Osteopathia Striata, Osteopetrosis, and Impaired Hearing: A Case Report

Bilateral cochlear implantation in a patient with osteopathia striata voorhoeve and cranial sclerosis

“…Bilateral sensorineural hearing loss of 40 dB or more has been found in about 1 to 2 in every 1000 newborns in developed countries [8]. Hearing loss is found to be usually conductive rather than mixed or sensorineural in patients with OS-CS sclerosis [9][10][11][12][13][14]. Mixed or sensorineural hearing loss [15].…”

When bone becomes marble: Head and neck manifestations of osteopetrosis

“…The hypothesis that hearing loss is primarily due to internal auditory canal stenosis secondary to bony overgrowth does not always hold true (13). The conductive hearing loss has been attributed to exostosis projecting into the middle ear cavity, calcified and sclerotic ossicles, and recurrent otitis media (14)(15)(16)(17). Ear infections can also be due to compromised mucociliary clearance and thickening mucosa lining the eustachian tube, with the presence of microscopic mucus inclusion cycts as decribed by Blum and Hines ( 13) and Hawke et al (17).…”

Osteopathia striata with cranial sclerosis: Variable expressivity in a four generation pedigree

“…Most cases presented with conductive hearing loss (78.6%). Surgical attempts to mobilize the middleear ossicles were unsuccessful because of sclerotic bone thickening and overgrowth from all boundaries of the tympanic cavity [Jones and Mulcahy, 1968;Gay et al, 19941.…”

“…*Bass et al, 1980;Bloor, 1954;de Boer and van Gool, 1974;Clement et al, 1982;Clementi et al, 1993;Cortina et al, 1981;Currarino and Friedmann, 1986;Culver and Thumasathit, 1972;Franklyn and Wilkinson, 1978;Gay et al, 1994;Goodmann and Robertson, 1993;Hoeffel and Merle, 1990;Horan and Beighton, 1978;Hurt, 1953;Jones and Mulcahy, 1968;de Keyser et al, 1983;Kornreich et al, 1988;Nakamura et al, 1985;Odrezin and Krasikov, 1993;Paling et al, 1981;Piechowiak et al, 1986;Robinow and Unger, 1984;Sevaux and Galmiche, 1970;Schnyder, 1980;Taybi and Nurock, 1969;Walker, 1969;Winter et al, 1980; own cases 11-2, 111-1,111-2, IV-1, and IV-2.…”