Author(s)
Sriprachodaya Gaddam, BS
Corinne R. Stonebraker, BA
Mingyang Gray, MD
Maura Cosetti, MD
Zachary Schwam, MD
Affiliation(s)
Icahn School of Medicine at Mount Sinai
Abstract:
Objective: To investigate the relationship between microtia grade and Jahrsdoerfer score in non-syndromic patients with congenital aura atresia (CAA), a previously unestablished association, to assess whether external ear severity can serve as a reliable predictor of middle ear anatomy.
Study Design: Retrospective review
Setting: Tertiary referral center
Patients: Patients with unilateral microtia and non-syndromic CAA who underwent evaluation for microtia and/or atresia repair
Interventions: Non-contrast fine-cut computed tomography of the temporal bone imaging (CTTB)
Main Outcome Measures: Grade of microtia (I-VI) using the Marx classification and the Jarhsdoerfer score.
Results: Twenty patients with unilateral CAA and microtia (average age of 15.7 years, range 7 - 40 years) were evaluated (75% male). The distribution of microtia grades was as follows: Grade 1 (n = 3), Grade 2 (n = 10), Grade 3 (n= 5), Grade 4 (n = 2). Mean Jarhsdoerfer scores based on grade were 8.7 for Grade 1, 5.8 for Grade 2, 6.4 for Grade 3, 6.0 for Grade 4. Ordinal logistic regression model did not find a statistically significant relationship between Jarhsdoerfer score and microtia grade (ß = –0.12, SE = 0.16, Wald = 0.57, p = 0.449), indicating that in this cohort, microtia severity was not a significant predictor of Jarhsdoerfer scores.
Conclusions: No significant correlation was found between Marx microtia grade and Jahrsdoerfer score. Surgeons cannot rely solely on the appearance of the ear to estimate surgical candidacy or expected anatomic complexity. Even severe microtia (Grade 3-4) may still have favorable middle ear anatomy for atresia repair. Individualized imaging assessment to guide surgical planning is thus critical. However, further research is encouraged given the small sample size of the study.
Learning Objective: To understand the correlation between external ear severity and middle ear anatomy.
Desired Result: Attendees will understand how the severity of microtia relates to the Jahrsdoerfer score and the implications of this relationship for surgical candidacy and counseling in aural atresia.
Level of Evidence: Level V
Indicate IRB or IACUC: STUDY 21-01768, Icahn School of Medicine at Mount Sinai