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July 1970

Congenital Ectodermal Dysplasia With Hearing Loss

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.

Arch Otolaryngol. 1970;92(1):85-89. doi:10.1001/archotol.1970.04310010111016

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.

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