Paget’s disease and cochlear implantation

2004 ◽  
Vol 118 (10) ◽  
pp. 810-813 ◽  
Author(s):  
A. Bacciu ◽  
E. Pasanisi ◽  
V. Vincenti ◽  
F. Ingegnoli ◽  
M. Guida ◽  
...  
Keyword(s):  
Cochlear Implantation ◽  
Signal To Noise Ratio ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Postoperative Evaluation ◽  
Sentence Recognition ◽  
Open Set ◽  
Profound Deafness ◽  
History Of ◽  
Nucleus 24

Paget’s disease of bone is a common disorder of unresolved etiology characterized by excessive bone resorption followed by excessive bone formation. If the skull isaffected this may result in hearing loss and eventually develop into profound deafness. To date, no cases of cochlear implantation in patients with Paget’s disease have been reported.The authors present a case of radiographically confirmed Paget’s disease of the skull in a 77-year-old man with a 20-year history of progressive bilateral sensorineural hearing losswho underwent cochlear implantation. A successful insertion of the Nucleus 24 Contour electrodearray was achieved without surgical and postoperative complications. At the 10 months’ postoperative evaluation, the patient had gained useful open-set speech perception. In quiet conditions, his performance scores on the word and sentence recognition tests were 100 and 98 per cent, respectively. In the presence of noise (at +10 dB. signal-to-noise ratio), his performance scores on the word and sentence recognition tests were 96 and 94per cent, respectively.

Implanting outside the Guidelines: A Case Study

2008 ◽  
Vol 19 (03) ◽  
pp. 197-203
Author(s):  
Millicent K. Seymour ◽  
Larry Lundy
Keyword(s):  
Cochlear Implantation ◽  
Signal To Noise Ratio ◽  
Percent Correct ◽  
Sentence Recognition ◽  
Speech In Noise ◽  
The Past ◽  
Open Set ◽  
Hearing Aid Use ◽  
Communication Difficulties

An 81-year-old female was referred for cochlear implantation due to difficulty communicating in her daily activities despite the use of appropriate amplification. The poorer ear was unable to tolerate amplification for the past 15 years. The open-set sentence-recognition test score in quiet in her "good" ear was 85 percent correct, indicating that the patient was not a traditional cochlear implant candidate. However, the sentence-recognition score in noise at +10 dB signal-to-noise ratio was 0 percent, demonstrating a significant breakdown in the patient's speech understanding in more difficult listening situations. This speech-in-noise score appeared to correlate with the patient's reported communication difficulties as well as with the communicative breakdowns that were observed clinically. The patient underwent cochlear implantation in the better ear. Cochlear implantation in this nontraditional patient provided objective and subjective benefit over hearing aid use. Una mujer de 81 años de edad fue referida para implante coclear debido a sus dificultades para comunicarse en sus actividades diarias, a pesar del uso de amplificación apropiada. El oído peor había sido incapaz de tolerar la amplificación durante los últimos 15 años. El puntaje de la prueba de reconocimiento de palabras de lista abierta en silencio en su oído "bueno" fue de un 85%, indicando que le paciente no era una candidata tradicional para un implante coclear. Sin embargo, los puntajes de reconocimiento de frases en ruido, a una tasa señal/ruido de ±10 dB fueron de 0%, demostrando un colapso en la comprensión del lenguaje por parte de la paciente en situaciones auditivas más difíciles. Este puntaje de lenguaje en ruido pareció correlacionar con las dificultades reportadas por la paciente, así como con el colapso comunicativo que se observó clínicamente. La paciente fue sometida a una implantación coclear en el mejor oído. El implante coclear en esta paciente no tradicional aportó un beneficio objetivo y subjetivo sobre el uso de auxiliares auditivos.

Bilateral cochlear implantation in a patient with petrous bone cholesteatoma in the only hearing ear: case report

2011 ◽  
Vol 125 (12) ◽  
pp. 1272-1274 ◽  
Author(s):  
S T Husseini ◽  
M Guida ◽  
M Negri ◽  
M Falcioni
Keyword(s):  
Case Report ◽  
Cochlear Implantation ◽  
English Language ◽  
Internal Auditory Canal ◽  
Petrous Bone ◽  
Successful Outcome ◽  
Sentence Recognition ◽  
Open Set ◽  
History Of ◽  
Petrous Bone Cholesteatoma

AbstractObjective:We report a case of successful cochlear implantation in a patient with petrous bone cholesteatoma in the only hearing ear.Case report:A 63-year-old man presented with a four-year history of right-sided, progressive hearing loss in his only hearing ear. Computed tomography and magnetic resonance imaging revealed a right supralabyrinthine petrous bone cholesteatoma, with erosion of the superior semicircular canal and the roof of the internal auditory canal. Due to the high risk of post-operative right-sided deafness, we decided first to perform left cochlear implantation. Five months later, the patient had a 40 per cent score for open-set two-syllable word recognition and an 85 per cent score for sentence recognition. Given these good performances, we decided to eradicate the cholesteatoma via a translabyrinthine approach, with insertion of a second cochlear implant, as a single-stage procedure. A successful outcome was achieved.Conclusion:Cochlear implantation can be an effective method of hearing rehabilitation in patients with petrous bone cholesteatoma, following total eradication of disease, if the cochlea remains intact. To our best knowledge, this is the first English language report of cochlear implantation in a patient with petrous bone cholesteatoma.

scholarly journals A rare case of Paget’s disease affecting the cervical spine

2019 ◽  
Vol 101 (2) ◽  
pp. e38-e42
Author(s):  
J Holton ◽  
M Jones ◽  
Z Klezl ◽  
M Czyz ◽  
M Grainger ◽  
...  
Keyword(s):  
Cervical Spine ◽  
Independent Living ◽  
Paget’S Disease ◽  
Posterior Part ◽  
Cord Compression ◽  
Bone Cell ◽  
Paget's Disease ◽  
History Of ◽  
Posterior Instrumented Fusion ◽  
Degenerative Cervical Spine

We present the case of a 75-year-old man with a rapidly progressive cervical myelopathy on a background of a 3-year history of neck pain and a severely degenerative cervical spine. The patient developed progressive myelopathy over a six-month period and suffered from worsening kyphosis. Suspicion of an underlying oncological process prompted transfer to our tertiary referral unit. Biopsy was consistent for Paget’s disease, an extremely rare diagnosis of the cervical spine. Magnetic resonance imaging revealed cord compression between C4 and C6 with associated cord signal change indicative of myelopathy. A three-level corpectomy and posterior instrumented fusion was performed. There was significant blood loss (3.5l) intraoperatively, consistent with a diagnosis of Paget’s disease of the bone. Cell salvage was used, as was neuromonitoring for both the anterior and posterior part of the procedure. Postoperatively, neurological function improved slightly and the patient required community neurorehabilitation to allow independent living.

Cochlear implantation in a patient with paget's disease

2011 ◽  
Vol 121 (2) ◽  
pp. 358-360 ◽  
Author(s):  
Amit Wolfovitz ◽  
Rabia Shihada ◽  
Talma Shpak ◽  
Jacob Braun ◽  
Michal Luntz
Keyword(s):  
Cochlear Implantation ◽  
Paget’S Disease ◽  
Paget's Disease

scholarly journals Extramammary Paget's Disease: A Case Report

2020 ◽  
Vol 8 (1) ◽  
pp. 83-85
Author(s):  
Anita Shah ◽  
Yogesh Poudel ◽  
Anuj Poudel
Keyword(s):  
Paget’S Disease ◽  
Paget's Disease ◽  
Extramammary Paget’S Disease ◽  
Erythematous Plaque ◽  
Eosinophilic Cytoplasm ◽  
Topical Glucocorticoids ◽  
Atypical Cells ◽  
History Of ◽  
White Scale ◽  
Extramammary Paget's Disease

A 60- year-old man presented with four year history of a non- healing rash. The rash was pruritic and painless. He had previously been treated empirically with oral and topical antibiotics, topical antifungal agents, and topical glucocorticoids without improvement. The physical examination revealed an erythematous plaque with superficial white scale affecting the skin of the groin and scrotum. Skin biopsy and histopathology revealed epidermal infiltration by large atypical cells that had eosinophilic cytoplasm and prominent nucleoli, findings were consistent with Pagets disease. Extramammary Paget's disease (EMPD) is an intraepithelial adenocarcinoma that is sometimes misdiagnosed as dermatitis clinically. It most commonly involves the vulva and in perianal skin, scrotum, penis and axilla. The patient was kept on regular surveillance. The patient has increased intensity of itching and increase in size of lesion after six months of diagnosis.

scholarly journals Molecular insights into an ancient form of Paget’s disease of bone

2019 ◽  
Vol 116 (21) ◽  
pp. 10463-10472 ◽  
Author(s):  
Barry Shaw ◽  
Carla L. Burrell ◽  
Darrell Green ◽  
Ana Navarro-Martinez ◽  
Daniel Scott ◽  
...  
Keyword(s):  
Direct Sequencing ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Paget’S Disease Of Bone ◽  
Paget's Disease Of Bone ◽  
North West ◽  
Sequestosome 1 ◽  
The North ◽  
History Of ◽  
Skeletal Samples

Paget’s disease of bone (PDB) is a chronic skeletal disorder that can affect one or several bones in individuals older than 55 y of age. PDB-like changes have been reported in archaeological remains as old as Roman, although accurate diagnosis and natural history of the disease is lacking. Six skeletons from a collection of 130 excavated at Norton Priory in the North West of England, which dates to medieval times, show atypical and extensive pathological changes resembling contemporary PDB affecting as many as 75% of individual skeletons. Disease prevalence in the remaining collection is high, at least 16% of adults, with age at death estimations as low as 35 y. Despite these atypical features, paleoproteomic analysis identified sequestosome 1 (SQSTM1) or p62, a protein central to the pathological milieu of PDB, as one of the few noncollagenous human sequences preserved in skeletal samples. Targeted proteomic analysis detected >60% of the ancient p62 primary sequence, with Western blotting indicating p62 abnormalities, including in dentition. Direct sequencing of ancient DNA excluded contemporary PDB-associated SQSTM1 mutations. Our observations indicate that the ancient p62 protein is likely modified within its C-terminal ubiquitin-associated domain. Ancient miRNAs were remarkably preserved in an osteosarcoma from a skeleton with extensive disease, with miR-16 expression consistent with that reported in contemporary PDB-associated bone tumors. Our work displays the use of proteomics to inform diagnosis of ancient diseases such as atypical PDB, which has unusual features presumably potentiated by yet-unidentified environmental or genetic factors.

Cochlear implantation in a patient with Paget’s disease

2014 ◽  
Vol 35 (3) ◽  
pp. 408-410 ◽  
Author(s):  
Kenichi Takano ◽  
Etsuko Saikawa ◽  
Noriko Ogasawara ◽  
Tetsuo Himi
Keyword(s):  
Cochlear Implantation ◽  
Paget’S Disease ◽  
Paget's Disease

scholarly journals Statin Use and Its Effects on Skeletal Related Events in Patients with Multiple Myeloma

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5774-5774 ◽  
Author(s):  
Fernando M Vargas Madueno ◽  
Amin Benyounes ◽  
Gentry King ◽  
Kuan-Hsiang Gary Huang ◽  
Gabor Varadi
Keyword(s):  
Multiple Myeloma ◽  
Vitamin D ◽  
Alcohol Abuse ◽  
Statin Therapy ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Exact Test ◽  
History Of ◽  
Skeletal Related Events ◽  
Statin Use

Abstract Introduction: Skeletal related events (SRE) in multiple myeloma (MM) patients are associated with significant morbidity and mortality. Studies assessing the anti myeloma effect of statins in conjunction with chemotherapy have shown conflicting results with regards to overall survival or disease response. To our knowledge there have been no studies evaluating the effect of statins on SRE. Here we sought to assess the relationship between statin use and the presence of SRE. Methods: We retrospectively reviewed 101 patients seen at our institution between the years 2007-2012 who had a diagnosis of MM separating them in 2 groups, patients on statin therapy (n=50) and those without statin therapy (n=51). Statin use was considered if present prior to the occurrence of a SRE. SRE were defined as pathologic fractures, necessity for orthopedic intervention, radiation therapy or spinal cord compression. MM stage as per the International Staging System (ISS), as well as history of osteoporosis, malignancy, hypothyroidism, Paget's disease of the bone, alcohol abuse, smoking status, calcium-vitamin D, and bisphosphonate use were also recorded. Results: In our cohorts the prevalence of SRE was significantly lower in patients on statin medication when compared to statin naive patients, (36% vs. 58.8% respectively, p = 0.029, Fisher's exact test, Figure 1). No significant differences were noted between statin treated group and statin naive group in the following variable subgroups (Fisher's exact test): the history of smoking or alcohol abuse, the documented diagnosis of osteoporosis, coexistent malignancy, hypothyroidism, or Paget's disease of the bone, and the use of other medications including bisphosphonates or calcium - vitamin D supplementation. There were no significant differences in distribution of cancer staging (according to ISS) when we compared between statin use status (chi-square test for trend). Conclusion: The use of statins was associated with a decrease in the prevalence of SRE in patients with MM. This was found independent of disease stage, history of osteoporosis or second malignancy, and bisphosphonate use. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals Hydrocephalus and Headaches in Paget’s Disease of the Skull: Complete Relief by Ventriculo-Atrial Shunt

1984 ◽  
Vol 11 (1) ◽  
pp. 69-72 ◽  
Author(s):  
Chantal Hausser ◽  
Georges Elie Ouaknine ◽  
Jacques Sylvestre
Keyword(s):  
Paget’S Disease ◽  
Paget's Disease ◽  
Neurological Dysfunction ◽  
Complete Relief ◽  
Communicating Hydrocephalus ◽  
Intellectual Function ◽  
History Of ◽  
Atrial Shunt ◽  
First Time ◽  
Shunting Procedure

SUMMARYA 76 year old patient with a long history of headaches was found to have Paget’s disease and communicating hydrocephalus. There were (otherwise) no neurological or musculo-skeletal manifestations of Paget’s disease, but moderate impairment of intellectual function was present. Treatment with disphosphonates did not bring any significant improvement, but three days following a ventriculo-atrial shunting procedure, the patient became headache-free for the first time in several years.In the literature, patients with hydrocephalus have been shown to respond quite unevenly to atrio-ventricular shunting, but in most instances the descriptions concerned advanced cases with well-established symptoms of dementia, ataxia and incontinence. Our case is reported to stress the importance of early diagnosis and management of hydrocephalus in Paget’s disease for the prevention of widespread neurological dysfunction.

scholarly journals Recent advances in understanding and managing Paget’s disease

F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 1485
Author(s):  
Ian R Reid
Keyword(s):  
Disease Progression ◽  
Paget’S Disease ◽  
Cost Effective ◽  
Paget's Disease ◽  
Symptomatic Disease ◽  
Joint Replacement Surgery ◽  
Replacement Surgery ◽  
Primary Endpoints ◽  
History Of ◽  
Biochemical Indices

Paget’s disease is a condition which continues to challenge and surprise. The dramatic fall in its incidence over the last three decades has been an enormous surprise, as is the capacity of a single infusion of the potent bisphosphonate, zoledronate, to produce biochemical remission in 90% of patients, remissions which usually persist for many years and raise the possibility of a cure in some patients. However, challenges in its management remain. The trials carried out in Paget’s disease have almost always had biochemical indices as their primary endpoints. From these studies, we also know that bone pain is relieved, quality of life improved, bone histology normalised, and radiological lesions healed. Thus, disease progression is halted. Studies have not been powered to assess whether clinically important endpoints such as fracture and the need for joint replacement surgery are diminished, although these complications are well established as part of the natural history of the condition. Since disease progression is prevented by potent bisphosphonates, it is likely that disease complications will also be prevented. Zoledronate also reduces the frequency of follow-up needed and therefore provides a very cost-effective intervention in those who have symptomatic disease or are at risk of complications.

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