Serial case report: Radiological images of congenital sensorineural hearing loss patients with and without hypoplasia of the vestibulocochlear nerve

Abstract

Background: Congenital Sensorineural Hearing Loss (SNHL) is a hearing loss caused by damage to the inner ear (cochlea), vestibulocochlear cranial nerve (N.VIII), or the innervation pathway from the inner ear to the brain that is acquired from birth. If hearing loss occurs from birth or before the period of speech development is not detected. Thus, this case report compares two patients with congenital sensorineural hearing loss with and without vestibulocochlear nerve hypoplasia. Case Description: The first case was a 4-year-old girl with the main complaint of not hearing and responding to calls since the age of 1.5 years. Temporal CT scan and MRI results did not show existing organ and structural abnormalities, so suspected retro-cochlear auditory neuropathy. Patients undergo the installation of a cochlear implant in the left ear with good results. Case second, a 3-year-old girl with Down Syndrome who has not been able to speak since birth. CT scan results showed left and right basal cochlea 1.5 turns and bilateral jugular notch dehiscence, while MRI results showed vestibulocochlear nerve hypoplasia, narrowing of the IAC opening width 1.5 turns cochlea. If this happens, a cochlear implant cannot be done because abnormality finding is a contraindication operation. Conclusion: CT-scan and MRI examination are necessary to evaluate the structures and organs in the outer, middle and inner ear because the presence or absence of structural abnormalities found in the patient will significantly impact further management.