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Bilateral Conductive Hearing Impairment With Hyperostosis of the Temporal Bone

Bilateral Conductive Hearing Impairment With Hyperostosis of the Temporal Bone CLINICAL NOTE Bilateral Conductive Hearing Impairment With Hyperostosis of the Temporal Bone A New Finding in Robinow Syndrome Dion F. Eijkenboom, MD; Berit M. Verbist, MD, PhD; Cor W. R. J. Cremers, MD, PhD; Henricus P. M. Kunst, MD, PhD obinow syndrome (RS) is characterized by hypertelorism, wide palpebral fissures with a short upturned nose, shortening of forearms and forelegs, and genital hypoplasia. The syndrome is classified into dominant (DRS) and recessive (RRS) types, each with distin- R guishing clinical findings. We evaluated a patient who was diagnosed as having RS with persistent hearing loss. Computed tomographic (CT) images showed ossicular chain changes and thickening of the skull and petrous bones, with appositional bone growth, causing a narrowing of the middle ear cavity and oval and round window niches. These findings were confirmed during an ex- ploratory tympanotomy. To our knowledge, this is the first report of conductive hearing loss in RS. frequencies 0.5 to 4.0 kHz. The PTA of the REPORT OF A CASE right ear was 25 dB (Figure 1A). Speech discrimination was in concordance with A 17-year-old girl who was previously di- the PTA. A CT scan of the temporal bones agnosed as having RS was http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA Otolaryngology - Head & Neck Surgery American Medical Association

Bilateral Conductive Hearing Impairment With Hyperostosis of the Temporal Bone

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Publisher
American Medical Association
Copyright
Copyright 2012 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.
ISSN
2168-6181
eISSN
2168-619X
DOI
10.1001/archoto.2011.1459
pmid
22431878
Publisher site
See Article on Publisher Site

Abstract

CLINICAL NOTE Bilateral Conductive Hearing Impairment With Hyperostosis of the Temporal Bone A New Finding in Robinow Syndrome Dion F. Eijkenboom, MD; Berit M. Verbist, MD, PhD; Cor W. R. J. Cremers, MD, PhD; Henricus P. M. Kunst, MD, PhD obinow syndrome (RS) is characterized by hypertelorism, wide palpebral fissures with a short upturned nose, shortening of forearms and forelegs, and genital hypoplasia. The syndrome is classified into dominant (DRS) and recessive (RRS) types, each with distin- R guishing clinical findings. We evaluated a patient who was diagnosed as having RS with persistent hearing loss. Computed tomographic (CT) images showed ossicular chain changes and thickening of the skull and petrous bones, with appositional bone growth, causing a narrowing of the middle ear cavity and oval and round window niches. These findings were confirmed during an ex- ploratory tympanotomy. To our knowledge, this is the first report of conductive hearing loss in RS. frequencies 0.5 to 4.0 kHz. The PTA of the REPORT OF A CASE right ear was 25 dB (Figure 1A). Speech discrimination was in concordance with A 17-year-old girl who was previously di- the PTA. A CT scan of the temporal bones agnosed as having RS was

Journal

JAMA Otolaryngology - Head & Neck SurgeryAmerican Medical Association

Published: Mar 1, 2012

References