1

Introduction

Paget disease is a chronic osteoclastic disorder of unknown etiology that occurs most frequently in the spine, pelvis, and skull. Excessive bone resorption and consequent secondary bone formation are the main pathology associated with this condition. Paget disease is common in Europe and North America, but relatively uncommon in Asia. There is only one case report of Paget disease of temporal bone in Korea . Seventy percent of Paget patients have skull lesions, and half of them have hearing impairment . The type of hearing loss includes conductive, sensorineural, or mixed varieties; however, total deafness is not common. There is only one prior case report of cochlear implantation in a patient with Paget disease . Here, another case of cochlear implantation in a patient with Paget disease is reported.

2

Case

A 60-year-old woman with Paget disease was referred to the ENT clinic for progressive bilateral hearing impairment that was aggravated for the past 2 years. The patient used a hearing aid in the left ear for 2 1/2 years, but it was of no benefit for the past 10 months.

The patient was diagnosed with Paget disease of the vertebrae 6 years before the onset of hearing loss. The patient reported severe bilateral tinnitus with hearing loss. The pure tone audiogram showed total deafness in both ears, and there was no response to auditory brainstem stimulation at 100 dB. A speech perception test was not possible.

After the diagnosis of total deafness due to progressive Paget disease, cochlear implantation was planned. Temporal bone computed tomography and magnetic resonance imaging showed normal cochlear patency in both cochleae. According to the PTA results and history of hearing aids, the left side was selected for the implant. A nucleus 24 contour advance (CA) cochlear implant electrode was inserted successfully ( Figs. 1 and 2 ).

Axial computed tomography image. There is diffuse glass ground opacity in the skull base and calvarium. These pathologies are not seen in the otic capsule.

Stenver’s view after implantation.

The speech perception tests 9 months postoperatively were much improved: 47 of 50 for the environmental test, 20 of 25 for 3 syllables, 20 of 25 for 2 syllables, and 7 of 25 for 1 syllable in the closed-set discrimination tests. The patient scored 24 of 30 in the closed-set sentence identification test, 23 of 50 in the closed sentence identification test (K-CID), 10 of 20 for 3 syllables, 13 of 20 for 2 syllables, 14 of 20 for 1 syllable in the open-set cognition test, and 5 of 10 in the open-set comprehension test (GASP).

Tests 4 years postoperatively were 50 of 50 for the environmental test, 25 of 25 for 3 syllables, 24 of 25 for 2 syllables, and 21 of 25 for 1 syllable in the closed-set discrimination test. In addition, the patient scored 28 of 30 in the closed-set sentence identification test, 47 of 50 in the closed sentence identification test, 18 of 20 for 3 syllables, 19 of 20 for 2 syllables, 18 of 20 for 1 syllable in the open-set cognition test, and 10 of 10 in the open-set comprehension test. This report was approved by the institutional review board of the Pusan National University Hospital, Busan, Korea.