<p>The aim was to investigate the hearing status of patients with choanal atresia and to gain new knowledge about the hearing status of patients with choanal atresia. The hearing status of 70 patients with bilateral (<i>n</i> = 35) and unilateral (<i>n</i> = 35) choanal atresia was evaluated in this cohort study. Demographic data, the prevalence of comorbidities and hearing disorders, as well as the type (conductive vs. sensorineural) and level of hearing loss (HL), and the received type of&#xa0;hearing rehabilitation were investigated. In total, 49% (<i>n</i> = 34) of the study cohort had a hearing disorder. Comorbidities were significantly associated to sensorineural (<i>p</i> &lt; 0.01) and conductive (<i>p</i> &lt; 0.001) HL in the total study cohort. Sensorineural HL was significantly more prevalent in the bilateral (<i>n</i> = 15, 43%) than in the unilateral group (<i>n</i> = 3, 9%; <i>p</i> &lt; 0.01). Conductive HL was observed in the bilateral (<i>n</i> = 6; 17%) and unilateral group (<i>n</i> = 10; 29%; <i>p</i> = 0.394). Rehabilitation consisted of paracentesis/ventilation tube insertion for conductive HL, and hearing aids and cochlear implants for sensorineural HL.</p><p><i>Conclusion</i>:&#xa0;Almost every second child in the study cohort presented a hearing disorder. Hearing disorders were correlated with comorbidities. Both, sensorineural and conductive HL were prevalent.This highlights the importance of performing adequate hearing tests and initiating hearing rehabilitation where necessary for patients with bilateral and unilateral choanal atresia. This is crucial for children’s development.<Table Float="No" ID="Taba"> <tgroup cols="1"> <colspec align="left" colname="c1" colnum="1" /> <tbody> <row> <entry align="left" colname="c1"> <p><b>What is Known:</b></p> </entry> </row> <row> <entry align="left" colname="c1"> <p>• <i>Normal hearing is essential for speech, language, and psychosocial development in children.</i></p> <p>• <i>Data on hearing outcomes in patients with choanal atresia are limited.</i></p> </entry> </row> <row> <entry align="left" colname="c1"> <p><b>What is New:</b></p> </entry> </row> <row> <entry align="left" colname="c1"> <p>• <i>Hearing impairment was present in almost half (49%) of a rare cohort of 70 patients with choanal atresia.</i></p> <p>•<i> Sensorineural hearing loss was more prevalent in bilateral choanal atresia and associated comorbidities, whereas conductive hearing loss predominated in unilateral cases.</i></p> </entry> </row> </tbody> </tgroup> </Table></p>