International Journal of Otorhinolaryngology

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An Unusual Association: Arnold Chiari Deformity and Meniere's Disease

Received: May 23, 2018    Accepted: Jun. 08, 2018    Published: Jul. 12, 2018
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Abstract

Rare and fairly unknown, Arnold Chiari deformity is defined by the abnormally low position of the cerebellar tonsils that engage through the foramen magnum. Its association with the triad of tinnitus-hypoacousia-vertigo causes an authentic Meniere’s disease worth discussing. We report an unusual association of Arnold Chiari deformity with Meniere’s disease. A 46-years-old patient was diagnosed with left Meniere’s disease in 1994 on the classical diagnostic triad and the mode of progression: rotatory vertigo evolving by iterative crises; Intermittent buzzing tinnitus; left perception deafness with notion of wadded left ear. For a decade (1994 to 2015), he has been put under hygieno-dietary measures, vestibular re-education and medical treatment. The evolution was marked by the worsening of vertigo becoming progressively incapacitating, as well as an aggravation of the left deafness and persistence of tinnitus. Audiometry highlighted a severe endocochlear left perception deafness. The videonystagmography revealed a well-compensated left vestibular deficit. Cerebral magnetic resonance imaging (MRI) revealed a cerebellar tonsils’ ptosis through the foramen magnum. The diagnosis of Arnold-Chiari deformity associated with Meniere’s disease was then retained. The patient received a surgical left labyrinthectomy in 2015. The clinical course was uneventful, marked by the disappearance of vertigo twenty four months later. Association of Meniere’s disease and Arnold Chiari deformity is rare and must be diagnosed. These two pathologies being manifested by peripheral vertigo, a meticulous clinical and Para clinical examination is necessary to guide the diagnosis. Cerebrospinal fluid flow and pressure anomaly due to Arnold Chiari malformation can truly impact labyrinthine physiology, which explains the correlation between these two entities.

DOI 10.11648/j.ijo.20180401.16
Published in International Journal of Otorhinolaryngology ( Volume 4, Issue 1, June 2018 )
Page(s) 21-26
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Vertigo, Meniere's Disease, Arnold Chiari

References
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[3] Elster AD, Chen MY. Chiari 1 malformations: clinical and radiologic reappraisal. Radiology 1992; 183: 347-53.
[4] Alexander T. H, Harris J. P. Current epidemiology of Meniere's syndrome. Otolaryngol Clin North Am. 2010; 43: 965-970.
[5] Weckel A, Marx M, Esteve-Fraysse M. J. Control of vertigo in Meniere's disease by intratympanic dexamethasone. European Annals of Otorhinolaryngology, Head and Neck Diseases. 2018; Volume 135: 7-10.
[6] Nevoux J, Barbara M, Dornhoffer J, Gibson W, Kitahara T, Darrouzet V. International consensus (ICON) on treatment of Ménière's disease. European Annals of Otorhinolaryngology, Head and Neck diseases. 2018; 135: S29–S32.
[7] Committee on hearing equilibrium guidelines for the diagnosis evaluation on therapy in Ménière’s disease. Otolaryngol Head Neck Surg. 1995; 113: 181–5.
[8] Lopez-Escamez JA, Carey J, Chung WH, Goebel JA, et al. Diagnostic criteria for Ménière’s disease. J vest Res. 2015; 25:1–7.
[9] Noël S, Delavallée M, Scory P, et al. La fatigue comme unique symptôme de la malformation d’Arnold-Chiari. Med Som. 2009; 6: 109–12.
[10] Benjamin M. D, Santiago J, Hebert J. C, Thirion S, Ranaivojaona S, Alvarez C, Atallah A, Sibille G, Bataille H, Porlys M, Ebrad P. Hémi-hypertrophie et scoliose révélatrices d'une malformation de Chiari de type 1 avec syringomyélie. Archives de Pédiatrie. 2011; 18: 1210-5.
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[15] Xiaofeng D, Chenlong Y, Jiahe G, Liang W, Tao Y, Jun Y, et al. Long term outcomes after small-bone-window posterior fossa decompression and duraplasty in adults with Chiari malformation type I. World Neurosurg 2015; 84 (4): 998-1004.
[16] Guerra Jimenez G, Mazon Gutierrez A, Marco De Lucas E, et al. Audio-vestibular signs and symptoms in Chiari Malformation type 1. Case series and literature review. Act Otorrinolaringol. Esp. 2015; 66 (1): 28-35.
[17] Muncie HL, Sirmans SM, James E. Dizziness: Approach to evaluation and management. Am Fam Physician 2017. 95 (3): 154-62.
[18] Alarcon AV, Hidalgo LO, Arevalo RJ. Labyrinthectomy and vestibular neurectomy for intractable vertiginous symptoms. Int Arch Otorhinolaryngol 2017; 21 (2): 184-90.
[19] Flores Garcia ML, Llata Segura C, Cisneros Lesser JC, Pane Pianese C. Endolymphatic sac surgery for Meniere's disease. Current opinion and literature review. Int Arch Otorhinolaryngol 2017; 21 (2): 179-83.
[20] Jumaily M, Faraji F, Mikulec AA Intratympanic triamcinolone and dexamethasone in the treatment of Meniere's syndrome. Otol Neurotol 2017; 38 (3): 386-91.
[21] Société Française d’Oto-Rhino-Laryngologie et de Chirurgie de la Face et du Cou. Recommandation pour la pratique clinique. RCP Stratégie diagnostique et thérapeutique dans la maladie de Menière. [Available from: https://www.orlfrance.org/wp-content/uploads/2017/06/Maladie-de-Meniere-stategie-diagnostique-et-therapeutique.pdf].
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  • APA Style

    do Santos Zounon Alexis, Molher Joffrey, Bonnard Damien, Darrouzet Vincent. (2018). An Unusual Association: Arnold Chiari Deformity and Meniere's Disease. International Journal of Otorhinolaryngology, 4(1), 21-26. https://doi.org/10.11648/j.ijo.20180401.16

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    ACS Style

    do Santos Zounon Alexis; Molher Joffrey; Bonnard Damien; Darrouzet Vincent. An Unusual Association: Arnold Chiari Deformity and Meniere's Disease. Int. J. Otorhinolaryngol. 2018, 4(1), 21-26. doi: 10.11648/j.ijo.20180401.16

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    AMA Style

    do Santos Zounon Alexis, Molher Joffrey, Bonnard Damien, Darrouzet Vincent. An Unusual Association: Arnold Chiari Deformity and Meniere's Disease. Int J Otorhinolaryngol. 2018;4(1):21-26. doi: 10.11648/j.ijo.20180401.16

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  • @article{10.11648/j.ijo.20180401.16,
      author = {do Santos Zounon Alexis and Molher Joffrey and Bonnard Damien and Darrouzet Vincent},
      title = {An Unusual Association: Arnold Chiari Deformity and Meniere's Disease},
      journal = {International Journal of Otorhinolaryngology},
      volume = {4},
      number = {1},
      pages = {21-26},
      doi = {10.11648/j.ijo.20180401.16},
      url = {https://doi.org/10.11648/j.ijo.20180401.16},
      eprint = {https://download.sciencepg.com/pdf/10.11648.j.ijo.20180401.16},
      abstract = {Rare and fairly unknown, Arnold Chiari deformity is defined by the abnormally low position of the cerebellar tonsils that engage through the foramen magnum. Its association with the triad of tinnitus-hypoacousia-vertigo causes an authentic Meniere’s disease worth discussing. We report an unusual association of Arnold Chiari deformity with Meniere’s disease. A 46-years-old patient was diagnosed with left Meniere’s disease in 1994 on the classical diagnostic triad and the mode of progression: rotatory vertigo evolving by iterative crises; Intermittent buzzing tinnitus; left perception deafness with notion of wadded left ear. For a decade (1994 to 2015), he has been put under hygieno-dietary measures, vestibular re-education and medical treatment. The evolution was marked by the worsening of vertigo becoming progressively incapacitating, as well as an aggravation of the left deafness and persistence of tinnitus. Audiometry highlighted a severe endocochlear left perception deafness. The videonystagmography revealed a well-compensated left vestibular deficit. Cerebral magnetic resonance imaging (MRI) revealed a cerebellar tonsils’ ptosis through the foramen magnum. The diagnosis of Arnold-Chiari deformity associated with Meniere’s disease was then retained. The patient received a surgical left labyrinthectomy in 2015. The clinical course was uneventful, marked by the disappearance of vertigo twenty four months later. Association of Meniere’s disease and Arnold Chiari deformity is rare and must be diagnosed. These two pathologies being manifested by peripheral vertigo, a meticulous clinical and Para clinical examination is necessary to guide the diagnosis. Cerebrospinal fluid flow and pressure anomaly due to Arnold Chiari malformation can truly impact labyrinthine physiology, which explains the correlation between these two entities.},
     year = {2018}
    }
    

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    AB  - Rare and fairly unknown, Arnold Chiari deformity is defined by the abnormally low position of the cerebellar tonsils that engage through the foramen magnum. Its association with the triad of tinnitus-hypoacousia-vertigo causes an authentic Meniere’s disease worth discussing. We report an unusual association of Arnold Chiari deformity with Meniere’s disease. A 46-years-old patient was diagnosed with left Meniere’s disease in 1994 on the classical diagnostic triad and the mode of progression: rotatory vertigo evolving by iterative crises; Intermittent buzzing tinnitus; left perception deafness with notion of wadded left ear. For a decade (1994 to 2015), he has been put under hygieno-dietary measures, vestibular re-education and medical treatment. The evolution was marked by the worsening of vertigo becoming progressively incapacitating, as well as an aggravation of the left deafness and persistence of tinnitus. Audiometry highlighted a severe endocochlear left perception deafness. The videonystagmography revealed a well-compensated left vestibular deficit. Cerebral magnetic resonance imaging (MRI) revealed a cerebellar tonsils’ ptosis through the foramen magnum. The diagnosis of Arnold-Chiari deformity associated with Meniere’s disease was then retained. The patient received a surgical left labyrinthectomy in 2015. The clinical course was uneventful, marked by the disappearance of vertigo twenty four months later. Association of Meniere’s disease and Arnold Chiari deformity is rare and must be diagnosed. These two pathologies being manifested by peripheral vertigo, a meticulous clinical and Para clinical examination is necessary to guide the diagnosis. Cerebrospinal fluid flow and pressure anomaly due to Arnold Chiari malformation can truly impact labyrinthine physiology, which explains the correlation between these two entities.
    VL  - 4
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Author Information
  • Department of Otolaryngology Head and Neck Surgery, Military Teaching Hospital, Cotonou, Benin; Department of Otolaryngology Head and Neck Surgery, CHU Bordeaux, University of Bordeaux, Bordeaux, France

  • Department of Otolaryngology Head and Neck Surgery, CHU Bordeaux, University of Bordeaux, Bordeaux, France

  • Department of Otolaryngology Head and Neck Surgery, CHU Bordeaux, University of Bordeaux, Bordeaux, France

  • Department of Otolaryngology Head and Neck Surgery, CHU Bordeaux, University of Bordeaux, Bordeaux, France

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