Skip to main content

Advertisement

SpringerLink
Log in

Long QT and Hearing Loss in High-Risk Infants Prospective Study Registry

  • Original Article
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

The objective of this study is to determine the prevalence of an abnormal electrocardiogram showing a prolonged QTc greater than 450 ms in infants with unilateral or bilateral sensorineural hearing loss. We conducted a prospective study of healthy term infants (≥37 weeks gestational age) who failed their newborn auditory brainstem response hearing screen, were seen by an audiologist and diagnosed as having sensorineural hearing loss during follow-up to 1 year of age. In infants with a diagnosis of hearing loss, we collected a detailed family history and performed an ECG between 2 and 6 months of age. We obtained follow-up for 1 year by calling the parent requesting the hearing and cardiac status of their child. Two of the 40 infants with sensorineural hearing loss (5%) had a QTc greater than 450 ms. Both had mild bilateral hearing loss and genetic testing did not identify a known mutation for long QT syndrome. The remaining 38 infants had QTc intervals of ≤ 450 ms. One patient diagnosed with bilateral severe sensorineural hearing loss had a normal ECG (QTc = 417 ms). Several months after the ECG was performed, the infant’s mother contacted the study cardiologist after she learned that the infant’s maternal grandmother was diagnosed with a cardiomyopathy and arrhythmias. Genetic testing was recommended even though the child was asymptomatic and was positive for a pathogenic mutation in the KCNQ1 gene. We speculate that molecular genetic testing in infants with hearing loss may become the standard of care rather than targeted electrocardiograms.

Clinical Trial Registration NCT02082431 https://www.clinicaltrials.gov/ct2/show/NCT02692521?cond=NCT02692521&rank=1.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Chang RK, Lan YT, Silka MJ, Morrow H, Kwong A, Smith-Lang J, Wallerstein R, Lin HJ (2014) Genetic variants for long QT syndrome among infants and children from a statewide newborn hearing screening program cohort. J Pediatr 164(3):590–595. https://doi.org/10.1016/j.jpeds.2013.11.011

    Article  CAS  PubMed  Google Scholar 

  2. Schwartz PJ, Crotti L, Insolia R (2012) Long-QT syndrome: from genetics to management. Circ Arrhythm Electrophysiol 5(4):868–877. https://doi.org/10.1161/CIRCEP.111.962019

    Article  PubMed  PubMed Central  Google Scholar 

  3. Schwartz PJ, Stramba-Badiale M, Crotti L, Pedrazzini M, Besana A, Bosi G, Gabbarini F, Goulene K, Insolia R, Mannarino S, Mosca F, Nespoli L, Rimini A, Rosati E, Salice P, Spazzolini C (2009) Prevalence of the congenital long-QT syndrome. Circulation 120(18):1761–1767. https://doi.org/10.1161/CIRCULATIONAHA.109.863209

    Article  PubMed  PubMed Central  Google Scholar 

  4. Jervell A, Lange-Nielsen F (1957) Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval and sudden death. Am Heart J 54(1):59–68. https://doi.org/10.1016/0002-8703(57)90079-0

    Article  CAS  PubMed  Google Scholar 

  5. El-Sherif N, Turitto G, Boutjdir M (2019) Acquired long QT syndrome and electrophysiology of torsade de pointes. Arrhythm Electrophysiol Rev 8(2):122–130. https://doi.org/10.15420/aer.2019.8.3

    Article  PubMed  PubMed Central  Google Scholar 

  6. Giudicessi JR, Wilde AAM, Ackerman MJ, Giudicessi JR, Wilde AAM, Ackerman MJ (2018) The genetic architecture of long QT syndrome: a critical reappraisal. Trends Cardiovasc Med 28(7):453–464. https://doi.org/10.1016/j.tcm.2018.03.003

    Article  PubMed  PubMed Central  Google Scholar 

  7. Schwartz PJ, Ackerman MJ, Antzelevitch C, Bezzina CR, Borggrefe M, Cuneo BF, Wilde AAM (2020) Inherited cardiac arrhythmias. Nat Rev Dis Primers 6(1):58. https://doi.org/10.1038/s41572-020-0188-7

    Article  PubMed  PubMed Central  Google Scholar 

  8. Moore JP, Gallotti RG, Shannon KM, Bos JM, Sadeghi E, Strasburger JF, Wakai RT, Horigome H, Clur SA, Hill AC, Shah MJ, Behere S, Sarquella-Brugada G, Czosek R, Etheridge SP, Fischbach P, Kannankeril PJ, Motonaga K, Landstrom AP, Williams M, Patel A, Dagradi F, Tan RB, Stephenson E, Krishna MR, Miyake CY, Lee ME, Sanatani S, Balaji S, Young ML, Siddiqui S, Schwartz PJ, Shivkumar K, Ackerman MJ (2020) Genotype predicts outcomes in fetuses and neonates with severe congenital long QT syndrome. JACC Clin Electrophysiol 6(12):1561–1570. https://doi.org/10.1016/j.jacep.2020.06.001

    Article  PubMed  PubMed Central  Google Scholar 

  9. Bohnen MS, Peng G, Robey SH, Terrenoire C, Iyer V, Sampson KJ et al (2017) Molecular pathophysiology of congenital long QT syndrome. Physiol Rev 97:89–134

    Article  CAS  Google Scholar 

  10. Lim BG, Clark RH, Kelleher AS, Lin Z, Spitzer AR, Pediatrix SoundGene® Study Group Principal Investigators and Contributors (2013) Utility of genetic testing for the detection of late-onset hearing loss in neonates. Am J Audiol 22(2):209–215. https://doi.org/10.1044/1059-0889(2013/12-0078)

    Article  PubMed  Google Scholar 

  11. Niaz A, Rizvi SF, Khurram D (2011) Prevalence of long QT syndrome and other cardiac defects in deaf-mute children. J Ayub Med Coll Abbottabad 23(1):5–8

    PubMed  Google Scholar 

  12. Anto R, Maheswari S, Vadivu S, Kameswaran M (2019) Cochlear implantation in congenital long-qt syndrome: a comprehensive study. J Int Adv Otol 15(3):352–357. https://doi.org/10.5152/iao.2019.7404

    Article  PubMed  PubMed Central  Google Scholar 

  13. Sanyal SK, Kaul KK, Hussein A, Wilroy RS, Agarwal K, Sohel S (2013) Jervell and Lange-Nielsen syndrome in a father and daughter from a large highly inbred family: a 16-year follow-up of 59 living members. Cardiol Young 23(4):530–539. https://doi.org/10.1017/S1047951113000164

    Article  PubMed  Google Scholar 

  14. Sanecka A, Biernacka EK, Szperl M, Sosna M, Mueller-Malesińska M, Kozicka U, Baranowski R, Kosiec A, Łazarczyk H, Skarżyński H, Hoffman P, Bieganowska K, Piotrowicz R (2016) QTc prolongation in patients with hearing loss: electrocardiographic and genetic study. Cardiol J 23(1):34–41. https://doi.org/10.5603/CJ.a2015.0062

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

Principal investigators were as follows: Kartik Mody, MD, St. Joseph’s Hospital and Banner University Center, Phoenix, AZ; Sara Neumann, AuD, Hearts for Hearing, Oklahoma City, OK; Seema LaGree, AuD, Presbyterian Ear Institute, Albuquerque, and NM; Patricia Lake, AuD, Lawrence and Memorial Hospital, New London, CT. Melanie H. Drummond, RN, BSN, The Children’s Hospital of San Antonio, San Antonio, TX; Katy Kohlleppel, RN, BSN, The Children’s Hospital of San Antonio, San Antonio, TX; Joanna Shilling, Hearing Screen Coordinator/Clinical Care Coordinator, Swedish American Hospital, Rockford, IL. We would also like to recognize Jaclyn Cohen, RN, Nalina Fisher, BS, Octavia Reed, RN, BSN, Kathy McCarty, and Theresa Katich for administrative support with the study and Beverly Gail Lim, ARNP, AuD for her help with planning the study.

Funding

No funding was secured for this study.

Author information

Authors and Affiliations

  1. The MEDNAX Center for Research, Education, Quality and Safety, 1301 Concord Ter, Sunrise, FL, 33323, USA

    Amy S. Kelleher & Reese H. Clark

  2. Child Cardiology Associates, Fairfax, VA, USA

    Jill A. Young & Mitchell I. Cohen

  3. Children’s Cardiology Associates, Austin, TX, USA

    Arnold L. Fenrich & Daniel P. Shmorhun

  4. Banner University Medical Center, Phoenix, AZ, USA

    Gregory C. Martin

  5. Phoenix Childrenʼs Hospital, Phoenix, AZ, USA

    Gregory C. Martin

  6. Swedish American Hospital, Rockford, IL, USA

    Martin A. Anyebuno

  7. Childrens Hospital at Providence Alaska Medical Center, Anchorage, AK, USA

    Evelyn D. Rider

  8. The Childrens Hospital of San Antonio, San Antonio, TX, USA

    Cheryl L. Motta

Authors
  1. Arnold L. Fenrich
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Daniel P. Shmorhun
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Gregory C. Martin
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jill A. Young
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Mitchell I. Cohen
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Amy S. Kelleher
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Martin A. Anyebuno
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Evelyn D. Rider
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Cheryl L. Motta
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Reese H. Clark
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

MAA: collected the data, and critically analyzed, reviewed, and revised the manuscript for important intellectual content. RHC: conceptualized and designed the study, drafted the initial manuscript and critically analyzed, reviewed, and revised the manuscript for important intellectual content. MIC: conceptualized and designed the study, collected the data, and critically analyzed, reviewed, and revised the manuscript for important intellectual content. ALF: designed the study, collected the data, and critically analyzed, reviewed, and revised the manuscript for important intellectual content. ASK: conceptualized and designed the study, drafted the initial manuscript and critically analyzed, reviewed, and revised the manuscript for important intellectual content. GCM: designed the study, collected the data, and critically analyzed, reviewed, and revised the manuscript for important intellectual content. CLM: collected the data, and critically analyzed, reviewed, and revised the manuscript for important intellectual content. EDR: collected the data, and critically analyzed, reviewed, and revised the manuscript for important intellectual content. DPS: collected the data, and critically analyzed, reviewed, and revised the manuscript for important intellectual content. JAY: designed the study, collected the data, and critically analyzed, reviewed, and revised the manuscript for important intellectual content. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

Corresponding author

Correspondence to Amy S. Kelleher.

Ethics declarations

Conflict of interest

The authors have no conflict of interest to disclose.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Fenrich, A.L., Shmorhun, D.P., Martin, G.C. et al. Long QT and Hearing Loss in High-Risk Infants Prospective Study Registry. Pediatr Cardiol 43, 1898–1902 (2022). https://doi.org/10.1007/s00246-022-02939-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-022-02939-4

Keywords

Search

Navigation