Hearing dysfunction due to pre- and postsynaptic deficits at the hair cell ribbon synapse (A06)

• Subject Area: Otolaryngology, Phoniatrics and Audiology ; Cognitive, Systems and Behavioural Neurobiology
• Term: from 2011 to 2022
• Project identifier: Deutsche Forschungsgemeinschaft (DFG) - Project number 154113120

In project A06, we plan to use a systems physiology approach to elucidate the disease mechanisms of three forms of human auditory neuropathy with autosomal dominant inheritance. In a mouse model of human AUNA1, overexpression of the actin nucleation factor Diaphanous homologue 3 leads to a primary degeneration of inner hair cell stereocilia. AUNA2 and CAPOS are caused by mutations in Atp11a (unpublished) and Atp1A3, both likely leading to dysfunction of spiral ganglion neurons. In addition, we will continue our work on the role of the scaffold protein bassoon in sound encoding using a hair cell specific knockout mouse.

• DFG Programme: Collaborative Research Centres
• Subproject of: SFB 889: Cellular Mechanisms of Sensory Processing
• Applicant Institution: Georg-August-Universität Göttingen
• Project Head: Professorin Dr. Nicola Strenzke