progeroid syndrome Rare genetic endocrine disease Genetic subcutaneous tissue disorder Lipodystrophy - intellectual disability - deafness ICD10:Q78.8 Rajab-Spranger syndrome Lipodystrophy - intellectual disability - deafness is an extremely rare form of genetic lipodystrophy (see this term), reported in 3 patients from one family to date, characterized by generalized congenital lipodystrophy, low birth weight, progressive sensorineural deafness occurring in childhood, intellectual deficit, progressive osteopenia, delayed skeletal maturation, skeletal abnormalities described as slender, undermineralized tubular bones, and dense metaphyseal striations in the distal femur, ulna and radius of older patients. Autosomal recessive inheritance has been suggested. OMIM:608154 orphanet Syndromic genetic deafness Primary bone dysplasia with defective bone mineralization