Familial occurrence of retiniti punctata albecen and congenital enorineural deafne

PURPOE: To report the cotranmiion of retiniti punctata albecen (RPA) and congenital enorineural deafne. METHOD: Cae report of two ibling with nyctalopia and profound bilateral enorineural deafne. REULT: The affected ibling, an 11-year-old female and a 7-year-old male, preented with decreaed viual acuity and night blindne. In both eye of both ibling, ophthalmocopic evaluation dicloed numerou white pot at the level of the retinal pigment epithelium with macular paring. The rod threhold dark adaptation and electroretinogram tracing were conitent with advanced rod-cone degeneration. CONCLUION: Two affected member of a family were found to exhibit RPA and congenital enorineural deafne. Thi pedigree upport the genetic cotranmiion of the trait.