Mice heterozygous for the Serpinb6a null mutation show deficits in central auditory function after acoustic trauma

Abstract

Objectives Complete deficiency of the serine protease inhibitor gene, SERPINB6, is responsible for autosomal-recessive, nonsyndromic sensorineural hearing loss in humans. A mouse model of this deafness gene identifies Serpinb6a expression in the neurosensory epithelium and fibrocytes of the cochlea. Homozygous Serpinb6a mutant mice display an exaggerated hearing loss after exposure to moderate acoustic trauma. It is unknown if and how heterozygous Serpinb6a mice show increased vulnerability to acoustic trauma. Methods We exposed Serpinb6a+/-and Serpinb6a+/+mice to acoustic trauma and measured their hearing function prior to, 3 and 14 days postexposure, analysing shifts in hearing threshold a..