Masoud Motasaddi Zarandy - Otorhinolaryngology Research Center, Tehran University of Medical Sciences, Tehran, Iran
mohammad Jafar Mahmoudi - Department of Cardiology, Tehran University of Medical Sciences, Tehran, Iran.
Iran Malekzadeh - Department of Pediatrics, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
Sevil Nasirmohtaram - Otorhinolaryngology Research Center, Tehran University of Medical Sciences, Tehran, Iran

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.  

Congenital sensory-neural hearing loss, congenital heart disease, echocardiography, Electrocardiography