Congenital Deafness in: 13-15 Trisomy Syndrome

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Abstract
Histopathological studies were performed on a pair of temporal bones from a 6½-month-old patient born with 47 chromosomes. The extra autosomal chromosome is in the 13-15 or "D" group, characterizing the 13-15 (D) trisomy syndrome. The patient failed to respond to sound and had multiple congenital anomalies, clinically consistent with 13-15 trisomy syndrome. To our knowledge, this is the fourth case of this syndrome, with microscopic temporal bone findings, reported in the literature. The abnormal findings were as follows: malformations of the bony partitions of the coils of cochlea, and of the modiolus, anomalies of the saccule and cochlea; and a large teratoma on the right side, consisting of striated muscle fibers and whorls of nerve fibers was found in the internal auditory meatus. To our knowledge, this is the first instance of such a teratoma to be reported in the world literautre.

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