Adam is a 34-year-old maths teacher who is usually fit and well, with no significant past medical history. He presents to you complaining of a sudden onset of deafness in the right ear for the past 24 hours. He woke with it and it did not improve at all over the course of the day and evening. He denies any intercurrent illness or other symptoms like pain or tinnitus. It has never happened before.
On examination, his ear canal is clear and the drum normal. Tuning fork testing reveals that Weber test localises to the left (non-deaf) ear, and Rinne test confirms that bone conduction is better than air conduction on the right.
What is the most likely diagnosis of Adam’s deafness and what management should be instituted?
Adam’s clinical presentation is consistent with sudden sensorineural hearing loss (SSNHL). SSHNL is a medical emergency and requires urgent assessment and treatment.1 While there are range of identifiable aetiologies, 90% of cases of SSNHL are idiopathic.
The diagnosis of SSNHL is based on a careful history and examination, mainly to differentiate sensorineural from conductive hearing loss, and to exclude identifiable causes. Urgent audiology is required to evaluate the extent of the hearing loss, and imaging with MRI to exclude retrocochlear pathology (non-contrast CT has poor diagnostic yield).
A widely used treatment of SSNHL is the administration of corticosteroids, either systemically or intra-tympanically. While a 2013 Cochrane review2 found that the value of steroids remains unclear, 2019 practice guidelines recommend that corticosteroids should be offered as initial therapy.3 The recommended dose of oral prednisone is 1 mg/kg/day (up to 60 mg) in a single dose for 7 to 14 days, then tapered over a similar period of time. Early treatment at sufficiently high dose is critical.