Abstract
In the clinical setting, the pathophysiology of sensorineural hearing loss is poorly defined and there are currently no diagnostic tests available to differentiate between subtypes. This often leaves patients with generalized treatment options such as steroids, hearing aids, or cochlear implantation. The gold standard for localizing disease is direct biopsy or imaging of the affected tissue; however, the inaccessibility and fragility of the cochlea make these techniques difficult. Thus, the establishment of an indirect biopsy, a sampling of inner fluids, is needed to advance inner ear diagnostics and allow for the development of novel therapeutics for inner ear disease. A promising source is perilymph, an inner ear liquid that bathes multiple structures critical to sound transduction. Intraoperative perilymph sampling via the round window membrane of the cochlea has been successfully used to profile the proteome, metabolome, and transcriptome of the inner ear and is a potential source of biomarker discovery. Despite its potential to provide insight into inner ear pathologies, human perilymph sampling continues to be controversial and is currently performed only in conjunction with a planned procedure where the inner ear is opened. Here, we review the safety of procedures in which the inner ear is opened, highlight studies where perilymph analysis has advanced our knowledge of inner ear diseases, and finally propose that perilymph sampling could be done as a stand-alone procedure, thereby advancing our ability to accurately classify sensorineural hearing loss.
Highlights
Permanent hearing loss affects more than 5% of the world’s population and is the most common sensory deficit in developed countries [1]
Perilymph can potentially be sampled during inner ear surgery such as stapedectomy, labyrinthectomy, and cochlear implantation, which allows for comparison between patient subpopulations with sensorineural hearing loss (SNHL) and conductive hearing loss (CHL) [12,13]
We propose that perilymph sampling via the round window membrane (RWM) can be developed as a safe outpatient procedure and can serve as a “liquid biopsy” to guide diagnosis and treatment of SNHL
Summary
Permanent hearing loss affects more than 5% of the world’s population and is the most common sensory deficit in developed countries [1]. Without accurate localization of disease and disease mechanisms, only rehabilitative treatment options such as hearing aids or cochlear implants can be offered. Those with severe or profound SNHL who identify 50%. Perilymph can potentially be sampled during inner ear surgery such as stapedectomy, labyrinthectomy, and cochlear implantation, which allows for comparison between patient subpopulations with SNHL and CHL [12,13]. Postmortem perilymph sampling has allowed for comparisons between CSF, serum, and perilymph, furthering our understanding of inner ear anatomy and the role of each fluid in hearing. This study revealed distinct patterns of esterase concentration of endolymph and perilymph compared to serum and CSF, further suggesting that these are all separate fluids [17]. Over 500 miRNAs have been detected in the endolymph and perilymph of postmortem samples, with 481 differentially expressed in patients with a vestibular disorder called benign paroxysmal positional vertigo [20]
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