Abstract 11133: Prevalence and Types of Long QT Syndrome among Children with Hearing Loss

Introduction. Long QT syndrome (LQTS) associated with sensorineural hearing loss (SNHL) is known as the Jervell and Lang Nielsen syndrome (JLNS), an autosomal recessive disorder. Autosomal dominant LQTS is more common but has not been associated with SNHL. Hypothesis. Children with severe to profound hearing loss are at higher risk for LQTS, therefore ECG screening can be useful for early diagnosis of LQTS. Methods. We conducted statewide, prospective ECG screening of infants and children (≤6 y) with unilateral or bilateral, severe or profound, sensorineural or mixed hearing loss in California in 2009-2011. Subjects were identified through the state newborn hearing screening and special education programs. Participants were interviewed about medical and family histories and had initial screening by 12-lead ECGs. Children with histories suggesting arrhythmia, syncope, or sudden death, or a QTc ≥ 450 ms had a repeat ECG. DNA sequencing of 12 genes involved in LQTS was performed if the repeat QTc was ≥ 450 m...