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L. Albarzangi, MRCS; R. Persaud, MRCS; T. Beale, FRCR
Royal National Throat Nose and Ear Hospital, London, England

Arch Otolaryngol Head Neck Surg. 2004;130:1444.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

A 5-year-old boy presented with profound bilateral deafness. At the age of 3 years, he had been diagnosed as having a conductive hearing loss and had undergone a grommet insertion procedure. His hearing and language did not improve despite the use of a hearing aid, and he was assessed for a cochlear implant.

Clinical assessment showed a language and communication delay and severe profound mixed hearing loss in both ears. Genetic assessment revealed a number of genes on the X chromosome in the area of the breakpoint that can cause deafness.

Axial and coronal views of the petrous bone of one of the deaf ears are shown in Figure 1 and Figure 2, respectively. An axial view of a normal petrous bone is shown for comparison in Figure 3.

Figure appears in full text version. Figure 1.

Figure appears in full text version. Figure 2.

Figure appears in full text version. Figure 3.

What is your diagnosis?

SECTION EDITORS: R. NICK BRYAN, MD; PATRICIA A. HUDGINS, MD

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