November 6, 1981

Otosclerosis Associated With Osteopoikilosis

Author Affiliations

Sunnyview Hospital and Rehabilitation Center Schenectady, NY
St George's Medical School West Indies

JAMA. 1981;246(18):2030-2031. doi:10.1001/jama.1981.03320180022014

To the Editor.—  Osteopoikilosis is a rare bone dysplasia thought to be inherited by an autosomal dominant mechanism.1 It is characterized by. numerous spheroid nodules in the spongiosa of the metaphysis and epiphysis. The bones involved most often are the long bones and the small bones of the hands, feet, and pelvis. Osteopoikilosis usually is found incidentally on roentgenography. Its pattern is pathognomonic (ie, polka-dot or spotted bones).Patients with osteopoikilosis have been found to have an increased incidence of dermatofibrosis lenticularis disseminata (localized areas of elastic fiber hyperplasia of the skin). There have been reports of association with keloid formation,2 dwarfism,1 and dystocia.2We describe here cases of a daughter and mother with both osteopoikilosis and otosclerosis, as well as two cousins of the proband with otosclerosis.

Report of Cases.—Case 1.—  A 21-year-old woman went to her podiatrist for foot pain and was