Spiral ganglion degeneration in a mouse model of auditory neuropathy spectrum disorder

2014 Fiscal Year Final Research Report

• Project/Area Number: 24791819
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Otorhinolaryngology
• Research Institution: 独立行政法人国立病院機構(東京医療センター臨床研究センター)
• Principal Investigator: NAMBA Kazunori 独立行政法人国立病院機構(東京医療センター臨床研究センター), 聴覚・平衡覚研究部, 研究員 (60425684)
• Project Period (FY): 2012-04-01 – 2015-03-31
• Keywords: OTOF / Otoferlin / Auditory Neuropathy / ANSD / 蝸牛神経節 / Spiral Ganglion / 神経細胞 / neurodegeneration

Outline of Final Research Achievements

Auditory neuropathy spectrum disorder (ANSD) is a hearing disorder in which sound enters the inner ear normally but the transmission of signals from the inner ear to the brain is impaired. In this study, we compared the number of spiral ganglion (SG) neurons between otoferlin deficient (OTOF-/-) and normal mice in developmental stage.
Comparing to normal SG, the number of afferent type-1 neuron for OTOF-/- was apoptically decreased from post-natal day 7, and it over 50% in 12 weeks of age. However, a decrease was not seen in efferent type-II neuron.
Because the outer hair cell is mainly projected by efferent nerve, these data suit the definition of ANSD, and it is useful as the animal model of disease of ANSD.

Free Research Field

耳科学