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January 1983

Hereditary Inner-Ear Abnormalities in Animals: Relationships With Human Abnormalities

Author Affiliations

From the MRC Institute of Hearing Research, University of Nottingham, United Kingdom. Dr Steel is now with the Institut für Zoologie, Technische Universität München, West Germany.

Arch Otolaryngol. 1983;109(1):22-29. doi:10.1001/archotol.1983.00800150026005

• A classification scheme for inner-ear abnormalities is defined, based on animals with hereditary inner-ear defects. Three major groups are (1) morphogenetic abnormalities, involving gross structural deformities of the labyrinth; (2) neuroepithelial abnormalities, in which there appears to be a primary organ of Corti defect and Reissner's membrane remains in its normal position; and (3) cochleosaccular abnormalities, showing collapse of Reissner's membrane and restriction of vestibular abnormalities to the saccule. Recent experimental findings in hearingimpaired animal mutants are taken into account. The relevance of the classification scheme to conditions in humans is discussed. It is suggested that the Mondini and Michel deformities in humans are best considered together in the morphogenetic group. Some cases of inner-ear abnormality in humans might be considered as belonging to the neuroepithelial group.

(Arch Otolaryngol 1983;109:22-29)