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Klippel-Feil Syndrome and Deafness: A Study With Polytomography

Klippel-Feil Syndrome and Deafness: A Study With Polytomography Abstract Klippel-Feil syndrome, the triad of limitation of movement of the head, short neck, and low posterior hairline, is often associated with other congenital anomalies. Deafness is the second most common associated abnormality and has been estimated to occur in up to 30% of patients with Klippel-Feil syndrome. Histological and roentgenographic studies suggest this deafness to be secondary to abnormal development of the inner ear. We report a case of Klippel-Feil syndrome and deafness and illustrate the associated anatomical defects with polytomography, a relatively new radiological technique which is becoming of increased value in the evaluation of congenital and acquired deafness. The importance of early evaluation for deafness is stressed when conditions known to be associated with deafness are diagnosed. References 1. McLay K, Maran AGD: Deafness and the Klippel-Feil syndrome . J Laryng 83:175-184, 1969.Crossref 2. Gunderson CH, Greenspan RH, Glaser GH, et al: The Klippel-Feil syndrome: Genetic and clinical reevaluation of cervical fusion . Medicine 46:491-512, 1967.Crossref 3. Ulloa MG, Gonda TF: Klippel-Feil syndrome: Review of the literature and report of a case . Plast Reconstr Surg 4:109, 1949.Crossref 4. Klippel M, Feil A: Un cas d'absence des vertebres cervicales . Nouv Iconogr Salpet 25:223, 1912. 5. Jaffee IS: Congenital shoulder-neck-auditory anomalies . Laryngoscope 78:2119-2139, 1968.Crossref 6. Morrison SG, Perry LW, Scott LP III: Congenital brevicollis (Klippel-Feil syndrome) and cardiovascular anomalies . Amer J Dis Child 115:614-620, 1968. 7. Rubin A: Handbook of Congenital Malformations . Philadelphia, WB Saunders Co, 1967. 8. McKusick VA: Mendelian Inheritance in Man , ed 2. Baltimore, Johns Hopkins Press, 1968. 9. Gorlin RJ, Pindborg JJ: Syndromes of the Head and Neck . New York, McGraw-Hill Book Co Inc, 1964. 10. Sommerfeld RM, Schweiger JW: Cleft palate associated with Klippel-Feil syndrome . Oral Surg 27:737-739, 1969.Crossref 11. Haddad HM, Wilkins L: Congenital anomalies associated with gonadal dysplasia . Pediatrics 23:885-902, 1959. 12. Hugh-Jones K, Wallace J, Thornber JM, et al: Gonadal dysgenesis with unusual abnormalities . Arch Dis Child 40:274, 1965.Crossref 13. Stratton HJM: Gonadal dysgenesis and the ears . J Laryng 79:343-346, 1965.Crossref 14. Wildervanck LS, Hoeksema PE, Penning L: Radiological examination of the inner ear of deaf mutes presenting the cervico-oculo-acusticus syndrome . Acta Otolaryng 61:445-453, 1966.Crossref 15. Jalladeau J: Malformations Congenitales Associees au Syndrome de Klippel-Feil, thesis. Paris, 1936. 16. Wildervanck LS: Klippel-Feil syndrome associated with abducens paralysis, bulbar retraction and deaf-mutism. Case . Nederl T Geneesk 96:2752-2756, 1952. 17. Everberg G, Ratjen E, Sorensen H: Wildervanck's syndrome: Klippel-Feil's syndrome associated with deafness and retraction of the eyeball . Brit J Radiol 36:562-567, 1963.Crossref 18. Jensen J: Malformations of the inner ear in deaf children: A tomographic and clinical study . Acta Radiol , (suppl 286) , pp 1-97, 1969. 19. Franceschetti A, Klein D: Dysmorphie cervicooculo-faciale avec surdité familiale . J Genet Hum 3:176, 1954. 20. Siliquini PL, Cacaci F: A case of Klippel-Feil syndrome with associated abnormality of the middle ear . Minerva Ortop 18:734-737, 1967. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Klippel-Feil Syndrome and Deafness: A Study With Polytomography

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Publisher
American Medical Association
Copyright
Copyright © 1972 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1972.02110090088009
Publisher site
See Article on Publisher Site

Abstract

Abstract Klippel-Feil syndrome, the triad of limitation of movement of the head, short neck, and low posterior hairline, is often associated with other congenital anomalies. Deafness is the second most common associated abnormality and has been estimated to occur in up to 30% of patients with Klippel-Feil syndrome. Histological and roentgenographic studies suggest this deafness to be secondary to abnormal development of the inner ear. We report a case of Klippel-Feil syndrome and deafness and illustrate the associated anatomical defects with polytomography, a relatively new radiological technique which is becoming of increased value in the evaluation of congenital and acquired deafness. The importance of early evaluation for deafness is stressed when conditions known to be associated with deafness are diagnosed. References 1. McLay K, Maran AGD: Deafness and the Klippel-Feil syndrome . J Laryng 83:175-184, 1969.Crossref 2. Gunderson CH, Greenspan RH, Glaser GH, et al: The Klippel-Feil syndrome: Genetic and clinical reevaluation of cervical fusion . Medicine 46:491-512, 1967.Crossref 3. Ulloa MG, Gonda TF: Klippel-Feil syndrome: Review of the literature and report of a case . Plast Reconstr Surg 4:109, 1949.Crossref 4. Klippel M, Feil A: Un cas d'absence des vertebres cervicales . Nouv Iconogr Salpet 25:223, 1912. 5. Jaffee IS: Congenital shoulder-neck-auditory anomalies . Laryngoscope 78:2119-2139, 1968.Crossref 6. Morrison SG, Perry LW, Scott LP III: Congenital brevicollis (Klippel-Feil syndrome) and cardiovascular anomalies . Amer J Dis Child 115:614-620, 1968. 7. Rubin A: Handbook of Congenital Malformations . Philadelphia, WB Saunders Co, 1967. 8. McKusick VA: Mendelian Inheritance in Man , ed 2. Baltimore, Johns Hopkins Press, 1968. 9. Gorlin RJ, Pindborg JJ: Syndromes of the Head and Neck . New York, McGraw-Hill Book Co Inc, 1964. 10. Sommerfeld RM, Schweiger JW: Cleft palate associated with Klippel-Feil syndrome . Oral Surg 27:737-739, 1969.Crossref 11. Haddad HM, Wilkins L: Congenital anomalies associated with gonadal dysplasia . Pediatrics 23:885-902, 1959. 12. Hugh-Jones K, Wallace J, Thornber JM, et al: Gonadal dysgenesis with unusual abnormalities . Arch Dis Child 40:274, 1965.Crossref 13. Stratton HJM: Gonadal dysgenesis and the ears . J Laryng 79:343-346, 1965.Crossref 14. Wildervanck LS, Hoeksema PE, Penning L: Radiological examination of the inner ear of deaf mutes presenting the cervico-oculo-acusticus syndrome . Acta Otolaryng 61:445-453, 1966.Crossref 15. Jalladeau J: Malformations Congenitales Associees au Syndrome de Klippel-Feil, thesis. Paris, 1936. 16. Wildervanck LS: Klippel-Feil syndrome associated with abducens paralysis, bulbar retraction and deaf-mutism. Case . Nederl T Geneesk 96:2752-2756, 1952. 17. Everberg G, Ratjen E, Sorensen H: Wildervanck's syndrome: Klippel-Feil's syndrome associated with deafness and retraction of the eyeball . Brit J Radiol 36:562-567, 1963.Crossref 18. Jensen J: Malformations of the inner ear in deaf children: A tomographic and clinical study . Acta Radiol , (suppl 286) , pp 1-97, 1969. 19. Franceschetti A, Klein D: Dysmorphie cervicooculo-faciale avec surdité familiale . J Genet Hum 3:176, 1954. 20. Siliquini PL, Cacaci F: A case of Klippel-Feil syndrome with associated abnormality of the middle ear . Minerva Ortop 18:734-737, 1967.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Mar 1, 1972

References