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June 1969

Unusual Audiological Findings in Cranial Metaphyseal Dysplasia

Author Affiliations

Milwaukee; Wood, Wis
From the Department of Otolaryngology, Marquette School of Medicine; the Department of Audiology, Milwaukee Childrens Hospital (Dr. Miller); and the Department of Otolaryngology, Veterans Administration Hospital, Wood, Wis (Dr. Geretti).

Arch Otolaryngol. 1969;89(6):861-864. doi:10.1001/archotol.1969.00770020863012

C ranial metaphyseal dysplasia is a rare congenital abnormality characterized by increased density and thickness of the vault, facial bones, base of the skull, and diaphyses of the long bones. Other descriptive terms, such as leontiasis ossea and osteopetrosis may be variants of this disease. Inheritance may occur as an autosomal dominant or recessive trait. The neurologic sequelae may be late motor development, mental retardation,cranial nerve paralyses from atroatropphyd by the overgrowth of the foraminaforamina.1some type of hearing loss associated with thiproba-blysorsorineural, ecause the cochlear nerves become compressed as they pass through narrowed internal auditory canals. Less frequently, there can be a conductive type hearing loss as a result of deformities of the external auditory meatus or fixation of the stapes in the oval window. However, the literature has not reported the possibility of hearing loss deterioration or an apparent change in the status of the loss over

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