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Ensink Robbert Jan H.; Sleeckx, Jean-Pete; Cremers, Cor W. R. J.
The American Journal of Otology: May 1999
Pediatric Otology: PDF Only


Results of stapedectomy are reported in a Belgian 26-year-old woman and two Dutch brothers having the proximal symphalangism syndrome (McKusick 18580).

Study Design:

Case reports are presented. A review of the results of ear surgery for congenital conductive hearing loss in this syndrome is given.


The Belgian patient was treated in a general hospital. The Dutch patients were treated in a university hospital, which was a tertiary referral center.


Patients were referred to have an evaluation of their hearing impairment.


Based on the syndromal diagnosis and based on routine audiometric tests, a congenital ossicular fixation was considered to be the cause of the hearing loss. By exploratory tympanotomies, this was confirmed. Reconstaictive procedures including stapedotomy were performed.

Main Outcome Measures/Results:

Long-term audiometric data are presented to evaluate the outcome of the surgical interventions.


Congenital stapes ankylosis eventually combined with a congenital fixation of the short process of the incus in the fossa incudis, causing the congenital conductive hearing loss. Surgical intervention is very successful in most reported cases, but negative side effects are incidentally found as well.

© 1999, The American Journal of Otology, Inc.