Electrocardiogram screening of deaf children for long QT syndrome: are we following UK national guidelines?

Abstract

Despite clear national guidelines for electrocardiographic screening of deaf children, there is wide variation in practice. Our local practice of performing investigations, including electrocardiography, during magnetic resonance imaging sedation has been very successful. Electrocardiograms should be reviewed by trained clinicians, and corrected QT intervals should be calculated manually.

QTc prolongation in patients with hearing loss: Electrocardiographic and genetic study

“…The presence of such changes in a person with the hearing loss immediately raises a suspicion of a JL-N syndrome and allows implementation of prophylactic lifesaving treatment. Besides the British Association of Paediatricians in Audiology (BAPA) and British Association of Audiological Physicians (BAAP) to screen every patient with congenital hearing loss with ECG, other organizations do not recommend routine ECG in this group of patients [2]. However, guidelines clearly do emphasize ECG utility in patients with a history of loss of consciousness, arrhythmia or family history of sudden death [3].…”

Electrocardiogram screening of deaf children for long QT syndrome: An Egyptian experience

“…This highlights the very early and malignant presentation of this syndrome as emphasized by Schwartz . Because ECG is a simple, inexpensive, and noninvasive investigation, some organizations recommend routine ECG screening in patients with congenital hearing loss . Children with LQTS were treated according to European Society of Cardiology guidelines…”

“…5 Because ECG is a simple, inexpensive, and noninvasive investigation, some organizations recommend routine ECG screening in patients with congenital hearing loss. 25 Children with LQTS were treated according to European Society of Cardiology guidelines. 26 The limitations of this study were the absence of genetic testing for syndromic and nonsyndromic forms of hereditary SNHL (limited by facility and costs).…”

Improving the aetiological investigations of congenital hearing loss: our experience in an audit of 56 patients

“…For example, children with hearing loss due to Jervell Lange-Nielson syndrome can have cardiac dysrhythmias and are at increased risk of sudden death. 5 When recognised and treated appropriately with drugs and implanted cardiac defibrillators, the prognosis is significantly improved. Similarly, Usher syndrome is a condition in which congenital hearing loss is associated with retinitis pigmentosa, where children with initially normal sight to go on to develop visual impairment.…”

“…Despite its availability, there have been concerns regarding adherence to the guidelines and differing practices within varying units. 5 In a bid to address this, we have designed a simple proforma to help improve compliance to standards and enable efficient medical record-keeping and auditing.…”