Congenital sensorineural hearing loss (SNHL) is common. In the Western world, the incidence is 1-3 per 1000 live births. The aetiology encompasses genetic and non-genetic factors accounting for 55% and 45% of cases, respectively. Reports that describe the contribution of intrauterine infection to the occurrence of congenital SNHL are limited, and comparative analysis of the different pathogens is lacking. Lipopolysaccharide (LPS), a product of bacteriolysis, has been demonstrated to be associated with inner ear damage in experimental studies. To elucidate the potential role of this toxin in congenital SNHL and to identify the pathogenesis and transmission routes, we reviewed the literature. We speculate that different routes of exposure to LPS in utero may result in congenital inner ear damage.