Bubbico L, Rosano A, Spagnolo A
Department of Biomedical Sciences, Italian Institute of Social Medicine, Rome, Italy.
Acta Otorhinolaryngol Ital. 2007 Feb;27(1):17-21.
Neonatal hearing loss is the most frequent sensorial congenital defect in newborns. No data are available on worldwide prevalence of congenital deafness. World Health Organization (WHO) data indicate 1-4 cases per 1000 individuals, with a considerable increase in developing countries. A prevalence exceeding 1 per 1000 however, indicates a serious public health problem calling for urgent attention. Aim of the study was the evaluate the prevalence of prelingual deafness in the Italian population and determine the socio-demographic characteristics of the condition. Data were provided by the National Institute of Social Insurance (INPS) and the Italian Central Statistics Institute (ISTAT) and were collected in 18 out of the 20 Italian regions (98.2% of total population). All subjects recognized as deaf-mute by a special medical committee were included. According to law No. 509/1988, they had to present a mean bilateral sensorineural-hearing impairment, detected in neonatal age, which caused the damage in speech development and equal to 60 dB or more for 500-, 1000- and 2000-Hz frequency tones in the better ear. Prevalence rates were calculated according to region and age bracket using updated population data from census 2001. Statistical analyses were performed using the SPSS statistical software package. A total of 40,887 cases of prelingual profound sensorineural hearing loss > or =60 dB were detected in Italy in 2003, for a total prevalence rate of 0.72 per 1000. The hearing impairment prevalence differs according to sex. The overall prevalence is 0.78 per 1000 for males and 0.69 per 1000 for females (p < 0.001). The hearing impairment prevalence differs according to region of residence (p < 0.001). The geographic distribution of prelingual deafness was found to be: North 15,644 cases (0.63 per 1000), Central Italy 7111 cases (0.64 per 1000), South and Islands 18,132 (0.87 per 1000). The prelingual hearing loss is highly prevalent in South Italy (Basilicata, Calabria and Sicily). For the southern regions of Italy, the rate observed in the 50-64 and >64 age groups reached 1.27 and 1.15, respectively. This phenomenon may have been due, in part, to the epidemic incidence of maternal rubella which occurred in the 40's and 50's (in Italy, the rubella vaccination was only recommended starting from 1972), and, in part, to the habit of contracting consanguineous marriages. Data from the Vatican Archives on 520,492 consanguineous marriages, for which dispensation was requested in the period 1911-1964, indicate that in the years 1935-1939, in small villages in South Italy (Basilicata, Calabria, Sicily) consanguineous marriages accounted for over 40% of marriages.
新生儿听力损失是新生儿中最常见的先天性感觉缺陷。目前尚无关于先天性耳聋全球患病率的数据。世界卫生组织（WHO）的数据显示，每1000人中就有1 - 4例，在发展中国家这一数字有显著增加。然而，患病率超过每1000人1例，就表明这是一个严重的公共卫生问题，需要紧急关注。本研究的目的是评估意大利人群中语前聋的患病率，并确定该疾病的社会人口学特征。数据由国家社会保险局（INPS）和意大利中央统计局（ISTAT）提供，收集自意大利20个地区中的18个（占总人口的98.2%）。所有被一个特别医疗委员会认定为聋哑的受试者都被纳入研究。根据第509/1988号法律，他们必须呈现出平均双侧感音神经性听力障碍，这种障碍在新生儿期被检测到，导致言语发育受损，且较好耳在500、1000和2000赫兹频率音调下的听力损失等于或超过60分贝。使用2001年人口普查的最新人口数据，按地区和年龄组计算患病率。使用SPSS统计软件包进行统计分析。2003年在意大利共检测到40887例语前严重感音神经性听力损失≥60分贝的病例，总患病率为每1000人0.72例。听力障碍患病率因性别而异。男性的总体患病率为每1000人0.78例，女性为每1000人0.69例（p < 0.001）。听力障碍患病率因居住地区而异（p < 0.001）。语前聋的地理分布情况为：北部15644例（每1000人0.63例），意大利中部7111例（每1000人0.64例），南部和岛屿地区18132例（每1000人0.87例）。语前听力损失在意大利南部（巴西利卡塔、卡拉布里亚和西西里）非常普遍。对于意大利南部地区，在50 - 64岁和64岁以上年龄组中观察到的患病率分别达到1.27和1.15。这种现象部分可能是由于40年代和50年代发生的孕产妇风疹流行（在意大利，风疹疫苗接种直到1972年才开始推荐），部分也归因于近亲结婚的习俗。梵蒂冈档案馆关于1911 - 1964年期间申请豁免的520492例近亲婚姻的数据表明，在1935 - 1939年期间，意大利南部的小村庄（巴西利卡塔、卡拉布里亚、西西里）近亲婚姻占婚姻总数的40%以上。
