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April 1968

Unusual Congenital Anomalies in the Inner Ear

Author Affiliations

From the Department of Otolaryngology, Massachusetts Eye and Ear Infirmary and Harvard Medical School.

Arch Otolaryngol. 1968;87(4):335-349. doi:10.1001/archotol.1968.00760060337001

CONGENITAL malformations of the inner ear usually occur in association with other anomalies in well-known hereditary syndromes, and the affected individuals often are severely deaf from birth. These syndromes occur in consistent patterns of morphological deficit which can be classified according to the underlying anatomical, embryologic, or metabolic alteration.1

It is unusual however, to find congenital malformations of the inner ears of individuals who had no obvious hearing loss at birth. Minor morphologic alterations found incidentally in postmortem studies may be considered as anatomical variants of no functional significance.2

We wish to describe the anomalies found in six ears of three individuals who had bilateral progressive hearing losses acquired after birth, and to comment on the embryologic and functional significance of these changes.

Report of Cases  Case 1.—Otological History.—This 78-year-old patient first complained of bilateral hearing loss at the age of 47. Audiometric tests performed

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