[Update on physiology and pathophysiology of the inner ear: pathomechanisms of sensorineural hearing loss].
Abstract
Hearing impairment is the most common form of human sensory deficit. The most frequent form, sensorineural hearing loss (SNHL), which accounts for approximately 70% of cases, encompasses various pathologies in both the inner ear and the auditory nerve. The individual hearing impairment and its outcome following aiding with hearing devices critically depend on the underlying disorder. Here recent progress in our understanding of the cellular mechanisms of SNHL in genetically engineered mouse models is reviewed. First, insights gained from models for specific defects in cochlear sound amplification and ion homeostasis are discussed followed by a focus on disorders of the inner hair cell synapses (auditory synaptopathy) and the auditory nerve (auditory neuropathy). Both nosological entities have also attracted substantial clinical interest in recent years and share an impaired temporal processing of auditory stimuli. This results in poor speech recognition, often out of proportion to the pure tone threshold. Hearing loss can range from mild variants with exclusive deficits of temporal processing to complete deafness. At least initially, signs of normal outer hair cell function such as evoked otoacoustic emissions can be found. In ...Continue Reading
References
Mice with altered KCNQ4 K+ channels implicate sensory outer hair cells in human progressive deafness
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