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January 1968

Middle Ear Disease in Turner's Syndrome

Author Affiliations

Kraków, Poland
From the departments of pediatrics (Dr. Rybak), and otolaryngology (Dr. Szpunar) Medical Academy, Kraków, Poland.

Arch Otolaryngol. 1968;87(1):34-40. doi:10.1001/archotol.1968.00760060036007

IN 1938 HENRY TURNER1 reported a syndrome consisting of shortness of stature, sexual underdevelopment, short, "webbed" neck, and cubitus valgus, features he found in seven girls, aged 15 to 23. In his opinion this syndrome was brought about by pituitary insufficiency. It should be mentioned, however, that in 1930 Ulrich2 had already reported a somewhat similar "pterygium syndrome." Later, Varney et al3 and Albright et al4 proved that the cause of sexual underdevelopment in such cases lies in gonadal aplasia or dysgenesis with a secondary rise of urinary gonadotropins. Polani5 showed that in 80% of these cases sex chromatin was negative (male) and the chromosome constition was most often XO.

Isolated sexual underdevelopment is now called "pure" gonadal dysgenesis. On the other hand, gonadal dysgenesis combined with some characteristic anomalies of other organs is usually called Turner's syndrome, formerly also Ulrich-Turner's syndrome. Here the following