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DR ROOHIA
First described in 1944 by DeKleyn
Incidence: 5-20 per 100,000
4,000 new cases/year in US
Idiopathic
Hearing loss in 3 contiguous frequencies of at least 30 dB
Some authors use at least 20 dB loss
Onset of hearing loss occurs in less than 72 hours
Recovery rate without treatment 32% - 79%
Usually within 2 weeks of onset
Only 36% with complete recovery
No middle ear disease
Otologic emergency!
 The two principal indications for intratympanic
steroids are sudden sensorineural hearing loss
(SNHL) and Meniere's disease
• decreasing the number of circulating blood leukocytes and inhibiting the
formation and liberation of inflammatory mediators
• inhibit the release of chemoattractive and vasoactive factors, decrease the
secretion of lipolytic and proteolytic enzymes, and inhibit the release of
proinflammatory cytokines
• These actions decrease the damage from an inflammatory response,
whether the insult is secondary to mechanic, hypoxic, ischemic, infectious,
or autoimmunologic causes
 On exposure to lipopolysaccharide
 cultured endothelial modiolar cells and tissue exhibit a
generic response and release proinflammatory cytokines
 vasculitis, vascular leakage syndrome, entry of
immunocompetent cells, and perivasculitis, ultimately
leading to cochlear ischemia, intracochlear tissue
damage, and hearing loss
 DEXAMETHASONE-interrupt the beginnings of the
inflammatory cascade at the level of cytokine expression
 Serum glucocorticoid levels are directly correlated
with activity and concentration of Na+,K+-ATPase in
the inner ear
 potassium secretion by marginal cells is immediately
increased after the administration of steroids
 Intratympanic administration yields much higher
concentrations of steroids in the inner ear than either
intravenous or oral administration
 Parnes and colleagues:
 intravenous and intratympanic administration
successfully penetrated the blood-labyrinthine
barrier.
 Methylprednisolone had the highest concentration
and longest duration in perilymph and endolymph
 therapeutic efficacy may rely on other mechanisms
of action(Na+-K+ channel activity)
 choice for sudden SNHL and acute vestibular
vertigo
 protocol of oral steroids for inner ear disease is
60 mg of prednisone (or 1 mg/kg/day for adults)
taken for 10 to 14 days in idiopathic sudden SNHL
or for 1 month in suspected autoimmune inner ear
disease
 If hearing loss returns during the taper, a higher
dose of prednisone is restarted
 Relapse of hearing loss is often preceded by tinnitus
 Systemic and intratympanic steroid
therapy has also been used for
treatment of sudden SNHL
 prognostic factors predicting
response –
 initial severity of hearing loss and
time between onset and treatment.[
 There is a high spontaneous
recovery rate of 30% to 60%
 Oral steroid therapy within the
first 2 weeks has shown recovery
rates approaching 80% and
decreasing thereafter
 intratympanic steroids do provide
an excellent method for salvage of
hearing in the case of systemic
steroid treatment failure
 Gianoli and Li
 trial of intratympanic steroids for patients with sudden
SNHL who had failed to improve after high-dose
systemic steroids (1 mg/kg/day of prednisone for a
minimum of 1 week).
 tympanostomy tube placement
 0.5 mL of steroid solution consisting of either 25 mg/mL
of dexamethasone or 62.5 mg/mL of methylprednisolone
 Four treatments were administered over 10 to 14 days,
and audiometric data were recorded 1 to 2 weeks after
treatment
 Kopke and colleagues
 RWM microcatheter -62.5 mg/mL of methylprednisolone
at a continuous rate of 10 ?L/hour for 14 days with an
electronic pump
 Chandrasekhar
 10 patients treated with intratympanic dexamethasone
 6 experienced hearing improvements greater than 10 dB,
however
 Parnes and colleagues
 13 patients 6 showed hearing improvements of 10 dB or
more.
 If intratympanic steroids are to be used
 they should be used as soon as possible after it
becomes clear that oral steroids are not improving
hearing, preferably within the first 2 weeks of the
original insult
 dexamethasone, followed by methylprednisolone
 Intratympanic dexamethasone preparations vary from 1 to 25
mg/mL
 hyaluronic acid preparation consisting of a 1 : 1 mixture of 16
mg/mL of dexamethasone and 0.5 mg/mL of hyaluronate
sodium
 intratympanic methylprednisolone studies use a solution of
62.5 mg/mL
 protocol is designed to fill the middle ear space (which is 0.3 to
0.5 mL
 self-administration through tympanostomy tubes have every-
other-day dosing
 “shotgun” dosing with multiple injections over the first 2
weeks of treatment
 compromise of the immune system leading to
infections, osteoporosis, peptic ulcers, hypertension,
myopathy, ocular effects, impaired healing,
psychologic effects, and avascular necrosis
Advantages to IT steroids
May be used when systemic steroids are
contraindicated or refused
Greater concentration achieved at target
end organ
May be performed in outpatient setting
Possible use for salvage of hearing
Relatively low complication rate
Challenges for IT steroids
Not well established as primary
treatment strategy
Dosing?
Best delivery technique?
Long term effects?
Why does it work? .... Sometimes
 Take Home Messages:
 SSNHL is an otologic emergency
 Systemic steroids are mainstay of therapy
 Prednisone 60 mg/day for 3-5 days, tapered 5-7
days
 Better prognosis if treatment started early (within 4
weeks of onset)
 IT steroids may be an alternative when systemic
steroids are contraindicated
 IT steroids is another option when oral steroids fail
to restore hearing
Steroids in SSNHL

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Steroids in SSNHL

  • 2. First described in 1944 by DeKleyn Incidence: 5-20 per 100,000 4,000 new cases/year in US Idiopathic Hearing loss in 3 contiguous frequencies of at least 30 dB Some authors use at least 20 dB loss Onset of hearing loss occurs in less than 72 hours Recovery rate without treatment 32% - 79% Usually within 2 weeks of onset Only 36% with complete recovery No middle ear disease Otologic emergency!
  • 3.  The two principal indications for intratympanic steroids are sudden sensorineural hearing loss (SNHL) and Meniere's disease
  • 4. • decreasing the number of circulating blood leukocytes and inhibiting the formation and liberation of inflammatory mediators • inhibit the release of chemoattractive and vasoactive factors, decrease the secretion of lipolytic and proteolytic enzymes, and inhibit the release of proinflammatory cytokines • These actions decrease the damage from an inflammatory response, whether the insult is secondary to mechanic, hypoxic, ischemic, infectious, or autoimmunologic causes
  • 5.  On exposure to lipopolysaccharide  cultured endothelial modiolar cells and tissue exhibit a generic response and release proinflammatory cytokines  vasculitis, vascular leakage syndrome, entry of immunocompetent cells, and perivasculitis, ultimately leading to cochlear ischemia, intracochlear tissue damage, and hearing loss  DEXAMETHASONE-interrupt the beginnings of the inflammatory cascade at the level of cytokine expression
  • 6.  Serum glucocorticoid levels are directly correlated with activity and concentration of Na+,K+-ATPase in the inner ear  potassium secretion by marginal cells is immediately increased after the administration of steroids
  • 7.  Intratympanic administration yields much higher concentrations of steroids in the inner ear than either intravenous or oral administration  Parnes and colleagues:  intravenous and intratympanic administration successfully penetrated the blood-labyrinthine barrier.  Methylprednisolone had the highest concentration and longest duration in perilymph and endolymph  therapeutic efficacy may rely on other mechanisms of action(Na+-K+ channel activity)
  • 8.  choice for sudden SNHL and acute vestibular vertigo  protocol of oral steroids for inner ear disease is 60 mg of prednisone (or 1 mg/kg/day for adults) taken for 10 to 14 days in idiopathic sudden SNHL or for 1 month in suspected autoimmune inner ear disease  If hearing loss returns during the taper, a higher dose of prednisone is restarted  Relapse of hearing loss is often preceded by tinnitus
  • 9.  Systemic and intratympanic steroid therapy has also been used for treatment of sudden SNHL  prognostic factors predicting response –  initial severity of hearing loss and time between onset and treatment.[  There is a high spontaneous recovery rate of 30% to 60%  Oral steroid therapy within the first 2 weeks has shown recovery rates approaching 80% and decreasing thereafter  intratympanic steroids do provide an excellent method for salvage of hearing in the case of systemic steroid treatment failure
  • 10.  Gianoli and Li  trial of intratympanic steroids for patients with sudden SNHL who had failed to improve after high-dose systemic steroids (1 mg/kg/day of prednisone for a minimum of 1 week).  tympanostomy tube placement  0.5 mL of steroid solution consisting of either 25 mg/mL of dexamethasone or 62.5 mg/mL of methylprednisolone  Four treatments were administered over 10 to 14 days, and audiometric data were recorded 1 to 2 weeks after treatment
  • 11.  Kopke and colleagues  RWM microcatheter -62.5 mg/mL of methylprednisolone at a continuous rate of 10 ?L/hour for 14 days with an electronic pump  Chandrasekhar  10 patients treated with intratympanic dexamethasone  6 experienced hearing improvements greater than 10 dB, however  Parnes and colleagues  13 patients 6 showed hearing improvements of 10 dB or more.
  • 12.  If intratympanic steroids are to be used  they should be used as soon as possible after it becomes clear that oral steroids are not improving hearing, preferably within the first 2 weeks of the original insult
  • 13.  dexamethasone, followed by methylprednisolone  Intratympanic dexamethasone preparations vary from 1 to 25 mg/mL  hyaluronic acid preparation consisting of a 1 : 1 mixture of 16 mg/mL of dexamethasone and 0.5 mg/mL of hyaluronate sodium  intratympanic methylprednisolone studies use a solution of 62.5 mg/mL  protocol is designed to fill the middle ear space (which is 0.3 to 0.5 mL  self-administration through tympanostomy tubes have every- other-day dosing  “shotgun” dosing with multiple injections over the first 2 weeks of treatment
  • 14.  compromise of the immune system leading to infections, osteoporosis, peptic ulcers, hypertension, myopathy, ocular effects, impaired healing, psychologic effects, and avascular necrosis
  • 15.
  • 16.
  • 17. Advantages to IT steroids May be used when systemic steroids are contraindicated or refused Greater concentration achieved at target end organ May be performed in outpatient setting Possible use for salvage of hearing Relatively low complication rate
  • 18. Challenges for IT steroids Not well established as primary treatment strategy Dosing? Best delivery technique? Long term effects? Why does it work? .... Sometimes
  • 19.  Take Home Messages:  SSNHL is an otologic emergency  Systemic steroids are mainstay of therapy  Prednisone 60 mg/day for 3-5 days, tapered 5-7 days  Better prognosis if treatment started early (within 4 weeks of onset)  IT steroids may be an alternative when systemic steroids are contraindicated  IT steroids is another option when oral steroids fail to restore hearing

Editor's Notes

  1. interferon-γ, granulocyte/monocyte colony-stimulating factor, interleukins, and tumor necrosis factor-α
  2. other forms of steroids have been tolerated much better by middle ear tissues. Dexamethasone seems to be better tolerated and less irritative to middle ear tissues