Hearing loss is the most common sensory defect and it is estimated to affect 1 in 1000 newborns in a disabling way. The hearing deficit can strongly impact the proper development of speech and language skills, potentially also affecting the cognitive development of an individual, even when an early diagnosis is successful in identifying the defect.
Hearing loss can have a complex and multifactorial aetiology, ranging from genetic hereditary defects to pharmacological ototoxic effects and noise-induced or age-related degeneration of the hearing function. Several molecular candidates responsible for different types of hearing defects have been identified. The underlying cellular mechanisms are very diverse and often not fully elucidated. The understanding of the processes leading to the loss of auditory function starts from the definition of the pathophysiology of hearing and the development of the sensory organ.
The hearing apparatus consists of an ensemble of highly specialized cell types, synergistically involved in the conversion of mechanical sound waves at different frequencies into electrical impulses to be transmitted to the brain’s hearing center for processing. The development during embryogenesis as well as the homeostasis of the hearing organ involves multiple molecular players, including transcription factors, channels, transporters, structural proteins, and enzymes, and the alteration of the tightly regulated apparatus will affect the hearing function at different levels.
The origin of the sensory defect is often to be found in the developmental stages of the hearing apparatus, during embryogenesis, especially when structural malformations are associated with the loss of the hearing function, such as an enlargement of the vestibular aqueduct (EVA) or an incomplete partition of the cochlea (IP), among others. These malformations are irreversible alterations of the inner ear structure whose consequences on hearing cannot be rectified other than by preventing their occurrence during the delicate phase of embryonic development. Gene therapy or pharmacological interventions can be envisioned as possible approaches aiming to prevent the development of the abnormality and the consequent loss of hearing function. With this goal in mind, understanding the mechanisms leading to the malformation of the inner ear structures and their role in the associated development of hearing loss is of foremost importance.
A mechanistic definition of the causative molecular processes underlying the structural alterations and the associated hearing defects is essential to pave the way for the development of a therapeutic approach aimed at the rescue of the hearing function or the prevention of its degeneration. The authors are encouraged to contribute manuscripts dealing with the elucidation of the pathophysiology of hearing and the pathomechanisms leading to structural alterations and hearing defects. The present Research Topic aims to promote the investigation of potential therapeutical targets for the prevention or rescue of hearing loss, with a particular focus on malformative forms of the hearing defect.
Authors are invited to submit both original studies and review articles focusing on the investigation of the molecular mechanisms underlying the pathophysiology of hearing loss, with a strong focus on the underlying mechanistic aspect. Studies dealing with the development of innovative therapeutical approaches in the prevention or rescue of hearing loss are welcome.
Hearing loss is the most common sensory defect and it is estimated to affect 1 in 1000 newborns in a disabling way. The hearing deficit can strongly impact the proper development of speech and language skills, potentially also affecting the cognitive development of an individual, even when an early diagnosis is successful in identifying the defect.
Hearing loss can have a complex and multifactorial aetiology, ranging from genetic hereditary defects to pharmacological ototoxic effects and noise-induced or age-related degeneration of the hearing function. Several molecular candidates responsible for different types of hearing defects have been identified. The underlying cellular mechanisms are very diverse and often not fully elucidated. The understanding of the processes leading to the loss of auditory function starts from the definition of the pathophysiology of hearing and the development of the sensory organ.
The hearing apparatus consists of an ensemble of highly specialized cell types, synergistically involved in the conversion of mechanical sound waves at different frequencies into electrical impulses to be transmitted to the brain’s hearing center for processing. The development during embryogenesis as well as the homeostasis of the hearing organ involves multiple molecular players, including transcription factors, channels, transporters, structural proteins, and enzymes, and the alteration of the tightly regulated apparatus will affect the hearing function at different levels.
The origin of the sensory defect is often to be found in the developmental stages of the hearing apparatus, during embryogenesis, especially when structural malformations are associated with the loss of the hearing function, such as an enlargement of the vestibular aqueduct (EVA) or an incomplete partition of the cochlea (IP), among others. These malformations are irreversible alterations of the inner ear structure whose consequences on hearing cannot be rectified other than by preventing their occurrence during the delicate phase of embryonic development. Gene therapy or pharmacological interventions can be envisioned as possible approaches aiming to prevent the development of the abnormality and the consequent loss of hearing function. With this goal in mind, understanding the mechanisms leading to the malformation of the inner ear structures and their role in the associated development of hearing loss is of foremost importance.
A mechanistic definition of the causative molecular processes underlying the structural alterations and the associated hearing defects is essential to pave the way for the development of a therapeutic approach aimed at the rescue of the hearing function or the prevention of its degeneration. The authors are encouraged to contribute manuscripts dealing with the elucidation of the pathophysiology of hearing and the pathomechanisms leading to structural alterations and hearing defects. The present Research Topic aims to promote the investigation of potential therapeutical targets for the prevention or rescue of hearing loss, with a particular focus on malformative forms of the hearing defect.
Authors are invited to submit both original studies and review articles focusing on the investigation of the molecular mechanisms underlying the pathophysiology of hearing loss, with a strong focus on the underlying mechanistic aspect. Studies dealing with the development of innovative therapeutical approaches in the prevention or rescue of hearing loss are welcome.
