Increased susceptibility to acoustic trauma in a mouse model of non-syndromic sensorineural deafness, DFNB91

Abstract

Inactivating mutations of SERPINB6 in humans result in progressive hearing loss starting in early adulthood (DFNB91). We have previously shown that C57BL/6J mice lacking the orthologous gene, Serpinb6a, exhibit progressive hearing loss, which is associated with progressive loss of distinct cell types in the organ of Corti beginning with outer hair cells (OHCs). However, deafness in these animals occurs much earlier than expected, possibly because C57BL/6J mice also carry an age-related hearing loss mutation in the cadherin 23 gene (Cdh23ahl) that causes late onset hearing loss. The CBA/CaH strain of mice does not carry Cdh23ah/ahl and may represent a better model of the human DFNB91 patients..