Otologic manifestation of Melnick-Needles Syndrome

Abstract

Melnick-Needles syndrome is a disease of skeletal dysplasia including sclerosis of the skull base, metaphyseal flaring of short and long bones, cortical irregularities and ribbon appearance of ribs, increasement in vertebral body height with anterior concavity, decreased lumbar disc space, marked coxa valga, delayed in general ossification, a delay in closure of the anterior fontanelle, facial abnormalities that show exophthalmos, mild hypertelorism, micrognathia, malaligned teeth and large ears. There has been many reports of this syndrome since Melnick and Needles originally described this disease in 1966，but to date otologic description of this syndrome has been absent. So we report a case of this syndrome associated with stapes fixation.