Treatment for Progressive Hearing Loss Due to Paget’s Disease of Bone - A Case Report and Literature Review

Abstract

A 73-year old man seeking treatment for bilateral severe hearing loss visited our department. When he was 47 years old, he was found to have high levels of serum alkaline phosphatase (ALP) with normal serum calcium levels. However, a needle bone biopsy and a bone scan did not yield an accurate diagnosis. Since then, his hearing became progressively worse and required hearing aids. At his first visit to our department, audiological assessment revealed a bilateral severe hearing loss and an air-bone gap with 45 dB in the low frequency range (250-1000 Hz) with profound sensorineural hearing loss in the high frequency range (Figure 1). Neither the auditory brainstem response nor the stapedial response was found bilaterally. The Japanese monosyllabic open-set word recognition test in a sound field at 65 dB had a result of 20% with the hearing aid. Also, the conductive hearing loss was not ameliorated by a bone conduction hearing aid ( Figure 2, gray triangles).The image findings showed a geographic lucency in the skull bone in the X-ray and an increased uptake in the skull in the bone scan, which is useful in determining the extent and activity of the condition. Computed tomography (CT) findings revealed homogeneously diffused hyperostosis of the skull and a slight loss of bone mineral density in the cochlear capsule (Figure 3). The T2-weighted magnetic resonance imaging (MRI) analysis demonstrated that the eight nerves and the perilymph in the cochlea were visible bilaterally (Figure 4). The image findings suggested the diagnosis of Paget's disease, and the serum ALP level rose to 5309 U/L (normal serum range is from 20 to 141 U/L), with normal serum calcium levels.The creatinine clearance level was less than 40 mL/h, and the concentration of circulating 1,25-dihydroxyvitamin (373 pg/mL), intact parathyroid hormone (iPTH, 61 pg/mL), and % tubular reabsorption of phosphate (69%) were within the normal ranges. A potent bisphosphonate, oral risedronate (Actonel, Eisai Co., Ltd; Tokyo, Japan), was prescribed in daily adjusted dosages for Paget's disease is a common bone remodeling disorder that typically begins with excessive bone resorption in the elderly. Bilateral progressive hearing loss is the most frequently encountered complication of Paget's disease. The types of hearing loss identified by audiometry are conductive, sensorineural, or both. However, the precise mechanism of hearing loss remains unclear, and the treatment has been controversial. We present a 73-year-old man who suffered from bilateral progressive hearing loss due to Paget's disease. Potent bisphosphonates, oral risedronate in daily adjusted dosages for 6 months, did not decrease or suppress the worsening of the hearing loss. The Nucleus CI24 Contour electrode array was successfully inserted on the left side without surgical and postoperative complications. The Japanese open set monosyllable word recognition test in a sound field at 65 dB had a result of 74%. This cochlear implantation can be an indication for cases of profound hearing loss due to ...

Hormone Therapy: Challenges for Treating Hearing Impairments

“…[99] with isolated cases of conductive hearing loss reported for this reason [96]. Regarding calcitonin, several articles have described auditory improvement in patients with Paget's disease [97,98]. More recently, the door has been opened to use this hormone in the treatment of otosclerosis.…”

The defeat of the temporal bone in Paget’s disease as a cause of hearing loss in elderly and senile age

“…Как было сказано ранее, к самому распространенному неврологическому осложнению КБП относится снижение остроты слуха вследствие вовлечения пирамид височных костей [4,6,[22][23][24]. Недавние исследования выявили нарушение метаболического гомеостаза улитки из-за выработки местных цитокинов [24], также важную роль отводят дефициту остеопротегерина, ключевого ингибитора дифференциации и активации остеокластов, что приводит к де-миелинизации и дегенерации нейронов спирального ганглия [23].…”

“…Недавние исследования выявили нарушение метаболического гомеостаза улитки из-за выработки местных цитокинов [24], также важную роль отводят дефициту остеопротегерина, ключевого ингибитора дифференциации и активации остеокластов, что приводит к де-миелинизации и дегенерации нейронов спирального ганглия [23]. Прежние представления о роли в патогенезе тугоухости при КБП таких изменений, как фиксация слуховых косточек и компрессия VIII пары черепного нерва вследствие стеноза внутреннего слухового прохода не подтвердились [4,6,22]. Тугоухость у пациентов с БПК не имеет характерных особенностей и может относиться к кондуктивному, смешанному и сенсоневральному типам.…”

“…Клиническая картина КБП многообразна и во многом зависит от локализации «педжетовских очагов», хотя обычно протекает с минимальными клиническими проявлениями [2]. Поскольку пирамида височной кости и капсула внутреннего уха часто вовлекаются в патологический процесс, одним из самых распространенных неврологических ослож нений деформирующего остеита является тугоухость [4]. Так как снижение остроты слуха после 50 лет может быть единственной жалобой пациента с КБП, врач обычно относит это к возраст-ассоциированной тугоухости -пресбиакузису, в то время как раннее назначение препаратов из группы бифосфонатов пациентам с КБП, осложненной тугоухостью, может остановить прогрессирующее снижение остроты слуха [5][6][7].…”

NFAM1 signaling enhances osteoclast formation and bone resorption activity in Paget's disease of bone

“…Patients with PDB have symptoms including bone pain, fractures, neurological complications due to spinal cord compression, deafness and dental abnormalities [2]. It has been reported that approximately 1% PDB patients have an incidence of osteosarcoma [3,4]. PDB has a variable geographic distribution, with an increased incidence in Caucasians of European origin, but it also occurs in African-Americans and Asian descent.…”