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March 1991

Familial Progressive Vestibulocochlear Dysfunction

Author Affiliations

Department of Neurology Canisius-Wilhelmina Hospital PO Box 9015 6500 GS Nijmegen, the Netherlands
Department of Otolaryngology Academic Hospital PO Box 9101 6500 HB Nijmegen, the Netherlands

Arch Neurol. 1991;48(3):262. doi:10.1001/archneur.1991.00530150030012

To the Editor.  —In 1988, we described a family with vestibular areflexia and progressive hearing loss, presumably developing from the age of about 40 years onward, and caused by an autosomal dominant disorder.1 Among the family members examined in 1984, we found two affected members: the proband, aged 45 years (IV 9 on pedigree1), and his father, aged 71 years (III 16 on pedigree). All other family members examined were reported to show no abnormalities. This also concerned the then 42-year-old sister of the proband (IV 10 on pedigree1). However, her reexamination in 1989, when she was 47 years old, showed that she had also developed the disorder over the past 5 years, and revision of her examinations in 1984 made it clear that a more appropriate interpretation would have been that there were some indications of an impending dysfunction.In 1989, she returned to our department

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