Affects 1-3 of every thousand children born in the U.S., with higher rates in developing countries.
Usually not associated with other congenital abnormalities.
More than 50% of cases have a genetic causation.
Caused by cochlear or retrocochlear pathology.
SNHL (sensorineural hearing loss) can occur gradually over years, weeks to months or hours to days.
Children usually otherwise healthy.
Early childhood development usually normal and diagnosis may not be made until after the age of 1 year.
With little or no exposure to environmental sounds and speech auditory deprivation causes cortical reorganization.
Sudden deafness, or idiopathic sensorineural hearing loss refers to the acute onset of hearing loss of at least 30 dB in 3 connected frequencies occurring instantaneously or over a period of up to 3 days.
Idiopathic sudden sensorineural hearing loss with onset in less than 72 hours, has estimated incidence of 5-20 per 100,000 persons per year.
Idiopathic sudden sensorineural hearing loss typically seen between ages 43-53 years, with equal sex distribution.
Idiopathic sudden sensorineural hearing loss associated with transient vestibular symptoms in 28-57% of patients.
Sudden hearing loss shoul be treated as a medical emergency.
Natural course of sudden hearing loss characterized by spontaneous recovery in about 50% of patients.
Possible etiologies include infections, autoimmune processes, circulatory abnormalities, and neurological diseases.
Idiopathic sudden sensorineural hearing loss treated with oral corticosteroids, with improvement in approximately 45-60% of patients.
Treatment options include oral steroids, intratymphatic steroid injections, antiviral agents, vasodilators, diuretics, hyperbaric oxygen therapy and vitamins.
Present standard treatment is the emergent administration of tapering doses of steroids
Intratympanic corticosteroid decreases systemic exposure and and adverse effects compared to oral steroids.
In a randomized trial comparing efficacy oral prednisone to intratympanic methylprednisone for idiopathic sudden sensorineural hearing loss: primary endpoint was change in hearing at 2 months after treatment-intratympanic corticosteroid was non-inferior to oral corticosteroids (Rauch SD et al).