Potential role for lipopolysaccharide in congenital sensorineural hearingloss

A L Smit,B W Kramer,B Kremer,R J Stokroos,S G H Litjens

doi:10.1099/jmm.0.015792-0

Abstract

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.

Highlights

Congenital sensorineural hearing loss (SNHL) in childhood is common
We will focus on the infectious causes of congenital SNHL, of which infection with cytomegalovirus (CMV) is the most common (Nance et al, 2006)
Micro-organisms responsible for chorioamnionitis can reach the chorioamnion by the abdominal cavity through the Fallopian tubes or inadvertent needle contamination at the time of amniocentesis or chorionicvillus sampling, by passage through the cervix from the vagina (Goldenberg et al, 2000; Romero & Mazor, 1988), or transplacentally due to a maternal systemic infection (Goldenberg et al, 2000) (Fig. 2)

Summary

Potential role for lipopolysaccharide in congenital sensorineural hearing loss

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

Introduction

Transtympanical injection

Role of LPS in congenital hearing loss