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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
Arch Otolaryngol Head Neck Surg. 2012;138(3):309-312. doi:10.1001/archoto.2011.1459.
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Robinow 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 distinguishing 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 exploratory tympanotomy. To our knowledge, this is the first report of conductive hearing loss in RS.

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Figure 1. Audiograms before (A) and 1 month after (B) surgery. A, Audiograms 1 month before surgery show a more severe hearing impairment of the left ear, with a marked low-tone hearing impairment. B, Audiograms 1 month after surgery show improvement of the hearing impairment of the left ear (pure-tone averages [PTA] over frequencies 0.5-4.0 kHz).

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Figure 2. Computed tomographic (CT) images show generalized hyperostosis of the skull and temporal bones, without pneumatization of the mastoid. The ossicular chain is involved as well. A, An axial CT image of the right temporal bone shows a misshapen stapes (arrow). B, An axial image of the left temporal bone at the same level shows a normally shaped but markedly thickened stapes (arrow). ET indicates eustachian tube. C, An axial slice through the epitympanum illustrates the narrowing of the internal auditory canal (IAC) and the middle ear cavity due to bone growth. The ET is widened, pointing at dysfunction. D and E, Coronal images reveal stenosis of the oval window on the right (D) and narrowing of the oval window (OW) due to bone apposition and displacement of the facial nerve (n VII) on the left (E). Note also the exostosis in the external auditory canal. F, Broadening of the malleal handle (white arrow) and fusion of the incus to the epitympanic roof (black arrow) are evident on a coronal image.

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Figure 3. Lateral (A) and frontal (B) photographs of our patient at the age of 17 years. Prominent midfacial hypoplasia, hypertelorism, and low implanted auricles with underdeveloped superior helixes are present. Note that the patient underwent cosmetic rhinoplasty at the age of 14 years.




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