Familial microtia, meatal atresia, and conductive deafness in three siblings

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Abstract
We report on three sibs with right‐sided microtia, meatal atresia, and conductive deafness. Two of the sibs also had right‐sided palatoplegia. These sibs may have the autosomal‐recessive form of microtia (No. 25180, McKusick [1983]), of which few familial cases are known to date. The malformation is due to a disturbance of the development of the first and second branchial arches. Review of the literature shows that microtia and meatal atresia with or without middle‐ear involvement are developmental field defects which, either isolated or as a part of the facio‐auriculovertebral spectrum, may occur (1) sporadically, (2) as component manifestation of syndromes, (3) as a multifactorial, or (4) as an apparent Mendelian trait.