Several syndromes have been associated with pili torti. In Bjornstad syndrome, an autosomal dominantly inherited condition, pili torti occurs in association with sensorineural deafness. Menkes kinky-hair disease, a disorder in intestinal copper transport, is characterized by pili torti, depigmented hair, severe neurological abnormalities, bony changes, and early death. Other associations with pili torti include deafness and hypogonadism (Crandall syndrome), and basal cell carcinoma and follicular atrophoderma (Bazex syndrome). Twisted hairs have also been seen in hypohidrotic ectodermal dysplasia, pseudomonilethrix, and trichothiodystrophy in association with other characteristic findings.