Related Experiment Video
Updated: Jul 17, 2026
Testing a Cochlear Implant Electrode Insertion Training System for Optimal Electrode Array Placement in Different Inner Ear Anatomies
Published on: February 6, 2026
The symptoms and treatment of congenital inner ear malformation: a national-level study comparing the TriNetX
Gillian Michaelson1, Haley Baker2, Todd Otteson2
1Case Western Reserve University School of Medicine, Cleveland, OH, USA; Department of Otolaryngology - Head and Neck Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA; Department of Otolaryngology - Head and Neck Surgery, Lewis Katz School of Medicine at Temple University, Philadelphia, PA, USA.
Objective:
The current epidemiological understanding of inner ear malformation (IEM) patients is limited by the rarity of the condition. This study employs two national databases to describe the natural progression of this disease across thousands of electronic medical records.
Methods:
A retrospective analysis was conducted using de-identified data from TriNetX and Epic Cosmos. Patient characteristics of individuals with IEM and related diagnoses and treatments were extracted and chi-square analyses were used to compare between databases.
Results:
Overall, 8862 IEM patients were identified in Epic Cosmos and 8145 IEM patients in TriNetX. There was no difference in the number of cochlear implant patients between the two datasets (8% vs 9%, p = 0.226) and there was a statistically, but not clinically, significant difference between the number of hearing loss patients (81% vs 80%, p = 0.007). Patients were first diagnosed with IEM before age 20 at a rate of 42% in Epic Cosmos and 54% in TriNetX, and after 50 years old at a rate of 36% in Epic Cosmos and 12% in TriNetX.
Conclusion:
This analysis suggests most IEM patients will experience some degree of hearing loss, although onset timing and severity may vary widely and most patients will not require a cochlear implant. Additionally, more adults than previously recognized may have mild forms of IEM that contribute to late-onset symptoms. Finally, these large databases successfully present a similar patient population when queried using well-defined, consistently applied diagnosis or billing codes.
