Sudden and Fluctuant

Sensorineural Hearing Loss

Prakash Adhikari
2nd Year M.S.Resident (ENT-HNS)
Ganesh Man Singh Memorial Academy of ENT and
Head and Neck Studies,
TU Teaching Hospital, IOM,Kathmandu,Nepal
ROADMAP………
 Introduction
 Definition
 Causes
 ISSNHL
 Investigations
 Prognostic factors
 Treatment modalities
 Conclusion
 Take home message
Introduction-SSNHL
 Devastating to patients

 Frustrating for physicians

 Definitive diagnosis and treatment still unknown

 First described by De Klevn in 1944

Statistics

 15,000 reported cases per year worldwide

 4,000 cases per year in the U.S.
 Nepal et al study in TUTH-46 cases in between
2002-2005
 Highest:: 50-60 years
 Lowest:: 20-30 years
 M=F
 2% bilateral
 90% of cases are idiopathic
Definition

 Several
 Most accepted:
 30dB SNHL
 More or equal to 3 contiguous frequencies
 Less than 3 days (Wilson et al)
 More or equal to 20dB (Haberkamp and Tanyeri)
 More or equal to 20dB in no more than 1 week
(Kronenberg et al)
Causes of sudden or fluctuating
SNHL
COCHLEAR
 Traumatic
 Inflammatory
 Vascular
 Haematological conditions
 Autoimmune disease
 Endolymphatic hydrops
 Metabolic disorders
 Skeletal system
 Ototoxicity
 Miscellaneous
Retrocochlear and central nervous
system
 Meningitis
 Multiple sclerosis
 Friedrich’s ataxia
 Amyotrophic lateral sclerosis
 Xeroderma pigmentosum
 Tumors
 Central deafness
Idiopathic
Cochlear causes- Traumatic
 Electricity
 Rupture of tympanic membrane
 From telephone during thunderstorm
 Cordless-complex signal
 3 cases reported-HL-during use of
cordless during lightening (Singleton et al 1984)
Traumatic HL
 Breaks in the membranous labyrinth
 Intracochlear – Meniere’s
 Oval and/or round window – perilymph fistula

 History – inciting event

 Blow to the head
 Lifting a heavy object
 Exposure to sudden changes
in barometric pressure
 High risk population
 Post stapedectomy
 Inner ear anomalies
Traumatic SHL
 Treatment
 Avoid lifting > 10 lbs.
 Avoid straining
 After 5 days
 If improvement – 6 weeks of light
activity
 If no improvement – surgery
 Middle ear exploration
 Patching of perilymph fistula
Inflammatory-Bacterial

Acute otitis media

 Very few cases are reported
Typhoid fever
 2nd to 3rd weeks
 Usually unilateral
 Female preponderance
 Reversible in few cases
Spirochates- syphilis
Congenital
 B/L profound HL-fluctuation
 Involve low and high
frequencies\poor speech
discrimination
Late syphilis
 5%present with SSNHL
 Sudden detoriation in 15%
Mycoplasmas
 Case reported-1984 by Nishioka et al.
 B/L involvement
 Treated with minocycline n steroids
 No improvement
Bullous myringitis
 Rare association
 Untreated cases-
improvement in few
 M. pneumonia also isolated
Chlamydia
 C. trachomatis and C. psittaci
 Darouger et al, 1978, reported
 B/L involvement
 No improvement with steroids
Brucellosis
 B. melitensis. B.abortus, B. suis
 Vestibulocochlear nerve commonly
affected
 Serological tests
 Tetracycline and rifampicin
 Failed to reverse hearing loss
Lyme disease- B.burgdorferi
 Transmitted by tick
 Facial palsy commonly associated
 Ix- IFA.ELISA,IMMUNOBLOTTINGS
 Treated with Benzyl penicillin
 No satisfactory results
Rickettsiae-typhus
 R. prowazekii
 Hearing loss-early and serious

 two cases. (Tsiachris D et al.2008).

 resolved automatically without administration of specific
treatment.
Viral
Mumps
 Incidence-0.1%,80% unilateral
 Three common presentation
 Insidious unilateral complete
 Unilateral partial
 Bilateral complete
Murakami and Muzushima,1985,n=53
 HL- unilateral, profound and permanent
 Haematogenous infection theory
 Inflammatory change
 Measles
 5-10%
 Measles rash
 Assymetric permanent B/L HL affecting
higher frequencies
 Suzuki et al-mumps deafness in one
ear and rubella infection following HL
in opposite ear
 Improved with betamethasone
Chickenpox
 Reported in a 14 month old infant-
1983
 B/L sudden HL
 No improvement
Varicella zoster virus
 Sudden deafness with facial palsy
 Oral acyclovir
 Watch for RFT

 70% had herpetic infections (Wilson-1986,n-60)

 MRI- enhancement in facial and cochleovestibular
nerves

concluded relation of herpes simplex
Koide et al-1988,n-60 -
virus and sudden deafness
 reactivation of a latent infection - role in the aetiology
Human spumaretrovirus-HSRV
 Few cases were reported in 90s,
 isolated from patients neoplastic and
degenerative diseases
 Bilateral HL
Infectious mononucleosis
 1% nervous system involvement
 Temporary bilateral HL
Lassa fever
 Arenavirus infection
 Cummins et al-1990,n- 69
29% incidence HL
 Liao et al study 1992,n-12
88% bilateral
 Ribaviarin started
 Improvement in 8 cases
HIV AND AIDS
 one third had abnormal PTA (Brichall et al,1992,n-18)

 Reported as a presenting symptom – (Timon

and Wash, 1989)

 Bilateral HL
 Neurotropic and lymphotrophic virus-never
been cultured from 7th cranial nerve or
spiral ganglion cells. (Morris and prasad,1990)
 Temporal bone reports by AIDS. (Michaels et al
1994)
Protozoa
Toxoplasmosis
 Lymphadenopathy-commonest
 Hearing rarely affected
Katholm et al,1991
 SSNHL-Both ear.
 Improved hearing with sulphadiazine
and pyrimethamine
Buerger’s disease
Iatrogenic
 Radiotherapy
 Significant correlation between total
radiation dose to inner ear and
observed hearing impairment. (Garu et al,
1991,n-22)

 Higher frequencies involved

Postoperative

 Microembolism involving cochlear division of the IAC.

 Pump filters may prevent microemboli
 Hypothermia produces latencies increased of wave I-IV.
(Ness et al 1993)
 Almost always unilateral
 adrenalectomy (Journeaux et al 1990)

Hochermann and Reimer 1987 - bilateral HL
Spinal anaesthesia
 Transient hearing loss reported

Panning etal 1983,n-100::: in urological
patients found 8 cases.
 Audiogram done in 3 cases
 Low frequency involvement
 Improved with no therapy
 WANG 1987.Fog et al 1990 and Sundberg et al 1992
 Shape & position- tip of the needle
 26 was to preferred than 22G
Lee and Peachman,1986 reported
 SSNHL following C/S,
 Blood patch with 10ml
 Hearing improved.
Hardt,1998 reported similar result

 occurs in wholly or partially patent cochlear aqueduct

and occurs via the release of perilymph into the CSF.
Vascular cause
HYPERTENSION
 Dobozi nad Greig (1972)- no evidence of atherosclerosis

 Drettner et al (1975)- low frequency hearing was

related to cardiovascular disease events

 Kikuchi et al (1993),n=102 MRI - slow blood flow in

the vertebrobasilar system

 Mark et al (1992)- MRI with Gad scan showed

cochlear enhancement on the side of HL.
 Hypercoagulation
 Recurrent episodes of thrombosis
 Stria vascularis has a slow blood flow in those with a high
haematocrit value

 no difference with coagulation test. (Lalanne et al 1992 n=79)

Viscosity

 strong correlation between whole blood viscosity and hearing

impairment at all frequencies. Gatehouse et al (1989)

 hydroxyethyl starch pentoxifylline infusions-superior results.

(Desloovere et al 1991)
Haematological
 Anaemia- rarely associated
 Case reports on iron def, megaloblastic and aplastic anaemia
 Prognosis poor

Polycythaemia vera

 Cerebral blood flow significantly reduced

 B/L SSNHL reported

 Improved after phlebotomy ( Davis and Nilo 1995).

 Sickle cell disease
 Gradual onset/ fluctuant or SSNHL
 Sickling and impaired blood flow in the cochlear venous system
with secondary anoxia of the hair cells and stria vacularis.
 Bilateral SSNHL Mace AD et al J Laryngol Otol. 2008

Thalassaemia
 B/L high freq SNHL in a patient on desferrioxamine
(Back,1980, n=52)
 (Oliveri et al, 1986,n=89)-22::: abnormal Hearing
 Low serum Ferritin was also a risk factor
Waldenstrom’s macroglobulinaemia
 Characterized by retinal changes,
abnormal bleeding tendencies,
generalized weakness and dyspnoea
Leukaemia
 Common in ALL
 CLL- few reported
 Treatment with cyclophosphamide and
prednisolone improved hearing
 Cryglobulinaemia
 SLE, multiple myeloma or macroglobulinaemia

Autoimmune disease

 Closely related- 30% associated with SSNHL

 ANA,ATA +ve
 CD3+,CD4+ peripheral lymphocytes and
marked decrease of CD8+.
Non-organ specific autoimmune disease

SLE
 Cyclophosphamide and steroids
fail to improve hearing
 ANF, Ds DNA ACLA
 Systemic vasculitis
 ANCA, proteinase 3 and myeloperosidase
PAN
 b/L deafness, treated with prednisolone improved
 Obliterative vasculitis of the labyrinthine artery or its
branches resulting in diffuse or focal areas of
ischaemic necrosis
Cogan’s syndrome
 4% deafness in syphilitic keratitis
(Cogan 1945)
 Non syphilitic keartitis- progressive HL
 Aortitis – 10%

 Hearing fluctuates with disease exacerbations and

remissions
 Majority develop bilateral deafness (67%)
 MRI shows narrowing or obliteration of parts of the
vestibular labyrinth
 STEROIDS/ COCHLEAR IMPLANTATION
Relapsing polychondritis
 Ocular lesions, HL and dizziness
 U/L or B/L
 Usually accompanied by abrupt
cessation of steroids and reduction of
medicines
 Six diagnostic criteria
 Wegener’s granulomatosis
Three principal components
 NGL in upper or lower respiratory tract
 Generalized focal necrotizing vasculitis of arteries and veins
 GN
 Occ. reversible
 Treated with cyclophosphamides and steroids
Giant cell arteritis
 B/L, sudden
 Biopsy
 Protein electrophoresis
 Prednisolone
Kawasaki disease
 Self limited acute vasculitis
 7th cranial nerve palsy
 Diagnostic criteria
 Mechanism of SSNHL not known
 Usually B/L. CHL may be present
 Takayasus disease
 Absence of pulse in upper extremities due to
the obstruction of the aortic arch branches
 HPE- no vasculitis

Kanzaki et al 1993,n-17- 7 had SSNHL
 No improvement of steroids
Behcet’s disease

Brama and Fainaru,1980,n-16-
 10 had SSNHL.
Endolymphatic hydrops
 5% develop SSNHL

Hallberg 1956- 57/1270.

Takahara et al, 1974,Sando et al-1977: found two
cases at autopsy
 Fluctuant HL
 Classic symptoms- endolymphatic
hydrops
 GTT/PTA
Metabolic disorders
Renal failure
 80% of case with ARF and 52.4% with
CRF improved with treatment.
 B/L symmetrical
Alport’s syndrome

 Slowly progressive and high freq involved

 C/F: HTN, proteinuria and haematuria
 Gubler et al 1981,n-58, one present as deafness,
37 showed HL
 Always bilateral
 PTA- trough shaped, sloping and flat ( Pintelman,
1976)
 Temporal bone studies- degeneration of stria
vascularis, loss of cochlear neurons, atrophy of
spiral ligaments or loss of hair cells
Renal transplantation
 Improve hearing after transplant

Mc Donald et al 1978 - HL following
transplant,n-4.
 Neomycin/ cyclosporin
Renal tubular acidosis
IgA nephropathy
Diabetes Mellitus
 Diabetics- deafer in lower freq than
control
 Wilson et al 1982- diabetics fail to
recover as well in higher frequencies
 Nottingham 1981- did not find any
difference between two groups on PTA.
 Central disturbance of auditory
pathway and microvascular
complication
 Wackym and Linthicum 1986
 Microangiopathic involvement of
endolymphatic sac had significant greater
HL.
 Diet- severe HL,hypoglycemia oral, insulin-
less loss
Refsum’s disease
 AR
 Retinitis pigmentosa, chronic
polyneuropathy, ataxia
 Anosmia, HL (cochlear), cardiopathy
Hyperlipidaemia

 Not signficant correlation. Drettner et al 1975, n -1000.

Hypothyroidism
 Symmetrical B/L HL
 ? Association
 -13% improved hearing
Vant Hoff and Stuart 1979.n-48
when they became euthyroid
 No improvement- Parving et al 1983,n-15
TUTH STUDY
 45.7% HAD SYSTEMIC DISEASE
 DM alone-7
 HTN alone-3
 Hyperlipidemia alone-2
 DM+HTN-6
 DM+Hyperlidemia-2
 DM+HTN+Hyperlipidemia-1
Ototoxicity
 Interferon

 Contraceptive pills

 Dantrolene/Mianserin- sudden B/L HL- no imrovement

 Sudden sensorineural hearing loss after heroin

injection. Schrock A, Eur Arch Otorhinolaryngol. 2008
May;265(5):603-6.
 Iloprost-induced sudden hearing loss. Dursun E, J
Laryngol Otol. 2007 Jun;121(6):609-10.

 Iloprost may be a potentially ototoxic drug, causing sudden

hearing loss.
 completely reversed in eight days with conservative therapy.
Vaccination
 Tetanus antitoxin. n-1, in tetanus
toxoid. (Mair and Elverland,1977,n-8)
 Whoopong cough, rabies, small pox.
 But not on diptheria or oral polio
 Cause- local hypersensitivity or Ag-
Ab rxn
CO Intoxication
 CarboxyHb- deprive vital organs of
O2
 Chronic- permanent symmetrical high
freq loss
 Acute- B/L asymmetric.improved in 4
weeks.
 May be fluctuant
 U shaped audiogram
Miscellaneous
Ulcerative colitis
 B/L HL
 Steroids + immunosuppresives responded. Jacob et al
1990
Scleroderma
 Cyclophosphamide- B/L mixed HL- improved
Sarcoidosis
 SSNHL may be only complaint
 Fluctuant B/L
 Increase calcium, ESR and ACE,CXR- hilar adenopathy
 Biopsy- non caesating granuloma
 Prednisolone- improve hearing
Retrocochlear and CNS
 Meningitis- B/L
 Cryptococcal meningitis and sudden
deafness (Maslan et al 1985)
 Viral and TB can also cause
Multiple sclerosis
 3% have hearing problem
 25% vertigo
 Multiple areas of demyelination of CNS
 U/L
 Absence of wave II to IV
Friedrich’ ataxia
 No obvious cause
Amyotrophic lateral sclerosis (Van
Laere’s deiseae)
 Hearing loss first manifestation
 VKH syndrome
 B/L HL
 Returned to normal

Xeroderma pigmentosa
 B/L HL absence of tone decay, absent
stapedial reflexes
Tumors
 Vestibular schwannoma
 10% sudden (Higgs 1973,n-44)
 In between 10-15%
 MRI with Gadolinium- compression of
vasculature within the bony IAM

 Metastasis in CP angle: from breast,

bronchus, prostate.
Central deafness
 U/L or B/L

Earnest 1977, B/L cerebral infarcts
 B/L cerebral lesions- Tanka et al,1991.
 Creutzfeldt-Jakob disease (Tobias et
al,1994) report a case with cortical
deafness
Lacunar syndromes
 Cortical encephalitis
 No improvement no worsening
 HL- first symptom
 B/L assymetric HL
 Speech discrimination-poor
 Prednisolone + cyclophosphamide
Alzheimer’s disease
 Loss of dendritic spines and axionic
collaterals
Noise

SSNHL- following concerts
Emmett, 1994:
 Acute acoustic trauma
Idiopathic SSNHL
 Definitions
 Several
 Most accepted:
 30dB SNHL
 More or equal to 3 contiguous frequencies
 Less than 3 days (Wison et al)
 More or equal to 20dB (Haberkamp and
Tanyeri)
 Moe or equal to 20dB in no more than 1
week (Kronenberg et al)
 Proper history
 Noise trauma, barotrauma, direct
temporal bone trauma, medication
History
 Time course
 Associated symptoms
 Vertigo/dizziness
 Aural fullness
 Tinnitus
 Ototoxic drug use
 Symptoms of URTIs
 H/O head trauma, straining, sneezing, nose
blowing, intense noise exposure
 H/O flying or SCUBA diving
History
 PMH:
 Autoimmune disorders
 Vascular disease
 Malignancies
 Neurologic conditions
 Hypercoagulable states
 Sickle cell disease (African Americans)

 PSH: stapedectomy or other otologic

surgeries
Physical Exam
 Complete H&N exam in everyone
 Ears: r/o effusions, cholesteatoma, cerumen
impaction
 Weber/Rinne
 Neurologic exam – cerebellar findings
 Tandem gait
 Romberg
 Nose to finger, heal to shin
 Vestibular – Dix-Hallpike test
Suggested categorization of SSNHL

 SSNHL of specific cause

 Possible idiopathic SSNHL or probable

idiopathic SSNHL

 Definite idiopathic SSNHL

Epidemiology
 Incidence:
 Vary according to age and possibly
sex
 8 per 100,000 per year (Wu et al,2006).
 Specific cause found in less than 5%
 Equal in both gender
 Bilateral less than 1%
Age distribution
 Shai and Sheehy (1976)
 Three fourth are more than 40 years
 4% B/L
 Adult B/L-possibility of HIV
Etiopathogenesis
 Postulated causes
 Viral infection
 Vascular occlusion
 Membrane breaks
 Immunological
 Activation of cochlear nuclear factor
kappa B.
 Schuknecht- vascular lesons, membrane breaks and
viral cochleitis (Temporal bone studies)
 Hypothesis-activation of cochlear NFkB by
endogenous and exogenous stimuli.- no direct proof.
 Immunologic phenomena- Vasama and
Linthicum.2000,n-12.
 Vascular aetiology (Merchant et al) –extensive cochlear
fibrosis and inner ear ossifications-not convincing.
 Other studies-disturbance of microciculation of the
cochlea produce cochlear damage and hyperviscosity
with sludging of red cells and consequent hypoxia.
Temporal bone studies
 Shows viral cochleitis (schunknecht and
Denovan,1986,n-12).

ISSNHL due to
Kheterpal,1990.n-12:
neurotrophic viruses
Precipitating Factors
 Preceding viral infection:30-40%
 3 group of viruses
 1. virus causing ARD- Influenza,
parainfluenza, rhinoviruses
 No confirmed seasonal viruses
 2.Poliovirus,coxsackie,rubella,EBV,adenovir
us type 3 & HSV
 3. Mumps, measles and varicella zoster
Prognosis
 47%-63% spontaneously resolve

 Four prognostic variables

 Time since onset
 age
 Tinnitus / Vertigo
 Audiogram type
Prognosis
Prognosis
 Age
Tinnitus:
 80% alarming, 25% preceeding
 Dose not affect the outcome
 In Danino et al, 1984- favorable
prognostic sign
 Tinnitus present in 71% who recovered
compared with 39% who did not
Prognosis
 Vertigo – 29% affected vs. 55% not
affected
Audiogram type
Prognosis
 8KHz
Wilson (1980)
 Prognostic factors
 Vertigo not statistically significant
 Age less than 40 years favorable for recovery
 Type of audiogram
 Midfrequency loss with best recovery

 Profound loss less likely to have recovery

 Loss between 40 dB – 85 dB more likely to

respond to steroid therapy

Investigations
Battery of tests
 CBC
 RFT, Electrolytes
 Glucose
 Lipid profile
 Serology
 Autoantibodies
 TFT
 MRI

 +/- Auditory brainstem response (ABR) and otoacoustic

emission (OAE)
 ENG if vestibular symptoms and/or signs are present
 Audiogram
 Pure tone
 Speech discrimination
 Tympanometry
 Stapedial reflex
Our protocol
 Hb,TC,DC,ESR
 Bl.Glucose F/PP
 LFT
 Coagulation profile
 TFT
 Lipid profile
 RFT
 ANA
 Serlogy
 Urine RME
 CXR
 MRI in selected cases
 PTA- EVERY 3RD DAY
AIED
 Diagnosis
 Based on Hearing loss and response to
treatment
 Hughes –
 Lymphocyte transformation test
 Sensitivity – 50-80%
 Specificity – 93%
 Positive predictive value 56-73%
 Western blot
 Sensitivity – 88%
 Specificity – 80%
 Positive predictive value – 92%
Diagnostic Testing
 MRI:
 Rule out cerebellopontine angle tumors
 Multiple sclerosis
 ischemic changes
 13% of patients with acoustic tumors
present with SHL
 23% may recover hearing
 2000 survey of 100 ENTs (43%
otologists) in the United Kingdom
 78% - CBC, ESR, Syphilis serology
 38% - MRI on initial visit
 98.5% - steroids
 41% - Carbogen
 31% - acyclovir
Outcomes
 Complete spontaneous recovery seen in 50-
75%
 Wilson defined recovery into three cateories
 Complete-within 10dB
 Partial recovery-within 50%
 No recovery-less than 50%
 Cinamon et al- 75% spontaneous recovery,
Wilson et al 47% spontaneous recovery
  

Treatment
Treatment
 Therapy for ISSNHL is controversial
 Difficult to study
 High spontaneous recovery rate
 Low incidence
 Makes validation of empiric treatment
modalities difficult
TREATMENT
 Increasing cochlear blood flow
 No proven value
 Betahistine-histamine like effect on
H1 receptors in the cochlea
vasculature leading to increase in
cochlear blood flow
 Glycerol-iv in rabbit
Pentoxiflline / oxpentyfylline

 Phosphodiesterase inhibitor and

haemorheological agent
 Increase oxygen delivery to tissues
 Iv given in SSNHL of vascular origin
 Probst et al,1992: no significant
diffeence.
 Calcium antagonists
 Increase cerebral blood flow
 Nifedipine, nimodipine
 No significant difference with other drugs
(eg:nimodipine Vs naftidrduryl)
 Further trials needed
Diatizone meglumine (Hypaque)

 1 ml of iv hypaque prior to vetebral angiography-

significant hearing improvement
 Huang et al 1989-not better than spontaneous
recovery-65%.
Low molecular weight Dextran (Rheomacrodex)

 10%solution either in 5% dextrose or in normal saline

 no significant benefit
 Allergic rxn

Hydroxyethyl starch/Hetastarch-Hespan

 Artificial colloid derived from waxy starch-

myelopectin
 6% colloidal solution in 0.9% sodium chloride
 No difference in hearing
 Pruritis
5% carbon dioxide. 95% oxygen
(carbogen)

 Cerebral vasodilator
 10% co2 –dangerous
 After carbogen inhalation- perilymphatic oxygenation of
8.6mmHg rose to 14.8mmHg
 95% o2 and 5% Co2 for 30 mins 8 times per day at intervals of
one hour. (Fisch et al 1984)
 3/4th pts improved
Giger 1979,n-55:
 Carbogen or iv infusion with papaverine and Dextran
 No difference on 5th day but better result at 1 year
 It mat improve spontaneous rate recovery in sudden deafness

 Grandis et al study.1993- same as spontaneous recovery (65%)

Hyperbaric oxygen
 No proper trials
 Can be considered
Anticoagulants
 Heparin-chosen in initial stages
 Reduce serum lipid level
 Also binds with histamine
 Donaldson 1979.n-23: iv heparin sodium-
recovery (70%) same as spontaneous
(66%)
Defibrinogenation therapy
Batroxobin
 Reduction in serum fibrinogen
 Thrombin like enzyme-Gicopetide
 Prostaglandins
 Synthesized by vascular structures of the lateral wall
of the inner ear (stria vascularis and spiral ligament)
 PGI2 and PGF2alpha found in perilymph
 Inj Aspirin intraperitoneally-decrease in perilymph
 Olszewski et al 1985-n-22, prostacyclin:55%recovery,9
partial recovery
 Olszewski et al 1990: 87% complete recovery
Other therapies
 Vitamins- B1,B12,B6,C,A,E
 INTERFERON
 Iron therapy
 Zinc
 Ginko extract
 diazepam
Treatment
 1987 – Wilkins and associates
 “shotgun” regimen – dextran, histamine,
Hypaque, diuretics, steroids,
vasodilators, carbogen
 No difference between treated and non-
treated patients
 No control group
 Treatment for only three days
Steroid therapy
 Treatment of choice when loss is
retrocohclear
 Wilson et al,1980: double blinded clinical
trial-stastically significant effect on
hearing recovery in patients with
moderate HL

Dose of oral steroids:

RCTs
 acyclovir+ prednisolone Vs Prednisolone:
no difference (Stokroos 1998), (Uri 2003)
 prednisolone / placebo / carbogen:
no difference (Cinamon 2001)
 carbogen Vs carbogen+iv Mgso4:
later is better (Gordin 2002)
 prednisolone +acyclovir Vs prednisolone+
acyclovir+intratympanic methylprednisolone:
methylprednisolone better (Xenellis 2006)
Cinamon (2001)
 Results
 Overall improvement in PTA at follow-up (73%)
 Steroid 80%

 Placebo 81%

 Carbogen 55%

 Placebo inhalation 77%

 Not statistically significant

 Trends:
 Low frequency loss improved more
 High frequency loss improved less
 Patients without vertigo have better outcome
The Newest Treatment:
Intra-Tympanic Dexamethasone

 IT dexamethasone, methylprednisolone &

hydrocortisone all results in higher
perilymph concentration of steroid than
systemic delivery of steroids
Parnes (1999)
 Potency corrected
levels in perilymph
after IT
administration
Parnes (1999)
 Potency corrected
levels in endolymph
after IT
administration
Otolaryngology–Head and Neck Surgery (2007) 137, 74-78

 Intratympanic steroid treatment

improved hearing loss in (55%).
 Kopke (2001)

 IT Delivery via Microcatheter

 Methylprednisolone (62.5 mg/mL) delivered
continuously for 14 days at rate of 10 µL/hour
using pump
Silverstein (2002)
 N-48,
 refractory hearing loss after systemic steroids
 Dexamethasone 4-24 mg/mL with MicroWick
 23% had improvement of PTA of at least 10
dB
Silverstein (2002)
 Group Study Steroid Delivery Improvement

Xenelis Prospective, Methylprednisolone Injection 47% (PTA)

(2006) controlled

Batista Prospective, Dexamethasone Injection 20% (PTA)

(2005) PSSNHL
Slattery Prospective Methylprednisolone Injection 55% (PTA/SDS)
(2005)

Banerjee Retrospective Methylprednisolone Via PET 69%, ≤10 days (PTA)

(2005) review Dexamethasone 31%, >10 days

Herr Retrospective Dexamethasone MicroWick 53% (PTA/SDS)

(2005) review Microcatheter

Guan-Min Prospective, Dexamethasone Injection 53% Treatment (PTA)

(2004) randomized, 7% Control
contolled

Silverstein Academy Dexamethasone MicroWick 23% (PTA)

(2002) Presentation 35% (SDS)

Gianoli Prospective Dexamethasone Via PET 44% (PTA)

(2001)

Kopke (2001) Prospective Methylprednisone Microcatheter 100%,≤6wk(PTA/SDS)

0% >6wk
Sudden Sensorineural Hearing Loss
 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
Sudden Sensorineural Hearing Loss
 Challenges for IT steroids
 Not well established as primary
treatment strategy
 Dosing?
 Best delivery technique?
 Long term effects?
 Why does it work? .... Sometimes
 No high quality evidence to support
the contention that any particular
treatment in ISSNHL is more effective
than placebo
 ? Risk of short course of oral steroids
Ongoing Study
 National Institutes for Health (NIH)
study
 Multi-centre RCT
 Oral steroids vs. intra-tympanic steroids
Conclusion
 No statistically significant benefit
of:
 Steroids over placebo
 Anti-virals plus steroids over steroids alone
 Steroids over other active treatment

 Wilson’s often-misquoted study was not

a randomized controlled trial
Conclusion
 Questionable internal & external
validity but positive results favouring:
 Batroxobin
 Magnesium
 Vitamin E
 Hyperbaric oxygen
 Intra-tympanic dexamethasone
Conclusion
 SHL is devastating to patients
 Frustrating for physicians to dx and tx
 Thorough H&P
 Rule out treatable cause
 Directed labs, Audiogram, MRI
 Discuss risks, benefits, and alternatives of treatment with
the patient
 Treat the disorder aggressively
 Rehabilitate those whose hearing does not improve
 Follow patients for development of associated diseases
and for contralateral ear disease
Sudden Sensorineural Hearing Loss
 Challenges
 True incidence is not known
 Patients with spontaneous recovery
usually do not present to an
otolaryngologist
 Patients may present beyond what is considered
to be therapeutic window
 Etiology still unclear
 Relative paucity of studies examining treatments
based on prospective, double-blind, randomized,
controlled trials
Sudden Sensorineural Hearing Loss
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