The plainfin midshipman fish, Porichthys notatus, is a seasonal breeding teleost fish for which vocal-acoustic communication is essential for its reproductive success. Female midshipman use the saccule as the primary end organ for hearing to detect and locate "singing" males that produce multiharmonic advertisement calls during the summer breeding season. Previous work has shown that female aud...

Auditory neuropathy is a form of hearing loss in which cochlear inner hair cells fail to correctly encode or transmit acoustic information to the brain. Few genes have been implicated in the adult-onset form of this disease. Here we show that mice lacking the transcription factor Foxo3 have adult onset hearing loss with the hallmark characteristics of auditory neuropathy, namely, elevated audit...

The inner ear, which manages our senses of hearing and balance, has mechanosensitive hair cells, which convert vibration into electronic signal to depolarize auditory or vestibular neurons. Inner ear functions depend largely on hair cells, their associated neurons and cochlear lateral wall, and defects in these cells result in hearing loss and deafness. Although some investigations indicated ha...

Hair cells of the inner ear, the mechanosensory receptors, convert sound waves into neural signals that are passed to the brain via the auditory nerve. Little is known about the molecular mechanisms that govern the development of hair cell-neuronal connections. We ascertained a family with autosomal recessive deafness associated with a common cavity inner ear malformation and auditory neuropath...

Acoustic overstimulation can lead to sensory cell (hair cell) loss in the auditory epithelium. Damaged hair cells in the organ of Corti (the mammalian auditory end-organ) degenerate and are replaced by non-sensory cells (supporting cells) which construct an irreversible scar. In birds, however, auditory hair cells which are damaged by acoustic trauma or ototoxic drugs may be replaced by new hai...

Fishes can regenerate lateral line and inner ear sensory hair cells that have been lost following exposure to ototoxic antibiotics. However, regenerative capabilities following noise exposure have not been explored in fish. Moreover, nothing is known about the functional relationship between hair cell damage and hearing loss, or the time course of morphological versus functional recovery in fis...

We investigate the importance of the degree of peripheral or central target differentiation for mouse auditory afferent navigation to the organ of Corti and auditory nuclei in three different mouse models: first, a mouse in which the differentiation of hair cells, but not central auditory nuclei neurons is compromised (Atoh1-cre; Atoh1f/f ); second, a mouse in which hair cell defects are combin...

Rab interacting molecules (RIMs) are multi-domain proteins that positively regulate the number of Ca2+ channels at the presynaptic active zone (AZ). Several molecular mechanisms have been demonstrated for RIM-binding to components of the presynaptic Ca2+ channel complex, the key signaling element at the AZ. Here, we report an interaction of the C2B domain of RIM2α and RIM3γ with the C-terminus ...

Spontaneous neuronal activity during the development of the auditory sensory system is important in establishing mature connectivity. Wang et al. show that glia-like cells drive spontaneous spiking in neighboring cochlear inner hair cells via a process that involves osmotic cell shrinkage and the secretion of potassium ions.

This paper summarizes recent research on functions of the cochlea of the inner ear. The cochlea is described as the seat of the first step in the auditory sound analysis and transduction of mechanical vibration into electrochemical processes leading to the generation of neural action potentials. The cochlea is also described as a frequent seat of auditory disorders. This research summary addres...