Deletion of Limk1 and Limk2 in mice does not alter cochlear development or auditory function.

Inherited hearing loss is associated with gene mutations that result in sensory hair cell (HC) malfunction. HC structure is defined by the cytoskeleton, which is mainly composed of actin filaments and actin-binding partners. LIM motif-containing protein kinases (LIMKs) are the primary regulators of actin dynamics and consist of two members: LIMK1 and LIMK2.

Accelerated Development of the First-Order Central Auditory Neurons With Spontaneous Activity.

In developing sensory systems, elaborate morphological connectivity between peripheral cells and first-order central neurons emerges via genetic programming before the onset of sensory activities. However, how the first-order central neurons acquire the capacity to interface with peripheral cells remains elusive. By making patch-clamp recordings from mouse brainstem slices, we found that a subset of neurons in the cochlear nuclei, the first central station to receive peripheral acoustic impulses, exhibits spontaneous firings (SFs) as early as at birth, and the fraction of such neurons increases during the prehearing period.

Auditory Neuropathy after Damage to Cochlear Spiral Ganglion Neurons in Mice Resulting from Conditional Expression of Diphtheria Toxin Receptors.

Auditory neuropathy (AN) is a hearing disorder characterized by normal cochlear amplification to sound but poor temporal processing and auditory perception in noisy backgrounds. These deficits likely result from impairments in auditory neural synchrony; such dyssynchrony of the neural responses has been linked to demyelination of auditory nerve fibers. However, no appropriate animal models are currently available that mimic this pathology.

[Development of improving speech perception of cochlear implants in noisy environment].

Cochlear implantation has been a standard therapy for treating severe deafness because patients who receive it have better speech perception. However, the hearing performance of cochlear implantation in noisy environment is far from satisfaction. Efforts have been made to reverse such condition, such as EAS, bimodal stimulation, environment-adaptive speech enhancement and multipolar stimulation, and patients who receive it get more or less better speech perception in noisy environment than traditional cochlear implantation.