A Mutation in the gene TBC1 Domain Family Member 24 (TBC1D24), encoding for a GTPase activating the proteins Rab5 and Rab35, involved in endosome to lysosome trafficking as a mechanism for degrading synaptic vesicles associated proteins, has been shown to cause severe neurodegeneration and deafness [100]. Here, TBC1D24 is linked to deafness.