Paget’s disease of the temporal bone can cause hearing loss that eventually develops into profound deafness. However, the treatment options for hearing loss associated with Paget’s disease are limited. Thus far, only 3 cases of cochlear implantation in patients with Paget’s disease have been reported. In this study, we describe a 62-year-old woman who underwent cochlear implantation for progressive bilateral sensorineural hearing loss. The patient gained useful open-set speech perception over a 5-year postoperative period. Cochlear implantation can be beneficial in patients with profound hearing loss associated with Paget’s disease.
Paget’s disease is a localized bone-remodeling disorder, involving one or more bone sites in the systemic skeleton, and little is known about its pathogenesis . Ethnic and geographic clustering of this disease has been known, with observations that the disorder is more common in regions such as Europe and North America, but that it is relatively rare in Asia. The skull is involved in approximately 70% cases, and hearing is affected in approximately 50% of these patients . Hearing loss is generally conductive, sensorineural, or mixed; however, profound sensorineural hearing loss is the last common. To the best of our knowledge, only 3 cases of cochlear implantation have been reported in patients with Paget’s disease . We present a female patient with a 17-year history of Paget’s disease of the skull who developed profound bilateral sensorineural hearing loss and underwent successful cochlear implantation.
A 62-year-old female with a history of Paget’s disease since the age of 45 years presented with severe bilateral tinnitus, hearing loss, and dizziness. She was currently receiving treatment for hypertension, but reported no other medical complaints. Although she used a hearing aid in the left ear, it was not providing any benefit for the past 6 months. Otoscopic examination was unremarkable. However, an audiogram showed bilateral profound sensorineural hearing loss ( Fig. 1 ), with speech discrimination scores not possible on the right and 40% on the left with the use of the hearing aid. Blood chemical analysis revealed that alkaline phosphatase, calcium, and phosphate levels were within the normal range. A computed tomography (CT) of the temporal bone revealed that the basal turn of the right cochlear showed osteoblastic changes and attenuation, and the bilateral semicircular canals showed osteolytic changes and were indistinct ( Fig. 2 ).
On the basis of the audiology results, we selected the right side for cochlear implantation. During the surgery ( Fig. 3 ), marked osteolysis of the otic capsule was observed, and the bones surrounding the semicircular canals were completely resorbed and replaced by a perilymph-like substance. The bone surrounding the cochlea along the facial canal and perilymph appeared transparent. Most of the lateral semicircular canal was destroyed, leaving only a thin rim of bone surrounding the internal cavity. The bone was extremely fragile during removal for histological examination. When creating the cochlear fenestration, the bone close to the round window of the cochlea was observed to be fragile, whereas that near the cochlear fenestration was relatively strong. A Med-EL Combi 40 cochlear implant electrode was successfully inserted. Histologic examination of the temporal bone revealed a typical mosaic pattern with few osteoblasts and osteoclasts around the trabeculae ( Fig. 4 ).