Hearing impairment is common in patients with Apert syndrome. As with most syndromic craniosynostoses, hearing loss in Apert syndrome is primarily conductive, although mixed and sensorineural hearing loss have also been reported in the literature. The leading cause of conductive hearing loss in Apert syndrome is otitis media with effusion (OME). While OME is frequent in children, it is even more prevalent in Apert syndrome due to Eustachian tube dysfunction, which results from cranial base malformations. This condition leads to mild-to-moderate conductive hearing loss and increases the risk of recurrent acute otitis media. Proper diagnosis and management are essential in addressing hearing loss and preventing complications such as retraction pockets or cholesteatoma. As a result, ventilation tube placement is commonly performed in patients with Apert syndrome. Given the crucial role of FGFR2 signaling in the development of the external, middle, and inner ear, a wide range of malformations has been described in the literature. Although otolaryngologic management in Apert syndrome primarily focuses on nasal obstruction and obstructive sleep apnea, lifelong otologic follow-up is mandatory. Regular otoscopic examinations and radiologic assessments are essential to monitor and manage evolving ear conditions. This chapter provides a comprehensive overview of the otologic manifestations of Apert syndrome, detailing the diagnostic approach and available treatment options.