Biomaterial Database

Osteogenesis imperfecta and hearing loss. 1996

M Verstreken, and J Claes, and P H Van de Heyning

Department of E.N.T., Head and Neck Surgery, University of Antwerp, Belgium.

A review of the literature of osteogenesis imperfecta (OI) and hearing loss in the last century is given. This includes the first association in 1912 by Adair-Dighton of OI with hearing loss, the triad of VAN DER HOEVE and DE KLEYN and different clinical and audiometric studies and classifications of the disease. A case report of a 14 year old patient is also included. The genetics of OI and the complexity in correlating genetics with clinical classifications is elucidated. Finally, the findings reported after middle ear surgery, the results of surgery and comparisons with otosclerosis are reviewed.

UI	MeSH Term	Description	Entries
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D010013	Osteogenesis Imperfecta	COLLAGEN DISEASES characterized by brittle, osteoporotic, and easily fractured bones. It may also present with blue sclerae, loose joints, and imperfect dentin formation. Most types are autosomal dominant and are associated with mutations in COLLAGEN TYPE I.	Fragilitas Ossium,Lobstein Disease,Brittle Bone Disease,Lobstein's Disease,Osteogenesis Imperfecta Tarda,Osteogenesis Imperfecta with Blue Sclerae,Osteogenesis Imperfecta, Type 1,Osteogenesis Imperfecta, Type I,Disease, Lobstein,Disease, Lobstein's,Lobsteins Disease,Ossiums, Fragilitas,Osteogenesis Imperfecta Tardas
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002886	Chromosomes, Human, Pair 17	A specific pair of GROUP E CHROMOSOMES of the human chromosome classification.	Chromosome 17
D002897	Chromosomes, Human, Pair 7	A specific pair of GROUP C CHROMOSOMES of the human chromosome classification.	Chromosome 7
D003094	Collagen	A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of SKIN; CONNECTIVE TISSUE; and the organic substance of bones (BONE AND BONES) and teeth (TOOTH).	Avicon,Avitene,Collagen Felt,Collagen Fleece,Collagenfleece,Collastat,Dermodress,Microfibril Collagen Hemostat,Pangen,Zyderm,alpha-Collagen,Collagen Hemostat, Microfibril,alpha Collagen
D004432	Ear, Middle	The space and structures directly internal to the TYMPANIC MEMBRANE and external to the inner ear (LABYRINTH). Its major components include the AUDITORY OSSICLES and the EUSTACHIAN TUBE that connects the cavity of middle ear (tympanic cavity) to the upper part of the throat.	Tympanic Cavity,Tympanum,Middle Ear,Cavities, Tympanic,Cavity, Tympanic,Ears, Middle,Middle Ears,Tympanic Cavities,Tympanums
D005260	Female		Females
D006314	Hearing Loss, Conductive	Hearing loss due to interference with the mechanical reception or amplification of sound to the COCHLEA. The interference is in the outer or middle ear involving the EAR CANAL; TYMPANIC MEMBRANE; or EAR OSSICLES.	Conductive Hearing Loss
D006320	Hearing Tests	Part of an ear examination that measures the ability of sound to reach the brain.	Hearing in Noise Test,Quick Speech-in Noise Test (QuickSIN),Real Ear Measurement,Speech in Noise Hearing Test,Ear Measurement, Real,Ear Measurements, Real,Hearing Test,Measurement, Real Ear,Measurements, Real Ear,Quick Speech in Noise Test (QuickSIN),Real Ear Measurements,Test, Hearing,Tests, Hearing