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Mixed and Sensorineural Hearing Loss in Otosclerosis: Incidence, Pathophysiology, and Treatment

  • OTOLOGY: Otosclerosis and Stapes Surgery (JP Harris and A Quesnel, Section Editors)
  • Published:
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Abstract

Purpose of Review

Otosclerosis can lead to mixed or rarely pure sensorineural hearing loss (SNHL). Herein, we review the incidence, pathophysiology, and management of mixed hearing loss in otosclerosis, often termed “cochlear otosclerosis.”

Recent Findings

Histopathological studies of human temporal bones demonstrate that extension of otosclerotic foci to the cochlear endosteum, which leads to hyalinization of the spiral ligament and upregulation of TGFß-1, is associated with mixed hearing loss. Peri-cochlear extension of otosclerosis can be assessed in life via computed tomography imaging. In small retrospective series, bisphosphonate treatment stabilized hearing in patients with cochlear otosclerosis. Stapedectomy and/or amplification and cochlear implantation remain the main treatment modalities for patients with mixed hearing loss due to cochlear otosclerosis.

Summary

Approximately one-third of patients diagnosed with otosclerosis will develop a sensorineural hearing loss component, resulting in mixed hearing loss. Further work is needed to characterize the molecular/cellular mechanisms of sensorineural hearing loss in otosclerosis. Current management includes stapedectomy, amplification, consideration of bisphosphonate treatment (off-label), and cochlear implantation.

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    Acknowledgements

    The authors report no proprietary or commercial interest in any product mentioned or concept discussed in this article.

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    1. Maimuna Ahmad and Divya A. Char contributed equally to the manuscript.

    Authors and Affiliations

    1. Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, MA, USA

      Maimuna Ahmad, Divya A. Chari & Alicia M. Quesnel

    2. Department of Otolaryngology, UMass Memorial Medical Center, Worcester, MA, USA

      Maimuna Ahmad & Divya A. Chari

    3. University of Massachusetts Medical School, Worcester, MA, USA

      Maimuna Ahmad & Divya A. Chari

    4. Department of Otolaryngology, Harvard Medical School, Boston, MA, USA

      Divya A. Chari & Alicia M. Quesnel

    5. Akouos Inc, Boston, USA

      Michael J. McKenna

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    1. Maimuna Ahmad
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    Correspondence to Alicia M. Quesnel.

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    MJM — Co-Founder and Chief Medical Officer of Akouos Inc. AMQ — Frequency Therapeutics (sponsored research agreement), Grace Medical (sponsored research agreement, licensed patent), Akouos (research participant). DAC — Grace Medical (licensed patent). The other authors declare that they have no conflict of interest.

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    Ahmad, M., Chari, D.A., McKenna, M.J. et al. Mixed and Sensorineural Hearing Loss in Otosclerosis: Incidence, Pathophysiology, and Treatment. Curr Otorhinolaryngol Rep 10, 8–15 (2022). https://doi.org/10.1007/s40136-021-00390-2

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