title: Sensorineural hearing loss in insulin-like growth factor I-null mice: a new model of human deafness.
abstract: It has been reported that mutations in the gene encoding human insulin-like growth factor-I (IGF-I) cause syndromic hearing loss. To study the precise role of IGF-I in auditory function and to hypothesize the possible morphological and electrophysiological changes that may occur in the human inner ear, we have analysed the auditory brainstem response in a mouse model of IGF-I deficiency. We show here that homozygous Igf-1(-/-) mice present an all-frequency involved bilateral sensorineural hearing loss. Igf-1(-/-) mice also present a delayed response to acoustic stimuli; this increases along the auditory pathway, indicating a contribution of the central nervous system to the hearing loss in Igf-1(-/-) mice. These results support the use of the Igf-1(-/-) mouse as a new model for the study of human syndromic deafness.
authors.affiliation: Instituto de Investigaciones Biomédicas 'Alberto Sols', Consejo Superior de Investigaciones Científicas (CSIC), Universidad Autónoma de Madrid (UAM), Arturo Duperier 4, Madrid 28029, Spain.
authors.forename: R
authors.initials: R
authors.lastname: Cediel
authors.forename: R
authors.initials: R
authors.lastname: Riquelme
authors.forename: J
authors.initials: J
authors.lastname: Contreras
authors.forename: A
authors.initials: A
authors.lastname: Díaz
authors.forename: I
authors.initials: I
authors.lastname: Varela-Nieto
mesh_terms: D000161:Acoustic Stimulation / Q000379:methods; D000818:Animals; D001309:Auditory Threshold / Q000502:physiology; D003638:Deafness / Q000235:genetics / Q000503:physiopathology*; D004195:Disease Models, Animal; D004307:Dose-Response Relationship, Radiation; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology / Q000528:radiation effects; D006319:Hearing Loss, Sensorineural / Q000235:genetics* / Q000503:physiopathology; D007334:Insulin-Like Growth Factor I / Q000172:deficiency / Q000502:physiology*; D051379:Mice; D018345:Mice, Knockout; D011930:Reaction Time / Q000502:physiology / Q000528:radiation effects
chemical_list: D007334:Insulin-Like Growth Factor I