comprehensive note on SSNHL

1. Sudden SensoriNeural Hearing Loss
SSNHL
Dr. K.D.N.U.D.Jayasena
Registrar in ENT Head and Neck Surgery

2. Definition
• Hearing loss greater than
30 dB
• Over 3 contiguous pure-
tone frequencies
• occurring within 3 days'
period.

3. Introduction
Abrupt and rapidly progressive losses .
• Awakening with a hearing loss,
• Hearing loss noted over a few days,
• Selective low- or high-frequency loss,
• Distortions in speech perception.

4. Side
• Commonly Unilateral
• Bilateral is rare
• Simultaneously B/L is very rare. (relatively high mortality
rate 45%)*
*https://www.ncbi.nlm.nih.gov/pubmed/26944135

5. Epidermiology
• Incidence 8-10 per 100000 per year (Scott brown 7th ed)
{but figures may vary due to self limiting disease, non reporting cases}
Sex- Male and female equally affected*
Age- Peak incidence in sixties, median age is 40-54
Rare in children
Bilateral loss is common in childhood, pooerer recovery and 35-45%
mortality rate. (Meta analysis - Sara et. al.)

6. Pathophysiology
• Only 10% to 15% have an identifiable cause. (5% Scott
&Brown)
• The most common causes are:
Infectious diseases,
Trauma such as a head injury, Neoplasms
Autoimmune diseases such as Cogan’s syndrome
Ototoxic drugs

7. Idiopathic ...
The postulated pathophysiology for idiopathic sudden sensory hearing
loss (ISSHL) has theoretical pathways,
• Labyrinthine viral infection
History, (fever)
Examination (rash) and investigations (Lymphocytosis)
Mumps* - not proven
*Okamoto M, Shitara T, Nakayama M, et al. Sudden deafness accompanied by asymptomatic mumps. Acta
Otolaryngol Suppl. 1994. 514:45-8. [Medline].

8. Idiopathic Contd....
• Other infections
Syphilis
Encephalitis

9. Idiopathic contd..
• Labyrinthine vascular compromise
Cochlea is an end organ - No co-laterals.
Thrombosis, Embolus, Spasm
In one study, a partial overlap was found between classical coronary
risk factors and risk factors for sudden hearing loss. ( Chang et al)
Smoking is a risk factor.

10. Blood supply of cochlea

11. Idiopathic..
Intracochlear membrane ruptures.
 Allow mixing of perilymph and endolymph,
 Effectively altering the endocochlear potential.
Immune-mediated inner ear disease.
“The association of hearing loss in Cogan syndrome, systemic
lupus erythematosus, and other autoimmune rheumatologic
disorders has been well documented”.

12. Idiopathic.....
• Other causes
Hypoxia
Barotrauma
Drugs
Sickle cell disease
Meniere's Disease
Tumors

13. Initial workup
• Its an otological emergency.
• Early presentation and management increases the chance of
recovery.
• Immediate goal is to find a treatable cause and treat.

14. Initial workup,
• History-
Onset,
time ,
course ,
associated symptoms - Fever, Myalgia, Rash etc.
• PMH- DM,Ht, Hypercholestorelemia, Use of aspirin(cause reversible
hearing loss- neurotoxicity)

15. Examination
• Otoscopy - For exclude external and middle ear diseases
• Hearing assesment. -To confirm
• Tuning fork tests .- To confirm
• Fistula test using pneumatic speculum must be performed.
-To exclude fistula in bony labyrynth
• Pure Tone Audiometry. - To Confirm, Objective value
• Tympanogramme- Exclude other causes

16. Investigations
• FBC- ( infections, Polycythemia vera, Leukemia)
• VDRL (Syphilis)
• MRI (CP angle tumors, acaustic neuroma etc)
• TSH (Hypothyroidism)
• FBS (DM)
• ESR ( Autoimmune disease)
• Cholesterol (Vascular risk factor)
• SE

17. Treatments - No preferred treatment regimen
exists - 2017
No high-quality, randomized, controlled trial exists demonstrating the
efficacy of any medical therapy in ISSHL.
Hence, no single treatment has been unequivocally shown to be
effective

18. Treatments
Steroids - (NCBI pubmed- Steroids Improves)
Short or reducing doze course. (Oral/IV)
Intra-tympanic
0.5 to 0.8 ml
Posterior lower quadrent
Under LA.
Pt should kept 1hr facing affected ear upword after injecting.
weekly for 1 month

19. Oral steroid vs Intra tympanic

21. Other treatment options
• Antiviral agents. - Aciclovir, amantadine
• Vasodilators.(Nitroglycerine patches for 10 days under hospital
supervision and blood pressure monitoring. The patch contained 10
mg nitroglycerine.)
• Diuretics (Oral diuretics in the form of acetazolamide (250 mg twice a
day) for a total period of one month.)
• Hyperbaric oxygen. (small number of evidence)
• Supportive therapy in the form of multi–vitamins.

22. Recovery...
• Complete - < 10dB
• Partial >/= 50% improvement
• None - Less than 50 % improvement

23. Follow up
• Needed in partial or not recovering patients.
• Hearing aids etc
• Psychological support.

24. Prognosis and prognostic factors
• Good prognosis- presented early and with tinnitus. *
• The outcome was poor in subjects presenting late, and having
associated diabetes or hypertension.
• No relation with vertigo.
• The prognosis for hearing recovery for idiopathic SSNHL is
dependent on a number of factors including the severity of
hearing loss, age, presence of vertigo**
*https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4639688/
**https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4040829/

25. Prognosis
• Without treatments- 32%-79% get some degree of recovery
usually within 2 weeks
• Only 50% get a complete recovery.

26. Mobile phones and SSNHL?
• No evidence.
• Only some case reports available.

27. Take home massages
• SSHNL is an otological emergency
• Better prognosis with early treatment( within 4 wks of onset)
• Systemic steroids are mainstay in theraphy.
• IT steroids is another option. (can use with hyperglycaemics)

28. References
1. Okamoto M, Shitara T, Nakayama M, et al. Sudden deafness accompanied by
asymptomatic mumps. Acta Otolaryngol Suppl. 1994. 514:45-8. [Medline].
2.Idiopathic sudden sensorineural hearing loss,Scott Brown's
Otorhinolaryngology, Head & Neck Surgery 7th Ed pp. 3577-3590). (2008)
3 https://www.ncbi.nlm.nih.gov/pubmed/17676220
4 https://www.ncbi.nlm.nih.gov/pubmed/21610239
5 Rupasinghe R.A.S.T.,Comparison - Effectiveness of oral steroid versus
intratympanic Dexamethasone for sudden onset sensorineural hearing
loss.;Ceylon Journal of Otolaryngology. Volume : 5; Issue1; pp 07-13
6.https://www.ncbi.nlm.nih.gov/pubmed/26944135
7.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4040829/

29. •Thank you...!