Otosclerosis

Author Credentials 

Robert J. Yawn, MD
Alejandro Rivas, MD
Otology Group of Vanderbilt
Vanderbilt University Medical Center
Nashville, TN

Module Summary

Otosclerosis is a common form of hearing loss diagnosed and treated by the otolaryngologist. This module is designed to describe the embryology and anatomy of the normal middle ear and otic capsule, and then to understand how this differs in otosclerosis. A variety of treatments have been proposed and will be reviewed including amplification, medical therapy, and surgical therapy via stapedectomy or stapedotomy. Common complications from surgery will also be discussed.

Module Learning Objectives 
  1. Understand the clinical presentation, natural history of, and treatment of otosclerosis.
  2. Describe the histologic and embryologic considerations in otosclerosis.
  3. Describe various treatments including amplification, medical management, and surgical treatment.
  4. Describe the proper surgical technique for stapedectomy/stapedotomy and understand potential complications.
     

Embryology

Learning Objectives 
  1. Understand the natural formation of the otic capsule and stapes footplate.
  2. Describe the embryologic origins of the stapes footplate and suprastructure.
References 
  1. Isaacson G. Endoscopic anatomy of the pediatric middle ear. Otolaryngol Head Neck Surg. 2014 Jan;150(1):6-15.

Anatomy

Learning Objectives 

To understand the relevant anatomy as it pertains to surgical exposure required for stapedectomy/stapedotomy. Specifically:

  • External auditory canal
  • Tympanic membrane annulus
  • Chorda tympani nerve
  • Malleus/Incus/Stapes
  • Anatomic variations of the footplate
References 
  1. Isaacson G. Endoscopic anatomy of the pediatric middle ear. Otolaryngol Head Neck Surg. 2014 Jan;150(1):6-15.
  2. Rask-Andersen H, Schart-Moren N, Stromback K, Linthicum F, Li H. Special Anatomic Considerations in Otosclerosis Surgery. Otolaryngol Head Neck Surg. 2018 Apr;51(2):357-374.

Pathogenesis

Learning Objectives 

Describe the factors that play into bony remodeling in otosclerosis (hormonal, remodeling, immunologic).

References 
  1. Markou K, Goudakos J. An overview of the etiology of otosclerosis. Eur Arch Otorhinolaryngol. 2009 Jan;266(1):25-35.

Basic Science

Learning Objectives 
  1. Describe changes seen in temporal bones of patients with otosclerosis versus those without the disease.
  2. Describe rationale for proposed nonsurgical therapy (fluoride, cytokine inhibitors, bisphosphonates.
References 
  1. Michaels L, Soucek S. Origin and growth of otosclerosis. Acta oto-laryngologica. 2011 May;131(5):460-8.
  2. Chole RA, McKenna M. Pathophysiology of otosclerosis. Otol Neurotol. 2001 Mar;22(2):249-57.

Incidence

Learning Objectives 
  1. Describe the incidence of otosclerosis.
  2. Describe the incidence of progression of hearing loss in patients with otosclerosis versus those age-related changes.
References 
  1. Ishai R, Halpin CF, Shin JJ, McKenna MJ, Quesnel AM. Long-term Incidence and Degree of Sensorineural Hearing Loss in Otosclerosis. Otol Neurotol. 2016 Dec;37(10):1489-1496.

Genetics

Learning Objectives 
  1. Describe the most common forms of inheritance of otosclerosis.
  2. Describe the genes involved.
References 
  1. Bittermann AJ, Wegner I, Noordman BJ, Vincent R, van der Heijden GJ, Grolman W. An introduction of genetics in otosclerosis: a systematic review. Otolaryngol Head Neck Surg. 2014 Jan;150(1):34-9. 
  2. Thys M, Van Camp G. Genetics of otosclerosis. Otol Neurotol. 2009 Dec;30(8):1021-32.

Patient Evaluation

Learning Objectives 
  1. Describe the clinical evaluation of the patient with otosclerosis
    • Clinical History
    • Physical Exam Findings
    • Tuning Fork Exam
    • Audiologic Evaluation
References 
  1. Foster MF, Backous DD. Clinical Evaluation of the Patient with Otosclerosis. Otolaryngol Clin North Am. 2018 Apr;51(2):319-326.

Imaging

Learning Objectives 
  1. Describe the use computed tomography in the evaluation of otosclerosis.
  2. Describe the features on imaging that may suggest intraoperative difficulty.
  3. Describe the benefits of neuroradiology in evaluating the disease process.
References 
  1. Kanona H, Rana I, Offiah C, Patel N. Importance of a dedicated neuroradiologist in reporting high-resolution computed tomography for otosclerosis: a retrospective comparison study of 40 patients. J Laryngol Otol. 2017 Jun;131(6):492-496.
  2. Parra C, Trunet S, Granger Bet al. Imaging Criteria to Predict Surgical Difficulties During Stapes Surgery. Otol Neurotol. 2017 Jul;38(6):815-821.
  3. Dudau C, Salim F, Jiang D, Connor SE. Diagnostic efficacy and therapeutic impact of computed tomography in the evaluation of clinically suspected otosclerosis. Eur Radiol. 2017 Mar;27(3):1195-1201.
  4. Virk JS, Singh A, Lingam RK. The role of imaging in the diagnosis and management of otosclerosis. Otol Neurotol. 2013 Sep;34(7):e55-60.

Pathology

Learning Objectives 
  1. Describe the pathologic changes seen in temporal bones of patients with otosclerosis (bony resorption, new bone deposition, vascular proliferation).
  2. Describe pathologic mechanisms for sensorineural hearing loss in otosclerosis.
References 
  1. Quesnel AM, Ishai R, McKenna MJ. Otosclerosis: Temporal Bone Pathology. Otolaryngologic clinics of North America 2018.

Treatment

Learning Objectives 
  1. Describe the major modalities of treatment of otosclerosis:
    • Amplification
    • Medical therapy
    • Surgical Therapy
References 
  1. Batson L, Rizzolo D. Otosclerosis: An update on diagnosis and treatment. JAAPA. 2017 Feb;30(2):17-22.

Medical Therapies

Learning Objectives 

Describe common proposed medical therapies for otosclerosis including fluoride and bisphosphonates.

References 
  1. Jan TA, Remenschneider AK, Halpin C, Seton M, McKenna MJ, Quesnel AM. Third-generation bisphosphonates for cochlear otosclerosis stabilizes sensorineural hearing loss in long-term follow-up. Laryngoscope Investig Otolaryngol. 2017 Sep 21;2(5):262-268.
  2. Cruise AS, Singh A, Quiney RE. Sodium fluoride in otosclerosis treatment: review. The Journal of laryngology and otology 2010; 124:583-586.
     

Pharmacology

Learning Objectives 

Describe the pharmacologic implications of medical therapies of otosclerosis.

References 
  1. Liktor B, Szekanecz Z, Batta TJ, Sziklai I, Karosi T. Perspectives of pharmacological treatment in otosclerosis. Eur Arch Otorhinolaryngol. 2013 Mar;270(3):793-804.

Surgical Therapies

Learning Objectives 
  1. Describe the difference between stapedectomy and stapedotomy for treatment of otosclerosis.
  2. Describe the surgical steps of stapes surgery.
  3. Describe the use of surgical prostheses in stapes surgery.
  4. Describe the use of the endoscope in stapes surgery.
  5. Describe possible pitfalls seen in revision surgery.
References 
  1. Cheng HCS, Agrawal SK, Parnes LS. Stapedectomy Versus Stapedotomy. Otolaryngol Clin North Am. 2018 Apr;51(2):375-392.
  2. Hunter JB, Rivas A. Outcomes Following Endoscopic Stapes Surgery. Otolaryngol Clin North Am. 2016 Oct;49(5):1215-25.
  3. Hunter JB, O'Connell BP, Rivas A. Endoscopic techniques in tympanoplasty and stapes surgery. Curr Opin Otolaryngol Head Neck Surg. 2016 Oct;24(5):388-94.
  4. Husain Q, Lin KF, Selesnick SH. Stapes prosthesis length and hearing outcomes. Laryngoscope. 2018 Mar;128(3):722-726.
  5. Van Rompaey V, Claes G, Potvin J, Wouters K, Van de Heyning PH. Systematic review of the literature on nitinol prostheses in surgery for otosclerosis: assessment of the adequacy of statistical power. Otol Neurotol. 2011 Apr;32(3):357-66. 
  6. Battista RA, Wiet RJ, Joy J. Revision stapedectomy. Otolaryngol Clin North Am. 2006 Aug;39(4):677-97, v-vi.

Staging

Learning Objectives 
  1. Understand the dilemma in patients with far-advanced otosclerosis.
  2. Describe the differences in outcome of stapes surgery in these patients versus cochlear implantation.
References 
  1. Abdurehim Y, Lehmann A, Zeitouni AG. Stapedotomy vs Cochlear Implantation for Advanced Otosclerosis: Systematic Review and Meta-analysis. Otolaryngol Head Neck Surg. 2016 Nov;155(5):764-770.

Complications

Learning Objectives 

Describe the most common complications seen with stapes surgery and the management of these complications.

References 
  1. Antonelli PJ. Prevention and Management of Complications in Otosclerosis Surgery. Otolaryngol Clin North Am. 2018 Apr;51(2):453-462.

Review

Review Questions 
  1. What is the embryologic origin of the stapes?
  2. What is the pathologic difference of the otic capsule in patients with otosclerosis?
  3. What is the inheritance of otosclerosis?
  4. What is the most common clinical presentation of otosclerosis?
  5. What findings can be seen on the audiogram with respect to pure-tone audiometry?
  6. What findings will be seen on acoustic reflexes?
  7. What tuning fork findings will you see?
  8. What are the non-surgical options for otosclerosis?
  9. What is the difference in stapedotomy and stapedectomy?
  10. How does the endoscope play a role in stapes surgery?
  11. What options are there for patients with far-advanced otosclerosis?
  12. What are the most common complications of stapes surgery?
Resources 

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