Jervell and lange-nielsen syndrome in four jordanian families

الملخص EN

Objective: To report the importance of the cardiologic aspect in the evaluation of syncope in children with congenital deafness.

Methods: A total of 16 deaf-mute siblings in four Jordanian families were studied.

Each family had at least one sibling with congenital deafness and syncopal attacks.

Audiogram confirmed the profound sensorineural hearing loss in all the siblings.

Electrocardiogram was done in all symptomatic siblings and corrected QT interval was calculated.

Screening for corrected QT interval prolongation was performed in the asymptomatic siblings and parents.

Results: Eight siblings had abnormal prolongation of corrected QT interval and were diagnosed as Jervel- Lange-Nielsen syndrome (congenital sensorineural deafness and abnormal prolongation of corrected QT interval).

The syncope in all symptomatic siblings was controlled with the use of propranolol while the asymptomatic siblings were given propranolol for prophylaxis.

Conclusion: Electrocardiogram is an important diagnostic test in congenitally deaf children, especially those with syncopal episodes.