TBL1X and ocular albinism with late-onset sensorineural deafness: TBL1 may be required for the long-term survival of sensory neurons in the cochlea, because patients with OASD have a mutation in the TBL1 locus that leads to a small deletion and produces a mutant form of TBL1 in which the C-terminal half, including some of the WD40 repeats, is deleted (Figure S2) [17].