems.
With a Jervell and Lange-Nielsen syndrome diagnosis, your child will need regular follow-up visits to prevent complications. If your child has a medical device for their heart or ears, their provider may check it regularly.
Questions to ask your child’s provider may include:
Without treatment, half of children with this syndrome don’t survive their teen years. Early treatment with beta-blockers can improve their outlook (prognosis).
The prognosis depends on sex and which gene has the variation. The prognosis is better for females and those with a variation in the KCNE1 gene.
Healthcare providers may advise children with this syndrome to avoid activities that can trigger a fainting spell. These may include things like sports or amusement parks. It may also help to do things that can keep their QT interval (contraction phase) from getting longer. These include:
Learning that your child has a rare condition like Jervell and Lange-Nielsen syndrome isn’t easy. But it explains why your child faints and has hearing issues. One of the best things that you can do for your child is to make sure they’re getting the treatment they need. This includes making sure they take the medicine their healthcare provider prescribes for them. Don’t be afraid to ask questions or seek support for yourself as you ensure your child has what they need.
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Cleveland Clinic’s health articles are based on evidence-backed information and review by medical professionals to ensure accuracy, reliability and up-to-date clinical standards.
Cleveland Clinic’s health articles are based on evidence-backed information and review by medical professionals to ensure accuracy, reliability and up-to-date clinical standards.
When your child has long QT syndrome, you’ll want care from experts familiar with this rare heart condition. Cleveland Clinic Children’s has the providers you need.
