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Idiopathic Hearing Loss(ISSHL)- Perceptive hearing loss, etiology remains unknown after clinical, laboratory and imaging studies, hearing loss occurred within 24 hours, hearing loss is nonfluctuating, severity of the hearing loss averages at least 30 dB HL for three subsequent one octave steps in frequency, blank otological history in an otherwise healthy individual
Sudden Hearing Loss (SSHL) - a sensorineural hearing loss of 30 dB over less than three days affecting three contiguous frequencies, symptom of a greater condition

 Unilateral (only 2% of cases experience bilateral deafness)
 Roaring tinnitus
 Short- lived dysequilibrium/vertigo

 Possible Slopes of HL
 Low Frequency
 Low through Mid-High Frequency
 High Frequency - downward sloping loss has a worse prognosis than low and midfrequency loss

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The term “idiopathic” indicates an unknown origin
Research suggests SSHL etiology as:
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Compromised Vascular Supply
Intracochlear Membrane Breaks, Perilymph Fistula
Neurologic lesions
Viral Infections
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Traumatic insults
Autoimmune Inner Ear Disease
Enlarged Vestibular Acqueduct Syndrome
Syphillis

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ISSHL can often be mistakenly diagnosed as a middle ear disorder
Testing will reveal
 Normal Tympanometry; Abnormal Reflexes
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Tuning fork tests will indicate a sensorineural loss
OAE/ABR abnormal
Audiometry will usually show a unilateral loss
CT Scan/MRI needed to rule out neuroma
Negative fistula test
Urinalysis, blood work

 Depends on identification of lesion
 Vascular
 Hyperbaric oxygen therapy (HBOT) involves breathing pure oxygen in a specially designed chamber and it is sometimes used as a treatment to increase the supply of oxygen to the ear and brain in an attempt to reduce the severity of hearing loss
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Carbogen treatment: 95% oxygen and 5% carbon dioxide.
Carbogen inhalation therapy is given for about 10 minutes each 6 –8 hours over a three-day period by a respiratory therapist. This treatment is thought to increase the oxygen in the perilymph by dilating the cochlear artery
These treatment routes may not be covered by insurances

 Structural defects may require surgical treatment
 Fistulas
 Acoustic neuromas

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If no site of lesion is found, aggressive steroid treatment is usually prescribed
Prednisone: 1mg/kg per day for 2 –4 weeks, rapidly tapering the drug if there is a complete recovery of hearing. If hearing does not recover, reduction of medication is slowed.
The best outcome: when steroids are administered as quickly as possible
Some may benefit from antivirals, diuretics, a lowsodium diet, a restriction in the use of stimulants,
(alcohol and tobacco) and avoidance of excessive physical activity and noise exposure.

 35-50% of people have hearing return to normal levels
 If the hearing does not return, hearing aids, cochlear implants or assistive listening devices may be prescribed
 ASHA recommends a multi-memory, digitally programmable hearing aid, or with a volume wheel for flexibility.

 Most studies find no seasonal, geographic, ethnic, racial or sexual predilection for SHL.
 The right and left ears appear equally vulnerable.
 It affects about 4,000, usually between
40-60 years old

 Test to rule out middle ear pathology and confirm sensorineural lesion
 Understand the emotional aspect to this type of hearing loss and need for counseling
 Three step approach: administrative, medical, rehabilitative

 46-year-old female
 Sudden onset of unilateral tinnitus and decreased hearing while at work
 Awoke in the morning to limited hearing in left ear

 MRI indicated no structural anomalies
 Audiometry = Profound loss across all frequencies tested
 Diagnosed as an idiopathic viral infection, treated with steroids
 Currently, hearing has not improved

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Complains of inability to localize
Habit of answering the phone with poor ear
Discussed possibilities for ALD’s for phone use and CROS hearing aids
 Any other suggestions?

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Menner, A. (2003) A pocket guide to the ear. New York: Thieme.
 Vause, N. (2002) Idiopathic Sudden Sensorineural Hearing
Loss —On the Other Side of the Audiometer. Military Audiology
Short Course. http://www.militaryaudiology.org/masc2002/07_ISSHL.html
.
Retrieved April 15, 2008.
 Wynne, M., Diefendorf, A., Fritsch, M. (2001) Sudden Hearing
Loss. The ASHA Leader Online, http://www.asha.org/about/publications/leader-online/archives/2001/ .
Retrieved April 20, 2008.

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“An autoimmune disorder is a condition that occurs when the immune system mistakenly attacks and destroys healthy body tissue”
Medline Plus
Women are more commonly affected than men
Autoimmune disorders can cause
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Destruction of different body tissues
Changes in organ function
Abnormal growth of an organ

 Syndrome with progressive, fluctuating bilateral sensorineural hearing loss, dizziness and sometimes tinnitus which progresses over weeks to months
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First proposed in 1979
Can be confused with Meniere’s
Disease
Responsible for a very small number of hearing impairment cases (< 1%)
Most common in middle-aged women

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Caused by antibodies or immune cells that damage the inner ear
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Bystander damage= inner ear damage causes cytokines to be released which create further immune reactions after a delay (fluctuating symptoms)
Cross- reactions*= antibodies or T-cells accidentally damage the inner ear if the ear shares common antigens with a harmful substance the body is already trying to fight off
(COCH5B2)
Intolerance= the body may not know all of the antigens in the inner ear. When they are released (after surgery, trauma or infection), the body attacks them (partially immune privileged locus)
Genetics= some people are genetically pre-disposed to immune disorders
* This is the currently favored theory

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Audiological Evaluation
Vestibular Testing
ABR (to rule out AN)
ECochG (to rule out Meniere’s)
Responsiveness to steroids
Blood tests for general autoimmune disorders
Blood tests for inner ear disorders
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Anti-cochlear antibodies (HSP70)
Lymphocyte transformation assay
Blood tests for diseases/problems that mimic AIED
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FTA (syphilis infection)
Lyme disease
Diabetes

Corticosteroids (managed by a
Rheumatologist)
 Prolonged usage is shown to have serious negative side effects
Broughton, Meyerhoff and Cohen, 2004
 Dosage is often tapered to the lowest one that prevents fluctuations in hearing
Broughton et.al
 Benefit is not found in all patients and high dosages may be needed occasionally as a
“booster”

 Cytotoxic Agents (chemotherapy-type medications)
 Methotrexate
 Highly toxic and studies show limited benefit
 Cochlear Implants
 For individuals who do not respond to medical treatment and profound hearing loss is permanent

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“AIED is one of the few reversible causes of sensorineural hearing loss”
Gopen, Keithley and Harris, 2006
 Early diagnosis and treatment are crucial to reversal or progression!

 Mathews, J., Kumar, B.N. (2003), Autoimmune sensorineural hearing loss, Clinical Otolaryngology , 28:479-488.
 Broughton, S.S., Meyerhoff, W.E., Cohen, S.B. (2004), Immunemediated inner ear disease: 10-year experience, Seminars in Arthritis and Rheumatism , 34:544-548
 Gopen, Q., Keithley, E.M., Harris, J.P. (2006), Mechanisms underlying autoimmune inner ear disease, Drug Discovery Today: Disease
Mechanisms , 3(1):137-142.
 Vestibular Disorders Association
 http://www.vestibular.org/vestibular-disorders/specificdisorders/autoimmunity.php
 American Hearing Research Foundation
 http://www.americanhearing.org/disorders/autoimmune/autoimmune.html