Progress in deciphering the genetic architecture of human sensorineural hearing
impairment (SNHI) or loss, and multidisciplinary studies of mouse models, have led to the …

In recent years, sensory neuroscientists have made major efforts to dissect the structure and
function of ribbon synapses which process sensory information in the eye and ear. This …

The classic view of sensorineural hearing loss (SNHL) is that the “primary” targets are hair
cells, and that cochlear-nerve loss is “secondary” to hair cell degeneration. Our recent work …

Unlike nonmammalian vertebrates, mammals cannot convert inner ear cochlear supporting
cells (SCs) into sensory hair cells (HCs) after damage, thus causing permanent deafness …

Clarin-1, a tetraspan-like membrane protein defective in Usher syndrome type IIIA (USH3A),
is essential for hair bundle morphogenesis in auditory hair cells. We report a new synaptic …

Cochlear inner hair cells (IHCs), the mammalian auditory sensory cells, encode acoustic
signals with high fidelity by Graded variations of their membrane potential trigger rapid and …

Sensorineural hearing impairment is the most common sensory disorder and a major health
and socio-economic issue in industrialized countries. It is primarily due to the degeneration …

Hearing relies on rapid, temporally precise, and sustained neurotransmitter release at the
ribbon synapses of sensory cells, the inner hair cells (IHCs). This process requires otoferlin …

Ferlins are a family of multiple C 2 domain proteins with emerging roles in vesicle fusion and
membrane trafficking. Ferlin mutations are associated with muscular dystrophy (dysferlin) …

Ribbon synapses of cochlear inner hair cells (IHCs) undergo molecular assembly and
extensive functional and structural maturation before hearing onset. Here, we characterized …