Usher syndrome associated with a variant of Dandy-Walker malformation.J Pediatr Ophthalmol Strabismus. 2010 May 21; 47 Online:e1-4.JP
Three cases of Usher syndrome associated with a variant of Dandy-Walker malformation in three siblings from consanguineous Turkish parents are described. The siblings had retinitis pigmentosa and hearing loss. Two of the siblings also had mental retardation, which is not a constant finding in Usher syndrome. Dandy-Walker malformation might have contributed to the mental retardation in two of these patients and might be a coincidental finding with Usher syndrome.