This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Yukiko lino, MD; Minoru Toriyama, MD; Yasunaga Sarai, MD; Tomoko Hasegawa, MD; Tetsuo Ishii, MD

Arch Otolaryngol Head Neck Surg. 1987;113(12):1325-1329.

Abstract
• We report the histopathological findings of the temporal bones and the nasal and paranasal specimen of a 7-month-old girl diagnosed as having Wolf-Hirschhorn or 4p- syndrome (deletion of the short arm of chromosome 4). This syndrome is characterized by growth retardation, mental retardation, and multiple congenital abnormalities, including craniofacial anomalies and hearing disturbance. These temporal bones displayed malformation of the ossicles, absence of the oval windows, abnormal course of the facial nerve with incomplete bony canal, and depression of the cochlear duct and the saccule. In addition, cholesteatoma, which might be of congenital origin, was present behind the eardrum. The nasal and paranasal specimen showed bilateral complete cleft palate with normal development of paranasal sinuses, nasal septum, and conchae.

(Arch Otolaryngol Head Neck Surg 1987;113:1325-1329)

Author Affiliations
From the Department of Otolaryngology (Drs lino, Toriyama, and Sarai) and the Division of Genetics, Clinical Research Institute (Dr Hasegawa), National Medical Center Hospital, Tokyo; and the Department of Otolaryngology, Tokyo Women's Medical College (Dr Ishii).; Reprint requests to Department of Otolaryngology, National Medical Center Hospital, 21-1, Toyama 1-chome, Shinjuku-ku, 162 Tokyo, Japan (Dr lino).

Footnotes
Accepted for publication April 17, 1987.

Read in part at the Fourth International Congress of Pediatric Otorhinolaryngology, Eger, Hungary, June 24, 1986.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?