Is gross motor delay secondary to bilateral vestibular hypofunction in Jervell and Lange-Nielsen syndrome?

AbstractJervell and Lange-Nielsen syndrome is a rare autosomal recessive condition characterized by bilateral profound sensorineural hearing loss, and prolonged QTinterval that may result in ventricular tachycardialeading to syncope, cardiac arrest or sudden death. Treatment of the arrhythmias is often life-saving as this condition has a high mortality if untreated. We describe 3 cases of Jervelland Lange-Nielsen syndrome, who presented with profound sensorineural hearing lossand delayed gross motor milestones and have evidence of bilateral vestibular hypofunction on vestibular testing. We suggest that vestibular hypofunction is associated with this syndrome and evaluation of vestibular function may increase the diagnostic yield in the asymptomatic stage of this rare, but severe form of long QT syndrome. Clinicians should consider Jervell Lange-Nielsen in the differential diagnosis of ‘non-syndromic’ profound sensorineural hearing losspresenting with vestibular failure, along with Usher syndrometype...