SINGLE-GENE INFLUENCES ON RADIOLOGICALLY-DETECTABLE MALFORMATIONS OF THE INNER-EAR

Inner ear malformations associated with hearing loss or vestibular dys function are discussed from the viewpoint of the etiologies of the mal formation. Symptoms of classification of inner ear malformations are d iscussed. The significance of malformations of the cochlea and vestibu lar aqueduct to auditory function are discussed. Genetics features and characteristics of Branchio-oto-renal, Waardenburg's, Pendred's, DiGe orge's, Wildervanck, Fountain, and Treacher Collins syndromes are disc ussed in relation to ear abnormalities and hearing. Similar attention is given to genetic studies of nonsyndromic hearing loss. (C) 1998 by Elsevier Science Inc.