Objectives: To report outcomes of surgery for severe congenital external auditory canal (EAC) stenosis with or without partial atretic plate (PAP). Design: Retrospective review. Subjects: Thirteen patients (18 ears) had surgery for severe EAC stenosis with (n = 10, 56%) or without PAP (n = 8, 44%). Indications included severe stenosis with hearing loss, cerumen impactions, and/or canal cholesteatoma. Mean age = 7.8 years (range 0.4-19.9 years). Mean follow up = 5.2 years (range 0.4-10.0 years). Setting: Tertiary care children's hospital. Interventions: Nineteen endaural canaloplasties were performed in 17 ears (2 revisions). There was one post-auricular approach. Fifteen tympanoplasties were performed in 13 ears (2 revisions). PAP was reconstructed with drilling to enlarge the bony annulus and fascia grafting to enlarge the tympanic membrane in 10 (56%) ears. Outcome measures: Patency of EACs; otologic findings; pure tone averages (PTA): complications. Results: All (100%) EACs had improved patency (>= 4 mm). Findings included canal cholesteatoma (2/18, 11%), ossicular fixation (4/18, 22%), stapes abnormality (2/18, 11%), and incudostapedial discontinuity from cholesteatoma (1/18, 6%). Mean preoperative PTA = 38.7 dB HL (range 60-20 dB HL). Mean postoperative PTA = 23.6 dB HL (range 50-6.7 dB HL). Audiologic results were significantly better for cases without PAP (p < .01) and without ossicular fixation (p < .01). There were seven minor and no major complications. Conclusions: Endaural canaloplasty is safe and effective for providing patent EACs and hearing improvement for severe congenital EAC stenosis. However, since hearing outcomes were worse for cases with PAP and ossicular fixation, alternatives such as hearing aids or BAHA (R) may be considered. (C) 2009 Elsevier Ireland Ltd. All rights reserved.