Misdiagnosis of otosclerosis in a patient with enlarged vestibular aqueduct syndrome: a case report

INTRODUCTION: In the present case we report on the mismanagement of a patient misdiagnosed with otosclerosis, who was subsequently found to have enlarged vestibular aqueduct syndrome bilaterally. This highlights the need to not only be vigilant in pre-operative assessment of otosclerosis but also in post-operative investigations of stapedectomy failures. CASE PRESENTATION: Our patient, a 56-year-old Caucasian Australian woman, lost the hearing in her right ear following a stapedectomy approximately 25 years ago. It is thought that preoperative imaging was not conducted, while an inadequate (unmasked) audiogram was used to formulate the initial diagnosis of otosclerosis. The hearing in her left ear deteriorated to the point that a cochlear implant was now being considered for her right ear. Imaging performed as part of our pre-cochlear implant battery revealed bilateral enlarged vestibular aqueducts and thus the decision to proceed with a right cochlear implant was made following discussion with our patient and her family in regard to not only general surgical risks but specifically the remote risk that the surgical drilling required during the procedure could risk a deterioration of the hearing in her left ear because of the enlarged vestibular aqueduct on that side. CONCLUSIONS: This report illustrates a case of misdiagnosis and mismanagement of bilateral enlarged vestibular aqueduct resulting in profound hearing loss. Fortunately our patient has been successfully implanted with a right cochlear implant with remarkable outcomes.