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Two unusual case of congenital conductive hearing loss related to anomaly of stapes

Year 2010, Volume: 32 Issue: 3, 345 - 351, 12.01.2010

Abstract

Abstract

Congenital abnormalities may occur in an isolated form of congenital ossicular or combined with external ear. Isolated ossicular anomalies are rarely seen in clinical practice only, a few reports include series of patients and vast majority of publications are consisted of case reports. Treatment of congenital ossicular anomalies depends on the condition of ossicular and oval window anomalies. In presented article two case reports with severe oval window anomalies and absence of suprastructure of stapes with mobile footplate were published. Management of these cases was discussed with the review of the literature.

Key words: Congenital conductive hearing loss; congenital absence of the oval window; absence of the stapes.

Özet

Konjenital anomaliler izole kemikçik anomalisi halinde veya dış kulakla kombine anomali olarak görülebilir. İzole kemikçik anomalisi klinik pratikte çok ender olarak rastlanır. Bu anomalilerle ilgili literatürün çoğunluğunu vaka takdimleri oluşturmaktadır ve vaka serisi halindeki bildiriler oldukça sınırlıdır. Konjenital ossiküler anomalinin tedavisi kemikçiklerdeki ve oval penceredeki anomalinin varlığına ve biçimine bağlı olarak değişiklik göstermektedir. Bu makalede ileri derecede oval pencere anomalisi ve hareketli tabanla birlikte stapes suprastriktürünün yokluğu ile karakterize iki ender olgunu ve tedavisi literatür taraması ışığında sunulmuştur.

Anahtar sözcükler: Konjenital iletim tipi işitme kaybı; oval pencerenin konjenital yokluğu; stapesin yokluğu.

 

References

  • Swartz JD, glazer AU, Faerber EN, Capitanio MA, Popky GL. Congenital middle ear deafnes: CT study. Radiology 1986; 159: 187-90.
  • Teunissen EB, Cremers WR. Classification of congenital middle ear anomalies. Report on 144 ears. Ann Otol Rhinol Laryngol 1993; 102: 606-12.
  • Nandapalan V, Tos M. Isolated congenital stapes ankylosis: An embryologic survey and literature review. Laryngoscope. 2000; 21: 71-80.
  • Yı Z, Yang J, Li Z, Zhou A, Zhou A, Lin Y. Bilateral congenital absence of stapes and oval window in 2 members of a family : Etiology and management. Otolarygol Head Neck Surg 2003; 128: 777-82.
  • Nakamura S, Sando I. Congenital absence of the oval window (Abs). Arch Otolaryngol 1966;84:131-36.
  • POU JW. Congenital absence of the oval window. Report of two cases.Laryngoscope 1963; 73: 384-91.
  • Zeifer B, Sabini P, Sonne J. Congenital absence of the oval window: Radiologic diagnosis and associated anomalies. Am J Neuroradiology 2000; 21: 322-27.
  • Jahrsdoerfer RA. Congenital absence of the oval window.ORL J Otorhinolarygol Relat Spec 1977; 84: 904-14.
  • Lambert PR. Congenital absence of the oval window. Laryngoscope 1990; 100: 37-40.
  • Teunissen E, Cremers CWRJ. Surgery for congenital anomalies of the middle ear with mobile stapes. Eur Arch Otorhinolaryngol 1993;250:327-31.
  • Cousins VC, Milton CM. Congenital ossicular abnormalities:A review of 68 cases. Am J Otol 1988; 9: 76-80.
  • Isenberg SF, Tubergen TB. An unusual congenital middle ear ossicular anomaly. Arch Otolaryngol 1980; 106: 179-81.
  • Al-Mazrou KA, Alorainy IA, Al-Dousary SH, Richardson MA.Facial nerve anomailes in association with congenital hearing loss. Int J Pediatr Otorhinolarygol 2003; 67: 1347-53.
  • Durcan DJ, Shea JJ, Sleechx JP. Bifurcation of the facial nerve. Arch Otolaryngol 86; 1967: 619-31.
  • Alarcon A, Jahrsdoerfer RA, Kesser BW. Congenital absence of the oval window: Diagnosis, Surgery, and Audiometric Outcomes. Otol Neurotol 2008; 29: 23-28.
  • Jahrsdoerfer RA. Transposition of the facial nerve in congenital aural atresia. Am J Otol 1995; 16; 290-94.
  • Jahrsdoerfer RA. Congenital malformations of the ear. Analysis of 94 operations. Ann Otol Rhinol Laryngol 1980; 89: 348-52.

Konjenital stapes anomalisine bağlı iletim tipi işitme kayıplı nadir iki olgunun sunumu

Year 2010, Volume: 32 Issue: 3, 345 - 351, 12.01.2010

Abstract

Konjenital anomaliler izole kemikçik anomalisi halinde veya dış kulakla kombine anomali olarak görülebilir. İzole kemikçik anomalisi klinik pratikte çok ender olarak rastlanır. Bu anomalilerle ilgili literatürün çoğunluğunu vaka takdimleri oluşturmaktadır ve vaka serisi halindeki bildiriler oldukça sınırlıdır. Konjenital ossiküler anomalinin tedavisi kemikçiklerdeki ve oval penceredeki anomalinin varlığına ve biçimine bağlı olarak değişiklik göstermektedir. Bu makalede ileri derecede oval pencere anomalisi ve hareketli tabanla birlikte stapes suprastriktürünün yokluğu ile karakterize iki ender olgunu ve tedavisi literatür taraması ışığında sunulmuştur

References

  • Swartz JD, glazer AU, Faerber EN, Capitanio MA, Popky GL. Congenital middle ear deafnes: CT study. Radiology 1986; 159: 187-90.
  • Teunissen EB, Cremers WR. Classification of congenital middle ear anomalies. Report on 144 ears. Ann Otol Rhinol Laryngol 1993; 102: 606-12.
  • Nandapalan V, Tos M. Isolated congenital stapes ankylosis: An embryologic survey and literature review. Laryngoscope. 2000; 21: 71-80.
  • Yı Z, Yang J, Li Z, Zhou A, Zhou A, Lin Y. Bilateral congenital absence of stapes and oval window in 2 members of a family : Etiology and management. Otolarygol Head Neck Surg 2003; 128: 777-82.
  • Nakamura S, Sando I. Congenital absence of the oval window (Abs). Arch Otolaryngol 1966;84:131-36.
  • POU JW. Congenital absence of the oval window. Report of two cases.Laryngoscope 1963; 73: 384-91.
  • Zeifer B, Sabini P, Sonne J. Congenital absence of the oval window: Radiologic diagnosis and associated anomalies. Am J Neuroradiology 2000; 21: 322-27.
  • Jahrsdoerfer RA. Congenital absence of the oval window.ORL J Otorhinolarygol Relat Spec 1977; 84: 904-14.
  • Lambert PR. Congenital absence of the oval window. Laryngoscope 1990; 100: 37-40.
  • Teunissen E, Cremers CWRJ. Surgery for congenital anomalies of the middle ear with mobile stapes. Eur Arch Otorhinolaryngol 1993;250:327-31.
  • Cousins VC, Milton CM. Congenital ossicular abnormalities:A review of 68 cases. Am J Otol 1988; 9: 76-80.
  • Isenberg SF, Tubergen TB. An unusual congenital middle ear ossicular anomaly. Arch Otolaryngol 1980; 106: 179-81.
  • Al-Mazrou KA, Alorainy IA, Al-Dousary SH, Richardson MA.Facial nerve anomailes in association with congenital hearing loss. Int J Pediatr Otorhinolarygol 2003; 67: 1347-53.
  • Durcan DJ, Shea JJ, Sleechx JP. Bifurcation of the facial nerve. Arch Otolaryngol 86; 1967: 619-31.
  • Alarcon A, Jahrsdoerfer RA, Kesser BW. Congenital absence of the oval window: Diagnosis, Surgery, and Audiometric Outcomes. Otol Neurotol 2008; 29: 23-28.
  • Jahrsdoerfer RA. Transposition of the facial nerve in congenital aural atresia. Am J Otol 1995; 16; 290-94.
  • Jahrsdoerfer RA. Congenital malformations of the ear. Analysis of 94 operations. Ann Otol Rhinol Laryngol 1980; 89: 348-52.
There are 17 citations in total.

Details

Primary Language English
Journal Section Case Reports
Authors

Ahmet Kutluhan

Mehti Salviz

Hüseyin Çetin

Akif Bilgen

Publication Date January 12, 2010
Published in Issue Year 2010Volume: 32 Issue: 3

Cite

AMA Kutluhan A, Salviz M, Çetin H, Bilgen A. Two unusual case of congenital conductive hearing loss related to anomaly of stapes. CMJ. September 2010;32(3):345-351.