Otologic Manifestations of Chiari I Malformation

Sperling, Neil M.; Franco, Ramon A.; Milhorat, Thomas H.

doi:10.1097/00129492-200109000-00020

Cited by 40 publications

(38 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Analysis of the absolute latency values in patients with Chiari malformation type II disclosed a tendency to increased median value of potentials III and V when compared to normal values ( Table 1). The most plausible explanation for this discre p a n c y is that it is probably due to the association of type II brainstem abnormalities and the sensory-neural maturation delay typically found in this kind of malformation 5,11,12 .…”

Section: Graphic 1 Abnormalities Found In the Auditory Pathway Absomentioning

confidence: 99%

Abnormalities in auditory evoked potentials of 75 patients with Arnold-Chiari malformations types I and II

Filho¹,

Pratesi

2006

Arq. Neuro-Psiquiatr.

View full text Add to dashboard Cite

-Objective: To evaluate the frequency and degree of severity of abnormalities in the auditory pathways in patients with Chiari malformations type I and II. Method: This is a series-of-case descriptive study in which the possible presence of auditory pathways abnormalities in 75 patients (48 children and 27 adults) with Chiari malformation types I and II were analyzed by means of auditory evoked potentials evaluation. The analysis was based on the determination of intervals among potentials peak values, absolute latency and amplitude ratio among potentials V and I. Results: Among the 75 patients studied, 27 (36%) disclosed Arnold-Chiari malformations type I and 48 (64%) showed Arnold-Chiari malformations type II. F i f t y -t h re e (71%) of these patients showed some degree of auditory evoked potential abnormalities. Te s t s w e re normal in the remaining 22 (29%) patients. Conclusion: A u d i t o ry evoked potentials testing can be c o n s i d e red a valuable instrument for diagnosis and evaluation of brain stem functional abnormalities in patients with Arnold-Chiari malformations type I and II. The determination of the presence and degree of severity of these abnormalities can be contributory to the prevention of further handicaps in these patients either through physical therapy or by means of precocious corrective surgical intervention.KEY WORDS: Arnold-Chiari malformations type I and type II, auditory evoked potentials.A n o rmalidades nos potenciais evocados auditivos de 75 pacientes com os tipos I e II das malformações de Arnold-Chiari RESUMO -Objetivo: Avaliar a freqüência e grau de comprometimento das vias auditivas em tronco cerebral por meio de potencial evocado auditivo, em pacientes afetados por malformações de Arn o l d -C h i a r i de tipos I e II. Método: Foi efetuado um estudo descritivo de tipo série de casos, sendo selecionados 75 pacientes (48 crianças e 27 adultos) nos quais foi realizada avaliação dos potenciais evocados das vias auditivas, com base à determinação dos valores dos intervalos entre picos de potenciais, da latência absoluta e da razão entre as amplitudes dos potenciais V e I. Resultados: E n t re os 75 pacientes avaliados, 27 (36%) a p resentavam malformações de Arnold-Chiari de tipo I e 48 (64%) apresentavam malformações de tipo II. Em 53 (71%) do total de pacientes os potenciais evocados auditivos mostraram algum grau de anormalidade. Os testes foram normais nos restantes 22 (29%). Conclusão: O potencial evocado auditivo pode ser considerado valioso instrumento para o diagnóstico e avaliação da gravidade das anormalidades funcionais de tronco cerebral em pacientes port a d o res de malformações de Arnold-Chiari de tipo I e II. Esta avaliação pode contribuir de maneira significativa não somente no diagnóstico como também na prevenção de ulter i o res lesões, pela adoção de medidas preventivas, tanto por meio de fisioterapia como por interv e n ç ã o cirúrgica precoce. PALAVRAS-CHAVE: malformação de Arnold-Chiari de tipo I e de tipo II, potenciais evocados auditivos.

show abstract

Section: Graphic 1 Abnormalities Found In the Auditory Pathway Absomentioning

confidence: 99%

Abnormalities in auditory evoked potentials of 75 patients with Arnold-Chiari malformations types I and II

Filho¹,

Pratesi

2006

Arq. Neuro-Psiquiatr.

View full text Add to dashboard Cite

show abstract

“…Other studies support the notion that headache and neck pain are frequent complaints, occurring in 65% to 81% of patients; however, sufficient characteristics of headaches are lacking [26][27][28][29].…”

Section: Asymptomatic and Headache Clinical Presentationsmentioning

confidence: 89%

“…Sperling et al [29] reported otologic CMI symptoms in 16 patients who were observed prospectively. On a selfadministered questionnaire, 81% of the patients reported episodic aural fullness, 81% tinnitus, 69% vertigo, 56% fluctuating hearing loss, and 81% headaches.…”

Section: Other Clinical Presentationsmentioning

confidence: 99%

Headache and chiari i malformation: Clinical presentation, diagnosis, and controversies in management

Taylor

Larkins

2002

Current Science Inc

View full text Add to dashboard Cite

The Chiari I malformation is a cerebellar anomaly of uncertain origin, defined in part as tonsillar herniation of at least 3 to 5 mm below the foramen magnum; it is associated with crowding of the craniocervical junction. It is the most frequent of the Chiari malformations and is associated with headaches, syringohydromyelia, and hydrocephalus. The clinical manifestations are related to direct compression of neural tissues and abnormal cerebrospinal fluid dynamics. Common presentation is in adulthood, but there is increasing recognition in childhood. Incidental magnetic resonance imaging discovers Chiari I in one third of patients who do not have clinical symptoms. Headaches in the occipital-suboccipital region or those that are of cough-type suggest symptomatic Chiari I malformation. Suboccipital craniectomy is performed for patients with Chiari I malformation who have neural compression syndromes of the brainstem and spinal cord, select headache types, and other uncommon conditions that are not the topic of this review.

show abstract

“…Cranial nerve dysfunction is common in Chiari I [59,88], and auditory brainstem response (ABR) findings in Chiari II are consistent with delayed brainstem maturation and progressive cochlear nerve stretching [79,84]. Several studies have found evidence for a hydrodynamic cause of hearing loss; symptoms of endolymphatic hydrops have been reported in Chiari I [4,74,107], and one study found hydrocephalus to be an independent predictor of hearing loss, distinct from the presence of cranial nerve deficits [65]. However, Chiari malformation-related hearing loss is likely multifactorial in origin.…”

Section: Auditory Pathway Dysfunctionmentioning

confidence: 99%

“…Auditory or vestibular symptoms have been reported in 74 % of patients with Chiari I [74] and 57-69 % of patients with Chiari II [12,126]. Proposed etiologies of hearing loss include cranial nerve stretching, compression of the cochlear nerve or nuclei by the cerebellar tonsils, and transmission of abnormal CSF pressures to the inner ear [2,31,49,53,107]. Cranial nerve dysfunction is common in Chiari I [59,88], and auditory brainstem response (ABR) findings in Chiari II are consistent with delayed brainstem maturation and progressive cochlear nerve stretching [79,84].…”

Section: Auditory Pathway Dysfunctionmentioning

confidence: 99%

Hearing loss in hydrocephalus: a review, with focus on mechanisms

Satzer

Guillaume

2015

Neurosurg Rev

View full text Add to dashboard Cite

While neither hydrocephalus nor cerebrospinal fluid (CSF) shunt placement is traditionally considered in the differential diagnosis of hearing loss, there is substantial evidence that CSF circulation and pressure abnormalities can produce auditory dysfunction. Several indirect mechanisms may explain association between hydrocephalus and hearing loss, including mass effect, compromise of the auditory pathway, complications of prematurity, and genetically mediated hydrocephalus and hearing loss. Nevertheless, researchers have proposed a direct mechanism, which we term the hydrodynamic theory. In this hypothesis, the intimate relationship between CSF and inner ear fluids permits relative endolymphatic or perilymphatic hydrops in the setting of CSF pressure disturbances. CSF is continuous with perilymph, and CSF pressure changes are known to produce parallel perilymphatic pressure changes. In support of the hydrodynamic theory, some studies have found an independent association between hydrocephalus and hearing loss. Moreover, surgical shunting of CSF has been linked to both resolution and development of auditory dysfunction. The disease burden of hydrocephalus-associated hearing loss may be large, and because hydrocephalus and over-shunting are reversible, this relationship merits broader recognition. Hydrocephalic patients should be monitored for hearing loss, and hearing loss in a patient with shunted hydrocephalus should prompt further evaluation and possibly adjustment of shunt settings.

show abstract

Otologic Manifestations of Chiari I Malformation

Abstract: Most patients with Chiari I malformation have symptoms that mimic primary otologic pathologic changes. The existence of common pathophysiologic mechanisms is proposed.

Cited by 40 publications

References 13 publications

Abnormalities in auditory evoked potentials of 75 patients with Arnold-Chiari malformations types I and II

Abnormalities in auditory evoked potentials of 75 patients with Arnold-Chiari malformations types I and II

Headache and chiari i malformation: Clinical presentation, diagnosis, and controversies in management

Hearing loss in hydrocephalus: a review, with focus on mechanisms

Contact Info

Product

Resources

About