Currently, we use several mouse models and strains to study central auditory processing in the midbrain in normal hearing and after hearing loss, as well as we plan to study visual cross-modal effects in deaf mice before and after implantation of cochlear implants.

C57BL/6J is the most widely used inbred strain and the first to have its genome sequenced. Although this strain is refractory to many tumors, it is a permissive background for maximal expression of most mutations. C57BL/6J mice are resistant to audiogenic seizures, have a relatively low bone density, and develop age related hearing loss (see

CBA/J inbred mice are widely used as a general purpose strain. CBA/J strain is the only CBA substrain that carries the Pde6brd1 mutation, which causes blindness by wean age. CBA/J mice are not histocompatible with the CBA/CaJ (Stock No. 000654) substrain (Green and Kaufer, 1965). The CBA/J inbred mouse strain is used to study granulomatous experimental autoimmune thyroiditis (G-EAT), are relatively resistant to diet-induced atherosclerosis (Paigen et al. 1990), and develop a mild hearing loss late in life, with most of the hearing loss occurring in the higher frequencies (Sweet et al. 1988) (see

Cacna1d (L-type channel Cav1.3)
Hearing impairment represents the most common sensory deficit in humans. Genetic mutations contribute significantly to this disorder. Mostly, only malfunction of the ear is considered. Here, we assessed the role of the peripheral deafness gene Cacna1d, encoding the L-type channel Cav1.3, in downstream processing of acoustic information. To this end, we generated a mouse conditional Cacna1d-eGFPflex allele. Upon pairing with Egr2::Cre mice, Cav1.3 was ablated in the auditory brainstem, leaving the inner ear intact (from

Expression of the otoferlin gene is not detected in these mutant mice, indicating that this missense mutation has a severe effect on the stability of the protein and potentially its localization. Auditory brainstem response analysis demonstrated that mice homozygous for the missense mutation are profoundly deaf, consistent with an essential role for otoferlin in inner hair cell neurotransmission. Vestibular-evoked potentials of mutant mice, however, is equivalent to those of wildtype mice, indicating that otoferlin is unnecessary for vestibular function even though it is highly expressed in both vestibular and cochlear hair cells (from .

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