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Craniometaphyseal Dysplasia-Induced Hearing Loss

A 25-year-old African American female presented with a chief complaint of bilateral hearing loss; she had used bilateral hearing aids for many years before presentation but was not satisfied as her hearing loss progressed. She had previously been diagnosed with craniometaphyseal dysplasia (CMD). She demonstrated frontonasal bossing, hypertelorism, and a left-sided facial nerve paresis that she stated was present since birth. Rinne tuning fork examination at 512 Hz was negative bilaterally; Weber examination lateralized to the right side. Preoperative audiogram revealed bilateral mixed hearing loss (Fig. 1A). Noncontrast computed tomography (CT) of the temporal bones revealed diffuse thickening of the calvarium (Fig. 2A). The external auditory canals were normal in size, whereas the mastoids were obliterated by heavily sclerotic bone bilaterally. Also noted was dense sclerosis of the otic capsules with narrowing of the semicircular canals, although the vestibule and cochlea were grossly normal in appearance. Figures 2B and C demonstrate portions of the ossicular chain. The patient underwent right endaural exploratory tympanotomy with canalplasty and ossiculoplasty. Because both the malleus and incus were fixed and could not be freed with good mobility, the incus was removed, and a titanium partial ossicular replacement prosthesis with hydroxyapatite head was placed. Results of a 2-month postoperative audiogram are shown in Figure 1B.