As for other vestibular schwannomas, intralabyrinthine schwannomas commonly cause a sensorineural hearing loss, contrary to more lateral ear pathology that can cause conductive or mixed hearing loss. This case report features a patient that presented with a mixed and thus partly pseudo-conductive hearing loss due to an intracochlear schwannoma, a finding that is very rare. As a result, the patient was initially misdiag-nosed as having otosclerosis and a stapedotomy was performed, without hearing improvement. We discuss the clinical implications of this atypi-cal presentation, which illustrates the importance of performing supplementary audiological testing (e.g., the Gellé test), and the importance of considering vestibular system testing when otosclerosis is suspected. In addition, the importance of imaging and considering differential diagnoses in cases of conductive hearing loss is stressed.