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Temporal Bone Histopathologic Features and Otolaryngologic Manifestations

Nobuhiro Tokita, MD; H. K. Chandra-Sekhar, MD; John F. Daly, MD; Melvin H. Becker, MD; Slobodan Aleksic, MD

Arch Otolaryngol. 1979;105(8):449-454.

Abstract
• The campomelic syndrome is characterized by dwarfism, craniofacial anomalies, bowing of the tibiae and femora, cutaneous dimpling overlying the tibial bend, respiratory distress, and early death. Otolaryngologic manifestations include flat facies with a broad nasal bridge, low-set ears, cleft palate, mandibular hypoplasia, and tracheobronchial malacia. The underlying pathologic feature appears to be disturbance in cartilage growth involving the affected bones and the respiratory tract cartilage. The cause is unknown. We report clinical and histopathologic features in two cases of this syndrome. The endochondral layer of the otic capsule contained no cartilage cells. The cochlea was short and flattened, presenting a scala communis. The vestibule and the canals were deformed by bone invasion. Defective endochondral ossification of the petrooccipital synchondroses possibly explains the shortened skull base seen in this syndrome. The tracheobronchial malacia significantly contributes to respiratory distress and neonatal death.

(Arch Otolaryngol 105:449-454, 1979)

Author Affiliations
From the Departments of Otolaryngology (Drs Tokita, Chandra-Sekhar, and Daly) Radiology (Dr Becker), and Neuropathology (Dr Aleksic), New York (NY) University Medical Center.

Footnotes
Accepted for publication Nov 24, 1977.

Reprint requests to Department of Otolaryngology, New York University Medical Center, 550 First Ave, New York, NY 10016 (Dr Daly).