Endolymphatic hydrops surgery

1. SURGICAL INTERVENTIONS FOR
ENDOLYMPHATIC HYDROPS
Dr Kristy Fraser-Kirk
Otologist + Auditory Implant Surgeon
Sunshine Coast University Hospital
Evolve ENT

2. MENIERE’S DISEASE
ENDOLYMPHATIC HYDROPS
Endolymphatic hydrops
 Endolyphatic sac maintains hydrostatic
pressure and endolymph homeostasis for
inner ear
 Sac dysfunction causes progressive accumulation
of endolymph
 Episodic distension, herniation and rupture of
endolymphatic system causes recurrent episodes
of vertigo (hours-days), low frequency SNHL
and tinnitus +/- aural fullness

3. MENIERE’S DISEASE
ENDOLYMPHATIC HYDROPS
Endolymphatic hydrops
 The cause of much despair for patients and
clinicians alike
 ‘Remit-relapse’ natural history
 Progressive but with potential for spontaneous
remission
 Disabling: most morbidity arises from vertigo
attacks
 Treatments have potential to do harm
Essential to have a cohesive, step-wise evidence-
based approach to guide treatment decisions

4. ICON ARTICLE, 2017

5. ICON ARTICLE: 2017
Meniere’s Disease Treatment Priorities
 Reduce the frequency of vertigo attacks
 Reduce the severity of vertigo attacks
 Impair hearing as little as possible
 +/- Improve hearing
 +/- Improve tinnitus

6. MENIERE’S DISEASE
Meniere’s Disease Treatment Priorities
 Reduce the frequency of vertigo attacks
 Reduce the severity of vertigo attacks
 Impair hearing as little as possible
 +/- Improve hearing
 +/- Improve tinnitus
Treatments should be
 Symptomatic
 Tailored to the individual patient’s main concern
 Conservative wherever possible
 Consider the natural history of MD, the
spontaneous resolution rate, and the powerful
impact of the placebo effect

7. MENIERE’S DISEASE
STEP 1: Medical treatment
 Lifestyle
 Sleep (Level 4 evidence)
 Sleep anpoea (Level 2)
 Stress, caffeine, alcohol, tobacco (Level 4)
 Low salt diet
 Vestibular rehabilitation: Between attacks (Level 2)
 Psychotherapy: CBT (Level 2)
 Diuretics: Hydrochlorothiazide > acetazolamide >
chlorthalidone (Level 4)
 Betahistine: 48-96mg/day – up to 288-480mg/d
(Level 1 and 3)
 Meniette: No evidence in Cochrane review (Level 2)

8. MENIERE’S DISEASE
STEP 2: Intratympanic steroids
 Improves both frequency and severity of vertigo
compared with placebo over 24 months (Level 2)
 Dexamethasone more common than
Methylprednisolone
 Methylprednisolone 65mg/mL 2 weeks apart as safe
and effective as gentamicin 40mg/mL

9. MENIERE’S DISEASE
STEP 3: Conservative Surgical Treatments
 Endolymphatic sac surgery
 Controversial, ‘placebo surgery’
 Declining in favour of intratympanic steroids
 Danish studies reviewed by Cochrane 2010, 2013
suggested no evidence of efficacy (Level 2)
 Meta-analysis 2015 Lim et al reported low level
evidence of efficacy
 ICON: Countered as ‘placebo treatments’ (grommet
and mastoidectomy) are not actually placebo, the
Danish studies are erroneous
 ICON: ESS first-line for medical/conservative
failures if patients are young and hearing

10. MENIERE’S DISEASE
STEP 4: Destructive Medical Treatments
 Gentamicin
 Titration protocol, 40mg/mL – repeat injections until
no further vertigo spells (Level 2, Level 4)
 Ablative: Risk of hearing loss and permanent
vestibular loss
 ICON: Gentamicin as destructive method when all
prior have failed in patients with little/no hearing and
good contralateral vestibular reserve
 Hypersensitivity Cohort: Carriers of mitochondrial
mutation of gene MTRNR1 experience complete
deafness following a single exposure of the
aminoglycoside

11. MENIERE’S DISEASE
STEP 5: Surgical Destructive Treatments
Vestibular Neurectomy
 No RCTs in the literature
 ICON: Only after all else has failed for patients suffering
intractable vertigo/drop attacks, poor but serviceable
hearing, good contralateral vestibular reserve
 VN superior to ITG for vertigo control
Labyrinthectomy
 Destroys hearing
 Rarely performed
 Simultaneous cochlear implant to rehabilitate hearing
 More common in Australia/US than Europe. Banned in France.

13. IS DIZZINESS A
“SURGICAL DISEASE?”
Why is it so important to
CRITICALLY EVALUATE
the role of surgery
in Meniere’s Disease?

14. IS DIZZINESS A
“SURGICAL DISEASE?”
The natural history of MD makes it difficult to know
whether what we are doing actually works.
Episodes naturally fluctuate in frequency and severity
Strong tendency to spontaneous remission
Strong placebo effect for medical and surgical treatments
High morbidity = ‘High stakes’, investigator bias
Treatments have potential to cause harm
Mechanisms by which surgery helps ill defined
Quality research difficult to design for surgical
interventions

15. MD AND THE PLACEBO EFFECT
The existence of a multitude of therapies
– all with enthusiastic defenders of their efficacy –
is the best proof of the lack of good therapy.
Hymans van den Bergh

16. MD AND THE PLACEBO EFFECT
Thomsen, 1976: Lithium Studies
 Lithium carbonate, effective in 70%
 Subsequent studies… Placebo effective in 70%
Thomsen, 1979: Glycerol Study
 Glycerol used to select patients for ELS
 Distinguishing treatment effect versus placebo difficult
 Patients with Meniere’s are ‘very good placebo responders’
Torok, 1977
 Review of all available literature
‘almost without exception advocates
of all treatments claimed success in 60-80%’

17. MD AND THE PLACEBO EFFECT
The SHAM Study: Thomsen et al, 1981

18. MD AND THE PLACEBO EFFECT
The Sham Study
 Double blind
 Endolymphatic sac surgery versus ‘placebo’
 Placebo treatment: Simple Mastoidectomy
 Inclusion Criteria:
 Fluctuating hearing loss, tinnitus, vertigo
 Attacks every fortnight
 Duration of disease 6months – 5 years
 Medically well, psychologically well

19. MD AND THE PLACEBO EFFECT
The Sham Study
 Two centres in Copenhagen
 Blinded: Operation and follow-up at different hospitals
 Op note standardised
 Daily dizziness questionnaire:
 Attack severity and frequency
 Hearing loss, tinnitus, dizziness, nausea, vomiting,
aural pressure
 30 patients: 15 ELS Versus 15 Sham
 Lower symptom scores in both groups
No difference between groups.
Placebo mastoidectomy was as effective as sac surgery.

20. MD AND THE PLACEBO EFFECT
Investigators interviewed patients at end of
study to determine ‘total value of the operation’
 Sac surgery ‘very beneficial to 73%’ of patients
 Sham mastoidectomy ‘very beneficial to 80%’

21. MD AND THE PLACEBO EFFECT
‘We have demonstrated that a purely placebo operation
(mastoidectomy) is as effective to all symptoms in MD
an an active endolymphatic shunt operation.
Major differences in symptoms between the
pre- and post-operative conditions NOT between
the active and placebo groups.
By blinding examiners and patients across two hospitals,
the differences between active and placebo groups
disappeared.

22. MD AND INVESTIGATOR BIAS

23. MD AND INVESTIGATOR BIAS
‘The bias effect of the investigator is particularly
pronounced in the dizzy patient’
‘The fluctuating intensity of MD symptoms and their
tendency for spontaneous remission make open
uncontrolled studies without value’.
‘Studies must be double blinded. Both patients and
investigators must be unaware of the treatment.’
‘Any leak in the blind must be considered disastrous. ‘

24. ETHICS OF DOUBLE BLIND
PLACEBO CONTROL

25. ETHICS OF DOUBLE BLIND
PLACEBO CONTROL
No More Sham because… ETHICS.

26. ETHICS OF DOUBLE BLIND
PLACEBO CONTROL
The Helinski Declaration
 ?unethical to withhold treatment thought to be
beneficial but not yet proven
 ?unethical to perform procedures if they are later
proven to be of no value
 ?to what extent should patients be informed about a trial
 ?will/should patients consent to a placebo operation
 ?can surgical risk and/or complications arising from
from placebo procedures be justified in the name of
science “harm today to help tomorrow”
 ?does a patient knowing their procedure may be placebo reduce
psychological ‘buy in’ and thereby reduce their placebo benefit?

27. BEYOND THE SHAM: ESS

28. BEYOND THE SHAM: ESS
Few procedures as controversial as ESS
 The ‘unicorn’ of vertigo surgery
 high rates of vertigo control with low rates
of hearing loss and minimal morbidity
 Many authors believe ESS to be safe and effective
 Vertigo control 80%
 Minimal hearing loss so considered ‘conservative’
BUT…
 Questions around long term efficacy
 Questions around efficacy relative to placebo

29. ENDOLYPHATIC SAC SURGERY

30. BEYOND THE SHAM: THE HISTORY OF
ESS
Endolymphatic Sac Surgery
 Various techniques:
 Decompression to mastoid
 Decompression to subarachnoid space
 Decompression + shunt
 Ligation
 Cochleosacculotomy:
 Shunt via round window to unite cochlear duct and
saccule
 Worsened hearing in 80%
 Poor vertigo control, worsens vestibular function

31. BEYOND THE SHAM: THE HISTORY OF
ESS
Endolymphatic Sac Surgery: Timeline
 1927 Portmann: first ELS surgery
 Mastoidectomy, incised endolymphatic sac
 1962 House: subarachnoid shunt
 1967 Kimura: obliterated endolymphatic duct in
guinea pigs to create hydrops, spiked interest in ESS
 1976: Paparella: ‘Endolymphatic Sac Enhancement’
 Wide dural incision, T-tube to decompress sac + duct
 75 patients, 94% vertigo control, 30% hearing gain
 1981 Thomsen Sham Study: casts doubts
 Patients re-examined 3 years later, still no difference
between ESS and mastoidectomy placebo

32. BEYOND THE SHAM: THE HISTORY OF
ESS
Endolymphatic Sac Surgery: Timeline
 1996 Sudemann: ESS first-line for ‘uncontrollable vertigo’
 2005 Durland: ESS improves symptoms and QOL
 Convert: 10 year follow up
 vertigo resolved in 64.5%, hearing gain in 14.8%
 Similar results by Goto and Kim
 2014 Sood: Systematic review and meta-analysis
 Found both decompression and shunt effective
 75% patients had vertigo control
 Effective short term (12-24mo),long term (>24mo)
 Shunting with sialastic: poorer hearing outcomes

33. SHOULD WE BLOCK THE DUCT
INSTEAD?
Endolymphatic duct ligation
 2015 Saliba: non-blinded RCT
 decompression V ligation
 Bone dissected off duct, ligated with titanium clips
 Decompression controlled vertigo in 37.5%
 Ligation controlled vertigo in 96.5%
 Hearing unaffected in both groups

34. ELS: IMPERFECT BUT OK?
Are we even doing what we think we are with ELS?

35. ELS: IMPERFECT BUT OK?
Are we even doing what we think we are with ELS?
 Temporal bone studies
 sac difficult to identify
 often not decompressed
 often not drained

36. ELS: IMPERFECT BUT OK?
Are we even doing what we think we are with ELS?
 Temporal bone studies
 sac difficult to identify
 often not decompressed
 often not drained
 ESS clearly not perfect, but can be considered an
acceptable alternative to IT gentamicin
 Sood et all: ELS conferred stable/better hearing in 72%,
compared with 1 in 4 hearing loss with gentamicin

37. ELS: IMPERFECT BUT OK?
Are we even doing what we think we are with ELS?
 Temporal bone studies
 sac difficult to identify
 often not decompressed
 often not drained
 ESS clearly not perfect, but can be considered an
acceptable alternative to IT gentamicin
 Sood et all: ELS conferred stable/better hearing in 72%,
compared with 1 in 4 hearing loss with gentamicin
However, is it a fair
alternative to IT steroid?

38. THE RISE OF STEROIDS IN MD

39. THE RISE OF STEROIDS IN MD
An immunological basis for MD?

40. THE RISE OF STEROIDS IN MD
An immunological basis for MD?
 Brookes: high circulating immune complexes in MD
 Endolymphatic sac and stria vascularis
targets for immunological activity
 1997 Shea, systemic and intratympanic steroid
 Vertigo control 63.4%
 Hearing gain 35.4%
 2001 Sennarouglu: IT Dexamethasone at 2 years
 Vertigo control 42%
 Hearing gain 16%

41. THE RISE OF STEROIDS
An immunological basis for MD?
 2005 Shea: Prospective, placebo, double blind RCT
 Intratympanic Dexamethasone versus placebo
 Excellent results for vertigo control and hearing

42. THE RISE OF STEROIDS
An immunological basis for MD?
 2005 Shea: Prospective, placebo, double blind RCT
 Intratympanic Dexamethasone versus placebo
 Excellent results for vertigo control and hearing
Additional benefits of intratympanic steroids over
endolyphatic sac surgery
 Cost effective
 Office based
 No complications
 No side effects
 Immediate availability: Start during acute phase
 No preoperative tests or consultations, no wait time

43. THE RISE OF STEROIDS
Steroid injections are a non-ablating,
safe and effective
treatment for Meniere’s disease

44. THE RISE OF STEROIDS:
PATEL 2016

45. THE RISE OF STEROIDS
Patel 2016
 Double-blind RCT intratympanic gentamicin versus
methylprenisolone
 Two centres in UK
 Inclusion:
 18-70 years
 Unilateral Menieres as per AAO-HNS
 Vertigo: At least 2 episodes over six months
 No response to medical treatment
 Randomised to MP 62.5mg/mL or Gent 40mg/mL
 Investigators blinded
 Two injections over two weeks

46. THE RISE OF STEROIDS
Patel 2016
 Followed over 24 months
 Assessed by:
 Vertigo self-report scales
 PTA
 Speech discrimination: AB words
 Vestibular function tests
 Outcomes:
 Vertigo relief
 Audiovestibular symptoms
 Hearing

47. THE RISE OF STEROIDS
Results
 Both drugs reduced vertigo attacks
 Both better than placebo
 Methylprednisolone superior: 90% versus 87%
 No difference in vestibular symptoms in either group
 MP better speech discrimination
 Same number of non-responders in each group
 No difference in number of injections required
 MP possibly more painful
 Gentamicin: 9 patients lost hearing
 Methylprednisolone: 5 patients lost hearing
 Gentamicin left patients with worse vestibular function
 Risk of acute iatrogenic vertigo, hearing loss, and worse
vestibular reserve post gentamicin therapy

48. THE RISE OF STEROIDS
‘Either steroid or gentamicin may be considered.
The latter in patients who have
no geographic access to repeat injections,
who is not afraid of a gentamicin-induced vertigo attack,
and who doesn’t rely [professionally] on hearing.’

49. THE RISE OF STEROIDS
Steroid injections are a non-ablating,
safe and effective
treatment for Meniere’s disease

50. ELS PLUS STERIODS?
Is there a role for combining surgery and steroids?
 2008 Kitahara:
 Sac decompression with instillation of steroid i
 Three groups
 Sac decompression
 Sac decompression with steroid
 Control group
 Decompression + steroid superior
to decompression alone, especially for hearing
 Both treatments superior to control group

51. JUST A GROMMET?
Kanegaonkar et al 2019
 Retrospective review
 33 patients
 Grommet insertion alone:
 Improved aural fullness
 Reduced vertigo attacks
 Some patients experienced improved SN hearing
 Mechanism?
 Altered tension of ossicular chain limits forces
exerted on basilar membrane
Cheap, office-based, safe, can be used for instillation of
steroid
 More research required: Poor quality study, small sample,
selection bias, retrospective review

52. DESTRUCTIVE SURGERY
Vestibular Neurectomy
 1961 House: Middle Fossa Approach
 1980 Silverstein/Norrell: Retrolabyrinthine approach
 Currently, 92% via posterior fossa approach
 retrosigmoid/retrolabyrinthine craniectomy
 Nerve transected at the cerebellopontine angle
or within the internal auditory canal
 Although ‘hearing preserving’ higher complications than
labyrinthectomy as intra-dural procedure:
 10% CSF leak, 3% wound infection
 Good results 90% vertigo control

53. DESTRUCTIVE SURGERY
Labyrinthectomy
 Transmastoid: SCC and vestibule ‘exonerated of
neuroepithelium’ directly via mastoidectomy

54. DESTRUCTIVE SURGERY
Labyrinthectomy
 Transmastoid: SCC and vestibule ‘exonerated of
neuroepithelium’ directly via mastoidectomy
 Transcanal: Tympanotomy, stapes removed,
bone between round and windows drilled out,
neuroepethelium removed with a hood and suction

59. VERTIGO
 Up to 15% GP presentations
 3-4% Emergency Dept. presentations
 Of all presentations:
HALF ARE OTOLOGIC
 The other half
 Neurologic
 Medical
 Psychological
 Other
Weinreich, HM et al. Overview of Dizziness in Practice. Otolaryngol Clin N Am 54 (2021) 839-852

60. ‘VERTIGO IS A SYMPTOM, NOT A
DISEASE'
 Poorly understood, poorly managed
Treatment priorities:
 Exclude critical differentials
 CVA / arrhythmia
 Rule in neuro-otologic disorders
 BPPV, Endolymphatic hydrops,
vestibular neuritis, labyrinthitis
 Appropriately on-refer others
 Work-up, treat, educate and empower
both patient and primary care colleagues
 Emphasise falls protection:
 US: Falls make up greatest percentage
injuries; 2.6 million non-fatal falls,
21,700 fatal falls per annum

61. WORK SMARTER, NOT HARDER
EXCLUDE RED FLAGS
 Ataxic gait
 Diplopia, vision loss, visual changes
 Cranial nerve deficits
 Slurred speech
 Paraesthesia of face, head, body
 Muscle weakness, incoordination

62. WORK SMARTER, NOT HARDER
ASK HIGH YIELD QUESTIONS
 How long does it last?
 Seconds, mins, hours, constant/days
 Is it triggered by head position?
 Seconds, mins, hours, constant/days
 Does your hearing change?
 Do you experience headache or visual change?
 What makes it better or worse?
 Vestibular suppressants, sleep, busy visual
fields

63. WORK SMARTER, NOT HARDER
APPLY THE SORTING HAT
 BPPV: Duration and positional nature
 Endolymphatic hydrops: Hearing loss, ear
symptoms
 Migraine: personal/family Hx, photophobia,
phonophobia, visual changes, motion sickness
 SCCD: Sound/pressure induced dizziness,
pulsatile tinnitus, ear fullness