It’s NF2 science, but summarised!
About 12 million people in the UK live with hearing loss, including people with NF2-related Schwannomatosis (NF2-SWN or NF2). For some people, hearing loss is associated with communication difficulties, poor social interaction, unemployment, anxiety and depression. Hearing loss resulting from damage to the inner ear (for example vestibular schwannoma tumour growth – see diagram below) is known as sensorineural hearing loss (SNHL) which is generally regarded as permanent. Currently, the management of SNHL is limited to hearing aids, cochlear implants or brain stem implants because there are no approved drugs for its treatment.
At the centre of many conditions that lead to SNHL lies inflammation. Inflammation is an immune system response vital for protecting the body against infection and injury although it can also lead to tissue damage, such as in areas involved in hearing. For example, the inflammatory cells called macrophages that are usually responsible for clearing infections can become active in the inner ear causing damage to the auditory nerve and inner ear hair cells. This activation relies on a sensor of inflammation inside macrophages being stimulated, known as the NLRP3 inflammasome. Once the inflammasome is active, the macrophages release a signalling particle called IL1B into the inner ear that leads to further inflammation and damage in a vicious cycle that can result in SNHL.
Interestingly, there is evidence to suggest drugs that inhibit the inflammatory signal IL1B can reduce the hearing loss experienced in patients with conditions that lead to SNHL. However, these drugs are yet to be tested as treatments for people with auto-inflammatory diseases and SNHL resulting from tumour growth such as the vestibular schwannomas that develop in people with NF2. The identification of the inflammasome in these cases of SNHL provides evidence that IL1B targeted drugs could be repurposed as a wider treatment of SNHL to benefit a larger number of people with hearing loss, including those with vestibular schwannoma.
This article has been written as a summary from the Gregory et al paper published in 2023 titled “The NLRP3 inflammasome as a target for sensorineural hearing loss” from the Geoffrey Jefferson Brain Research Centre in Manchester.
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