Sajjad M, Khattak A A, Bunn J E G, Mackenzie I
Child and Reproductive Health Group, Liverpool School of Tropical Medicine, Liverpool, UK.
J Laryngol Otol. 2008 Oct;122(10):1057-63. doi: 10.1017/S0022215108002235. Epub 2008 Apr 21.
Deafness is the hidden disability of childhood, and leads to poor educational and employment prospects. There is little published information on deafness in Pakistan. Profound hearing impairment is more prevalent in countries where consanguineous marriages are common, such as Pakistan. This study aimed to assess causes of childhood deafness and association with parental consanguinity, within deaf and hearing children in the Peshawar district of Pukhtoonkhwa Province, Pakistan.
One hundred and forty deaf children were identified from two schools for deaf children within the Peshawar district. These children were assessed via audiology, otoscopic examination, case note review and parental history, in order to attempt to ascertain the cause of their deafness. Two hundred and twenty-one attendees at a local immunisation clinic (taken as representative of the local childhood population) were also screened for hearing impairment. Parents of both groups of children were assessed by interview and questionnaire in order to ascertain the mother and father's family relationship (i.e. whether cousins or unrelated).
Of the 140 deaf school pupils, 92.1 per cent were profoundly hearing impaired and 7.9 per cent were severely hearing impaired. All these children had bilateral sensorineural hearing loss. A possible cause of deafness was identified in only six of these children. Parental consanguinity (i.e. first or second cousins) was established for 86.4 per cent of deaf school pupils and 59.7 per cent of immunisation clinic attendees. None of the control children were identified as having a hearing problem.
The prevalence of parental consanguinity was significantly higher in deaf children compared with non-hearing impaired children. However, the study also confirmed a high rate of consanguinity within the general Peshawar community. In this setting, prevention of consanguineous unions is the only means of reducing levels of congenital hearing impairment. The current levels of hearing disability represent both a prominent public health problem and an important, potentially preventable childhood disability.
耳聋是儿童时期的隐性残疾，会导致不良的教育和就业前景。关于巴基斯坦耳聋情况的公开信息很少。在巴基斯坦等近亲婚姻普遍的国家，重度听力障碍更为常见。本研究旨在评估巴基斯坦开伯尔-普赫图赫瓦省白沙瓦地区聋哑儿童和听力正常儿童中儿童期耳聋的病因及其与父母近亲结婚的关系。
从白沙瓦地区的两所聋哑学校中确定了140名聋哑儿童。通过听力学、耳镜检查、病历回顾和父母病史对这些儿童进行评估，以试图确定他们耳聋的原因。还对当地一家免疫诊所的221名就诊者（作为当地儿童人群的代表）进行了听力障碍筛查。通过访谈和问卷调查对两组儿童的父母进行评估，以确定父母的亲属关系（即是否为表亲或无亲属关系）。
在140名聋哑学生中，92.1%为重度听力障碍，7.9%为重度听力障碍。所有这些儿童均为双侧感音神经性听力损失。在这些儿童中，仅6名儿童的耳聋病因得以确定。86.4%的聋哑学生和59.7%的免疫诊所就诊者的父母为近亲结婚（即第一代或第二代堂/表亲）。未发现对照儿童有听力问题。
与听力正常的儿童相比，聋哑儿童中父母近亲结婚的比例显著更高。然而，该研究也证实了白沙瓦社区总体近亲结婚率较高。在这种情况下，预防近亲结合是降低先天性听力障碍水平的唯一途径。目前的听力残疾水平既是一个突出的公共卫生问题，也是一种重要的、潜在可预防的儿童残疾。
