Temporal Bone Findings in Cloverleaf Skull Syndrome

概要

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Temporal bone was examined in a case of cloverleaf skull syndrome. This syndrome was first reported in 1960 by Holtermüller and Wiedemann as kleeblattschädel-Syndrom. The patient was a 26-day-old male infant at the time of death. Besides a trilobed skull and abnormalities of extremities, he had low set ears, stenosis of both external auditory canals, beak-like nose, microphthalmia, retentio testis and congenital dermal sinus-like anormaly at coccygeal region. Chromosome study revealed the normal male karyotype. The right temporal bone showed some anomalies through the external and middle ears. The bony portion of external auditory canal was narrowed. The middle ear cavity was filled by mesenchymal tissue devoid of cell components. The incus was almost attached to the lateral tympanic wall. In addition, the stapes was thickened and bent anteriorly. The sensory and neural elements of the inner ear were normal.
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