American Journal of Otolaryngology - Head and Neck Medicine and Surgery
Volume 27, Issue 5 , Pages 334-340 , September 2006

Otosclerosis: etiopathogenesis and histopathology

  • Sebahattin Cureoglu, MD

      Affiliations

    • Department of Otolaryngology, University of Minnesota, Minneapolis, MN, USA
  • ,
  • Patricia A. Schachern, BS

      Affiliations

    • Department of Surgical Sciences, ENT Clinic, University of Udine, Udine, Italy
  • ,
  • Alfio Ferlito, MD, DLO, DPath, FRSM, FRCSEd, FRCS, FASCP, FRCSGlasg, FRCSI, MCAP, FACS, FRCPath, FHKCORL, FDSRCS

      Affiliations

    • Department of Surgical Sciences, ENT Clinic, University of Udine, Udine, Italy
    • Corresponding Author InformationCorresponding reviewer. Department of Surgical Sciences, ENT Clinic, University of Udine, Policlinico Universitario, Piazzale S. Maria della Misericordia, I-33100 Udine, Italy. Tel.: +39 0432 559302; fax: +39 0432 559339.
  • ,
  • Alessandra Rinaldo, MD

      Affiliations

    • Department of Surgical Sciences, ENT Clinic, University of Udine, Udine, Italy
  • ,
  • Vladimir Tsuprun, MD

      Affiliations

    • Department of Otolaryngology, University of Minnesota, Minneapolis, MN, USA
  • ,
  • Michael M. Paparella, MD

      Affiliations

    • Department of Otolaryngology, University of Minnesota, Minneapolis, MN, USA

References 

  1. Guild SR. Histologic otosclerosis. Ann Otol. 1944;53:246–267
  2. Schuknecht HF. Disorders of bone. In:  Schuknecht HF editors. Pathology of the ear. 2nd ed.. Philadelphia: Lea & Febiger; 1993;p. 365–414
  3. Valsalva AM. In: Valsalvae opera et Morgagni epistolae. Venetiis (Italy): Francescus Pitteri; 1741;p. 2
  4. Siebenmann F. Demonstration mikroskopischer Präparate von Otospongiosis. Proc Int Otol Congr. 1912;9:207
  5. Wang PC, Merchant SN, McKenna MJ, et al. Does otosclerosis occur only in the temporal bone?. Am J Otol. 1999;20:162–165
  6. Altmann F, Glasgold A, Macduff JP. The incidence of otosclerosis as related to race and sex. Ann Otol. 1967;76:377–392
  7. Morrison AW. Genetic factors in otosclerosis. Ann R Coll Surg Engl. 1967;41:202–237
  8. Hall JG. Otosclerosis in Norway, a geographical and genetical study. Acta Otolaryngol Suppl. 1974;324:1–20
  9. Hueb MM, Goycoolea MV, Paparella MM, et al. Otosclerosis: the University of Minnesota temporal bone collection. Otolaryngol Head Neck Surg. 1991;105:396–405
  10. Gordon MA. The genetics of otosclerosis: a review. Am J Otol. 1989;10:426–438
  11. Schuknecht HF, Barber W. Histologic variants in otosclerosis. Laryngoscope. 1985;95:1307–1317
  12. Gristwood RE, Venables WN. Otosclerosis of oval window niche: an analysis of the results of surgery. J Laryngol Otol. 1975;89:1185–1217
  13. Farrior B. Contraindications to the small hole stapedectomy. Ann Otol. 1981;90:636–639
  14. Shea JJ, Farrior JB. Stapedectomy and round window closure. Laryngoscope. 1987;97:10–12
  15. Schuknecht HF, Kirchner JC. Cochlear otosclerosis: fact or fantasy?. Laryngoscope. 1974;84:766–782
  16. Lim DJ, Robinson M, Saunders WH. Morphologic and immunohistochemical observation of otosclerotic stapes: a preliminary study. Am J Otolaryngol. 1987;8:282–295
  17. Parahy C, Linthicum FH. Otosclerosis and otospongiosis: clinical and histological comparisons. Laryngoscope. 1984;94:508–512
  18. Linthicum FH. Histopathology of otosclerosis. Otolaryngol Clin North Am. 1993;26:335–352
  19. Chole RA, McKenna M. Pathophysiology of otosclerosis. Otol Neurotol. 2001;22:249–257
  20. Chevance LG, Bretlau P, Jorgensen MB, et al. Otosclerosis. An electron microscopic and cytochemical study. Acta Otolaryngol Suppl. 1970;272:1–44
  21. Causse J, Chevance LG, Bretlau P, et al. Enzymatic concept of otospongiosis and cochlear otospongiosis. Clin Otolaryngol. 1977;2:23–32
  22. Chevance LG, Causse J, Bretlau P, et al. Hydrolytic activity of the perilymph in otosclerosis. A preliminary report. Acta Otolaryngol. 1972;74:23–28
  23. Manasse P. Die Ostitis Chronica Metaplastica der labyrinthkapsel. Wiesbaden (JF): Bergmann; 1912;
  24. Gussen R. Labyrinthine otosclerosis and sensorineural deafness. Pathologic findings of the spiral ligament. Arch Otolaryngol. 1975;101:438–440
  25. Lindsay JR, Beal DD. Sensorineural deafness in otosclerosis. Observations on histopathology. Ann Otol. 1966;75:436–457
  26. Parahy C, Linthicum FH. Otosclerosis: relationship of spiral ligament hyalinization to sensorineural hearing loss. Laryngoscope. 1983;93:717–720
  27. Cherukupally SR, Merchant SN, Rosowski JJ. Correlations between pathologic changes in the stapes and conductive hearing loss in otosclerosis. Ann Otol Rhinol Laryngol. 1998;107:319–326
  28. Elonka DR, Applebaum EL. Otosclerotic involvement of the cochlea: a histologic and audiologic study. Otolaryngol Head Neck Surg. 1981;89:343–351
  29. Hinojosa R, Marion M. Otosclerosis and sensorineural hearing loss: a histopathologic study. Am J Otolaryngol. 1987;8:296–307
  30. Jorgersen MB, Kristensen HK. Activity of otosclerosis assessed histologically. J Laryngol Otol. 1967;81:911–914
  31. Lim DJ, Saunders WH. Otosclerotic stapes: morphological and microchemical correlates. An electron microscopic and x-ray analytical investigation. Ann Otol. 1977;86:525–540
  32. Lindsay JR. Histopathology of otosclerosis. Arch Otolaryngol. 1973;97:24–29
  33. Sorensen MS. Temporal bone dynamics, the hard way. Formation, growth, modeling, repair and quantum type bone remodeling in the otic capsule. Acta Otolaryngol Suppl. 1994;512:1–22
  34. Cawthorne T. Otosclerosis. J Laryngol Otol. 1955;69:437–456
  35. Paparella MM, Mancini F, Liston SL. Otosclerosis and Meniere's syndrome: diagnosis and treatment. Laryngoscope. 1984;94:1414–1417
  36. Paparella MM, Schachern PA, Goycoolea MV. Multiple otopathologic disorders. Ann Otol Rhinol Laryngol. 1988;97:14–18
  37. Liston SL, Paparella MM, Mancini F, et al. Otosclerosis and endolymphatic hydrops. Laryngoscope. 1984;94:1003–1007
  38. Yoon TH, Paparella MM, Schachern PA. Otosclerosis involving the vestibular aqueduct and Meniere's disease. Otolaryngol Head Neck Surg. 1990;103:107–112
  39. Lawrence M. Possible influence of cochlear otosclerosis on inner ear fluids. Ann Otol. 1966;75:553–558
  40. Ghorayeb BY, Linthicum FH. Otosclerotic inner ear syndrome. Ann Otol. 1978;87:85–90
  41. Gristwood RE, Venables WN. Otosclerosis and chronic tinnitus. Ann Otol Rhinol Laryngol. 2003;112:398–403
  42. Gros A, Vatovec J, Sereg-Bahar M. Histologic changes on stapedial footplate in otosclerosis. Correlations between histologic activity and clinical findings. Otol Neurotol. 2003;24:43–47
  43. Takasaki K, Sando I, Hirsch BE. Loss of facial nerve fibers in a case of otosclerosis. Am J Otol. 2000;21:766–768
  44. Saim L, Nadol JB. Vestibular symptoms in otosclerosis—correlation of otosclerotic involvement of vestibular apparatus and Scarpa's ganglion cell count. Am J Otol. 1996;17:263–270
  45. Sando I, Hemenway WG, Miller DR, et al. Vestibular pathology in otosclerosis: temporal bone histopathological report. Laryngoscope. 1974;84:593–605
  46. Powers WH, Sheehy JL, House HP. The fixed malleus head. A report of 35 cases. Arch Otolaryngol. 1967;85:177–181
  47. Lippy WH, Schuring AG, Ziv M. Stapedectomy for otosclerosis with malleus fixation. Arch Otolaryngol. 1978;104:388–389
  48. Causse J, Causse JB. Eighteen-year report on stapedectomy. I: Problems of stapedial fixation. Clin Otolaryngol. 1980;5:49–59
  49. Covell WP, Feinmesser M. Further studies on the pathology of ossicles in otosclerosis. Laryngoscope. 1959;69:164–173
  50. Moon CN, Hahn MJ. Primary malleus fixation: diagnosis and treatment. Laryngoscope. 1981;91:1298–1307
  51. House HP. Diagnostic aspects of congenital ossicular fixation. Trans Am Acad Ophthalmol Otolaryngol. 1956;60:787–790
  52. Hilding DA. Postinflammatory fixation of the malleus. Its surgical correction. Arch Otolaryngol. 1965;81:17–19
  53. Nandapalan V, Pollak A, Langner A, et al. The anterior and superior malleal ligaments in otosclerosis: a histopathologic observation. Otol Neurotol. 2002;23:854–861
  54. Huber A, Koike T, Wada H, et al. Fixation of the anterior mallear ligament: diagnosis and consequences for hearing results in stapes surgery. Ann Otol Rhinol Laryngol. 2003;112:348–355
  55. Orecchio FA. In:  Lanza G editors. 2nd ed.. Anatomia patologica sistematica. vol. II:Padova: Piccin; 1985;p. 2286–2307
  56. Barnes L, Peel RL. Diseases of the external auditory canal, middle ear, and temporal bone. In:  Barnes L editors. 2nd ed.. Surgical pathology of head and neck. vol. I:New York: Marcel Dekker; 2001;p. 557–599
  57. Arnold W, Plester D. Active otosclerosis of the stapes footplate: histological and clinical aspects of its influence on the perilymph. Arch Otorhinolaryngol. 1977;215:159–178In German
  58. Arnold W, Plester D. Vascular degeneration in otosclerosis and its influence on the mesenchymal reaction of the mucoperiost. Arch Otorhinolaryngol. 1975;209:127–143In German
  59. Tomek MS, Brown MR, Mani SR, et al. Localization of a gene for otosclerosis to chromosome 15q25-q26. Hum Mol Genet. 1998;7:285–290
  60. Van Den Bogaert K, Govaerts PJ, Schatteman I, et al. A second gene for otosclerosis, OTSC2, maps to chromosome 7q34-36. Am J Hum Genet. 2001;68:495–500
  61. Chen W, Campbell CA, Green GE, et al. Linkage of otosclerosis to a third locus (OTSC3) on human chromosome 6p21.3-22.3. J Med Genet. 2002;39:473–477
  62. Van Den Bogaert K, De Leenheer EM, Chen W, et al. A fifth locus for otosclerosis, OTSC5, maps to chromosome 3q22-24. J Med Genet. 2004;41:450–453
  63. McKenna MJ, Kristiansen AG, Haines J. Polymerase chain reaction amplification of a measles virus sequence from human temporal bone sections with active otosclerosis. Am J Otol. 1996;17:827–830
  64. Arnold W, Niedermeyer HP, Lehn N, et al. Measles virus in otosclerosis and the specific immune response of the inner ear. Acta Otolaryngol. 1996;116:705–709
  65. Ferlito A, Arnold W, Rinaldo A, et al. Viruses and otosclerosis: chance association or true causal link?. Acta Otolaryngol. 2003;123:741–746
  66. Karosi T, Konya J, Szabo LZ, et al. Measles virus prevalence in otosclerotic stapes footplate samples. Otol Neurotol. 2004;25:451–456
  67. McKenna MJ, Mills BG. Immunohistochemical evidence of measles virus antigens in active otosclerosis. Otolaryngol Head Neck Surg. 1989;101:415–421
  68. McKenna MJ, Mills BG. Ultrastructural and immunohistochemical evidence of measles virus in active otosclerosis. Acta Otolaryngol Suppl. 1990;470:130–139[discussion 139-40]
  69. Niedermeyer H, Arnold W, Neubert WJ, et al. Evidence of measles virus RNA in otosclerotic tissue. ORL J Otorhinolaryngol Relat Spec. 1994;56:130–132
  70. Miyazawa T, Tago C, Ueda H, et al. HLA associations in otosclerosis in Japanese patients. Eur Arch Otorhinolaryngol. 1996;253:501–503
  71. Gregoriadis S, Zervas J, Varletzidis E, et al. HLA antigens and otosclerosis. A possible new genetic factor. Arch Otolaryngol. 1982;108:769–771
  72. Chobaut JC, Bertrand D, Raffoux C, et al. HLA antigens in otosclerosis. Am J Otol. 1982;3:241–242
  73. Majsky A, Novotny Z, Fajstavr J. HLA and otosclerosis. Tissue Antigens. 1982;20:306–307
  74. Pedersen U, Madsen M, Lamm LU, et al. HLA-A, -B, -C antigens in otosclerosis. J Laryngol Otol. 1983;97:1095–1097
  75. McKenna MJ, Kristiansen AG, Bartley ML, et al. Association of COL1A1 and otosclerosis: evidence for a shared genetic etiology with mild osteogenesis imperfecta. Am J Otol. 1998;19:604–610
  76. McKenna MJ, Kristiansen AG, Tropitzsch AS. Similar COL1A1 expression in fibroblasts from some patients with clinical otosclerosis and those with type I osteogenesis imperfecta. Ann Otol Rhinol Laryngol. 2002;111:184–189
  77. Rodriguez L, Rodriguez S, Hermida J, et al. Proposed association between the COL1A1 and COL1A2 genes and otosclerosis is not supported by a case-control study in Spain. Am J Med Genet. 2004;128A:19–22
  78. Yoo TJ, Tomoda K, Stuart JM, et al. Type II collagen–induced autoimmune otospongiosis. A preliminary report. Ann Otol Rhinol Laryngol. 1983;92:103–108
  79. Bujia J, Alsalameh S, Jerez R, et al. Antibodies to the minor cartilage collagen type IX in otosclerosis. Am J Otol. 1994;15:222–224
  80. Yoo TJ, Stuart JM, Kang AH, et al. Type II collagen autoimmunity in otosclerosis and Meniere's disease. Science. 1982;217:1153–1155
  81. Gordon MA, McPhee JR, Van de Water TR, et al. Aberration of the tissue collagenase system in association with otosclerosis. Am J Otol. 1992;13:398–407
  82. McPhee JR, Gordon MA, Ruben RJ, et al. Evidence of abnormal stromelysin mRNA expression in suspected carriers of otosclerosis. A possible molecular marker. Arch Otolaryngol Head Neck Surg. 1993;119:1108–1116
  83. Thalmann I, Thallinger G, Thalmann R. Otosclerosis: a local manifestation of a generalized connective tissue disorder?. Am J Otolaryngol. 1987;8:308–316

 This study was supported by Hubbard Broadcasting Foundation, Saint Paul, MN and The Starkey Hearing Foundation Eden Prairie, MN.

PII: S0196-0709(05)00217-6

doi: 10.1016/j.amjoto.2005.11.001

American Journal of Otolaryngology - Head and Neck Medicine and Surgery
Volume 27, Issue 5 , Pages 334-340 , September 2006