Congenital Deafness, Spiny Hyperkeratosis, and Universal Alopecia

Congenital deafness has been reported in association with a variety of dermatologic abnormalities. Morris et al1described a five-year-old Negro boy with generalized spiny hyperkeratosis, universal alopecia, and severe, bilateral, congenital, sensorineural deafness. The authors believe that this child is unique, and neither clinically nor histologically resembles any previously described syndrome. We recently acquired the temporal bones of an infant with the same skin disorder. This is the first child with this dermatologic condition whose temporal bones have been studied. The presence of Schiebe's cochleosaccular abnormality in the temporal bones of this infant is offered as a possible explanation for the severe deafness described clinically in the first reported case. In addition, it adds support to the thesis that this syndrome may represent a multifaceted ectodermal defect rather than a group of unrelated findings.