JOURNAL OF CLINICAL AND BIOMEDICAL SCIENCES

Article

Journal of Clinical and Biomedical Sciences

Year: 2025, Volume: 15, Issue: 1, Pages: 62-66

Case Report

Hearing and Balance Problems in a Chiari-1 Malformation Associated with Communicating Hydrocephalus - A Case Report

Received Date:27 November 2024, Accepted Date:13 January 2025, Published Date:25 March 2025

Abstract

Chiari malformation is characterized by an anatomical alteration at the base of the skull, cerebellar herniation, or brainstem herniation via the magnum foramen and up to the cervical spinal canal. It is categorized into five subtypes from type 0 to type IV. The effect of Chiari malformation is reported on the auditory as well as vestibular system due to the nature of the condition. A case of the 36-year-old female came with the complaint of reduced hearing sensitivity and ringing sensation in both ears along with episodes of vertigo, imbalance while walking, and headache and was diagnosed with bilateral cerebellar tonsillar herniation with mild communicating hydrocephalus. Detailed audiological evaluation revealed normal middle ear functioning in both ears, hearing sensitivity within normal limits in the right ear, and severe- profound hearing loss in the left ear, which correlated well with Auditory brainstem response testing. Tinnitus handicap evaluation revealed that catastrophic form of tinnitus and dizziness handicap evaluation revealed a severe form of dizziness. subjective vestibular evaluation revealed nystagmus and instability while testing indicative of vestibular impairment of central origin. The present case report supports the evidence-based practice and the outcomes of a complete audiological evaluation and subjective vestibular evaluation in an adult female with Chiari-1 malformation and communicating hydrocephalus are highlighted in this case report. Hearing loss and balance difficulties can be progressive, thus audiological and vestibular evaluations are vital to understanding pathophysiology, seeking the appropriate therapeutic approach, and enhancing the overall quality of life in these patients.

Keywords: Cerebellar Herniation, Tonsillar ectopia, Chiari malformation, Communicating hydrocephalus, Nystagmus, Sensori-neural hearing loss, Fukuda stepping test

References

  1. Jiménez GG, Gutiérrez ÁM, Lucas EMD, Román NVS, Laez RM, Angulo CM. Audio-vestibular signs and symptoms in Chiari malformation type i. Case series and literature review. Acta Otorrinolaringologica. 2015;66(1):28–35. Available from: https://doi.org/10.1016/j.otorri.2014.05.002
  2. Vázquez MEA, Fernández AA, Fernándezj JB, ECiO, JdlCL, Silva ME, et al. Malformaciones de la unión craneo-cervical (Chiari tipo Iy siringomielia). Documento de consenso. Madrid: AWWE. 2009. Available from: https://www.sen.es/pdf/2010/Consenso_Chiari_2010.pdf
  3. Sarnat HB. Disorders of segmentation of the neural tube: Chiari malformations. Handbook of Clinical Neurology. 2007;87:89–103. Available from: https://doi.org/10.1016/S0072-9752(07)87006-0
  4. Khoury C, Patterson MC, Dashe JF. Chiari malformations. 2013. Available from: http://www.uptodate.com
  5. Sclafani AP, DeDio RM, Hendrix RA. The Chiari-I malformation. Ear, Nose, & Throat Journal. 1991;70(4):208–212. Available from: https://pubmed.ncbi.nlm.nih.gov/1874153/
  6. Rydell RE, Pulec JL. Arnold-Chiari malformation: neuro-otologic symptoms. Archives of Otolaryngology. 1971;94(1):8–12. Available from: https://doi.org/10.1001/archotol.1971.00770070044002
  7. Hendrix RA, Bacon CK, Sclafani AP. Chiari-I malformation associated with asymmetric sensorineural hearing loss. The Journal of Otolaryngology. 1992;21(2):102–107. Available from: https://pubmed.ncbi.nlm.nih.gov/1583702/
  8. Satzer D, Guillaume DJ. Hearing loss in hydrocephalus: a review, with focus on mechanisms. Neurosurgical review. 2016;39(1):13–24. Available from: https://doi.org/10.1007/s10143-015-0650-2
  9. Khan AA, Bhatti SN, Khan G, EA, AA, AA, et al. Clinical and radiological findings in Arnold Chiari malformation. Journal of Ayub Medical College Abbottabad. 2010;22(2):75–78. Available from: https://pubmed.ncbi.nlm.nih.gov/21702272/
  10. Ahmmed AU, Mackenzie I, Das VK, Chatterjee S, Lye RH. Audio-vestibular manifestations of Chiari malformation and outcome of surgical decompression: a case report. The Journal of Laryngology & Otology. 1996;110(11):1060–1064. Available from: https://doi.org/10.1017/s0022215100135753
  11. Prabhu P, Anish AS, Vijayan D, Shiju AM, Shanthala SP, Sreenivas R. Audio-Vestibular Findings in an Adult With Arnold-Chiari Malformation. Journal of Hearing Science. 2020;10(4):85–90. Available from: https://doi.org/10.17430/JHS.2020.10.4.8

Copyright

This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Published By Sri Devaraj Urs Academy of Higher Education, Kolar, Karnataka

DON'T MISS OUT!

Subscribe now for latest articles and news.