Audiometric Analyses Confirm a Cochlear Component, Disproportional to Age, in Stapedial Otosclerosis

Objective: To report the preoperative audiometric profile of surgically confirmed otosclerosis. Study Design: Retrospective, multicenter study. Setting: Four tertiary referral centers. Patients: One thousand sixty-four surgically confirmed patients with otosclerosis. Interventions: Therapeutic ear surgery for hearing improvement. Main Outcome Measures: Preoperative audiometric air conduction (AC) and bone conduction (BC) hearing thresholds were obtained retrospectively for 1064 patients with otosclerosis. A cross-sectional multiple linear regression analysis was performed on audiometric data of affected ears. Influences of age and sex were analyzed and age-related typical audiograms were created. Bone conduction thresholds were corrected for Carhart effect and presbyacusis; in addition, we tested to see if separate cochlear otosclerosis component existed. Corrected thresholds were than analyzed separately for progression of cochlear otosclerosis. Results: The study population consisted of 35% men and 65% women (mean age, 44 yr). The mean pure-tone average at 0.5, 1, and 2 kHz was 57 dB hearing level. Multiple linear regression analysis showed significant progression for all measured AC and BC thresholds. The average annual threshold deterioration for AC was 0.45 dB/yr and the annual threshold deterioration for BC was 0.37 dB/yr. The average annual gap expansion was 0.08 dB/year. The corrected BC thresholds for Carhart effect and presbyacusis remained significantly different from zero, but only showed progression at 2 kHz. Conclusion: The preoperative audiological profile of otosclerosis is described. There is a significant sensorineural component in patients with otosclerosis planned for stapedotomy, which is worse than age-related hearing loss by itself. Deterioration rates of AC and BC thresholds have been reported, which can be helpful in clinical practice and might also guide the characterization of allegedly different phenotypes for familial and sporadic otosclerosis.

[1]  Robert A Dobie,et al.  Estimating Noise-Induced Permanent Threshold Shift from Audiometric Shape: The ISO-1999 Model , 2005, Ear and hearing.

[2]  E. Nelson,et al.  Questioning the Relationship between Cochlear Otosclerosis and Sensorineural Hearing Loss: A Quantitative Evaluation of Cochlear Structures in Cases of Otosclerosis and Review of the Literature , 2004, The Laryngoscope.

[3]  C. Cremers,et al.  A fifth locus for otosclerosis, OTSC5, maps to chromosome 3q22–24 , 2004, Journal of Medical Genetics.

[4]  R. Tange,et al.  The aetiology of otosclerosis: a review of the literature. , 2003, Clinical otolaryngology and allied sciences.

[5]  C. Cremers,et al.  Characterizing and Distinguishing Progressive Phenotypes in Nonsyndromic Autosomal Dominant Hearing Impairment , 2003 .

[6]  S. Manolidis,et al.  Linkage of otosclerosis to a third locus (OTSC3) on human chromosome 6p21.3-22.3 , 2002, Journal of medical genetics.

[7]  J. Timmermans,et al.  Prevalence of Otosclerosis in an Unselected Series of Temporal Bones , 2001, Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology.

[8]  R. Chole,et al.  Pathophysiology of Otosclerosis , 2001, Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology.

[9]  F. E. Offeciers,et al.  A second gene for otosclerosis, OTSC2, maps to chromosome 7q34-36. , 2001, American journal of human genetics.

[10]  P. Willems,et al.  Localization of a gene for otosclerosis to chromosome 15q25-q26. , 1998, Human molecular genetics.

[11]  W. Arnold,et al.  Otosclerosis: a measles virus associated inflammatory disease. , 1995, Acta oto-laryngologica.

[12]  L. Michaels,et al.  Pathology of the otic capsule , 1990, The Journal of Laryngology & Otology.

[13]  M. McKenna,et al.  Immunohistochemical Evidence of Measles Virus Antigens in Active Otosclerosis , 1989, Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery.

[14]  J. Uriel,et al.  Etiology of otospongiotic sensorineural losses. , 1989, The American journal of otology.

[15]  O. Ribári,et al.  Proteolytic enzymes in otosclerosis , 1987 .

[16]  O. Ribári,et al.  Proteolytic enzymes in otosclerosis. Significance of proteolytic enzymes in otosclerotic bone remodelling. , 1987, ORL; journal for oto-rhino-laryngology and its related specialties.

[17]  S. Gatehouse,et al.  Sensorineural Hearing Loss in Stapedial Otosclerosis , 1984, The Annals of otology, rhinology, and laryngology.

[18]  S Gatehouse,et al.  A re-examination of the Carhart effect. , 1982, British journal of audiology.

[19]  J. M. Cole,et al.  Long-term effect of otosclerosis on bone conduction. , 1979, The Laryngoscope.

[20]  S. R. Hoffman,et al.  Stapedectomy — in depth analysis of 2405 cases , 1978, The Laryngoscope.

[21]  R. Caparosa,et al.  A comparison of fenestration of the horizontal canal and stapedectomy in the opposite ear , 1978, The Laryngoscope.

[22]  R. Miyamoto,et al.  Cochlear reserve in otosclerosis. A long-term follow-up study of fenestration cases. , 1978, Archives of otolaryngology.

[23]  J. Kirchner,et al.  Cochlear otosclerosis: Fact or fantasy , 1974, The Laryngoscope.

[24]  S. Bundey,et al.  The inheritance of otosclerosis , 1970, The Journal of Laryngology & Otology.

[25]  R. Naunton,et al.  Sensorineural hearing loss in otosclerosis. , 1969, Archives of otolaryngology.

[26]  H. Schuknecht,et al.  FURTHER OBSERVATIONS ON THE PATHOLOGY OF PRESBYCUSIS. , 1964, Archives of otolaryngology.

[27]  R. Carhart.  Clinical application of bone conduction audiometry. , 1950, Archives of otolaryngology.