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January 1997

Congenital Conductive Hearing Loss in the Lacrimoauriculodentodigital Syndrome

Author Affiliations

From the Departments of Otorhinolaryngology (Drs Ensink and Cremers) and Human Genetics (Dr Brunner), University Hospital Nijmegen, Nijmegen, the Netherlands.

Arch Otolaryngol Head Neck Surg. 1997;123(1):97-99. doi:10.1001/archotol.1997.01900010107017

An inherited middle ear anomaly that was causing hearing impairment in a 12-year-old girl was treated successfully by a stapedotomy combined with a malleovestibulopexy. Cup-shaped ears, abnormal or absent thumbs, and skeletal deformities of the forearms were present in several members of 3 generations of a family. An autosomal dominant pattern of inheritance was recognized. These features are present in a number of previously described syndromes, but they correspond best with the lacrimoauriculodentodigital syndrome.

Arch Otolaryngol Head Neck Surg. 1997;123:97-99