A Porcine Congenital Single-Sided Deafness Model, Its Population Statistics and Degenerative Changes

Frontiers in Cell and Developmental Biology

Fan-jun Zheng¹  Wei-wei Guo¹  Shi-ming Yang¹  Hui Zhao¹  Wei Ren¹  Yue Zhang¹  Cong Xu¹  Peng Jin³  Xiao-yang Zhou⁴  Ting-ting Lin⁴  Lu-lu Wang⁴  Chuan-hong Liu⁴  Yong Wang⁴ 

[1] Beijing Key Lab of Hearing Impairment for Prevention and Treatment, Beijing, China;College of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Beijing, China;Department of Human Genetics, Emory University School of Medicine, Atlanta, GA, United States;Department of Laboratory Animal Science, College of Basic Medical Science, Army Medical University, Chongqing, China;Key Lab of Hearing Science, Ministry of Education, Beijing, China;National Clinical Research Center for Otolaryngologic Diseases, Beijing, China;

关键词: porcine (pig) model;    deafness (hearing loss);    ABR;    DPOAE;    pathology;   

DOI  :  10.3389/fcell.2021.672216

来源: DOAJ

【 摘 要 】

ObjectiveTo describe and study the population statistics, hearing phenotype, and pathological changes of a porcine congenital single-sided deafness (CSSD) pedigree.MethodsClick auditory brainstem response (ABR), full-frequency ABR, and distortion product otoacoustic emission (DPOAE) were used to assess the hearing phenotype of the strain. Tympanogram was used to assess the middle ear function since birth. Celloidin embedding–hematoxylin–eosin (CE-HE) stain and scanning electron microscopy (SEM) were used to study the pathological changes of cochlear microstructures. Chi-square analysis was used to analyze the relation between hearing loss and other phenotypes.ResultsThe mating mood of CSSD with CSSD was most efficient in breeding-targeted CSSD phenotype (47.62%), and the prevalence of CSSD reached 46.67% till the fifth generation, where 42.22% were bilateral hearing loss (BHL) and 9.00% were normal hearing (NH) individuals. Hearing loss was proved to have no relation with coat color (P = 0.0841 > 0.05) and gender (P = 0.4621 > 0.05) by chi-square analysis. The deaf side of CSSD offspring in the fifth generation had no relation with that of their maternal parent (P = 0.2387 > 0.05). All individuals in this strain exhibited congenital severe to profound sensorineural hearing loss with no malformation and dysfunction of the middle ear. The good hearing ear of CSSD stayed stable over age. The deaf side of CSSD and BHL presented cochlear and saccular degeneration, and the hair cell exhibited malformation since birth and degenerated from the apex to base turn through time. The pathology in BHL cochlea progressed more rapidly than CSSD and till P30, the hair cell had been totally gone. The stria vascularis (SV) was normal since birth and degenerated through time and finally exhibited disorganization of three layers of cells.ConclusionThis inbred porcine strain exhibited high and stable prevalence of CSSD, which highly resembled human non-syndromic CSSD disease. This porcine model could be used to further explore the etiology of CSSD and serve as an ideal tool for the studies of the effects of single-sided hearing deprivation on neural, cognitive, and behavioral developments and the benefits brought by CI in CSSD individuals.

【 授权许可】

Unknown