EarBase
News
FDA Approves First-Ever Gene Therapy for Treatment of Genetic Hearing Loss Under National Priority Voucher Program
Groundbreaking AAV-based gene therapy offers potential treatment for patients with OTOF gene-associated severe-to-profound and profound hearing loss.
Read full announcementCochlear Neural Degeneration Is Widespread in Ménière’s Disease: A Temporal Bone Study (Journal of the Association for Research in Otolaryngology)
Ménière's disease presents with endolymphatic hydrops and disproportionately poor word-recognition compared to other forms of sensorineural hearing loss, yet the underlying patterns of cochlear degeneration are not well defined. Here, we analyzed 97 human cochleas from the Mass Eye and Ear archives, including 43 Ménière's ears, 10 contralateral (clinically unaffected) ears, and 44 age-matched controls. Quantitative histopathology assessed survival of hair cells, spiral ganglion cells, auditory-nerve peripheral axons, stria vascularis, spiral-ligament fibrocytes, and the degree of endolymphatic hydrops. Ménière's ears displayed consistent cochlear and saccular hydrops, with roughly half the normal complement of cochlear hair cells but only 25% of the normal complement of auditory-nerve peripheral axons, despite preservation of most spiral ganglion cells. We show that the disconnection of the great majority of spiral ganglion cells from their hair cell targets, which was previously unrecognized, is likely the key driver of the poor word-recognition scores associated with Ménière's disease.
Read articleRapid Postmortem Temporal Bone Extraction and Dissection of Human Inner Ear Organs from Organ Donors (The Laryngoscope)
We developed a postmortem protocol for temporal bone extraction and dissection in organ donors, enabling atraumatic bilateral temporal bone retrieval within 1–2 h of ischemia, followed by stepwise microsurgical dissection of neurosensory organs. Combining rapid extraction, abbreviated decalcification, and atraumatic microsurgical dissection, this protocol establishes a scientifically versatile framework for human inner ear tissue acquisition.
Read article