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The otologic manifestations of mandibulofacial dysostosis.
Trans Sect Otolaryngol Am Acad Ophthalmol Otolaryngol. 1977 May-Jun; 84(3 Pt 2):ORL520-8.TS

Abstract

Sixteen patients (32 ears) with diagnoses of mandibulofacial dysostosis were reviewed. The characteristic otologic manifestations of the syndrome were delineated, found to be bilateral, and consist of the following: 1. Mild symmetric deformity of the auricle (grade 1 microtia). 2. Agenesis or hypoplastic development of the mastoid and mastoid antrum. 3. Absence of the external auditory canal. 4. Marked narrowing or agenesis of the middle ear cleft. 5. Agenesis or severe malformation of the malleus and incus. When present, the malleus and incus are most often rudimentary, fused to form a conglomerate mass, and ankylosed to either the atretic plate, epitympanum, or both. 6. Stapedial malformations which usually consist of a deformed suprastructure. 7. Frequently the tegmen assumes a more inferior (low lying) position than normal. 8. Occasionally, the facial nerve pursues an abnormal course and is located more anteriorly than would be expected. 9. A normal inner ear. 10. Normal bone conduction with a marked (greater than 50 dB) conductive hearing loss. 11. Marked disparity between the degree of auricular deformity (mild; grade 1 microtia) and the degree of deformity of the remaining first and second branchial arch derivatives that constitute the external and middle ears (severe).

Authors

No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, U.S. Gov't, Non-P.H.S.
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

888269

Citation

Hutchinson, J C., et al. "The Otologic Manifestations of Mandibulofacial Dysostosis." Transactions. Section On Otolaryngology. American Academy of Ophthalmology and Otolaryngology, vol. 84, no. 3 Pt 2, 1977, pp. ORL520-8.
Hutchinson JC, Caldarelli DD, Valvassori GE, et al. The otologic manifestations of mandibulofacial dysostosis. Trans Sect Otolaryngol Am Acad Ophthalmol Otolaryngol. 1977;84(3 Pt 2):ORL520-8.
Hutchinson, J. C., Caldarelli, D. D., Valvassori, G. E., Pruzansky, S., & Parris, P. J. (1977). The otologic manifestations of mandibulofacial dysostosis. Transactions. Section On Otolaryngology. American Academy of Ophthalmology and Otolaryngology, 84(3 Pt 2), ORL520-8.
Hutchinson JC, et al. The Otologic Manifestations of Mandibulofacial Dysostosis. Trans Sect Otolaryngol Am Acad Ophthalmol Otolaryngol. 1977 May-Jun;84(3 Pt 2):ORL520-8. PubMed PMID: 888269.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The otologic manifestations of mandibulofacial dysostosis. AU - Hutchinson,J C,Jr AU - Caldarelli,D D, AU - Valvassori,G E, AU - Pruzansky,S, AU - Parris,P J, PY - 1977/5/1/pubmed PY - 2001/3/28/medline PY - 1977/5/1/entrez SP - ORL520 EP - 8 JF - Transactions. Section on Otolaryngology. American Academy of Ophthalmology and Otolaryngology JO - Trans Sect Otolaryngol Am Acad Ophthalmol Otolaryngol VL - 84 IS - 3 Pt 2 N2 - Sixteen patients (32 ears) with diagnoses of mandibulofacial dysostosis were reviewed. The characteristic otologic manifestations of the syndrome were delineated, found to be bilateral, and consist of the following: 1. Mild symmetric deformity of the auricle (grade 1 microtia). 2. Agenesis or hypoplastic development of the mastoid and mastoid antrum. 3. Absence of the external auditory canal. 4. Marked narrowing or agenesis of the middle ear cleft. 5. Agenesis or severe malformation of the malleus and incus. When present, the malleus and incus are most often rudimentary, fused to form a conglomerate mass, and ankylosed to either the atretic plate, epitympanum, or both. 6. Stapedial malformations which usually consist of a deformed suprastructure. 7. Frequently the tegmen assumes a more inferior (low lying) position than normal. 8. Occasionally, the facial nerve pursues an abnormal course and is located more anteriorly than would be expected. 9. A normal inner ear. 10. Normal bone conduction with a marked (greater than 50 dB) conductive hearing loss. 11. Marked disparity between the degree of auricular deformity (mild; grade 1 microtia) and the degree of deformity of the remaining first and second branchial arch derivatives that constitute the external and middle ears (severe). SN - 0161-696X UR - https://www.unboundmedicine.com/medline/citation/888269/The_otologic_manifestations_of_mandibulofacial_dysostosis_ DB - PRIME DP - Unbound Medicine ER -