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David Morgan, FRCS; Martin Bailey, FRCS; Peter Phelps, FRCR, FRCS; Susan Bellman, MRCP, FRCS; Andrew Grace, FRCS; Richard Wyse, PhD

Arch Otolaryngol Head Neck Surg. 1993;119(1):49-54.

Abstract
• A comprehensive evaluation of the otolaryngological abnormalities in 50 patients with colobomata, heart defect, atresia of the choanae, retarded growth or development, genital hypoplasia, and ear anomalies or deafness (CHARGE) was performed. All the patients had ear abnormalities; 96% (48/50) had malformed pinnae, and 54% (27/50) had facial nerve palsies. Only 8% (4/50) had normal hearing, the commonest hearing defect being severe conductive or mixed loss. Eighty-four percent (42/50) of computed tomographic scans of the temporal bone were abnormal, the characteristic abnormality being the combination of a hypoplastic incus and absent semicircular canals. Eighty-six percent (43/50) of patients had upper airway abnormalities. Posterior choanal abnormalities occurred in 56% (28/50), and 42% (21/50) had retrognathia leading to intubation difficulties. Laryngotracheal abnormalities occurred in 38% (19/50), and 14% (7/50) required tracheostomies. Careful upper airway assessment is essential to avoid potentially lethal complications such as aspiration.

(Arch Otolaryngol Head Neck Surg. 1993;119:49-54)

Author Affiliations
From the Hospital for Sick Children, London, England. Dr Morgan is now with the East Birmingham (England) Hospital.

Footnotes
Accepted for publication December 3, 1991.

Reprint requests to East Birmingham Hospital, Bordesley Green E, Birmingham, England B9 5ST (Dr Morgan).

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