The Otosclerotic Syndrome

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Abstract
To illustrate the occurrence of ectodermal abnormalities in otosclerosis, a family is discussed where, in a married couple, exploration of the middle ear has disclosed typically otosclerotic changes. Numerous cases of perceptive loss of hearing are found among the woman's siblings, and one of her three children also suffers from this disease. Furthermore, chronic caries have been found in the milk teeth of three of the couple's 13 grandchildren. Clinical examination of 256 women with otosclerosis who were examined for occurrences of ectodermal and mesodermal abnormalities demonstrated that these abnormalities were very frequent, especially in patients with hypermobility of the little finger. The results of the above-mentioned examinations, when related to information found in the literature and the previously demonstrated microscopic abnormalities in the skin of patients having otosclerosis, indicate that it is probable that the hearing defect in this illness is but a single symptom in the otosclerotic syndrome.

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