There is growing evidence that the dynamics of biological systems that appear to be exponential over short time courses are in some cases better described over the long-term by power-law dynamics. A model of rate adaptation at the synapse between inner hair cells and auditory-nerve (AN) fibers that includes both exponential and power-law dynamics is presented here. Exponentially adapting compon...

OBJECTIVE To investigate auditory neural involvement in patients with Leber's hereditary optic neuropathy (LHON). METHODS Auditory assessment was undertaken in two patients with LHON. One was a 45 year old woman with Harding disease (multiple-sclerosis-like illness and positive 11778mtDNA mutation) and mild auditory symptoms, whose auditory function was monitored over five years. The other wa...

PURPOSE To determine whether activated Notch can promote a supporting cell fate during sensory cell differentiation in the inner ear. METHODS An activated form of the Notch1 receptor (NICD) was expressed in early differentiating hair cells using a Gfi1-Cre mouse allele. To determine the effects of activated Notch on developing hair cells, Gfi1-NICD animals and their littermate controls were a...

Auditory hair cell defect is a major cause of hearing impairment, often leading to spiral ganglia neuron (SGN) degeneration. The cell loss that follows is irreversible in mammals, because inner ear hair cells (HCs) have a limited capacity to regenerate. Here, we report that in the adult brain of both rodents and humans, the ependymal layer of the lateral ventricle contains cells with proliferat...

Previously reported changes in the gross sound-evoked cochlear potentials after intracochlear perfusion of nimodipine suggest that dihydropyridine-sensitive Ca2+ channels (L-type) control the sound-evoked release of transmitter from the inner hair cells of the mammalian cochlea. In the present study, we combined recording of the action potentials of single primary auditory afferent neurons with...

PURPOSE OF REVIEW For the perception of sound, acoustic signals need to be encoded into a neuronal code. This takes place at the inner hair cells of the organ of Corti and the afferent fibres of the auditory nerve. We will review the current knowledge of the anatomy and function of these elements as well as their connection - formed by the afferent inner hair cell synapse. RECENT FINDINGS Dep...

OBJECTIVE To review new insights into the pathophysiology of sensorineural hearing impairment. Specifically, we address defects of the ribbon synapses between inner hair cells and spiral ganglion neurons that cause auditory synaptopathy. DATA SOURCES AND STUDY SELECTION Here, we review original publications on the genetics, animal models, and molecular mechanisms of hair cell ribbon synapses ...

Laser scanning confocal microscopy was used to determine the distribution of actin, spectrin and tubulin in whole mounts of the organ of Corti of guinea pig, monkey, rat and chinchilla. Actin, spectrin and tubulin were localized in all cell types in the auditory epithelium. No specialized cytoskeletal organization of tubulin was detected in the cytoplasmic domain of hair cells. The only special...

Calcium buffers are important for shaping and localizing cytoplasmic Ca2+ transients in neurons. We measured the concentrations of the four main calcium-buffering proteins (calbindin-D28k, calretinin, parvalbumin-alpha, and parvalbumin-beta) in rat cochlear hair cells in which Ca2+ signaling is a central element of fast transduction and synaptic transmission. The proteins were quantified by cal...