Hearing loss and temporal bone structure in achondroplasia

American Journal of Medical Genetics
M ShohatD L Rimoin

Abstract

Characteristic temporal bone changes have recently been defined by high resolution CT in nine patients with achondroplasia (Cobb et al., Am J Neuroradiol 9:1195, 1988). These included narrowing of the skull base and "towering" petrous ridges resulting in abnormal orientation of the inner and middle ear structures. In order to determine whether these morphologic changes are the cause of the hearing deficit in achondroplasia, audiometric studies and ENT evaluation were performed in eight of the nine patients. All had a history of frequent otitis media and four had experienced tympanic membrane tube insertion. Three patients had significant sensorineural hearing loss, two had conductive hearing loss and one patient had combined hearing loss. None of the temporal bone morphologic changes were found to be correlated with the degree of either sensorineural or conductive hearing loss. Fusion of the ossicular chain was not present in any of our cases. Appropriate treatment of frequent acute otitis media and early awareness of middle ear effusions and conductive hearing loss in children with achondroplasia may be of great importance in preventing permanent hearing loss.

References

Jan 1, 1976·Headache·E Poźniak-Patewicz
Apr 1, 1981·International Journal of Pediatric Otorhinolaryngology·L GlassD L Rimoin

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Citations

Sep 27, 2008·Human Molecular Genetics·Suzanne L MansourAndrew O M Wilkie
Jan 1, 2011·Genetics Research International·Joseph P PillionJay Shapiro
Mar 26, 2013·Paediatric Respiratory Reviews·S AfsharpaimanKaren A Waters
May 17, 2012·Otolaryngologic Clinics of North America·Sofia Lyford-PikeDavid E Tunkel
May 28, 2011·The Anatomical Record : Advances in Integrative Anatomy and Evolutionary Biology·Cheryl A Hill
Dec 17, 2008·Biochimica Et Biophysica Acta·Stéphanie PannierLaurence Legeai-Mallet
Mar 22, 2014·Korean Journal of Audiology·Jongyoon JungJune Choi
Apr 17, 1999·American Journal of Medical Genetics·N M ThompsonJ M Fletcher
Jan 5, 2019·Orphanet Journal of Rare Diseases·Richard M Pauli

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