Conductive Hearing Loss in Individuals with Fibrodysplasia Ossificans Progressiva Fibrodysplasia ossificans progressiva (FOP) is a very rare genetic disorder that is characterized by progressive heterotopic ossification of soft tissues and congenital malformation of the great toes. Although previous case studies have reported hearing loss in individuals with FOP, there have been no large-scale studies regarding the nature or cause ... Clinical Focus: Grand Rounds
Clinical Focus: Grand Rounds  |   June 01, 1999
Conductive Hearing Loss in Individuals with Fibrodysplasia Ossificans Progressiva
 
Author Affiliations & Notes
  • Charles E. Levy
    The Ohio State University Medical Center, Department of Physical Medicine and Rehabilitation, 480 West 9th Avenue, Columbus, OH 43210-1290
  • Albert T. Lash
    The Ohio State University Medical Center, Department of Physical Medicine and Rehabilitation, 480 West 9th Avenue, Columbus, OH 43210-1290
  • Hal B. Janoff
    The University of Pennsylvania School of Medicine, Philadelphia
  • Frederick S. Kaplan
    The University of Pennsylvania School of Medicine, Philadelphia
Article Information
Hearing Disorders / Clinical Focus / Grand Rounds
Clinical Focus: Grand Rounds   |   June 01, 1999
Conductive Hearing Loss in Individuals with Fibrodysplasia Ossificans Progressiva
American Journal of Audiology, June 1999, Vol. 8, 29-33. doi:10.1044/1059-0889(1999/011)
History: Received October 23, 1998 , Accepted April 5, 1999
 
American Journal of Audiology, June 1999, Vol. 8, 29-33. doi:10.1044/1059-0889(1999/011)
History: Received October 23, 1998; Accepted April 5, 1999

Fibrodysplasia ossificans progressiva (FOP) is a very rare genetic disorder that is characterized by progressive heterotopic ossification of soft tissues and congenital malformation of the great toes. Although previous case studies have reported hearing loss in individuals with FOP, there have been no large-scale studies regarding the nature or cause of the hearing loss. Here, we report the findings of a two-part study. In Part I, we report the findings of a postal survey regarding hearing loss that was sent to 102 individuals with FOP. In Part II, we report the findings of on-site hearing evaluations of eight individuals with FOP. The findings of both studies indicate that individuals with FOP are at risk for hearing loss and that the type of loss is predominantly conductive in nature, similar to that seen in individuals who have otosclerosis.

Acknowledgments
We thank David Fabry, John Ferraro, and three anonymous reviewers for their comments on an earlier version of this manuscript. This work was supported by a grant from The Battelle Endowment for Technology and Human Affairs (Columbus, OH), Permobil (Woburn, MA), and the NIH K12 Rehabilitation Medicine Scientist Development Program Award.
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