The Mechanism of Hearing Loss in Paget's Disease of Bone

Monsell, Edwin M.

doi:10.1097/00005537-200404000-00002

Cited by 100 publications

(57 citation statements)

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“…Histopathologic temporal bone studies failed to detect abnormal changes within the middle ear associated with Paget's disease [4,8] . Further examination of temporal bones with Paget's disease revealed multiple microfractures within the otic capsule [9] .…”

Section: Discussionmentioning

confidence: 98%

“…Continuous remodeling of the pagetic otic capsule results predominantly in progressive sensorineural hearing loss. Degeneration of the spiral ligament is often found in patients with otosclerosis, but not in Paget's disease [4] . Abnormalities of the metabolic homeostasis of the cochlea as a result of the liberation of cytokines may also cause inner ear dysfunction in otosclerosis and Paget's disease [7] .…”

Section: Discussionmentioning

confidence: 99%

“…Monsell et al [4] demonstrated that a loss of bone mineral density in the cochlear optic capsule in the CT is associated with both high-tone hearing loss and a low-tone air-bone gap in Paget's disease. The otic capsule is composed of three layers: the endosteal layer, the endochondral layer, and the periosteal layer.…”

Section: Discussionmentioning

confidence: 99%

“…The air-bone gap may be associated with the acoustical mechanics of hearing by the enhanced conduction of low-frequency sound energy by pagetic bone. The diffuse lesions or microfractures act as a third window which may permit the dissipation of acoustic energy into the cranial cavity as an amplifier for bone-conducted sounds [4,10] .…”

Section: Discussionmentioning

confidence: 99%

“…Pure tone audiometry often presents an air-bone gap, which has been assumed to represent conductive hearing. However, surgery has been found to be less efficient in cases of Paget's disease [4] . We present a case of Paget's disease with profound hearing loss treated by cochlear implant surgery.…”

Section: Jamesmentioning

confidence: 99%

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

Aoki

Tanahashi²,

Mizuta³

et al. 2016

Int Adv Otol

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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 ...

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Jamesmentioning

confidence: 99%

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