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Harcourt and Cosentino are summarizing current problems and controversies surrounding the topic of Menière’s disease and express justified hopes that the results of the BEMED trial will act as a trigger to generate efforts to develop new and innovative approaches to the problem of Menière’s disease. We are sharing this hope and would like to comment on a few specific aspects mentioned in this editorial.
The authors mention endolymphatic hydrops as a „common pathophysiological element“ which has been observed in Menière’s disease (MD). In this context, it should not be forgotten that MD has been defined as the „idiopathic syndrome of endolymphatic hydrops“ [1]. A recent meta-analysis of all published histological reports on temporal bones of MD patients has confirmed that 100% of patients fulfilling the AAO-HNS criteria of definite MD show endolymphatic hydrops [2]. The presence of hydrops, however, is not strictly linked to the full-blown and simultaneously appearing clinical triad of symptoms, and recent evidence from imaging of endolymphatic hydrops has shown that the time delay between between hearing loss and vertigo is more than 5 years in 20% of the patients [3]. The emerging evidence from hydrops imaging has severely questioned the still widely remembered concept of so-called „asymptomatic hydrops“ . The latter was based on small series of hydropic temporal bones of patients that appeared not to suffer from definite MD [4]. These post-mortem cases, however, naturally lack the possibility of thorough clinical examination and – on a closer look - suffer from various inner ear symptoms like vertigo, hearing loss and tinnitus. Likewise, in our clinical experience, we have not yet encountered a patient with endolymphatic hydrops and an absence of inner ear symptoms. It is important to recognize two findings that have been known from histologic studies and are now being confirmed with more detailed clinical informations by imaging studies of endolymphatic hydrops:
1) hydrops may arise spontaneously, like in MD, but also as a consequence to a number of different inner ear pathologies including for example those caused by vestibular schwannoma [5] and
2) the clinical features associated with hydrops constitute a broad spectrum of inner-ear symptoms ranging from individually appearing auditory or vestibular symptoms (including vertigo attacks shorter than 20 minutes!) to the full-blown picture of simultaneous longer-lasting audiovestibular attacks and permanent functional deficits in the inter-paroxysmal phase (summarized in [6]).
Furthermore, recent studies have shown that the severity of hydrops is positively correlated with the extent of functional deficits in MD patients [7-10]. Therefore, the current state of knowledge supports the traditional notion that hydrops is the pathophysiological hallmark of Menière’s disease, even though we do not yet know the exact etiology of hydrops nor the exact mechanisms of how it may cause the various inner ear symptoms. Nevertheless, being able today to visualize endolymphatic hydrops in living patients at the same time as documenting their precise clinical and functional features , we will need to adapt our terminology, since clinical reality has shown us that there is more than just „MD“ or „not MD“. One possible solution to this dilemma is the terminology of „Hydropic Ear Disease“ which can be further specified by the prefix „primary“ (i.e. Menière’s disease) or „secondary“ and the suffix „auditory“ / „vestibular“ / „audiovestibular type“, including the specification of „suspected / definite / certain“ according to the availability of MR imaging or other objective evidence of endolymphatic hydrops.
The authors also mention endolymphatic sac surgery. While this is undoubtedly a controversial topic we feel it would be fair to also mention the other side of the coin, i.e. the re-evaluation of the highly popular Thomsen-Bretlau study, which has indeed claimed a significant benefit of sac surgery over placebo [11]. Unfortunately, the level of evidence on this topic has not been very high ever since. A recent and very enthusiastic report on the success of a procedure of endolymphatic duct blockage [12] is an example for new approaches in surgical treatments for MD that are still awaiting to receive the necessary attention and funding for controlled high-quality clinical trials.
References
1. AAO-HNS, Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere's disease. American Academy of Otolaryngology-Head and Neck Foundation, Inc. Otolaryngology - Head & Neck Surgery, 1995. 113(3): p. 181-5.
2. Foster, C.A. and R.E. Breeze, Endolymphatic hydrops in Meniere's disease: cause, consequence, or epiphenomenon? Otol Neurotol, 2013. 34(7): p. 1210-4.
3. Pyykko, I., et al., Meniere's disease: a reappraisal supported by a variable latency of symptoms and the MRI visualisation of endolymphatic hydrops. Bmj Open, 2013. 3(2).
4. Rauch, S.D., S.N. Merchant, and B.A. Thedinger, Menieres Syndrome and Endolymphatic Hydrops - Double-Blind Temporal Bone Study. Annals of Otology Rhinology and Laryngology, 1989. 98(11): p. 873-883.
5. Jerin, C., et al., Endolymphatic hydrops in a patient with a small vestibular schwannoma suggests a peripheral origin of vertigo. Austin J Radiol, 2015. 2015(6): p. 1033.
6. Gurkov, R., et al., What is Menière's disease? - a contemporary re-evaluation of endolymphatic hydrops. J Neurol, 2016. in press.
7. Gurkov, R., et al., MR volumetric assessment of endolymphatic hydrops. Eur Radiol, 2015. 25(2): p. 585-95.
8. Gurkov, R., et al., In vivo visualized endolymphatic hydrops and inner ear functions in patients with electrocochleographically confirmed Meniere's disease. Otology & Neurotology, 2012. 33(6): p. 1040-5.
9. Gurkov, R., et al., Herniation of the membranous labyrinth into the horizontal semicircular canal is correlated with impaired caloric response in Meniere's disease. Otology & Neurotology, 2012. 33(8): p. 1375-9.
10. Yamamoto, M., et al., Relationship between the Degree of Endolymphatic Hydrops and Electrocochleography. Audiology and Neuro-Otology, 2010. 15(4): p. 254-260.
11. Welling, D.B. and H.N. Nagaraja, Endolymphatic mastoid shunt: a reevaluation of efficacy. Otolaryngology - Head & Neck Surgery, 2000. 122(3): p. 340-5.
12. Saliba, I., et al., Endolymphatic duct blockage: a randomized controlled trial of a novel surgical technique for Meniere's disease treatment. Otolaryngol Head Neck Surg, 2015. 152(1): p. 122-9.
Competing interests:
No competing interests
23 February 2016
Robert Gürkov
otorhinolaryngologist
Prof. Eike Krause, Department of Otorhinolaryngology, LMU Munich
Re: Betahistine for Meniere’s disease
Harcourt and Cosentino are summarizing current problems and controversies surrounding the topic of Menière’s disease and express justified hopes that the results of the BEMED trial will act as a trigger to generate efforts to develop new and innovative approaches to the problem of Menière’s disease. We are sharing this hope and would like to comment on a few specific aspects mentioned in this editorial.
The authors mention endolymphatic hydrops as a „common pathophysiological element“ which has been observed in Menière’s disease (MD). In this context, it should not be forgotten that MD has been defined as the „idiopathic syndrome of endolymphatic hydrops“ [1]. A recent meta-analysis of all published histological reports on temporal bones of MD patients has confirmed that 100% of patients fulfilling the AAO-HNS criteria of definite MD show endolymphatic hydrops [2]. The presence of hydrops, however, is not strictly linked to the full-blown and simultaneously appearing clinical triad of symptoms, and recent evidence from imaging of endolymphatic hydrops has shown that the time delay between between hearing loss and vertigo is more than 5 years in 20% of the patients [3]. The emerging evidence from hydrops imaging has severely questioned the still widely remembered concept of so-called „asymptomatic hydrops“ . The latter was based on small series of hydropic temporal bones of patients that appeared not to suffer from definite MD [4]. These post-mortem cases, however, naturally lack the possibility of thorough clinical examination and – on a closer look - suffer from various inner ear symptoms like vertigo, hearing loss and tinnitus. Likewise, in our clinical experience, we have not yet encountered a patient with endolymphatic hydrops and an absence of inner ear symptoms. It is important to recognize two findings that have been known from histologic studies and are now being confirmed with more detailed clinical informations by imaging studies of endolymphatic hydrops:
1) hydrops may arise spontaneously, like in MD, but also as a consequence to a number of different inner ear pathologies including for example those caused by vestibular schwannoma [5] and
2) the clinical features associated with hydrops constitute a broad spectrum of inner-ear symptoms ranging from individually appearing auditory or vestibular symptoms (including vertigo attacks shorter than 20 minutes!) to the full-blown picture of simultaneous longer-lasting audiovestibular attacks and permanent functional deficits in the inter-paroxysmal phase (summarized in [6]).
Furthermore, recent studies have shown that the severity of hydrops is positively correlated with the extent of functional deficits in MD patients [7-10]. Therefore, the current state of knowledge supports the traditional notion that hydrops is the pathophysiological hallmark of Menière’s disease, even though we do not yet know the exact etiology of hydrops nor the exact mechanisms of how it may cause the various inner ear symptoms. Nevertheless, being able today to visualize endolymphatic hydrops in living patients at the same time as documenting their precise clinical and functional features , we will need to adapt our terminology, since clinical reality has shown us that there is more than just „MD“ or „not MD“. One possible solution to this dilemma is the terminology of „Hydropic Ear Disease“ which can be further specified by the prefix „primary“ (i.e. Menière’s disease) or „secondary“ and the suffix „auditory“ / „vestibular“ / „audiovestibular type“, including the specification of „suspected / definite / certain“ according to the availability of MR imaging or other objective evidence of endolymphatic hydrops.
The authors also mention endolymphatic sac surgery. While this is undoubtedly a controversial topic we feel it would be fair to also mention the other side of the coin, i.e. the re-evaluation of the highly popular Thomsen-Bretlau study, which has indeed claimed a significant benefit of sac surgery over placebo [11]. Unfortunately, the level of evidence on this topic has not been very high ever since. A recent and very enthusiastic report on the success of a procedure of endolymphatic duct blockage [12] is an example for new approaches in surgical treatments for MD that are still awaiting to receive the necessary attention and funding for controlled high-quality clinical trials.
References
1. AAO-HNS, Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere's disease. American Academy of Otolaryngology-Head and Neck Foundation, Inc. Otolaryngology - Head & Neck Surgery, 1995. 113(3): p. 181-5.
2. Foster, C.A. and R.E. Breeze, Endolymphatic hydrops in Meniere's disease: cause, consequence, or epiphenomenon? Otol Neurotol, 2013. 34(7): p. 1210-4.
3. Pyykko, I., et al., Meniere's disease: a reappraisal supported by a variable latency of symptoms and the MRI visualisation of endolymphatic hydrops. Bmj Open, 2013. 3(2).
4. Rauch, S.D., S.N. Merchant, and B.A. Thedinger, Menieres Syndrome and Endolymphatic Hydrops - Double-Blind Temporal Bone Study. Annals of Otology Rhinology and Laryngology, 1989. 98(11): p. 873-883.
5. Jerin, C., et al., Endolymphatic hydrops in a patient with a small vestibular schwannoma suggests a peripheral origin of vertigo. Austin J Radiol, 2015. 2015(6): p. 1033.
6. Gurkov, R., et al., What is Menière's disease? - a contemporary re-evaluation of endolymphatic hydrops. J Neurol, 2016. in press.
7. Gurkov, R., et al., MR volumetric assessment of endolymphatic hydrops. Eur Radiol, 2015. 25(2): p. 585-95.
8. Gurkov, R., et al., In vivo visualized endolymphatic hydrops and inner ear functions in patients with electrocochleographically confirmed Meniere's disease. Otology & Neurotology, 2012. 33(6): p. 1040-5.
9. Gurkov, R., et al., Herniation of the membranous labyrinth into the horizontal semicircular canal is correlated with impaired caloric response in Meniere's disease. Otology & Neurotology, 2012. 33(8): p. 1375-9.
10. Yamamoto, M., et al., Relationship between the Degree of Endolymphatic Hydrops and Electrocochleography. Audiology and Neuro-Otology, 2010. 15(4): p. 254-260.
11. Welling, D.B. and H.N. Nagaraja, Endolymphatic mastoid shunt: a reevaluation of efficacy. Otolaryngology - Head & Neck Surgery, 2000. 122(3): p. 340-5.
12. Saliba, I., et al., Endolymphatic duct blockage: a randomized controlled trial of a novel surgical technique for Meniere's disease treatment. Otolaryngol Head Neck Surg, 2015. 152(1): p. 122-9.
Competing interests: No competing interests