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Diran O. Mikaelian, MD; Vasken M. Der Kaloustian, MD; Nicolas A. Shahin, MD; Vergine M. Barsoumian, MA

Arch Otolaryngol. 1970;92(1):85-89.

Abstract
Congenital ectodermal dysplasias (EDs) are a group of familial disorders that affect tissues and organs of ectodermal origin to varying degrees. Since the otic capsule is of ectodermal origin, it is not surprising to find sensorineural hearing loss associated with ED. However, we could find only eight such cases in the literature. The present communication reports on two siblings affected with ED associated with sensorineural hearing loss. Results from a battery of hearing tests suggest a degeneration of the hair cells in the organ of Corti. The mode of inheritance in these cases is one of autosomal recessive form.

Author Affiliations
Beirut, Lebanon

From the department of otolaryngology (Drs. Mikaelian and Shahin and Miss Barsoumian) and pediatrics (Dr. Der Kaloustian), American University Hospital, Beirut, Lebanon.

Footnotes
Accepted for publication Feb 18, 1970.

Reprint requests to Department of Otolaryngology, Jefferson Medical College, Philadelphia, Pa 19107 (Dr. Mikaelian).

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