Overview
Sudden sensorineural hearing loss (SSHL) is defined as hearing loss that generally occurs over <72 hours and requires an immediate referral to ENT as it is considered to be a medical emergency.
In 90% of cases, there is no underlying cause and SSHL is idiopathic, however, it can lead to mild-severe hearing loss which may be permanent. Causes can include trauma, infection, malignancy, Meniere’s disease, and autoimmunity (e.g. multiple sclerosis or vasculitis).
Referral
Immediate referral
Any patient with the following should be referred for assessment within 24 hours by ENT or the emergency department:
- Sudden onset (<72 hours) unilateral or bilateral sensorineural hearing loss
- Unilateral hearing loss with focal neurological deficits e.g. facial drooping
- Hearing loss associated with a head or neck injury
- Hearing loss associated with severe infection such as necrotising otitis externa/Ramsay Hunt syndrome
Urgent referral (within 2 weeks)
Any patient with the following should be referred to ENT within 2 weeks:
- Sudden onset (<72 hours) unilateral or bilateral sensorineural hearing loss that developed more than 30 days ago
- Rapidly progressing (over 4-90 days) sensorineural hearing loss
- Features of a suspected head or neck malignancy:
- Unilateral hearing loss and effusion not associated with an upper respiratory tract infection
- Bloody discharge from the ear may suggest a malignant tumour of the external ear
Investigations
All patients
- Immediate referral to on-call ENT if not already done so
- MRI of the cerebellopontine angle and internal auditory meatus
- This is to rule out a vestibular schwannoma
Other investigations
If there are features that suggest an undiagnosed autoimmune condition, perform an antibody screen:
- FBC
- ESR and CRP
- U&Es
- Coagulation screen
- Antinuclear antibodies
- Anticardiolipin antibodies
- Lupus anticoagulant
- Antineutrophil cytoplasmic antibodies (ANCA)
- Syphilis serology
Management
All patients
- 1st-line: high-dose oral corticosteroids