Treatment The treatment of individuals with JLNS is aimed at treating hearing loss and preventing characteristic symptoms such as loss of consciousness or cardiac arrest. Specific medications, avoidance of triggering events such as competitive sports, triggers that elicit intense emotions (Tranebjaerg L et al, GeneReviews), and certain medical devices may all be used to treat individuals with JLNS.
Hearing loss in individuals with JLNS may be treated with a small device known as a cochlear implant. Unlike hearing aids, which increase and clarify sound, a cochlear implant improves hearing by stimulating nerve fibers within the inner ear.
The treatment of choice for cardiac abnormalities in most individuals with JLNS is drug therapy with beta-adrenergic agents (beta blockers). Beta blockers, which include propranolol, atenolol, and nadolol, reduce the workload of the heart by decreasing the electrical stimulation of the heart.
Individuals for whom beta blockers are unsuccessful may be treated by a surgical procedure in which certain nerves going to the heart are removed (left cardiac sympathetic denervation or sympathectomy). However, recently treatment with an implantable automatic cardioverter-defibrillator (ICD) has replaced sympathectomy as the treatment of choice in these individuals. This device detects the abnormal heartbeat automatically and selectively delivers an electrical impulse to the heart. ICDs are used in conjunction with antiarrhythmic drug therapy.
Some individuals with JLNS are encouraged to avoid potential triggering events such as jumping into cold water, amusement park rides or competitive sports. Individuals with JLNS and professionals treating these patients for various diseases including those unrelated to JLNS, are strongly encouraged to be familiar with all the types of medication which may provoke serious cardiac attacks (https://www.crediblemeds.org/)
Genetic counseling is recommended for affected individuals and their families. Other treatment is symptomatic and supportive.