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Objective: Endothelin 1 has many biological activities including regulating the functions of auditory system. The present study aimed to investigate the expression of the endothelin receptors in spiral ganglion neurons and its significance in the auditory transmission.
Methods: The cochleae of healthy guinea pigs were fixed, decalcified, embedded in paraffin and serially sectioned. The expression of endothelin receptor subunits, ET receptor A (ET-A) and ET receptor B (ET-B), was examined in the spiral ganglion neurons of guinea pig using immunohistochemical technique.
Results: Different degrees of ET-A and ET-B positive reactivity appeared in all spiral ganglion neurons from the basal turn to the apical turn.
Conclusions: These findings support the suggestion that endothelin via the endothelin receptor may play a physiological role in the spiral ganglion neurons.
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http://dx.doi.org/10.1016/j.ijporl.2009.11.003 | DOI Listing |
Neurotoxicology
September 2025
Department of Otolaryngology Head and Neck Surgery, The Third People's Hospital of Chengdu, The Affiliated Hospital of Southwest Jiaotong University, Chengdu 610031, China. Electronic address:
Gadolinium-based contrast agents (GBCAs) are widely used in systemic magnetic resonance imaging (MRI) and can be employed in otology to evaluate endolymphatic hydrops in patients with Ménière's disease. Given the heavy metal properties of gadolinium and its tendency to deposit in tissues, it is essential to assess its ototoxic risk. We evaluated the ototoxicity of gadodiamide using in vitro and in vivo models.
View Article and Find Full Text PDFHum Genet
September 2025
College of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Chinese PLA Medical School, 28 Fuxing Road, Beijing, 100853, China.
Recessive variants in TWNK cause syndromes arising from mitochondrial DNA (mtDNA) depletion. Hearing loss is the most prevalent manifestation in individuals with these disorders. However, the clinical and pathophysiological features have not been fully elucidated.
View Article and Find Full Text PDFJ Assoc Res Otolaryngol
September 2025
Biological Sciences Platform, Hurvitz Brain Sciences Program, Sunnybrook Research Institute, Sunnybrook Health Sciences Centre, 2075 Bayview Ave., Room M1 102, Toronto, ON, M4N 3M5, Canada.
Purpose: Delivery of therapeutics to the inner ear is complicated by their inaccessible location and the presence of the blood-labyrinth barrier that restricts most blood-borne compounds from entering the inner ear. This study addresses the challenge of optimal delivery in treating inner ear disease, focusing on magnetic targeting gene therapy using adeno-associated virus (AAV).
Methods: The investigation explores three AAV serotypes (AAV2 Quad Mut, AAV2 pANC80L65, and AAV9 PHP.
Mol Ther
September 2025
Department of Otorhinolaryngology, Graduate School of Medical Science, Brain Korea 21 Project, Yonsei University College of Medicine, Seoul 03722, Republic of Korea; Won-Sang Lee Institute for Hearing Loss, Seoul 03722, Republic of Korea; Severance Biomedical Science Institute, Yonsei University Col
Although gene editing therapy is applicable to human diseases, its efficiency and safety require further investigation. Further, non-virus-mediated gene editor delivery is challenging in the inner ear. Here, engineered virus-like particles (eVLPs) were used for inner-ear delivery of SpCas9 and single-guided RNA to delete the Kcnq4 dominant-negative mutant allele, which causes progressive hearing loss in a non-syndromic hearing loss murine model.
View Article and Find Full Text PDFFront Neurol
August 2025
The Eaton-Peabody Laboratories, The Massachusetts Eye and Ear Department of Otolaryngology - Head and Neck Surgery, Boston, MA, United States.
Sensorineural hearing loss (SNHL) is a hallmark symptom in patients with neurofibromatosis type 2-associated schwannomatosis (NF2-SWN), a genetic condition caused by mutations in the Neurofibromin II gene that encodes the tumor suppressor protein Moesin-Ezrin-Radixin-Like Protein (Merlin; also known as schwannomin). These mutations lead to the development of various tumors, including schwannomas, ependymomas and meningiomas along the vestibular nerve and the cerebellopontine angle. Original theories attributed SNHL in NF2-SWN to the mechanical compression of the vestibulocochlear nerve from the tumor itself, in addition to secretion of toxic tumor byproducts.
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