A family history of sudden cardiac death and congenital sensorineural deafness suggests a diagnosis of what syndrome?

A family history of sudden cardiac death combined with congenital sensorineural deafness indicates Jervell and Lange-Nielsen syndrome. This condition is a rare genetic disorder that is characterized primarily by bilateral sensorineural hearing loss and a prolonged QT interval, which can lead to life-threatening arrhythmias and sudden cardiac death.

In Jervell and Lange-Nielsen syndrome, the hearing loss is due to bilateral sensory-neural deafness, which is present from birth. The connection between deafness and cardiac issues is significant, as the genetic mutation affecting ion channels (typically involved in cardiac repolarization) also impacts the auditory system. This correlation is distinctive to this syndrome, underlining the importance of a comprehensive family history that reveals both sudden cardiac events and congenital deafness.

Other options, while related to cardiac conditions, do not encompass this unique combination of symptoms. Long QT syndrome, while also involving a prolonged QT interval and potential for arrhythmic events, does not specifically include congenital deafness as a feature. Marfan syndrome does encompass cardiac issues but primarily affects connective tissues and does not present with hearing loss. Brugada syndrome is characterized by a specific ECG pattern and a risk for sudden cardiac death but lacks the hearing loss component. Hence, the presence