Research Article | | Peer-Reviewed

Imaging in Otosclerosis: Behind the Echoes of the Cochlea

Received: 8 September 2025     Accepted: 22 September 2025     Published: 10 October 2025
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Abstract

Otosclerosis is a primary bone disorder of the otic capsule characterized by abnormal bone remodeling, leading to progressive conductive or mixed hearing loss, often accompanied by tinnitus. It affects approximately 0.3-0.4% of Caucasians, with lower prevalence in Asian and African populations, and accounts for 5-10% of adult-onset conductive hearing loss worldwide. Beyond auditory dysfunction, patients may present with associated symptoms such as tinnitus, vertigo, and, less commonly, rhinitis-like complaints due to Eustachian tube dysfunction, impacting quality of life. We present two cases—one fenestral and one cochlear otosclerosis—illustrating the clinical spectrum. HRCT remains the gold standard, while MRI assists in assessing cochlear involvement and excluding mimics. Advanced modalities, including ultra-high-resolution CT and photon-counting detector CT, further enhance lesion characterization. Given its global burden and potential for misdiagnosis, this study underscores the need for heightened awareness, timely imaging, and close collaboration between radiologists and otolaryngologists to optimize outcomes.

Published in International Journal of Otorhinolaryngology (Volume 11, Issue 2)
DOI 10.11648/j.ijo.20251102.17
Page(s) 37-41
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This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2025. Published by Science Publishing Group

Keywords

Otosclerosis, Otospongiosis, HRCT Temporal Bone, MRI Temporal Bone, Hearing Loss, Tinnitus, Cochlea

1. Introduction
Otosclerosis is a hereditary bone dysplasia or focal osseous dyscrasia primarily affecting the endochondral bone of the otic capsule within the temporal bone . It is characterized by abnormal resorption of mature dense bone by osteoclasts, replaced by spongy, vascular woven bone . In the early active phase (otospongiosis), lesions appear demineralized and vascular, while in the inactive phase they become dense and sclerotic . The most frequent site is the fissula ante fenestram, located anterior to the oval window . Progressive hearing loss and tinnitus in otosclerosis impose a significant psychosocial and functional burden, often impairing communication, reducing social interaction, and affecting work productivity. These symptoms can also negatively impact quality of life, contributing to emotional distress, anxiety, and depression in affected individuals . Here, we present two illustrative cases of otosclerosis followed by a comprehensive discussion of its imaging features, differential diagnosis, and treatment considerations.
2. Methodology
High-resolution CT (HRCT) of the temporal bone was performed using a 16 slice GE brightspeed multidetector CT scanner with thin collimation. Images were acquired at 120 kV and 200-250 mA with a slice thickness of 0.5 mm and a high-resolution bone reconstruction algorithm. Axial images were obtained parallel to the infra-orbito-meatal line, followed by multiplanar reformats (coronal and sagittal) to assess the otic capsule, oval window, round window niche, cochlea, and semicircular canals.
3. Case Presentation
Case 1:
A 42-year-old female presented with a two-year history of progressive bilateral conductive hearing loss, with pulsatile tinnitus more pronounced in the right ear. Audiometry confirmed a conductive hearing loss with an air-bone gap. Otoscopic examination was unremarkable.
HRCT Findings: Temporal bone thin coronal and axial sections (0.625 mm thickness) showed small foci of demineralization in the form of hypodense foci anterior to the oval window at the fissula ante fenestram. There was thickening and fixation of the stapes footplate on the right side (Figure 1).
Case 2:
A 38-year-old male presented with a one-year history of progressive bilateral mixed hearing loss (conductive and sensorineural), pulsatile tinnitus, and recurrent episodes of vertigo. Audiometry confirmed mixed hearing loss with an air-bone gap. Otoscopic examination revealed a reddish area behind the tympanic membrane interpreted as the Schwartze sign, a clinical marker of active otospongiosis due to vascular spongiotic lesions.
HRCT Findings: Temporal bone thin coronal and axial sections revealed diffuse demineralization around and behind the cochlea in a spiral fashion, termed the “halo sign” (Figure 2).
4. Figures
Figure 1. Fenestral Otosclerosis.
1) Figure 1 (A): Axial HRCT of the right temporal bone shows characteristic hypodense (lucent) foci anterior to the oval window at the fissula ante fenestram (blue arrow) indicating bone demineralization. The stapes footplate is thickened and fixed, consistent with stapes fixation (star).
2) Figure 1 (B): Axial HRCT of the left temporal bone shows characteristic hypodense (lucent) foci anterior to the oval window at the fissula ante fenestram (blue arrow) indicating bone demineralization.
3) Figure 1 (C): Coronal HRCT of the right temporal bone shows characteristic hypodense (lucent) foci anterior to the oval window at the fissula ante fenestram (blue arrow) indicating bone demineralization.
Figure 2. Cochlear Otosclerosis.
1) Figure 2 (A): Axial HRCT of the right temporal bone reveals diffuse hypodense demineralization around the cochlea, known as the "halo sign“ (orange arrow).
2) Figure 2 (B): Axial HRCT of the left temporal bone reveals diffuse hypodense demineralization around the cochlea (orange arrow).
3) Figure 2 (C): Coronal HRCT of the right temporal bone reveals diffuse hypodense demineralization around the cochlea (orange arrow).
5. Discussion
5.1. Prevalence and Demographics
Otosclerosis is a major cause of acquired hearing loss, affecting an estimated 15 million people in the United States . The prevalence of clinical otosclerosis is 0.3-0.4% in Caucasians, but only 0.5% in Asians and 0.1% in African-Americans . Histological otosclerosis is more common, reported in up to 13% of autopsy studies . Otosclerosis shows bilateral involvement in up to 70-80% of cases. It may be syndromically associated with Van der Hoeve syndrome, osteogenesis imperfecta (blue sclera, bone fragility), and Paget’s disease The disease shows a female predominance (2:1), with onset usually between the second and fifth decades .
5.2. Genetic Predisposition and Pathogenesis
Genetic predisposition plays a major role, with autosomal dominant inheritance and incomplete penetrance described . A positive family history is seen in ~60% of cases . To date, eight loci (OTSC1-5, 7, 8, 10) have been mapped. Candidate genes include COL1A1/COL2A1 (collagen synthesis), TGF-β1, BMP2, BMP4 (bone metabolism), RELN (neuronal migration), VDR (vitamin D regulation), and SERPINF1 . Osteoprotegerin (OPG) has been identified as a protective factor preventing abnormal otic capsule remodeling; OPG-deficient mice mimic otosclerosis . Environmental triggers such as the measles virus may also play a role .
5.3. Clinical Presentation and Diagnosis
Patients usually present with progressive conductive hearing loss, sometimes progressing to mixed or rarely pure SNHL . Associated symptoms include tinnitus and less commonly vertigo . Audiometry typically shows air-bone gap, Carhart notch, and absent stapedial reflexes . Otoscopy is often normal, though in some patients the Schwartze sign (promontory blush) may be visible .
5.4. Imaging Modalities and Findings
High-Resolution CT (HRCT)
HRCT is the gold standard for diagnosing and staging otosclerosis .
Findings: Fenestral hypodensities at the fissula ante fenestram, footplate thickening, and the “double-ring/halo sign” around the cochlea in retrofenestral disease .
Sensitivity/Specificity: Reported sensitivity ranges 34-95%, with an average pooled sensitivity ~58% and specificity ~95% . Positive predictive value is >90% . HRCT is most reliable for fenestral disease but less sensitive for tiny foci (<1 mm), retrofenestral lesions, and inactive dense foci .
Limitations: Vulnerable to slice thickness >1 mm; hypodense vascular connective tissue can mimic disease leading to false positives .
Bone density quantification studies have shown significantly reduced Hounsfield units in otosclerosis compared to normal otic capsule .
Magnetic Resonance Imaging (MRI)
MRI is less sensitive than HRCT but can identify active disease and exclude mimics .
Findings: Pericochlear intermediate T1 signal, perilabyrinthine enhancement post-contrast, and curvilinear T2 hyperintensities .
Utility: Useful in cases of predominant SNHL/tinnitus or when HRCT is negative but suspicion remains. MRI also rules out mimics such as vestibular schwannoma, Paget’s disease, or osteogenesis imperfecta .
Advanced Imaging Techniques
Recent advances have significantly improved diagnostic yield
Cone-beam CT (CBCT): Ultra-high spatial resolution and reduced dose; useful in preoperative assessment but limited by density calibration and artifacts .
Photon-counting detector CT (PCDCT): Converts photons directly to electrical signals, yielding better contrast, fewer artifacts, improved dose efficiency, and superior visualization of foci and prosthesis positioning .
Ultra-high-resolution CT (UHRCT): Submillimeter isotropic imaging with improved detection of small fenestral foci compared with standard HRCT .
Comparative studies demonstrate that while HRCT remains the standard, PCDCT and UHRCT offer superior visualization of both early fenestral lesions and postoperative prostheses, and may become the new benchmark for otosclerosis imaging . However, they are not yet standard of care due to cost, accessibility, and technical constraints. They are instead positioned as potential future directions.
5.5. Radiological Classification and Prognosis
The Veillon classification is commonly applied :
1) Group I: No radiological abnormality.
2) Group II (Ia-II): Minimal lesions without cochlear contact (footplate thickening, small pre-stapedial foci).
3) Group III: Foci in contact with the cochlear lumen.
4) Group IV (IVa/IVb): Extensive pericochlear or perivestibular lesions.
Prognostic implications:
Extensive (IV): Associated with poor pre/postoperative thresholds, reduced bone conduction gain, and higher complication rates .
Type III lesions: Despite minimal cochlear contact, patients show excellent postoperative outcomes, with >50% achieving >10 dB BC gain .
Localized fenestral disease: Predicts favorable outcomes with air-bone gap closure after stapes surgery .
Variability in HRCT findings of otosclerosis arises from differences in scanner technology (detector type, slice thickness, reconstruction algorithms), radiologist expertise, and the stage of disease at presentation. Early otospongiotic foci may be subtle and easily overlooked, whereas advanced sclerotic changes are more conspicuous. These factors explain the wide range of reported detection rates and diagnostic accuracy in the literature .
5.6. Differential Diagnosis
Several pathologies can mimic otosclerosis radiologically:
1) Osteogenesis imperfecta: Diffuse otic capsule demineralization plus systemic skeletal fragility, blue sclera .
2) Paget’s disease: “Cotton-wool” skull with generalized thickening and coarsening of trabeculae .
3) Cholesteatoma: Soft-tissue lesion with bony erosion of middle ear cavity .
4) Osteonecrosis: Patchy sclerosis and lucency not localized to fissula ante fenestram .
5) Otosyphilis: Aggressive destructive lesions with contrast-enhancing soft tissue .
5.7. Treatment
Conventional Approaches
Hearing aids: Beneficial in early/mild cases but do not halt progression .
Surgery: Stapedotomy/stapedectomy remains the gold standard for fenestral disease, with air-bone gap closure in most patients . Laser-assisted stapedotomy (CO₂/KTP lasers) has improved safety and precision.
Advanced and Adjunctive Treatments
HRCT-assisted surgical planning: Allows preoperative prosthesis length prediction and assessment of anatomical risk factors .
Medical therapy:
Sodium fluoride: Previously used, but evidence remains limited .
Bisphosphonates: Promising in preliminary studies for stabilizing cochlear disease by inhibiting osteoclast-mediated bone resorption but robust clinical trial data is still lacking to recommend them routinely.
Cochlear implantation: Reserved for profound SNHL or failed stapes surgery, with good functional outcomes in advanced retrofenestral disease .
6. Conclusion
HRCT remains the gold standard for diagnosing and staging otosclerosis, while MRI complements by detecting cochlear involvement and excluding mimics. Recent CT innovations further enhance lesion and prosthesis visualization. Timely imaging, combined with multidisciplinary management, is critical for optimal hearing preservation and improved patient outcomes.
7. Teaching Points
1) HRCT is most sensitive for fenestral disease and essential for surgical planning.
2) MRI helps detect active disease, cochlear involvement, and rule out differential diagnoses.
3) Advanced CT techniques (CBCT, UHRCT, PCDCT) provide higher resolution and better postoperative assessment.
4) Collaborative management between radiologists and ENT specialists ensures the best outcomes.
Abbreviations

BC

Bone Conduction

BMP2/BMP4

Bone Morphogenetic Protein 2 / 4

CBCT

Cone-Beam Computed Tomography

COL1A1/COL2A1

Collagen Type I Alpha 1 / Collagen Type II Alpha 1

ENT

Ear, Nose, and Throat (Otorhinolaryngology)

HRCT

High-Resolution Computed Tomography

MRI

Magnetic Resonance Imaging

OPG

Osteoprotegerin

PCDCT

Photon-Counting Detector Computed Tomography

SNHL

Sensorineural Hearing Loss

TGF-β1

Transforming Growth Factor Beta 1

UHRCT

Ultra-High-Resolution Computed Tomography

VDR

Vitamin D Receptor

Acknowledgments
We extend our heartfelt gratitude to the staff, residents, and technicians of the Radiodiagnosis Department at Kempegowda Institute of Medical Sciences for their unwavering support. We also acknowledge the invaluable assistance provided by the Postgraduates. We sincerely thank the Otorhinolaryngology Department for their significant contributions. Lastly, we are deeply appreciative of the patients who participated in this research.
Author Contributions
Pramod Vijayasarathy is the sole author. The author read and approved the final manuscript.
Patient Consent for Publication
Written informed consent has been obtained. The images are anonymized to protect patient identity and privacy.
Data Availability Statement
Not applicable.
Conflicts of Interest
The author declares no conflicts of interest.
References
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[2] Kanzara T, Virk JS. Diagnostic performance of high resolution computed tomography in otosclerosis. World J Clin Cases. 2017 Jul 16; 5(7): 286-291.
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[5] NHS. Otosclerosis [Internet]. 2023 [cited 2024 May 16]. Available from:
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[7] Leposavić L, Leposavić I, Jasović-Gasić M, Milovanović S, Nikolić-Balkoski G. Psychosocial aspects of acquired hearing impairment in the patients with otosclerosis. Psychiatr Danub. 2006 Jun; 18(1-2): 30-8. PMID: 16804497.
[8] Purohit B, Op de Beeck K, Hermans R. Role of MRI as first-line modality in the detection of previously undiagnosed otosclerosis: a single tertiary institute experience. Insights Imaging. 2020 May 19; 11(1): 71.
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  • APA Style

    Vijayasarathy, P. (2025). Imaging in Otosclerosis: Behind the Echoes of the Cochlea. International Journal of Otorhinolaryngology, 11(2), 37-41. https://doi.org/10.11648/j.ijo.20251102.17

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    ACS Style

    Vijayasarathy, P. Imaging in Otosclerosis: Behind the Echoes of the Cochlea. Int. J. Otorhinolaryngol. 2025, 11(2), 37-41. doi: 10.11648/j.ijo.20251102.17

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    AMA Style

    Vijayasarathy P. Imaging in Otosclerosis: Behind the Echoes of the Cochlea. Int J Otorhinolaryngol. 2025;11(2):37-41. doi: 10.11648/j.ijo.20251102.17

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  • @article{10.11648/j.ijo.20251102.17,
      author = {Pramod Vijayasarathy},
      title = {Imaging in Otosclerosis: Behind the Echoes of the Cochlea
    },
      journal = {International Journal of Otorhinolaryngology},
      volume = {11},
      number = {2},
      pages = {37-41},
      doi = {10.11648/j.ijo.20251102.17},
      url = {https://doi.org/10.11648/j.ijo.20251102.17},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijo.20251102.17},
      abstract = {Otosclerosis is a primary bone disorder of the otic capsule characterized by abnormal bone remodeling, leading to progressive conductive or mixed hearing loss, often accompanied by tinnitus. It affects approximately 0.3-0.4% of Caucasians, with lower prevalence in Asian and African populations, and accounts for 5-10% of adult-onset conductive hearing loss worldwide. Beyond auditory dysfunction, patients may present with associated symptoms such as tinnitus, vertigo, and, less commonly, rhinitis-like complaints due to Eustachian tube dysfunction, impacting quality of life. We present two cases—one fenestral and one cochlear otosclerosis—illustrating the clinical spectrum. HRCT remains the gold standard, while MRI assists in assessing cochlear involvement and excluding mimics. Advanced modalities, including ultra-high-resolution CT and photon-counting detector CT, further enhance lesion characterization. Given its global burden and potential for misdiagnosis, this study underscores the need for heightened awareness, timely imaging, and close collaboration between radiologists and otolaryngologists to optimize outcomes.
    },
     year = {2025}
    }
    

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    AB  - Otosclerosis is a primary bone disorder of the otic capsule characterized by abnormal bone remodeling, leading to progressive conductive or mixed hearing loss, often accompanied by tinnitus. It affects approximately 0.3-0.4% of Caucasians, with lower prevalence in Asian and African populations, and accounts for 5-10% of adult-onset conductive hearing loss worldwide. Beyond auditory dysfunction, patients may present with associated symptoms such as tinnitus, vertigo, and, less commonly, rhinitis-like complaints due to Eustachian tube dysfunction, impacting quality of life. We present two cases—one fenestral and one cochlear otosclerosis—illustrating the clinical spectrum. HRCT remains the gold standard, while MRI assists in assessing cochlear involvement and excluding mimics. Advanced modalities, including ultra-high-resolution CT and photon-counting detector CT, further enhance lesion characterization. Given its global burden and potential for misdiagnosis, this study underscores the need for heightened awareness, timely imaging, and close collaboration between radiologists and otolaryngologists to optimize outcomes.
    
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Author Information
  • Department of Radiodiagnosis, Kempegowda Institute of Medical Sciences Hospital and Research Centre, Bangalore, India

    Biography: Pramod Vijayasarathy, MBBS, MD, DNB, MICR, EDIR, DICR, is a radiologist with extensive expertise in diagnostic imaging. He is currently a Senior Resident at Kempegowda Institute of Medical Sciences, Bangalore, Karnataka, India. Previously, he served as a Specialist Radiologist at Aster Multispecialty Hospital, Bangalore. where he delivers comprehensive radiological services including MRI, CT, ultrasound, Doppler, mammography, elastography, fluoroscopy, and image-guided procedures. Dr. Pramod has contributed significantly to medical literature with multiple publications in reputed national and international journals. He has also authored book chapters, presented at national and international conferences, and contributed to academic radiology education. His clinical and research interests include cardiac imaging, fetal imaging, neuroimaging, fusion imaging, and interventional techniques. A recipient of academic and professional honors, Dr. Pramod remains committed to advancing imaging sciences and improving patient care through innovation and collaboration.

    Research Fields: Temporal bone imaging, cardiac imaging, neuroimaging, fetal imaging, fusion imaging, genitourinary imaging, artificial intelligence.