PDF herunterladen
J Neurol Surg A Cent Eur Neurosurg 2023; 84(03): 285-287
DOI: 10.1055/s-0041-1725959
Case Report

Chiari Type I Malformation Presenting with Unilateral Hearing Loss

Abdulhamid Ciçek 
1   Department of Neurosurgery, AZ Delta, Roeselare, Belgium
2   Department of Neurosurgery, Jan Yperman Hospital, Ypres, Belgium
,
Jeroen Cortier
1   Department of Neurosurgery, AZ Delta, Roeselare, Belgium
,
Sarah Hendrickx
1   Department of Neurosurgery, AZ Delta, Roeselare, Belgium
,
Johan Van Cauwenbergh
3   Department of Neurology, Jan Yperman Hospital, Ypres, Belgium
,
Lien Calus
4   Department of Ear, Nose and Throat Surgery, Jan Yperman Hospital, Ypres, Belgium
,
Johan Dehem
5   Department of Radiology, Jan Yperman Hospital, Ypres, Belgium
,
Dimitri Vanhauwaert
1   Department of Neurosurgery, AZ Delta, Roeselare, Belgium
2   Department of Neurosurgery, Jan Yperman Hospital, Ypres, Belgium
› Institutsangaben
› Weitere Informationen
Auch verfügbar auf
    Lizenzen und Reprints

Abstract

Introduction Chiari type I malformations can present in different ways, but the most frequent symptom is an occipitocervical headache. Hearing loss as the main presenting symptom is rare.

Case A young woman with progressive left-sided unilateral hearing loss was diagnosed with a Chiari type I malformation. She underwent a suboccipital craniectomy with C1 laminectomy and duraplasty. The hearing loss had resolved postoperatively with normalization of the audiometry.

Conclusion Chiari type I malformation can present solely with hearing loss. Improvement after surgical decompression is possible. This phenomenon is not emphasized well enough within the neurologic community. In this report, we present a summary of the pathophysiology and management in Chiari type I malformations.

Keywords

chiari - hearing loss - decompression


Publikationsverlauf

Eingereicht: 29. Oktober 2020

Angenommen: 16. Dezember 2020

Artikel online veröffentlicht:
07. Juni 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References

  • 1 Tubbs RS. Definitions and anatomic considerations in Chiari I malformation and associated syringomyelia. Neurosurg Clin N Am 2015; 26 (04) 487-493
    CrossrefPubMedSuche in Google Scholar
  • 2 Zhao JL, Li MH, Wang CL, Meng W. A systematic review of Chiari I malformation: techniques and outcomes. World Neurosurg 2016; 88: 7-14
    CrossrefPubMedSuche in Google Scholar
  • 3 Buell TJ, Heiss JD, Oldfield EH. Pathogenesis and cerebrospinal fluid hydrodynamics of the Chiari I malformation. Neurosurg Clin N Am 2015; 26 (04) 495-499
    CrossrefPubMedSuche in Google Scholar
  • 4 Kahn EN, Muraszko KM, Maher CO. Prevalence of Chiari I malformation and syringomyelia. Neurosurg Clin N Am 2015; 26 (04) 501-507
    CrossrefPubMedSuche in Google Scholar
  • 5 Greitz D. Unraveling the riddle of syringomyelia. Neurosurg Rev 2006; 29 (04) 251-263 , discussion 264
    CrossrefPubMedSuche in Google Scholar
  • 6 Öktemer H, Dilli A, Kürkçüoğlu A, Soysal H, Yazıcı C, Pelin C. Prevalence of Chiari type I malformation on cervical magnetic resonance imaging: a retrospective study. Anatomy 2016; 10 (01) 40-45
    CrossrefPubMedSuche in Google Scholar
  • 7 Oldfield EH. Pathogenesis of Chiari I: pathophysiology of syringomyelia: implications for therapy—a summary of 3 decades of clinical research. Neurosurg Clin N Am 2017; 64 (CN_suppl_1): 66-77
    CrossrefPubMedSuche in Google Scholar
  • 8 Pindrik J, Johnston Jr JM. Clinical presentation of Chiari I malformation and syringomyelia in children. Neurosurg Clin N Am 2015; 26 (04) 509-514
    CrossrefPubMedSuche in Google Scholar
  • 9 Sivakanthan S, Goldhagen C, Danner C, Agazzi S. Hearing loss and Chiari malformation: a clinical pearl. Clin Neurol Neurosurg 2014; 122: 20-22
    CrossrefPubMedSuche in Google Scholar
  • 10 Heuer GG, Gabel B, Lemberg PS, Sutton LN. Chiari I malformation presenting with hearing loss: surgical treatment and literature review. Childs Nerv Syst 2008; 24 (09) 1063-1066
    CrossrefPubMedSuche in Google Scholar
  • 11 Hendrix RA, Bacon CK, Sclafani AP. Chiari-I malformation associated with asymmetric sensorineural hearing loss. J Otolaryngol 1992; 21 (02) 102-107
    PubMedSuche in Google Scholar
  • 12 Henssen DJHA, Kurt E, Kozicz T, van Dongen R, Bartels RHMA, van Cappellen van Walsum AM. New insights in trigeminal anatomy: a double orofacial tract for nociceptive input. Front Neuroanat 2016; 10: 53
    PubMedSuche in Google Scholar
  • 13 Mangold SA, Das JM. Neuroanatomy, Cortical Primary Auditory Area. Treasure Island, FL: StatPearls Publishing; 2020
    Suche in Google Scholar
  • 14 Zhang CH, DeSouza RM, Kho JSB, Vundavalli S, Critchley G. Kernohan-Woltman notch phenomenon: a review article. Br J Neurosurg 2017; 31 (02) 159-166
    CrossrefPubMedSuche in Google Scholar
  • 15 Dammers R, Volovici V, Kompanje EJO. The history of the kernohan notch revisited. Neurosurgery 2016; 78 (04) 581-584
    CrossrefPubMedSuche in Google Scholar
  • 16 Safdarian M, Farzaneh F, Rahimi-Movaghar V. Contralateral radiculopathy: a Kernohan–Woltman notch-like phenomenon. Asian J Neurosurg 2018; 13 (01) 165-167
    Thieme ConnectPubMedSuche in Google Scholar
  • 17 Johnson GD, Harbaugh RE, Lenz SB. Surgical decompression of Chiari I malformation for isolated progressive sensorineural hearing loss. Am J Otol 1994; 15 (05) 634-638
    PubMedSuche in Google Scholar
  • 18 Ahmmed AU, Mackenzie I, Das VK, Chatterjee S, Lye RH. Audio-vestibular manifestations of Chiari malformation and outcome of surgical decompression: a case report. J Laryngol Otol 1996; 110 (11) 1060-1064
    CrossrefPubMedSuche in Google Scholar
  • 19 Sperling NM, Franco Jr RA, Milhorat TH. Otologic manifestations of Chiari I malformation. Otol Neurotol 2001; 22 (05) 678-681
    CrossrefPubMedSuche in Google Scholar
  • 20 Kumar A, Patni AH, Charbel F. The Chiari I malformation and the neurotologist. Otol Neurotol 2002; 23 (05) 727-735
    CrossrefPubMedSuche in Google Scholar
  • 21 Simons JP, Ruscetta MN, Chi DH. Sensorineural hearing impairment in children with Chiari I malformation. Ann Otol Rhinol Laryngol 2008; 117 (06) 443-447
    CrossrefPubMedSuche in Google Scholar
  • 22 Dolgun H, Turkoglu E, Kertmen H, Yilmaz ER, Sekerci Z. Chiari Type I malformation presenting with bilateral hearing loss. J Clin Neurosci 2009; 16 (09) 1228-1230
    CrossrefPubMedSuche in Google Scholar
  • 23 Raybaud C, Jallo GI. Chiari 1 deformity in children: etiopathogenesis and radiologic diagnosis. Handb Clin Neurol 2018; 155: 25-48
    CrossrefPubMedSuche in Google Scholar