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Sudden sensori-neural hearing loss

Sudden sensori-neural hearing loss. Dr. Vishal Sharma. Defining triad (Wilson, 1980). Sensori-neural deafness of > 30 dB HL over > 3 contiguous frequencies occurring in < 3 days Within 12 hrs: Cummings. Synonyms & alternatives.

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Sudden sensori-neural hearing loss

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  1. Sudden sensori-neural hearing loss Dr. Vishal Sharma

  2. Defining triad (Wilson, 1980) • Sensori-neural deafnessof > 30 dB HL • over > 3 contiguous frequencies • occurring in < 3 days • Within 12 hrs: Cummings

  3. Synonyms & alternatives • Sudden sensorineural hearing loss is also called acute cochlear dysfunction • Sudden sensorineural hearing loss accompanied by acute vertigo is also calledacute cochleo-vestibular dysfunction • Deafness occuring over days or weeks is called rapidly progressive hearing loss

  4. Epidemiology • Annual incidence (USA) is5 - 20 cases / 1 lakh • 47-70 % resolve spontaneously (do not report) • True incidence rate is higher • Gender not a risk factor • Unilateral cases: 96-99% • Bilateral cases: 1-4% • Left ears are affected more (55%)

  5. Etiology • Idiopathic (single largest group: 90 - 95%) • Cochlear causes • Retro-cochlear causes • Miscellaneous • Psychogenic • Malingering

  6. Criteria for idiopathic SSNHL • SSNHL present • No other cranial nerve involvement except eighth cranial nerve • No other etiology is known

  7. Idiopathic SSNHL Various hypotheses are: 1. Labyrinthine viral infection (viral cochleitis) 2. Labyrinthine vascular compromise 3. Membrane rupture 4. Immune-mediated inner ear damage 5. Activation of cochlear nuclear factor kappa B

  8. 1. Labyrinthine viral Infection (20 – 40 %) • Herpes, mumps, measles, maternal rubella, cytomegalovirus, varicella zoster 2. Labyrinthine vascular compromise • caused by thrombosis, embolus, reduced blood flow, vasospasm • Western diet (rich in saturated fat), alcohol intake & tobacco smoking are predisposing factors

  9. 3. Membrane rupture (Simmons) • Pts hear pop sound before sudden deafness • Oval & round window perilymph fistulae leak perilymph into middle ear  low perilymph pressure & relative endolymphatic hydrops • Rupture of intra-cochlear membranes  mixing of perilymph & endolymph altering endo-cochlear potential

  10. 4. Immune-mediated inner ear damage • Antigen-antibody complex mediated destruction of cochlea • Cross-reacting circulating antibodies seen in 65 % pt of SSNHL. Associated conditions are:  Cogan syndrome  Relapsing polychondritis  Systemic lupus erythematosus  Polyarteritis nodosa  Temporal arteritis

  11. 5. Activation of cochlear nuclear factor kappa B Merchant et al (2005) proposed this new theory Nuclear factor kappa B (NFҚ B) functions by:  regulating inflammatory response + apoptosis  regulating intracellular Ca & neuronal excito-toxicity NFҚ B activation is associated with destruction of spiral ganglion neurons & cochlear hair cells causing ISSNHL

  12. Cochlear causes 1. Infection:bacterial, viral, spirochaetal, mycoplasma 2. Trauma:temporal bone #, acoustic trauma, barotraumas, perilymph fistula, radiotherapy 3. Vascular:hyper-coagulable states, thrombo- embolism, hypertension, migraine 4. Hematological:polycythemia, leukemia, anemia

  13. Cochlear causes 5. Oto-toxicity:aminoglycoside, aspirin, frusemide, antimalarials, cisplatin 6. Endolymphatic hydrops 7. Metabolic:diabetes mellitus, hypothyroidism, hyperlipidemia, renal failure 8. Auto-immune:Cogan syndrome, systemic lupus erythematosus, relapsing polychondritis

  14. Retro-Cochlear causes • Meningitis • Encephalitis • Tumor: Vestibular schwannoma, other tumors of cerebello-pontine angle • Multiple sclerosis • Metastasis

  15. Clinical Features • Medical Emergency • Sensori-neural hearing loss • Tinnitus:seen in 60 - 70% pt • Vertigo: seen in 20 - 40% pt • Aural fullness:seen in 15 - 30% pt • Viral URTI: seen in 20 - 40% pt

  16. Patient Evaluation

  17. Early diagnosis & Tx improves prognosis • Deafness: onset, duration, severity, previous HL • Associated vertigo / tinnitus / aural fullness • Exclude trauma (noise / baro / temporal bone #) • Exclude ototoxicity / DM / hypothyroidism / blood dyscrasia / hyperlipidemia / renal failure • Tuning fork tests & fistula test • Perform careful neurological examination

  18. Basic Laboratory Investigations

  19. Complete Blood Count + ESR: forinfection • BT, CT, PT, aPTT & INR: forbleeding disorder • VDRL, FTA-Abs, TPHA, TPI: forsyphilis • ANA, Rh factor, other auto-antibody titre • T3, T4, TSH:for hypothyroidism • FBS & PPBS:for diabetes mellitus • Fasting lipid profile:for hyperlipidemia • Urea & Creatinine:for renal failure

  20. Imaging Studies 1. MRI with gadolinium contrast (gold standard):  1-2% pt with ISSNHL have IAC or CPA tumors  3-12% pt with acoustic neuroma have SSNHL 2. CT scan temporal bone + contrast • Detect anatomic defects (Mondini dysplasia or enlarged vestibular aqueduct syndrome)

  21. Contrast M.R.I.: acoustic neuroma

  22. Mondini dysplasia sac-like cochlea (black arrow) amorphous vestibule without any defined semicircular canals (white arrow) enlarged vestibular aqueduct (red arrow)

  23. Enlarged vestibular aqueduct

  24. Audiometry • Pure-tone Audiometry • Speech Audiometry • Tympanometry & acoustic reflex tests • SISI & Tone Decay Test • Oto-acoustic emission • BERA

  25. High-frequency hearing loss:PTA at 4 & 8 kHz exceeds PTA at 250 & 500 Hz by > 30 dB • Low-frequency hearing loss:PTA at 250 & 500 Hz exceeds PTA at 4 & 8 kHz by > 30 dB • Flat-type hearing loss:equal hearing losses at each frequency • Profound hearing loss:no response at maximum intensity for > 2 frequencies • Reference : Nakashima T, et al. Laryngoscope 1993;103:1145-49.

  26. Presence of OAE indicates preservation of some outer hair cell function • ABR reflects function of neural pathways • ABR & OAE results also assist in diagnosing psychogenic hearing loss & malingering • Vestibular tests are obtained when indicated by history & physical examination

  27. Treatment

  28. Treatment options (a) Vasodilators (b) Rheologic agents (c) Anti-inflammatory agents(Steroids) (d) Anti-viral agents (e) Diuretics (f) Hyperbaric oxygen (g) Surgery

  29. General Treatment • Bed rest & avoid strenuous exercise • Avoid following aggravators:  Alcohol Smoking  Stress Sleep deprivation  CNS stimulants Fatty diet  Straining Loud noise

  30. Vasodilators: reverse hypoxia • Betahistine: 16 mg TID, PO for 3 wk • Xanthinol nicotinate:300 mg slow IV Q12H  500 mg BD, PO for 3 wk • Carbogen (5% CO2 + 95% O2) inhalation: for 30 min, 8 times / day at 1 hour intervals in O.T. • Nimodipine:30 mg BD-TID, PO for 3 wk

  31. Rheologic Agents  blood viscosity to  blood flow & O2 delivery • Low-molecular-weight dextran:10 ml / kg / d X 7d • Pentoxifylline:400 mg TID, PO for 3-4 wk • Diatrizoate meglumine infusion:40 ml/d X 7d • Hydroxy-ethyl starch:500-1000 ml/d X 7d • Anticoagulants (heparin & warfarin):obsolete

  32. Cortico-Steroids • Anti-inflammatory agents • Prednisolone:1mg / kg / d in single or divided doses for 10 d  taper over 3 weeks • Intratympanic dexamethasone solution(8 mg/mL): 0.3–0.4 mL with hyaluronidase on alternate days after grommet insertion in PIQ

  33. Grommet in P.I.Q.

  34. Post-steroid recovery

  35. Side-effects of Steroids • Hyperglycemia • Hypertension • Gastric ulceration • Osteoporosis • Flaring of infection & delayed wound healing • Psychiatric disturbance (insomnia, euphoria) • Weight gain & trunk obesity

  36. Anti-virals & Diuretics • Anti-virals • Acyclovir: 800 mg PO, 5 times / day for 7 days • Famciclovir: 250 mg PO, TID for 7 days • Diuretics • Used in SSNHL due to endolymphatic hydrops • Hydrochlorothiazide: 25 mg PO, BD for 3-4 wk

  37. Hyperbaric oxygen • Consists of exposure to 100% oxygen at pressure of 250 kPa for 60 minutes in a multi-place hyperbaric chamber along with high doses of gluco-corticoids • Best results achieved if treatment started early

  38. Surgery • Repair of oval & round window perilymph fistulae has been used in cases of ISSNHL associated with positive fistula test or history of recent trauma or barotrauma • No standard methods are detailed

  39. Result evaluation (Wilson) • Complete recovery: • PTA or SRT: < 10 dB of pre-SSNHL value • Partial recovery: • PTA / SRT: > 50% recovery of pre-SSNHL value • No recovery: • PTA / SRT: < 50% recovery of pre-SSNHL value

  40. Result evaluation • Patient with pre-SSNHL value of: • Pure Tone Average = 30 dB • Speech Reception Threshold = 30 dB • Complete recovery: PTA or SRT  30 - 40 dB • Partial recovery: PTA or SRT  41 - 45 dB • No recovery: PTA or SRT > 45 dB

  41. Spontaneous Recovery • Spontaneous recovery rates for SSNHL range from 47 - 70%, combining categories of complete & partial recovery • Most spontaneous recoveries occur within 2 weeks

  42. Results • No high-quality, randomized, controlled trial shows efficacy of any medical therapy • Most studies don't show significant beneficial effect of vasodilators, acyclovir, rheological agents, hyperbaric oxygen over placebo • Corticosteroid therapy is only accepted therapy for ISSNHL. Recovery rates = 40 - 60%

  43. Favorable prognosis • Tx starting <10 days after onset of SSHL • Mild to moderate SNHL • Low or mid frequency SNHL • Presence of tinnitus (doubtful significance)

  44. Unfavorable prognosis • High frequency deafness (especially 8 kHz) • Hearing loss > 90 dB HL • Vertigo / vestibular changes evident on ENG • Bilateral sensori-neural deafness • Tx starting >15 days after onset of deafness • Age < 15 years or > 65 years • Elevated ESR (>25) • Poor speech discrimination score

  45. Further Study

  46. Leong, A.C. et al. (2007). Sudden hearing loss - A 12 minute consultation. Clinical Otolaryngology. 32: 391–394 • Xenellis J. et al. (2006). Idiopathic sudden sensorineural hearing loss: prognostic factors. J.L.O. 120, 718–724 • Xenellis J. et al. (2006) Intra-tympanic steroid treatment in ISSNHL. Otolaryngol. Head Neck Surg. 134, 940–945 • Aoki D. et al. (2006) Evaluation of superhigh-dose steroid for SSNHL. Otolaryngol. Head Neck Surg. 134, 783–787 • Bennett M. et al. (2005) Hyperbaric oxygen therapy for ISSNHL & tinnitus: J. Laryngol. Otol. 119: 791-798, • Wilson W. et al. (1980) The efficacy of steroids in the treatment of ISSNHL. Arch. Otolaryngol. 106, 772–776

  47. Thank You

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