Screening of long Q-T syndrome in patients with congenital sensorineural hearing loss

Document Type : Research Paper




ntroduction: The idiopathic long Q-T syndrome is an infrequent disorder, in which affected individuals have an unusual ECG repolarization abnormality presenting as syncope. Congenial long Q-T prolongation can be associated with congenital deafness in an autosomal recessive manner (Jerval and lange Nielson Syndrome).
Materials and Methods: This descriptive study was accomplished in 2003, on 100 cases with congenital deafness as the case group and 100 cases with intact hearing as the control group. The two groups were similar with respect to sex and age. ECG performed for the both groups and Q-T duration was measured according to Bazet formula. Data recorded in a questionnaire and analyzed by descriptive statistics.
Results: The cases were between 7- 12 years old, with the mean age of 9. Almost 50% of cases were female and 50 % were male. The mean Q-Tc duration was 0.42 sec in the control group, 0.42 sec in both females and males. The mean Q-Tc duration was 0.43 sec in the case group, 0.44 sec in female and 0.42 sec in male. In the case group, 44% had Q-Tc>0.46 sec (29% female and 15% male). In the control group, 28% had QTc= 0.44 sec (14% male and 14% female).
Conclusion: The present study's data supported the presence of long Q-T syndrome in patients with sensorineural hearing loss in the studied population. So, Routine ECG screening is recommended in patients with congenital deafness.


1- NigeL A, GLIBER WB. LVY RC Cardiac arrhythmia in the neonate and child. Prolonged QT syndrome. Am.J 2001;
180: 2O2-5.
2- Moss A, Lee TH. Ezra A. Heart disease in infant, children, adolescents, autosomal recessive syndrome with
associated cardiovascular abnormalities. Ann Emer Med 2005; Chap.3. 17.
3- Kenji Hoshino, Kiyoshi O. A sibling case of Jervel Lange Neilsen Syndrome. Pointed out at cardiac check screening
in school children. Pediatric cardiology & cardiac surgery. 2005; 10(3): 415-420.
4- Braunwald Eug. Vent tachycardia Braunwald A text book of Cardiovas Med 2005; 7th ed. Chp. 27- 32-33. 118-845.
5- Paparella B. Elio A. Saro B. Jervel's syndrome.Am J 2003; 37: 1586.
6- Locati EH. Zareba W. Moss AJ et al: Age and sex related difference in clinical manifestation in patients with
congenital long QT syndrome, finding from international LQTs registry, Netherland. Circulation 97: 2006; 2237.
7- Carson E. Long QT syndrome. Long QT interval syndrome, idiopathic long QT syndrome, aquired long QT
syndrome.Pediatric cardiology 2005; 1888, 724.
8- Malcol M. Silver D. Case E. Congenial cardiac dysrrhythmia with prolognged QT interval (isolated. Romanoward
Syndrome) with congenial nerve deafness (jevall Syndrome).Cardiovascular pathology 2005; 662-663.
9- Ballenger K. Jervel and Lange Nielsen Syndrome.Otorhinolaryngology 2001; 5: 1081.