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We report a kindred with autosomal dominantly inherited sensory neuropathy associated with sensorineural hearing loss and early-onset dementia. This kindred provides further evidence of the clinical variability among kindreds with hereditary sensory neuropathy, suggesting genetic heterogeneity.

A family is described in which 4 of 10 siblings developed a dementing illness that culminated in death within five to six years of onset. The pathological findings in 3 members were strikingly similar, and consisted of widespread nerve cell loss and astrocytosis within the cerebral cortex, status spongiosus within the outer cortical layers and, in 2, nerve cell loss and astrocytosis within the dorsomedial nucleus of the thalamus. It is concluded that the disorder described in this report does not conform precisely to any of the currently recognized categories of familial dementing disease.

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