Clinical Policy Bulletin: Meniere's Disease Surgery
Aetna considers any of the following surgical procedures medically necessary for the treatment of chronic refractory Meniere's disease (see selection criteria in the Appendix of the background section):
Surgical procedures non-destructive to hearing:
Endolymphatic mastoid shunt
Endolymphatic sac decompression
Lateral semi-circular canal plugging
Perilymphatic fistula patching
Tympanostomy tube insertion
Vestibular nerve decompression
Vestibular neurectomy (nerve section) or neurotomy (including middle fossa or retrosigmoid vestibular neurotomy).
Surgical procedures destructive to hearing:
Translabyrinthine vestibular neurectomy
Note: For bilateral Meniere's disease, ablative treatments are relatively contraindicated due to the risks of bilateral vestibular and cochlear hypofunction.
Meniere's disease (MD) is a pathological condition of the inner ear characterized by vertigo, tinnitus and a progressive loss of hearing. Delayed MD can develop in an ear that was damaged years earlier, usually by viral or bacterial infection. The majority of patients with MD also experience a sense of fullness and pressure in the area of the affected ear. This disorder affects male and female equally; it may occur in children, but has a peak onset between 20 and 50 years of age. The incidence of disease affecting both ears increases to over 40 % with long-term follow-up. Moreover, it has been reported that a high percentage of patients (57 %) had complete resolution of symptoms in 2 years.
The key pathological finding for patients with MD is an increase in the volume of endolymph, in conjunction with distention of the whole endolymphatic system (known as endolymphatic hydrops). However, the exact cause of MD is still unclear, and no treatment has prospectively modified the clinical course of the condition and thereby prevented the progressive hearing loss. Conservative management of patients with MD may include dietary salt restriction (1 to 2 g sodium daily) with or without diuretic (e.g., hydrochlorothiazide, dyazide, furosemide, amiloride, acetazolamide, and methazolamide), avoidance of caffeine, alcohol and nicotine. For acute attacks, vestibular suppressants (e.g., benzodiazepines and diazepam) or anti-emetics (e.g., anti-cholinergics such as glycopyrrolate, anti-dopaminergics such as droperidol, prochlorperazine, and anti-histamines such as dimenhydrinate, diphenhydramine, meclizine, and promethazine) have been used. Medical ablation of the inner ear with systemic administration of ototoxic aminoglycosides, such as streptomycin and gentamicin, has been useful in advanced bilateral MD when poor but aidable hearing precludes surgical intervention. Moreover, the indications for this approach are limited, especially with the advent of intra-tympanic placement of gentamicin.
For MD patients whose vertiginous symptoms are disabling and refractory to dietary and medical treatments, surgery may be the last resort in achieving relief. In general, surgical procedures for MD can be categorized as non-destructive or destructive regarding to hearing. The former includes endolymphatic sac surgery and vestibular nerve section (vestibular neurectomy), while the latter includes labyrinthectomy (extirpation of the labyrinth) and cochleosacculotomy.
Endolymphatic sac surgery is the most frequently employed conservative surgical approach for patients with MD when hearing is still serviceable. It has been reported to achieve complete or substantial control of vertigo in 81 % of patients, with significant improvement in hearing in about 20 % of patients. Although it has shown to be less likely to completely eliminate vertigo than vestibular nerve section, endolymphatic sac surgery has been reported to be a low morbidity procedure. Endolymphatic sac decompression and sacculotomy are two common types of endolymphatic sac surgery. In the former procedure, some of the bone surrounding the inner ear is removed. In some cases, endolymphatic sac decompression is coupled with the placement of an endolymphatic shunt. Sacculotomy entails the implantation of a permanent device that allows endolymph to drain out of the inner ear whenever pressure builds up.
Brinson et al (2007) compared the effectiveness of endolymphatic mastoid shunt (EMS, n = 88) versus endolymphatic sac decompression (ESD, n = 108) without sac incision for the treatment of MD. The American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) guidelines for the diagnosis and evaluation of therapy in MD were used to retrospectively identify suitable candidates for the study. All patients who failed medical management and underwent either EMS or ESD were selected for review using the AAO-HNS guidelines. The study was carried out at a tertiary care neurotology private practice. EMS and ESD were equally effective in reducing the incidence and severity of vertigo attacks with significant improvement in 67 % and 66 % of patients, respectively. The authors concluded that both EMS and ESD are effective, non-destructive alternatives for patients who have failed medical management of MD with similar long-term hearing outcomes.
Available medical literature suggests that vestibular nerve section can be performed by exposing the middle fossa or the posterior fossa. The posterior fossa vestibular neurectomy can be further divided into the retrolabyrinthine approach, the retrosigmoid-internal auditory canal approach, and the combined retrolabyrinthine-retrosigmoid approach. Vestibular nerve section has been shown to be generally very effective in the control of vertigo. According to available literature, vestibular nerve section is contraindicated in persons with vertigo arising from the only hearing ear (i.e., the other ear is deaf); central nervous system disease; persons in poor medical condition; persons with ataxia; and in most cases of bilateral MD.
Labyrinthectomy is indicated for patients with symptoms who have poor or non-serviceable hearing. There are several approaches to the labyrinth -- transcanal labyrinthectomy with section of the posterior ampullary nerve, transmastoid labyrinthectomy, and transmeatal cochloevestibular neurectomy. For the older patients (greater than 60 years of age), available medical literature suggests that a transmastoid labyrinthectomy may be preferable to a transcanal labyrinthectomy because the incidence of permanent post-surgical imbalance is less with the former approach. Labyrinthectomy has been reported to produce similar or better results than vestibular neurectomy. Cochleosacculotomy creates a permanent fistula in the cochlear duct by passing a small pick through the round window. It is mainly used for elderly patients with poor hearing who are unable to tolerate a prolonged operation.
Lacombe (2009) stated that spontaneous recovery or central compensation makes surgical procedures rare in patients with vertigo. The main target in treating MD is to promote vestibular compensation, which is possible only with a non-progressive and stable deficit leading to readjustment of vestibular reflexes. Surgical procedures can be classified as non-destructive (endolymphatic sac decompression, vestibular nerve decompression, patching of perilymphatic fistulas), selectively destructive (middle fossa or retrosigmoid vestibular neurotomy, lateral semi-circular canal plugging) and destructive (labyrinthectomy). Surgical indications essentially concern incapacitating vertigo and depend mainly on hearing status. In MD, vestibular neurotomy can be regarded as the gold standard considering its good results on vertiginous episodes; however, scoring with functional and quality-of-life scales bring out residual deficiency in some cases.
In a prospective, follow-up study, Charpiot and colleagues (2010) assessed the safety and effectiveness lateral semi-circular canal plugging to control vertigo in severe MD. A total of 28 MD patients with refractory vertigo and severe disability (functional scale 5 or 6) were included in this study. Lateral semi-circular canal plugging was performed in the pathological ear for each patient. The evaluation of therapy followed the guidelines for diagnosis and evaluation of therapy in MD. Hearing, frequency of vertigo, and functional disability were assessed in the early follow-up (6 months) for all the patients and in the late follow-up (2 years) for 16 patients. In addition, canal paresis was evaluated by the caloric test. No vital complication occurred. The hearing was preserved in 82 % of cases. Lateral semi-circular canal plugging induced in all cases canal paresis that was persistent after 2 years. After 2 years (n = 16), the control of vertigo was complete or substantial in 75 % of cases (restoration of a normal life = 62.5 %; no functional restriction =12.5 %). The authors concluded that lateral semi-circular canal plugging is a safe procedure that induces canal paresis and allows a good control of vertigo. In view of these results, lateral semi-circular canal plugging should be a therapeutic option for controlling rotatory vertigo in severe MD.
In a review on bilateral MD, Nabi and Parnes (2009) stated that for bilateral MD, ablative treatments are relatively contraindicated due to the risks of bilateral vestibular and cochlear hypofunction.
Selection criteria for surgery for Meniere's disease:
Member has disabling vertigo; and
Member has exhibited symptoms (hearing loss, tinnitus, vertigo) for at least 2 years; and
Member has failed conservative management, including dietary restrictions (avoidance of caffeine, alcohol and nicotine; low sodium), and medical therapy (anti-emetic, diuretic, and vestibular suppressants); and
Member has unilateral Meniere's disease and hearing loss is severe to profound in the involved ear (for labyrinthectomy only).
CPT Codes / HCPCS Codes / ICD-9 Codes
CPT codes covered if selection criteria are met:
ICD-9 codes covered if selection criteria are met:
Meniere's disease, unspecified
Active Meniere's disease, cochleovestibular
Active Meniere's disease, cochlear
Active Meniere's disease, vestibular
ICD-9 codes not covered for indications listed in the CPB:
Inactive Meniere's disease
Other ICD-9 codes related to the CPB:
320 - 349.9
Diseases of the central nervous system
388.30 - 388.32
389.00 - 389.9
Dizziness and giddiness
Abnormality of gait
Lack of coordination
The above policy is based on the following references:
Goksu N, Bayazit Y, Beder L. Posterior fossa vestibular nerve section for the management of peripheral vertigo. Eur Arch Otorhinolaryngol. 1999;256(5):230-232.
Rosenberg SI. Vestibular surgery for Meniere's disease in the elderly: A review of techniques and indications. Ear Nose Throat J. 1999;78(6):443-446.
Tewary AK, Riley N, Kerr AG. Long-term results of vestibular nerve section. J Laryngol Otol. 1998;112(12):1150-1153.
Sajjadi H, Paparella MM, Williams T. Endolymphatic sac enhancement surgery in elderly patients with Meniere's disease. Ear Nose Throat J. 1998;77(12):975-982.
Langman AW, Lindeman RC. Surgical labyrinthectomy in the older patient. Otolaryngol Head Neck Surg. 1998;118(6):739-742.
Saeed SR. Diagnosis and treatment of Meniere's disease. Br Med J. 1998;316:368-372.
Grant IL, Welling DB. The treatment of hearing loss in Meniere's disease. Otolaryngol Clin North Am. 1997;30(6):1123-1144.
Silverstein H, Rosenberg S, Arruda J, Isaacson JE. Surgical ablation of the vestibular system in the treatment of Meniere's disease. Otolaryngol Clin North Am. 1997;30(6):1075-1095.
Brookes GB. The role of vestibular nerve section in Meniere's disease. Ear Nose Throat J. 1997;76(9):652-663.
Knox GW, McPherson A. Meniere's disease: Differential diagnosis and treatment. Am Fam Physician. 1997;55(4):1185-1194.
Atlas JT, Parnes LS. Intratympanic gentamicin titration therapy for intractable Meniere's disease. Am J Otol. 1999;20(3):357-363.
Silverstein H, Arruda J, Rosenberg SI, et al. Direct round window membrane application of gentamicin in the treatment of Meniere's disease. Otolaryngol Head Neck Surg. 1999;120(5):649-655.
Moffat DA. Endolymphatic sac surgery: Analysis of 100 operations. Clin Otolaryngol. 1994;19:261-266.
Schwager K, Baier G, El-Din N, et al. Revision surgery after saccotomy for Meniere's disease: Does it make sense? Eur Arch Otorhinolaryngol. 2002;259(5):239-242.
Fukuhara T, Silverman DA, Hughes GB, et al. Vestibular nerve sectioning for intractable vertigo: Efficacy of simplified retrosigmoid approach. Otol Neurotol. 2002;23(1):67-72.
Moody-Antonio S, House JW. Hearing outcome after concurrent endolymphatic shunt and vestibular nerve section. Otol Neurotol. 2003;24(3):453-459.
Kitahara T, Kondoh K, Morihana T, et al. Surgical management of special cases of intractable Meniere's disease: Unilateral cases with intact canals and bilateral cases. Ann Otol Rhinol Laryngol. 2004;113(5):399-403.
Kaylie DM, Jackson CG, Gardner EK. Surgical management of Meniere's disease in the era of gentamicin. Otolaryngol Head Neck Surg. 2005;132(3):443-450.
Van de Heyning PH, Wuyts F, Boudewyns A. Surgical treatment of Meniere's disease. Curr Opin Neurol. 2005;18(1):23-28.
Durland WF Jr, Pyle GM, Connor NP. Endolymphatic sac decompression as a treatment for Meniere's disease. Laryngoscope. 2005;115(8):1454-1457.
Paparella MM. Endolymphatic sac revision for recurrent intractable Meniere's disease. Otolaryngol Clin North Am. 2006;39(4):713-721, vi.
Brinson GM, Chen DA, Arriaga MA. Endolymphatic mastoid shunt versus endolymphatic sac decompression for Meniere's disease. Otolaryngol Head Neck Surg. 2007;136(3):415-421.
Kitahara T, Kubo T, Okumura S, Kitahara M. Effects of endolymphatic sac drainage with steroids for intractable Meniere's disease: A long-term follow-up and randomized controlled study. Laryngoscope. 2008;118(5):854-861.
Li CS, Lai JT. Evaluation of retrosigmoid vestibular neurectomy for intractable vertigo in Ménière's disease: An interdisciplinary review. Acta Neurochir (Wien). 2008;150(7):655-661.
Nabi S, Parnes LS. Bilateral Ménière's disease. Curr Opin Otolaryngol Head Neck Surg. 2009;17(5):356-362.
Lacombe H. Surgery for vertigo. Neurochirurgie. 2009;55(2):268-271.
Charpiot A, Rohmer D, Gentine A. Lateral semicircular canal plugging in severe Ménière's disease: A clinical prospective study about 28 patients. Otol Neurotol. 2010;31(2):237-240.
Pullens B, Giard JL, Verschuur HP, van Benthem PP. Surgery for Ménière's disease. Cochrane Database Syst Rev. 2010;(1):CD005395.
Albera R, Canale A, Parandero F, et al. Surgical indication in Menière's disease therapy: Clinical and epidemiological aspects. Eur Arch Otorhinolaryngol. 2011;268(7):967-972.
Goto F, Tsutsumi T, Ogawa K. Lateral semicircular canal plugging with endolymphatic sac decompression as new surgical treatment for intractable Meniere's disease. Acta Otolaryngol. 2012 Mar 11. [Epub ahead of print]
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