1. By Saisuree Nivatwongs ENT PMK
SSNHL
By Saisuree Nivatwongs ENT PMK

2. SSNHL Incidence: 5-20 per 100,000 4,000 new cases/year in US
Idiopathic
Hearing loss at least 30 dB over 3 contiguous frequency
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. SSNHL Clinical Presentation Sudden onset hearing loss
Less than 3 days
Usually unilateral
Left side possibly more common (55%)
Bilateral 2%
Median age 40-54
Male = Female
Awakening from sleep
Hearing a “popping” prior to hearing loss
Aural fullness
Tinnitus
Vertigo

4. Etiology Viral infection
Association of SSNHL with viral URI in 25% - 63%
Serology confirming active viral infection
HSV, VZV, CMV, influenza, measles parainfluenza, rubeola, mumps, rubella
Immunoreactivity against virus
Histopathology of human temporal bones
Atrophy of organ of Corti, spiral ganglion, tectorial membrane
Hair cell loss
Unraveling of myelin

5. Etiology Vascular injury Sudden onset suggesting infarction
Perlman (1959) demonstrated loss of cochlear microphonic 60 seconds after occlusion of labyrinthine artery in guinea pig
Polycythemia ,Buerger’s, macroglobulinemia, sickle cell, fat embolism, DM

6. Etiology Intracochlear membrane rupture Autoimmune
Loss of endocochlear potential due to mixing of endolymph and perilymph
Gussen (1981) histologic evidence
Fallen out of favor
Autoimmune
cross-reacting circulation Ab

7. DDX Infectious Bacterial: meningitis, labyrinthitis, syphilis
Viral: Mumps, CMV
Inflammatory
Autoimmune, Cogan syndrome, Lupus, MS
Traumatic
Temporal bone fracture, acoustic trauma, perilymph fistula
Neoplastic
CPA tumor, temporal bone metastasis
Toxic
Aminoglycosides, aspirin
Vascular
Thromboembolism, macroglobulinemia, sickle cell disease, cerebral infarct, TIA
Congenital
Mondini malformation, enlarged vestibular aqueduct

8. Clinical Evaluation Hx Complete ENT exam
Audiogram include PTA, SRT, SDS
Tympanogram
ABR

9. Radiograph
MRI with Gd
0.8-2 %of pt with SSNHL have been diagnosed CPA /IAC tumors
Non-contrasted CT temporal bone : R/o congenital malformation

10. Laboratory Evaluation
CBC with diff
Polycythemia, leukemia, thrombocytosis
FBS , Electrolytes
Erythrocyte sedimentation rate (ESR)
FTA-Abs (Syphilis)
Coagulation profile
Thyroid function testing
Lipid profile
BUN, Creatinine UA
ANA , rheumatoid factor , viral study

11. Prognosis Severity of HL Audiogram shape Presence of vertigo Age
Without Rx : % will experience complete or partial recovery

12. Medical Rx Bed rest Low salt diet < 2 gm/day Diuretics : HCTZ
Steroids

13. Steroids Historical perspective: Reduce inner ear inflammation
Rx as early as posible
Oral, IV 10 days
Cannot be used for all patients
Diabetics, ulcers, TB, glaucoma
Intratympanic steroids

14. Medical Rx Antivirals Volume expanders : Dextran , hypaque
Vasodilators : Histamine phosphate , Ca antagonist , nicotinic acid
Anticoagulants : heparin
Carbogen inhalation (95%O2,5%CO2)

15. SSNHL Cochrane Database of Systematic Reviews
Wei (2003, Updated 2006): Steroids for idiopathic sudden sensorineural hearing loss
Only 2 prospective, double-blind, randomized, controlled trials evaluating therapy of SSNHL

16. Intratympanic steroid
Administration of steroids to middle ear round window niche/membrane directly targeting the inner ear
Very little systemic absorption
May benefit patients for whom systemic steroids are contraindicated
Higher concentration to end organ
May salvage hearing loss when non-responsive to systemic steroids

17. Advantage of 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. Take Home Messages: SSNHL is an otologic emergency
Systemic steroids are mainstay of therapy
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

19. Thank you for your attention