2b. Laryngoscope, 128:789-793, 2018.

Objective: To systematically appraise the implementation of cochlear implantation (CI) in Usher Syndrome (USH) Types 1, 2, and 3 patients, and analyze who would benefit from CI. Data Sources: A comprehensive search of PubMed, Embase, CINAHL, and Cochrane Library electronic databases from inception through June 2020 was performed. There were no language restrictions. Study Selection: The PRISMA strategy was followed. Included studies discuss USH patients who underwent CI regardless of age, nationality, or clinical subtype. All included studies report post-implantation functional, cognitive, or quality of life outcomes. Only reviews were excluded. Results: Fifteen studies met the inclusion criteria. USH patients experienced improvements in PTA and speech perception and expression outcomes after CI, as well as improvements in phonological memory and quality of life measures. Overall, patients implanted at younger ages outperformed older patients in audiological testing. Similarly, patients with prolonged auditory deprivation had relatively poor performance outcomes in sentence recognition and speech detection following CI. Conclusions: Most USH patients benefit from CI. USH patients who undergo CI at younger ages generally achieve better hearing, speech, and cognitive outcomes. CI at older ages can still prove beneficial if appropriate auditory amplification is started at the right time. Further research is warranted to fill the gap in understanding regarding the gene mutations underlying the pathophysiology of USH that have favorable CI outcomes as well as the optimal time to perform CI.

Background Sensorineural hearing loss (SNHL) is a significant cause of morbidity worldwide and currently has no curative treatment. Technological advancements in stem cell therapy have led to numerous studies that examine the generation of otic sensory cells from progenitors to restore inner ear function. Recently, organoids have emerged as a promising technique to further advance the process of creating functional replacement cells after irreversible hearing loss. Organoids are the three-dimensional generation of stem cells in culture to model the tissue organization and cellular components of the inner ear. Organoids have emerged as a promising technique to create functioning cochlear structures in vitro and may provide crucial information for the utilization of stem cells to restore SNHL. Purpose The purpose of this review is to discuss the recent advancements in stem cell-based regenerative therapy for SNHL. Results Recent studies have improved our understanding about the developmental pathways involved in the generation of hair cells and spiral ganglion neurons. However, significant challenges remain in elucidating the molecular interactions and interplay required for stem cells to differentiate and function as otic sensory cells. A few of the challenges encountered with traditional stem cell therapy may be addressed with organoids. Conclusion Stem cell-based regenerative therapy holds a great potential for developing novel treatment modalities for SNHL. Further advancements are needed in addressing the challenges associated with stem cell-based regenerative therapy and promote their translation from bench to bedside.

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Given the non-labile, terminal differentiation of inner-ear sensory cells, preserving their function is critical since sensory cell damage results in irreversible hearing loss. Gentamicin-induced cytotoxicity is one of the major causes of sensory cell damage and consequent sensorineural hearing loss. However, the precise molecular mechanisms and target proteins involved in ototoxicity are still unknown. The objective of the present study was to identify target proteins involved in gentamicin-induced cytotoxicity to better characterize the molecular pathways involved in sensory cell damage following ototoxic drug administration using House Ear Institute-Organ of Corti 1 (HEI-OC1) cells and high-performance liquid chromatography coupled with tandem mass spectrometry (HPLC-MS/MS). We identified several unique proteins involved in gentamicin-induced cytotoxicity, expression of which were further confirmed using confocal microscopy. Further investigation of these pathways can inform the design and discovery of novel treatment modalities to prevent sensory cell damage and preserve their function.

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