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 casesimprovement 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 infant1983 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
Koide et al-1988,n-60 -
concluded relation of herpes simplex 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
Reported as a presenting symptom –
(Brichall et al,1992,n-18)
(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, insulinless loss Refsum’s disease AR Retinitis pigmentosa, chronic polyneuropathy, ataxia Anosmia, HL (cochlear), cardiopathy
Hyperlipidaemia
Not signficant correlation.
Hypothyroidism Symmetrical B/L HL ? Association
Drettner et al 1975, n -1000.
-13% improved hearing when they became euthyroid No improvement- Parving et al 1983,n-15 Vant Hoff and Stuart 1979.n-48
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 AgAb 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 Acute acoustic trauma Emmett, 1994:
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 neurotrophic viruses Kheterpal,1990.n-12:
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 5075% 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 angiographysignificant 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 starchmyelopectin 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 sodiumrecovery (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 nontreated 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 (2006)
Prospective, controlled
Methylprednisolone
Injection
47% (PTA)
Batista (2005)
Prospective, PSSNHL
Dexamethasone
Injection
20% (PTA)
Slattery (2005)
Prospective
Methylprednisolone
Injection
55% (PTA/SDS)
Banerjee (2005)
Retrospective review
Methylprednisolone Dexamethasone
Via PET
69%, ≤10 days (PTA) 31%, >10 days
Herr (2005)
Retrospective review
Dexamethasone
MicroWick Microcatheter
53% (PTA/SDS)
Guan-Min (2004)
Prospective, randomized, contolled
Dexamethasone
Injection
53% Treatment (PTA) 7% Control
Silverstein (2002)
Academy Presentation
Dexamethasone
MicroWick
23% (PTA) 35% (SDS)
Gianoli (2001)
Prospective
Dexamethasone
Via PET
44% (PTA)
Methylprednisone
Microcatheter
100%,≤6wk(PTA/SDS) 0% >6wk
Kopke (2001) Prospective
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