The DNMT1:c.4001C>T was reported as VUS for Hereditary Sensory Neuropathy-Deafness-Dementia Syndrome (OMIM #614116), a degenerative disorder of the central and peripheral nervous systems characterized by sensorineural hearing loss, cerebellar ataxia, narcolepsy and dementia. This evidence concerns the gene DNMT1 and hereditary sensory neuropathy-deafness-dementia syndrome.