Hostname: page-component-848d4c4894-4hhp2 Total loading time: 0 Render date: 2024-05-01T11:30:26.681Z Has data issue: false hasContentIssue false

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

Published online by Cambridge University Press:  17 November 2010

S L Kang
Affiliation:
Department of Paediatrics, Basildon and Thurrock University Hospitals NHS Foundation Trust, Basildon, UK
C Jackson
Affiliation:
Emmeline Centre for Hearing Implants, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
W Kelsall*
Affiliation:
Neonatal Intensive Care Unit, Addenbrooke's Hospital, Cambridge, UK
*
Address for correspondence: Dr Wilf Kelsall, Neonatal Intensive Care Unit, Box 226 Addenbrooke's Hospital, Hills Road, Cambridge CB2 0QQ, United Kingdom Fax: +44 (0)1223 217064 E-mail: wilf.kelsall@addenbrookes.nhs.uk

Abstract

Introduction:

Jervell–Lange-Nielsen syndrome is characterised by congenital deafness and a long QT interval on electrocardiography.

Aim:

(1) To survey UK national practice regarding electrocardiography screening of deaf children referred to cochlear implant centres, performed to evaluate for prolonged QT interval as recommended by national guidelines, and (2) to review local practice.

Methods:

Data were collected via a questionnaire sent to all UK cochlear implant centres, and via review of the medical records of a local cochlear implant centre database.

Results:

Eight (42 per cent) of the 19 cochlear implant centres surveyed performed electrocardiographic screening. Thirteen cases of long QT syndrome were reported in seven centres, with two related deaths. In our local cochlear implant centre, 14 (7.1 per cent) of 193 children had abnormal electrocardiograms; one definite long QT syndrome case and 13 borderline cases were identified.

Conclusion:

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.

Type
Main Articles
Copyright
Copyright © JLO (1984) Limited 2010

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1Fortnum, HM, Summerfield, AQ, Marshall, DA, Davis, AC, Barnford, JM. Prevalence of permanent childhood hearing impairment in the United Kingdom and implications for universal neonatal hearing screening: questionnaire based ascertainment study. BMJ 2001;323:536–40CrossRefGoogle ScholarPubMed
2Cochlear implants for children and adults with severe to profound deafness. 2009. In: http://www.nice.org.uk/nicemedia/pdf/TA166Guidancev2.pdf [25 January 2010]Google Scholar
31. Aetiological Investigation into Bilateral Severe to Profound Permanent Hearing Loss in Children. 2. Aetiological investigation of infants with congenital hearing loss identified through newborn hearing screening. 2008. In: http://www.baap.org.uk/?q=documents/guidelines [25 January 2010]Google Scholar
4Schwartz, PJ, Moss, AJ, Vincent, GM, Crampton, RS. Diagnostic criteria for the long QT syndrome: an update. Circulation 1993;88:782–4CrossRefGoogle Scholar
5Bitner-Glindzics, M, Tranebjoerg, L. The Jervell and Lange Nielsen syndrome. Genetics in otorhinolaryngology. Adv Otorhinolaryngol 2000;56:4552CrossRefGoogle Scholar
6Schwartz, PJ, Periti, M, Malliani, A. The long QT syndrome. Am Heart J 1975;89:378–90CrossRefGoogle Scholar
7Wilson, C, Roberts, A, Stephens, D. Aetiological investigation of sensorineural hearing loss in children. Arch Dis Child 2005;90:307–9CrossRefGoogle ScholarPubMed
8Yoong, S, Spencer, N. Audit of local performance compared with standards recommended by the national guidelines for aetiologic investigation of permanent childhood hearing impairment. Child Care Health Dev 2005;31:649–57CrossRefGoogle ScholarPubMed
9Schwartz, PJ, Spazzolini, C, Crotti, L, Bathen, J, Amlie, JP, Timothy, K et al. The Jervell and Lange–Nielsen syndrome: natural history, molecular basis and clinical outcome. Circulation 2006;113:783–90CrossRefGoogle ScholarPubMed
10Daneshi, A, Ghassemi, MM, Talee, M, Hassanzadeh, S. Cochlear implantation in children with Jervell Lange-Nielsen syndrome. J Laryngol Otol 2008;122:314–17CrossRefGoogle ScholarPubMed
11Hutchin, T, Coy, NN, Lonlon, H, Telford, E, Bromelow, K, Blaydon, D. Assessment of the genetic causes of recessive childhood non-syndromic deafness in the UK – implications for genetic testing. Clin Genet 2005;68:506–12CrossRefGoogle ScholarPubMed
12Miller, MD, Porter, CJ, Ackerman, MJ. Diagnostic accuracy of screening electrocardiograms in long QT syndrome. Pediatrics 2001;108:812CrossRefGoogle ScholarPubMed
13Yanmei, F, Yaqin, W, Haibo, S, Huiqun, Z, Zhengnong, C, Dongzhen, Y et al. Cochlear implantation in patients with Jervell and Lange-Nielsen syndrome, and a review of literature. Int J Pediatr Otorhinolaryngol 2008;72:1723–9CrossRefGoogle Scholar