فهرست نشریات علمی دسترسی آزاد ایران

Background: It seems that there is a relationship between consanguinity and profound hearing loss but there is little data aboutthe association of consanguinity and hearing loss in Iran.Objectives: The aim of this study is to demonstrate the causes of profound bilateral sensorineural hearing loss among Iraniansamples who are candidates for cochlear implantation.Methods: This study was retrospective, analytical, and designed to collect information about profound hearing impaired casesreferred to the Baqiyatallah Cochlear implantation center using enumeration. A total of 310 children with profound hearing impairmentsparticipated in this study. They were aged from 6 months to 4 years old. The study was done between January 2007 andApril 2009. Chi-square tests were used to show whether there was any statistical difference between the incidence of marital consanguinityof their parents and the normal population.Results: Sixty-five percent of those 310 children had parents who had married with their relatives. Of the 203 (65%) parents thathad consanguineous marriages, 132 were first cousins, which includes the children of two brothers (37 [11.8%] patrilateral parallelcousins), the children of two sisters (38 [12.2%] multi-lateral parallel cousins), or the children of a brother and a sister (57 [18.3%] crosscousins). Fifty-four (17.4%) of the parents were second cousins and 17 (5.2%) were beyond second cousins. Also, hearing loss etiologywas obvious in 237 (76.3%) of the patients with profound hearing loss but was unknown in 73 (23.7%). Hereditary was identified asthe most common cause in 33% of the cases.Conclusions: Our data demonstrated a 65% occurrence of consanguineous marriage among the parents of deaf children, which isstatistically different from the percentage of consanguineous marriage among Iranian population (38%). This indicates an obviousrelationship between severe hearing loss and consanguineous marriage.