Medical management of sensorineural hearing loss PART II: Musculo-skeletal system

Booth, J. B.

doi:10.1017/s0022215100093129

Cited by 11 publications

(3 citation statements)

References 74 publications

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“…Systemic bone disease will often produce other characteristic features in the skull, long bones and spine (Brookes and Booth, 1987). Many of these diseases will also produce characteristic abnormalities of serum levels of calcium, phosphate and alkaline phosphatase (Booth, 1982) which if detected can direct further investigation to screening for temporal bone disease. In the present study, however, all three patients with positive densitometry findings had normal serum levels of these electrolytes and enzymes.…”

Section: Discussionmentioning

confidence: 99%

CT screening for temporal bone abnormalities in idiopathic bilateral sensorineural hearing loss

et al. 1997

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Bilateral sensorineural hearing loss can be caused by a variety of temporal bone abnormalities including primary cochlear otoscierosis, local and systemic bony diseases and some metabolic conditions. These may be identified using computerized tomography (CT), with attenuation recordings taken across the cochlear capsule (CT densitometry). Eighty patients with bilateral sensorineural hearing loss were screened over a period of six and a half years using this technique, and only three cases (3.8 per cent) of treatable disease were detected. Positive yields may be increased by screening selected cases with other clinical or biochemical stigmata of temporal bone disease.

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Section: Discussionmentioning

confidence: 99%

CT screening for temporal bone abnormalities in idiopathic bilateral sensorineural hearing loss

et al. 1997

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“…These disorders include osteitis deformans (Paget's disease), osteogenesis imperfecta (Van der Hoeve syndrome), osteitis fibrosa cystica, osteoporosis, osteopetrosis and acromegaly (Morrison, 1979). In addition, several rare genetic conditions are associated with craniotabular hyperosteosis and secondary hearing loss (Booth, 1982). Brookes and Morrison (1981) recently reported two cases in which Vitamin D deficiency was associated with bilateral cochlear deafness.…”

Section: Introductionmentioning

confidence: 99%

Vitamin D deficiency—a new cause of cochlear deafness

Brookes¹

1983

J. Laryngol. Otol.

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“…Hearing loss and visual impairment have also been reported. 11,12 Other malformations include syndactyly, radial deviation of the terminal phalanges Figure 1. Sclerostin: From genes to antibodies.…”

Section: Sclerosteosismentioning

confidence: 99%

The sclerostin story: From human genetics to the development of novel anabolic treatment for osteoporosis

Yavropoulou

Xygonakis

Lolou

et al. 2014

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Sclerosteosis and Van Buchem disease are two rare bone sclerosing disorders characterized by increased bone mineral density, tall stature and entrapment of cranial nerves due to overgrowth of a highly dense bone. Recent advances in human genetics have revealed the genetic background of these disorders by cloning the SOST gene, which is localized on chromosome region 17q12-q21 and codes for sclerostin. Sclerostin is a protein produced almost exclusively from osteocytes inhibiting bone formation by both osteoblasts and osteocytes. At the molecular level, sclerostin inhibits the Wnt signaling pathway, which plays a critical role in osteoblast development and function. Induced sclerostin deficiency in mice reproduces the bone sclerosing human diseases, while sclerostin excess leads to bone loss and reduced bone strength. The extracellular nature of sclerostin has rendered it a promising target for the development of novel anti-osteoporotic treatment. Otherwise healthy carriers of the SOST mutation present with increased bone mass and low levels of sclerostin in serum in contrast to patients with sclerosteosis, who exhibit undetectable levels, thus pointing to the possibility of titration of sclerostin levels in the circulation. Based on these unique characteristics, human anti-sclerostin antibodies have been developed and tested in ovariectomized rats and monkeys, demonstrating very promising results in bone formation. Clinical phase II and III trials are currently underway thereby translating human genetics to drug development.

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Medical management of sensorineural hearing loss PART II: Musculo-skeletal system

Cited by 11 publications

References 74 publications

CT screening for temporal bone abnormalities in idiopathic bilateral sensorineural hearing loss

CT screening for temporal bone abnormalities in idiopathic bilateral sensorineural hearing loss

Vitamin D deficiency—a new cause of cochlear deafness

The sclerostin story: From human genetics to the development of novel anabolic treatment for osteoporosis

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