WS and Hearing Manifestations

Sensorineural hearing loss is one of the common symptoms of Wolfram syndrome and observed in around 70 % of patients. The hearing loss affects the high frequencies first and progresses relatively slowly. Routine audiometry is recommended every year or every 2 years. Auditory brainstem response (ABR) audiometry should be performed at diagnosis to confirm the pathophysiology and examine the efficacy of any treatments. Management includes hearing aids and cochlear implant. Hearing manifestations in Wolfram syndrome should be carefully examined because dominant mutations in the WFS1 gene are a 

common cause of low-frequency sensorineural hearing loss, which is different from Wolfram syndrome. These patients develop low-frequency sensorineural hearing loss but do not develop other symptoms seen in patients with Wolfram syndrome, such as diabetes mellitus and ataxia.