Cochlear nerve deficiency

Abstract

Cochlear nerve deficiency (CND) is defined as a small or absent cochlear nerve. It is most often diagnosed in the setting of congenital sensorineural hearing loss and represents around 1?% of cases within this population. It is associated with a recognized syndrome in 30-50?% of cases, most commonly CHARGE syndrome. Treatment planning requires a combination of anatomic and functional assessments using both electrophysiologic and behavioral testing of hearing and imaging of the temporal bones, and central nervous system with MRI and CT. Treatment poses many management challenges and should be tailored to the families’ expectations and those options which provide the greatest chance for succes..