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Article
September 1940

BRITTLE BONES ASSOCIATED WITH DEAFNESS AND BLUE SCLERAS: REPORT OF THE SYNDROME IN TWO PERSONS OF ONE FAMILY

Author Affiliations

MILWAUKEE
From the Departments of Otolaryngology and Medicine of Mount Sinai Hospital.

Arch Otolaryngol. 1940;32(3):506-511. doi:10.1001/archotol.1940.00660020510008
Abstract

The syndrome of brittle bones, blue scleras, relaxed ligaments and deafness is an interesting hereditary condition which to the otologist is of particular interest because of the nature of the hearing impairment and its similarity to the deafness accompanying otosclerosis. Patients afflicted with this set of symptoms are usually first seen in the orthopedic service, because of frequent and multiple fractures, and the condition is promptly recognized by the blue scleras of the patients themselves and by the histories of frequent fractures both among the patients and among other members of the family. Reports in the literature regarding brittle bones and blue scleras have been numerous, but the associated deafness was reported first by van der Hoeve and de Kleyn1 and later by Bronson.2 These writers brought forth the interesting observation that persons with this syndrome have a tendency to become deaf in the third or fourth decade of life.

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