• auditory brainstem response;
  • bilateral sensorineural hearing loss;
  • inner ear;
  • syndromic hearing loss


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.