A systematic review of pediatric sensorineural hearing loss in congenital syphilis.

INTRODUCTION: Congenital syphilis is a known cause of progressive sensorineural hearing loss. The prevalence of syphilitic sensorineural hearing loss (SNHL) in childhood is not clearly defined.

OBJECTIVE: To determine the frequency and characteristics of pediatric SNHL following intrauterine infection with or exposure to Treponema pallidum in order to develop evidence-based guidelines for audiologic monitoring.

DATA SOURCES: Medline (1950-March 2008), EMBASE (1980-March 2008), CINAHL (1982-March 2008), BIOSIS Previews (1969-March 2008), and Cochrane databases. Manual search of references of identified articles and book chapters.

STUDY SELECTION: Articles with an inception cohort of children infected with T. pallidum during pregnancy, positive serological identification of syphilis infection in the antenatal period or pathognomonic clinical signs of congenital syphilis infection, and longitudinal serial audiologic evaluations to identify the prevalence and progression of SNHL.

DATA EXTRACTION: Patient information, maternal and infant serologic status, and audiometric data extracted in an independent fashion. Discrepancies resolved through mutual consensus.

DATA SYNTHESIS: Descriptive statistics.

RESULTS: One prospective cohort study met the inclusion criteria. No cases of SNHL in infants with early congenital syphilis treated with antibiotics in the neonatal period were identified.

CONCLUSIONS: There have been no reports of children with confirmed congenital SNHL secondary to in utero syphilis infection. Newborns with positive syphilis serology should have hearing screening performed at birth and receive treatment with an appropriate course of penicillin therapy. Longitudinal hearing screening is recommended for all pediatric patients with congenital syphilis, as further studies documenting longitudinal audiometric data for patients previously treated either fully or partly for congenital syphilis are required.