Frequency of Congenital Heart Diseases in Prelingual Sensory-Neural Deaf Children

Document Type: Original

Authors

1 Otorhinolaryngology Research Center, Tehran University of Medical Sciences, Tehran, Iran

2 Department of Cardiology, Tehran University of Medical Sciences, Tehran, Iran.

3 Department of Pediatrics, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran.

Abstract

Introduction:
Hearing impairment is the most frequent sensorial congenital defect in newborns and has increased to 2–4 cases per 1,000 live births. Sensory-neural hearing loss (SNHL) accounts for more than 90% of all hearing loss. This disorder is associated with other congenital disorders such as renal, skeletal, ocular, and cardiac disorders. Given that congenital heart diseases are life-threatening, we decided to study the frequency of congenital heart diseases in children with congenital sensory-neural deafness.
 Materials and Methods:
All children who had undergone cochlear implantation surgery due to SNHL and who had attended our hospital for speech therapy during 2008–2011 were evaluated by Doppler echocardiography.
 Results:
Thirty-one children (15 boys and 16 girls) with a mean age of 55.70 months were examined, and underwent electrocardiography (ECG) and echocardiography. None of the children had any signs of heart problems in their medical records. Most of their heart examinations were normal, one patient had expiratory wheeze, four (12%) had mid-systolic click, and four (12%) had an intensified S1 sound. In echocardiography, 15 children (46%) had mitral valve prolapse (MVP) and two (6%) had minimal mitral regurgitation (MR). Mean ejection fraction (EF) was 69% and the mean fractional shortening (FS) was 38%.
 Conclusion: 
This study indicates the need for echocardiography and heart examinations in children with SNHL.
 

Keywords

Main Subjects


1. Bubbico L, Rosano A, Spagnolo A. Prevalence of preligual deafness in Italy. Acta Otorhinolaryngol Ital 2007; 27(1):17-21.

2. Abreu Alves F.R, Andrade Quintanitha Ribero F. Diagnosis routine and approach in genetic sensorineural hearing loss. Braz J Otorhinolaryngol 2007;73(3):412-7.

3. Motasaddi Zarandi M, Rutka J. Diseases of the inner ear: a clinical, radiologic, and pathologic atlas. Canada: Springer; 2010.

4. Olusanya B.O. Addressing the global neglect of childhood hearing impairment in developing countries. PLoS Med 2007; 4(4):e74.

5. Guillermo H, Sr. Economist, Nedim J. A note on disability issues in the middle east and north africa. Document of the World Bank. Human Development Department Middle East and North Africa; 2005;40.

6. Bubbico L. Bartolucci MA., Broglio D., Boner A. Societal cost of pre-lingual deafness. Ann Ig 2007; 19(2):143-52.

7. Hain T.C. Hearing loss. (online) dizziness-and-balance.com (Dec 2014). Available at: www.dizziness,balance.com/disorders/hearing/ hearing.html.

8. Moody Antonio SA, Strasnick B. Syndromic sensorineural hearing loss. (online) Emedicine (March 2014). Available at: http:// emedicine. medscape.com/article/856116-overview.

9. Motasaddi Zarandi M, Rohanizadegan M, Salmasian H, Nikzad N, Bazazzadegan N, Malekpour M. Clinical application of screening for GJB2 mutations before cochlear implantation in a heterogenous population with high rate of autosomal recessive nonsyndromic hearing loss. Genet Res Int 2011; 787026.

 10. Hoeve HL, Brooks AS, Smit LS. JS-X syndrome: A multiple congenital malformation with vocal cord paralysis, ear deformity, hearing loss, shoulder musculature underdevelopment, and X-linked recessive inheritance. Int J Pediatr Otorhinolaryngol 2015; 79(7):1164-70.

11. Shim YS, Choi W, Hwang IT, Yang S. Hypoparathyroidism, sensorineural deafness, and renal dysgenesis syndrome with a GATA3 mutation. Ann Pediatr Endocrinol Metab 2015; 20(1):59-63.

12. Carignec C. Le, Lefevre M, Schott JJ, Chaventre A, Gayet M, Calais C, Moisan JP. Familial deafness, congenital heart defects, and posterior embryotoxon caused by cysteine substitution in the first Epidermal-Growth-Factor-Like domain of Jagged 1. Am J Hum Genet 2002; 71(1):180-6.

13. Broomfield SJ, Bruce IA, Henderson L,Ramsden RTGreen KM. Cochlear implantation in children with Jervell and Lange-Nielsen syndrome - a cautionary tale, Cochlear Implants Int. Cochlear Implants Int. 2012 Aug;13(3):168-72.

14. Lahbil D, Souldi L, Lamari H, El Kettani AZaghloul K. [Manifestation of congenital rubella syndrome: clinical and epidemiologic aspects]. Bull Soc Belge Ophtalmol. 2007; 303: 13-20.

15. Solorzano-Santos F, Lopez-Kirwan A, Alvarezy Munoz MT, Miranda-Novales MGGadea-Alvarez TVázquez-Rosales G. [Congenital rubella syndrome in infants treated at a pediatric hospital]. Gac Med Mex. 2001; 137(2): 105-9.

16.  Sadler T.W. Langman’s Medical Embbryology, 12th Edition. Lippincott Williams & Wilkins, Philadelphia; 2004:117 &225.

17. Arnold SA, Brown OE, Finitzo T. Hearing loss in children with congenital heart disease; a preliminary report. Int J pediatr Otorhinolaryngol. 1986; 11(3): 287-93.

18. Makaryus AN, Zubrow ME, Marshall JD, Gillam LD, Mangion JR. Cardiac Manifestations of Alstrom syndrome: echocardiographic findings. J Am Soc Echocardiogr. 2007; 20(12): 1359-63.

19. Cunningham ET Jr, Eliott D, Miller NR, Maumenee IH, Green WR. Familial Axenfeld-Rieger anomaly, Atrial Septal Defect, and sensorineural hearing loss.1998; Arch ophthalmol. 116:78-82.

20. Kliegman RM, Staton BF, Gene JW, et al. Nelson Textbook of Pediatrics 20th. United States of America: Elsevier; 2015:2167.

21. El Habbal MH, Mahoney CO. QT interval in children with sensory neural hearing loss: Pacing Clin Electrophysiol. 2002 Apr; 25(4 Pt 1):435-9.

22. Sopontammarak S, Khongphatthanayothin A, Sa-Nguanchua P. Prevalence of idiopathic long QT syndrome in congenital sensori-neural hearing loss students of Songkhla School for the Deaf. J Med Assoc Thai. 2003; 86(12):1149-55.

23. Pfisterer, M. E,Battler A , Zaret BL. Range of normal values for left and right ventricular ejection fraction at rest and during exercise assessed by radionuclide angiocardiography. Eur Heart J. 1985; 6 (8): 647-655.

24. Kurtz D, Ahnberg DS, Freed M, LaFarge CGTreves S. Quantitative radionuclide angiocardiography.  Determination of left ventricular ejection fraction in children: Br Heart J. 1976; 38(9): 966–973.

25.Groves A, Kooner GS, Functional Assessments: Fractional Shortening. (internet) Neonatal EchoSkills. (Dec 2011)Available at: http:// neonatalechoskills. com/FracShort.html.

26.Normal Values of TEE (online). Echo Pedia.org (Aug 2014). Available at: http://www. echopedia. org/wiki/Normal_Values_of_TTE.

27. Hepner AD, Ahmadi-Kashani M, Movahed MR. The prevalence of mitral valve prolapse in patients undergoing echocardiography for clinical reason: Int J Cardiol. Dec 2007; 123(1):55-7.

28. Piórecka-Makuła A, Wróblewska-Kałuzewska M. [Mitral valve prolapse in children]. Wiad Lek. 2000; 53(7-8):434-8.

29. Venugopalan P. Pediatric Mitral Valve Prolapse. (internet) Medscape (Feb 2014). Available at: http:// emedicine. medscape. com/ article/890425-overview.

30. Markiewicz-Łoskot G, Łoskot M, Moric-Janiszewska E, Dukalska M, Mazurek B, Kohut J, Szydłowski L. Electrocardiographic abnormalities in young athletes with mitral valve prolapse.Clin Cardiol. Aug 2009; 32(8):E36-9.

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