This rare neurologic disease is a slowly progressive disorder resembling Refsum disease. It presents with peripheral neuropathy, late-onset hearing loss, cataracts, and retinitis pigmentosa typically appearing in the third decade of life. Clinical features include pes cavus, ataxic or spastic gait disturbances, sensorimotor peripheral neuropathy, hyporeflexia, hyperreflexia, and extensor plantar responses. The syndrome is known as Fiskerstrand type peripheral neuropathy and is characterized by polyneuropathy, hearing loss, cerebellar ataxia, retinitis pigmentosa, and early-onset cataracts.