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Audiovestibular Findings in a Cohort of Patients with Chiari Malformation Type I and Dizziness.

ABSTRACT: Chiari Malformation Type I (CM1) is a neurological condition in which the cerebellar tonsils extend past the foramen magnum. While many studies have reported dizziness symptoms in patients with CM1, the prevalence of peripheral labyrinthine lesions is largely unknown. This study aimed to comprehensively describe the audiovestibular phenotype in a cohort of patients with CM1 expressly referred for dizziness. Twenty-four patients with CM1 and a complaint of dizziness/vertigo were evaluated. Hearing and auditory brainstem tract function were essentially normal. While vestibular abnormalities were most prevalent during rotational testing (33%), abnormal functional balance was the most common finding (40%). Patients with CM1 had a greater likelihood of exhibiting an abnormal sensory organization test (SOT) postural stability score for fixed platform conditions, and for the somatosensory analysis score. While no significant associations were identified between tonsillar ectopia extent and any vestibular/balance outcome measure, a significant negative association was identified between neck pain and the somatosensory sensory analysis score. Abnormal functional balance in the somatosensory domain was remarkable, with poorer scores associated with neck pain. An isolated peripheral vestibulopathy was present in only 8% of patients. Despite the low prevalence of vestibulopathy, vestibular/balance assessment is warranted to identify patients who may benefit from referral to specialized medical disciplines.

SUBMITTER: Famili HP

PROVIDER: S-EPMC10142081 | biostudies-literature | 2023 Apr

REPOSITORIES: biostudies-literature

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Audiovestibular Findings in a Cohort of Patients with Chiari Malformation Type I and Dizziness.

Famili Hannah P HP Zalewski Christopher K CK Ibrahimy Alaaddin A Mack Jessica J Cantor Fredric F Heiss John D JD Brewer Carmen C CC

Journal of clinical medicine 20230407 8

Chiari Malformation Type I (CM1) is a neurological condition in which the cerebellar tonsils extend past the foramen magnum. While many studies have reported dizziness symptoms in patients with CM1, the prevalence of peripheral labyrinthine lesions is largely unknown. This study aimed to comprehensively describe the audiovestibular phenotype in a cohort of patients with CM1 expressly referred for dizziness. Twenty-four patients with CM1 and a complaint of dizziness/vertigo were evaluated. Hearin ...[more]

PMID: 37109107

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Project description:OBJECT:Chiari malformation Type I (CM-I) is characterized by hindbrain deformity. We investigated the effects of craniocervical decompression surgery on the anatomical features of hindbrain deformity with a prospective MRI study of patients with CM-I. METHODS:A prospective longitudinal study was conducted in 48 patients with CM-I (39 with syringomyelia) treated with craniocervical decompression. Clinical examinations and cervical MRI were performed before surgery and 1 week, 3-6 months, and annually after surgery. Hindbrain deformity was defined by tonsillar ectopia, pointed cerebellar tonsils, and/or cervicomedullary protuberance. The length of the clivus, basiocciput (sphenooccipital synchondrosis to basion), supraocciput (internal occipital protuberance to opisthion), and anteroposterior (AP) width of CSF pathways at the foramen magnum were measured and compared with those from 18 healthy volunteers (control group). RESULTS:Before surgery, the patients' posterior fossa bones were short and their CSF pathways were narrow. All patients had tonsillar ectopia (mean [± SD] 12.3 ± 5.1 mm; normal 0.3 ± 1.0). The majority of patients had pointed tonsils and more than two-thirds exhibited a cervicomedullary protuberance. Clivus and basiocciput lengths were significantly shorter than the values obtained in the control group. However, the supraocciput length did not differ significantly from control measurements. The mean bulbopontine sulcus distance superior to the basion was 9.5 ± 2.6 mm (vs 13.6 ± 2.8 mm in controls; p < 0.0001). The AP widths of the CSF pathways at the level of the foramen magnum were significantly narrowed. After surgery, CSF pathways significantly expanded both ventrally and dorsally. By 3-6 months after surgery, pointed tonsils became round, cervicomedullary protuberance disappeared, and tonsillar ectopia diminished by 51% (to 6.0 ± 3.3 mm; p < 0.0001). CONCLUSIONS:The cerebellar tonsils and brainstem assumed a normal appearance within 6 months after craniocervical decompression. These findings support the concept that the CM-I is not a congenital malformation of the neural elements but rather an acquired malformation that arises from pulsatile impaction of the cerebellar tonsils into the foramen magnum. Clinical trial registration no.: NCT00001327.

Project description:BackgroundThe best surgical treatment of Chiari malformation patients with syringomyelia remains controversial, and whether cerebellar tonsillectomy should be performed has not been decided.ObjectiveTo evaluate the efficacy of posterior fossa decompression with duraplasty (PFDD) and Posterior fossa decompression with resection of tonsils (PFDRT) in patients of Chiari malformation type I (CM-I) with syringomyelia and explore relevant factors affecting prognosis.Patients and methodsWe retrospectively analyzed 182 adult patients of CM-I with syringomyelia who underwent PFDD or PFDRT over a 6-year period, and analyzed their clinical manifestations, imaging features, and follow-up data. Clinical outcomes were assessed using the Chicago Chiari Outcome Scale (CCOS), and imaging outcomes were assessed using the syrinx remission rate. Difference comparisons were performed to compare the differences between different surgical groups. Influencing factors associated with outcome were investigated using bivariate analysis and multiple linear regression analysis.ResultsThere were statistically significant differences in CCOS score (p = 0.034) and syrinx remission rates (p = 0.046) between the PFDRT group and the PFDD group after surgery. Regression analysis showed that preoperative motor dysfunction, cerebellar-related symptoms and different surgical methods may have influenced the CCOS score and that brainstem-related symptoms and age may have influenced the syrinx remission rates in the total patient group (p < 0.05). Regression analysis showed that the duration of symptoms, cerebellar-related symptoms and preoperative syrinx diameter may have influenced the CCOS score and that the preoperative cerebellar tonsillar hernia distance may have influenced the postoperative syrinx remission rate in the PFDRT group (p < 0.05). Age and length of hospital stay may have influenced the CCOS score, and brainstem-related symptoms and age may have influenced the syrinx remission rates in the PFDD group (p < 0.05).ConclusionThis study showed that the CCOS score in the PFDRT group was better than that in the PFDD group. Preoperative motor dysfunction, cerebellar-related symptoms, and different surgical methods in patients of CM-I with syringomyelia affected postoperative CCOS score. Both the duration of symptoms and the age of the patients should be actively considered as factors influencing prognosis. Symptomatic CM-I patients with syringomyelia should undergo surgical treatment as early as possible.

Dataset Information

Audiovestibular Findings in a Cohort of Patients with Chiari Malformation Type I and Dizziness.

Publications

Audiovestibular Findings in a Cohort of Patients with Chiari Malformation Type I and Dizziness.

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