The symptoms and treatment of congenital inner ear malformation: a national-level study comparing the TriNetX research network with Epic Cosmos.

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.