Sudden deafness

Douglas J Lanska MD FAAN MS MSPH (Dr. Lanska of the Great Lakes VA Healthcare System and the University of Wisconsin School of Medicine and Public Health has no relevant financial relationships to disclose.)
Originally released September 10, 2001; last updated February 4, 2017; expires February 4, 2020

This article includes discussion of sudden deafness and sudden hearing loss. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

The author explains the clinical presentation, pathophysiology, diagnostic work-up, and management of sudden deafness. "Sudden" deafness is defined as sensorineural hearing loss of 30 decibels or more in at least 3 contiguous frequencies occurring over less than 3 days. The pathophysiology of sudden deafness is poorly understood. Various theories have been proposed, including those attributing sudden deafness to vascular insults, infectious (especially viral) agents, autoimmune or inflammatory mechanisms, or disruption of labyrinthine membranes. Despite extensive investigation, most cases remain idiopathic. Systemic steroids, or a combination of systemic and intratympanic steroids, are commonly recommended, but some employ intratympanic steroid therapy as a first-line therapy because systemic and transtympanic administration of corticosteroids has been found to result in similar clinical outcomes. Intratympanic steroid perfusion should be offered in patients with incomplete recovery from idiopathic sudden sensorineural hearing loss after failure of initial management, and when used as salvage therapy, intratympanic steroids can result in significant gains in hearing. The overall prognosis depends on the underlying etiology, but a high rate of spontaneous resolution occurs overall (ie, about two thirds of cases). For those who do not recover from idiopathic sudden deafness in their only hearing ear (ie, producing bilateral deafness), cochlear implantation can be considered as early as 3 months after initiating treatment of sudden deafness.

Key points

 

• "Sudden" deafness is defined as sensorineural hearing loss of 30 decibels or more in at least 3 contiguous frequencies, occurring over less than 3 days.

 

• In patients with sudden sensorineural hearing loss, tinnitus is associated with worse high-frequency hearing loss, whereas aural fullness and pressure sensations are typically associated with low-frequency hearing loss.

 

• The clinical manifestations of ischemia of the inner ear can include unilateral deafness and tinnitus as well as acute vertigo, nausea and vomiting, imbalance, and canal paresis.

 

• The spectrum of clinical presentation of anterior inferior cerebellar artery (AICA) infarction includes ipsilateral hearing loss with or without tinnitus as well as a range of labyrinthine, brainstem, and cerebellar symptoms and signs.

 

• The spectrum of clinical presentation of superior cerebellar artery syndrome includes ipsilateral Horner syndrome, ipsilateral limb ataxia, contralateral sensorineural hearing loss (due to involvement of the lateral lemniscus carrying decussated ascending auditory information), contralateral superficial sensory loss, vertigo, nystagmus, nausea, and vomiting.

 

• Acute bilateral hearing impairment suggests vertebrobasilar occlusive disease, but hearing loss associated with vertebrobasilar insufficiency is most frequently unilateral.

 

• The blood supply to the inner ear is via the internal auditory artery (also called the labyrinthine artery), which typically originates from the anterior inferior cerebellar artery (AICA).

 

• Patients with unilateral idiopathic sudden sensorineural hearing loss should be evaluated for retrocochlear pathology (eg, acoustic neuroma) using magnetic resonance imaging, brainstem auditory evoked potentials, or audiometric follow-up.

 

• The overall prognosis depends on the underlying etiology, but a high rate of spontaneous resolution occurs overall (ie, about two thirds of cases).

 

• Management is complicated, as the underlying etiology is not known in most patients. A presumptive approach is generally employed, but no consensus exists concerning the management of sudden hearing loss.

 

Systematic syntheses and metaanalyses have failed to support the use of corticosteroids for sudden deafness and, instead, have concluded that “systemic or intratympanic steroid administration does not have a significant treatment effect.”

 

• For those who do not recover from idiopathic sudden deafness in their only hearing ear (ie, producing bilateral deafness), cochlear implantation can be considered as early as 3 months after initiating treatment of sudden deafness.

Historical note and terminology

"Sudden" deafness is typically defined as sensorineural hearing loss of 30 decibels or more in at least 3 contiguous frequencies, occurring over less than 3 days. Some (explicitly or implicitly) consider the syndrome to apply only to "idiopathic" monophasic cases, but in this article such restrictions are not employed.

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