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Case Reports in Otolaryngology
Volume 2014, Article ID 901672, 3 pages
http://dx.doi.org/10.1155/2014/901672
Case Report

Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings

1Otolaryngology, Head and Neck Surgery Department, Cruces University Hospital, Plaza de Cruces s/n, Barakaldo, 48903 Bizkaia, Spain
2Ophthalmology, Dermatology and Otolaryngology, University of the Basque Country Medical School, Barrio Sarriena s/n, Leioa, 48940 Bizkaia, Spain
3Neurosurgery Department, Donostia University Hospital, Calle Doctor Begiristain 117, San Sebastián, 20080 Gipuzkoa, Spain

Received 14 April 2014; Accepted 30 May 2014; Published 22 June 2014

Academic Editor: Tamás Karosi

Copyright © 2014 Jose Ignacio Undabeitia et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Middle ear ossicle malformations are an uncommon event. Among them, the congenital absence of the stapes is a very rare condition that is seldom described in the literature. We report the cases of two women, aged 19 and 22 , who presented with a long history of conductive deafness. An exploratory tympanotomy was performed and the absence of the stapes superstructure and an abnormal position of the facial nerve could be observed. A bone anchored hearing aid (BAHA) was implanted in both patients with good results. It is believed that stapes agenesis is related to an abnormal development of the facial nerve, which by the 5th to 6th week of gestation would interpose between the otic capsule and the stapes blastema, preventing these structures from contacting. A long history of nonprogressive hearing loss from birth or early childhood is the key to reach a diagnosis. Several treatment options have been described. The authors opted for a hearing aid due to the high risk of facial nerve lesion, with good functional results.