Abstract
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.
Highlights
Single-sided deafness (SSD) is defined as profound sensorineural hearing loss in one ear with normal hearing on the opposite side
Under the strategy of Congenital SSD (CSSD) mating with CSSD individuals, the number of targeted CSSD phenotype reached the highest with 20 CSSDs accounting for 47.62% of all offspring, 19 bilateral hearing losses (BHLs) for 45.24%, and only 3 normal hearing (NH) for 7.14%
When CSSD was mating with BHL, the
Summary
Single-sided deafness (SSD) is defined as profound sensorineural hearing loss in one ear with normal hearing on the opposite side. Congenital SSD (CSSD) often refers to those who did not pass the newborn hearing screening and was diagnosed with unilateral hearing loss at birth. The incidence of sensorineural hearing loss is estimated to be 1.86 per 1,000 newborns; among them, 30–40% are unilateral (Fitzpatrick et al, 2017; van Wieringen et al, 2019), but the CSSD incidence varies among researches because populations of different ages were involved, for children from 6 to 19 years old. The estimated CSSD incidence was 0.7–0.8% (Ross et al, 2010). In South Korea, the prevalence of unilateral hearing loss (UHL) was 9.31%, among which SSD accounts for 5.98% in the population over 12 years old; the prevalence of CSSD was unknown (Jun et al, 2015). Because of the lack of international or regional epidemiology study, the incidence of CSSD needs further study
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