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age. However, sensorineural hearing loss does occur in
younger individuals with OI and when it does it is char-
acteristically found in patients with type I OI. Patients
with type I OI are more likely to have hearing loss than
patients with type III or IV OI. Conductive hearing loss
in older patients with OI typically involves disruption
of the ossicular chain or fixation of the stapes footplate.
Stapes surgery can be undertaken in patients with OI
but outcomes are less favorable than reported in patients
without OI. Use of bone-anchored and implantable hear-
ing aids have been undertaken in patients with OI to
only a limited extent. Digital hearing aids are beneficial
for many patients with OI. While surgical implantation
of cochlear implants is more challenging in patients with
OI, successful outcomes have been reported.
[13]
Monsell EM. The mechanism of hearing loss in Paget's disease
of bone. Laryngoscope 2004;114(4):598-606.
[14]
Vallejo Valdezate LA, Martin Gil J, Jose-Yacaman M, Martin Gil
FJ, Gil-Carcedo LM, Herrero Laso JL. Otosclerosis and Van der
Hoeven's syndrome: a contribution. Acta Otorrinolaringol Esp
2001;52(2):85-93.
[15]
Heimert TL, Lin DD, Yousem DM. Case 48: osteogenesis imper-
fecta of the temporal bone. Radiology 2002;224(1):166-70.
[16]
Alkadhi H, Rissmann D, Kollias SS. Osteogenesis imperfecta of
the temporal bone: CT and MR imaging in Van der Hoeve-de
Kleyn syndrome. AJNR Am J Neuroradiol 2004;25(6):1106-9.
[17]
Ziyeh S, Berger R, Reisner K. MRI-visible pericochlear lesions in
osteogenesis imperfecta type I. Eur Radiol 2000;10(10):1675-7.
[18]
Hall III JW, Bratt GW, Schwaber MK, Baer JE. Dynamic sensori-
neural hearing loss: implications for audiologists: case reports.
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[19]
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Adv Otorhinolaryngol 2007;65:119-26.
[20]
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