SELECT LANGUAGE BELOW

Multi-center gene treatment for OTOF-related hearing loss observed for 2.5 years

Multi-center gene treatment for OTOF-related hearing loss observed for 2.5 years
  • Yasunaga, S. et al. identified a mutation in OTOF, which encodes otoferlin, leading to DFNB9, a non-syndromic form of deafness, in their 1999 study published in Nature Genetics.

  • In a 2024 article in The Lancet, Lv, J. et al. discussed a single-arm trial on AAV1-hOTOF gene therapy targeting autosomal recessive deafness 9.

  • A study by Wang, H. et al. in Nature Medicine (2024) presents results from a single-arm trial involving bilateral gene therapy in children affected by autosomal recessive deafness 9.

  • Cheng, X. et al. compared gene therapy to cochlear implants for restoring hearing and speech perception in individuals with congenital deafness in a 2025 JAMA Neurology study.

  • Qi, J. et al. conducted a single-arm trial on AAV gene therapy for autosomal recessive deafness 9, published in Nature Medicine in 2025.

  • Morton, C. C. & Nance, W. E. highlighted newborn hearing screening as a significant advancement in their 2006 article in the New England Journal of Medicine.

  • Lieu, J. E. C. et al. reviewed hearing loss in children in a 2020 JAMA publication.

  • Sloan-Heggen, C. M. et al. analyzed comprehensive genetic testing in a study of 1119 patients with hearing loss published in Human Genetics in 2016.

  • Rodríguez-Ballesteros, M. et al. investigated mutations in the otoferlin gene (OTOF) among those with nonsyndromic hearing impairment in a multicenter study published in 2008.

  • In 2019, Iwasa, Y. I. et al. conducted a mutation analysis of OTOF using massively parallel DNA sequencing in a cohort of 2,265 patients with sensorineural hearing loss, showcased in PLoS ONE.

  • Choi, B. Y. et al. focused on the identities and mutation frequencies of the otoferlin gene causing DFNB9 deafness in Pakistan in a 2009 Clinical Genetics article.

  • Akil, O. et al. studied dual AAV-mediated gene therapy to restore hearing in a mouse model of DFNB9, published in Proceedings of the National Academy of Sciences USA in 2019.

  • Zhang, Q. J. et al. noted a high prevalence of OTOF mutations in Chinese infants with congenital auditory neuropathy spectrum disorder in their 2016 Clinical Genetics study.

  • Roux, I. et al. emphasized the role of otoferlin in exocytosis at the auditory ribbon synapse in a 2006 article in Cell.

  • Zhang, L. et al. evaluated the efficacy and safety of AAV1-hOTOF in both mice and nonhuman primates in their 2023 publication in Molecular Therapy: Methods & Clinical Development.

  • Reisinger, E. & Trapani, I. reported successful outcomes of gene therapy for hereditary deafness in a 2024 The Lancet article.

  • Necula, V. et al. reviewed vertigo related to otosclerosis and stapes surgery in a 2023 narrative review in Medicina.

  • Quatre, R. et al. analyzed long-term hearing outcomes from cochlear implantation in severe otosclerosis in a 2025 study in Otolaryngology Head and Neck Surgery.

  • Terry, B. et al. addressed delayed complications following cochlear implantation in a 2015 article in JAMA Otolaryngology: Head & Neck Surgery.

  • Keithley, E. M. reported on cochlear aging mechanisms in her 2020 publication in Journal of Neuroscience Research.

  • Ford, C. L. et al. conducted a systematic review on otoferlin-related hearing loss, exploring natural history and clinical outcomes in 2023 Human Genetics.

  • Paping, D. E. et al. examined distortion product otoacoustic emissions for detecting early high-frequency hearing loss stages in adolescents, in a 2022 article in Noise Health.

  • Goutman, J. D. et al. discussed cochlear hair cells and their functions in a 2015 article in FEBS Letters.

  • Farinetti, A. et al. presented international consensus guidelines for audiological hearing loss assessment in children in 2018 Eur. Ann. Otorhinolaryngol. Head Neck Dis..

  • Maddox, R. K. et al. evaluated auditory brainstem responses to natural speech in human listeners, published in eNeuro in 2018.

  • Gransier, R. et al. studied phase-locked activity along the human auditory pathway and its individual variability in a 2021 Scientific Reports article.

  • Abdala, C. & Visser-Dumont, L. discussed the use of distortion product otoacoustic emissions for hearing assessment since 2001 in Volta Review.

  • The World Health Organization released the World Report on Hearing in 2021.

  • Robbins, A. M. et al. focused on evaluating meaningful auditory integration in profoundly hearing-impaired children in a 1991 American Journal of Otology study.

  • Robbins, A. M. & Osberger, M. J. produced the Meaningful Use of Speech Scale (MUSS) in 1990.

  • Pennini, P. T. M. & Almeida, K. assessed hearing aid benefits using the Speech, Spatial and Qualities of Hearing Scale in a 2021 article in Codas.

  • Galvin, K. L. & Noble, W. adapted the speech, spatial, and qualities of hearing scale for use with children in a 2013 publication in Cochlear Implants International.

  • Archbold, S. et al. studied the inter-user reliability of auditory performance categories in their 1998 article in British Journal of Audiology.

  • McDaniel, D. M. & Cox, R. M. evaluated the Speech Intelligibility Rating test for hearing aid comparisons in a 1992 Journal of Speech and Hearing Research article.

  • Fu, Q. J. et al. developed and validated a Mandarin speech perception test in a 2011 Journal of Acoustical Society of America study.

  • Cheng, X. et al. found that music training can enhance music and speech perception in pediatric Mandarin-speaking cochlear implant users in a 2018 article in Trends in Hearing.

Facebook
Twitter
LinkedIn
Reddit
Telegram
WhatsApp

Related News