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Phytanic acid storage disease

Hearing maintained after 15 years of dietary treatment

Department of Otorhinolaryngology (Drs. Djupesland and Flottorp,) and the Department of Neurology (Dr. Refsum), The National Hospital (Rikshospitalet), Oslo, Norway.

Heredopathia atactica polyneuritiformis is a biochemically defined disease with a specific dietary treatment. It is an autosomal inborn error of metabolism. The phytanic acid is of exogenous origin and stems mainly from preformed phytanic acid in foods. In two Norwegian patients, serum phytanic acid has been brought down to normal levels and one of them has been followed for 15 years. During this period of dietary treatment there was no worsening of hearing.

Address correspondence and reprint requests to Dr. Flottorp, Department of Otorhinolaryngology, The National Hospital (Rikshopitalet), Oslo, Norway.

Accepted for publication June 24, 1982.