| Objective: 1 To know the prevalence of children aged 0 to 14 with severe sensorineural hearing loss in Hebei province. 2 To study the causes and risk factors of severe sensorineural deafness. 3 To investigate the standard of classification about congenital inner ear malformations. 4 To learn the treatment of severe sensorineural hearing loss, the status of application and problems about cochlear implants in Hebei. Accordingly, the studies want to elaborate the key directions of the disease’s prevention and to call for the community to concern about the treatment of deafness problems.Methods: We analyzed clinical data about severe sensorineural deaf children from 0 to 14 years old retrospectively, who applied for the cochlear relief projects in Hebei province. The general situations of pediatric patients, family history, motherhood, and these audiological and radiological materials were included.Results:1 There were 1482 cases with severe sensorineural deafness who applied for the cochlear relief projects from 2008 to 2014, followed by the numbers over these years, 100 cases, 188 cases, 92 cases, 148 cases, 321 cases, 302 cases, and 331 cases; of which less than 1 year of age is 13 cases, 26 cases, 18 cases, 22 cases, 38 cases, 35 cases, and 41 cases. There were 937 children with state aid in seven years. As the multiple applications, the applicants and receivers did not correspond.2 1482 cases of severe sensorineural hearing loss applied for cochlear. There were 834 male cases, accounting for 56.28%, and 648 female cases, accounting for 43.72%, male to female ratio was 1.29: 1. Within 2 years of age accounted for 48.79%, visits 723 cases, 668 cases of 3 to 6 years old accounted for 45.07%, from 7 to 14 years 91 cases of 6.14%. The applicants less than 6 years old accounted for the vast majority. The girl was younger than the boy obviously to see a doctor(P=0.000).3 There were 1482 children who applied for cochlear that was state-relieved project, according to the number of applicants from more to less, the regional distribution was Shijiazhuang, Xingtai, Handan, Baoding, Hengshui, Tangshan, Cangzhou/Langfang, Zhangjiakou, Chengde, Qinhuangdao.4 The same cases may involved multiple risk factors, so the data according to the attendance basis. A total of 1493 cases, 1329 cases of unknown cause(89.02%), there were 164 cases of suspicious risk factors(10.98%), in which there was a family history of deafness 78 cases(47.56%); 52 cases of the mother had a history of drug use in early pregnancy(31.71%), included four pregnant women had ototoxic drugs(gentamicin), one case of rabies vaccine, and viral infections(warts) 1 case; children with a history of preterm birth hypoxia were 19 cases(11.58%). 9 cases(5.49%) had fever medication history, in which there were four children with ototoxic drugs(gentamicin). There were 2 cases hearing impairment combined with congenital heart disease, 1 case of purulent meningitis sequelae, and 1 case of Hunt’s syndrome sequelae. Above these pathogenic or high-risk factors, genetic factors were the highest(47.56%), followed by early pregnancy medications.5 The audiological test results about 1482 cases of severe sensorineural deaf children conformed to the state relief project screening criteria, but there were unilateral inspection or an inspection of non-compliance situation.6 The image data(HRCT and MRI) of 1482 cases(2964 ears) with severe sensorineural deaf children were analyzed uniformly, and the results were as follows:(1) malformations of the membranous labyrinth 2056(69.36%);(2)malformations of the osseous labyrinth 855 ears(28.85%), contained all kinds of the osseous deformity from more to less in turn: large vestibular aqueduct syndrome 336 ears(11.34%), incomplete partition typeⅡ334 ears(11.27%), incomplete partition typeⅠ52 ears(1.75%), vestibule-lateral semicircular canal dysplasia 39 ears(1.32%), cochlear aplasia 26 ears(0.88%), cochlear hypoplasia 24 ears(0.81%), common cavity deformity 16 ears(0.54%), incomplete partition type Ⅲ16 ears(0.54%), Michel deformity 12 ears(0.40%).(3) Other simple malformations 53 ears(1.79%), included in only wide internal auditory canal 32 ears(1.08%); the purely narrow internal auditory canal 11 ears(0.37%); cochlear ossification 5 ears(0.17%); missing posterior semicircular canal 4 ears(0.14%); vestibular semicircular canal ossification 1 ear(0.03%). We could see that the large vestibular aqueduct syndrome and incomplete partition typeⅡ accounted for among the two inner ear malformations, while nearly 70% of the patients, with no visible image of the inner ear abnormalities, which were membranous labyrinth.7 The data of the Second National Sample Survey on Disability showed that hearing disability prevalence rate was 2.11%, and 0 to 14 age group accounted for 1.53%, and this age group with one or two hearing disability majority, GradeⅠ and Ⅱ hearing impairment account for 83.90% in the age group of 0~3. Combined with resident population per year in 2008-2014, and calculated over the several people with disabilities every year, and for the dynamic analysis of the number of columns, we could see the number of people with disabilities in hearing increased year by year, from the growth rate, was a slight increase year by year. According to the average growth rate formula, we could predict that the number of people with disabilities in hearing in 2015 was approximately 1.57 million, and the number of children within 14 years old was less than 24,000, about 20,000 people with severe hearing loss in children less than 3 years old.Conclusions:1 Genetic factors were the main causes to congenital sensorineural deafness(approximately 50%), so deafness gene screening was crucial, which should be included in premarital and pregnancy test project. Other risk factors such as early pregnancy medical history(especially ototoxic drugs), children with a history of preterm birth hypoxia, fever medication history and so on, were likely to affect auditory function. It is important to popularize the knowledge of deafness, propagandize and educate in pregnancy, screen for deafness genes, do prenatal care well and to screen newborn hearing screening, for those methods could prevent children from congenital sensorineural deafness, and which need to carry out comprehensively and deeply. The disease should be early prevention, early detection, early diagnosis and early treatment.2 The inner ear malformation refers the osseous labyrinth malformation, was divided into nine categories, including large vestibular aqueduct syndrome and incomplete partition typeⅡ malformations incidence rate in the top two. Different inner ear malformations have different treatments, and it is crucial to handle the detailed classification of inner ear malformations.3 Cochlear implants were the most effective treatment to severe and profound sensorineural hearing loss. Preoperative detailed audiological and radiological examinations directly related to the surgical procedure, selection of cochlear electrode and assessment of postoperative results.4 Hebei is the populous, cochlear annual aid amount was far less than in children with severe hearing loss amount of growth each year, to infer the national situation nor optimistic. And in order to get the best results of operations, implemented cochlear implant surgery should be done as soon as possible(verbal learning period). Only depend on the power of the State is not enough, the whole social resources(such as health insurance, charity organizations or individuals, etc.) should participate actively. It not only reduces the pressure of children’ own survival and the economic burden on families in the long run, but also makes a great contribution to the development of the whole society. |
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