Sensorineural Hearing Impairment in Children with Chiari I Malformation

Simons, Jeffrey P.; Ruscetta, Melissa N.; David, Helena Maria Scherlowski Leal

doi:10.1177/000348940811700607

Cited by 10 publications

(7 citation statements)

References 13 publications

(21 reference statements)

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“…On the other hand, two patients had fluctuating loss. Patient 1 had a Chiari I malformation, which has been associated with sensorineural hearing loss 12, 13. Although this may have contributed to the severity of her deficit as well as its fluctuating nature, it would not explain the conductive component of her hearing loss.…”

Section: Discussionmentioning

confidence: 99%

Clinical experience in diagnosis and management of superior semicircular canal dehiscence in children

et al. 2011

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In contrast to adults, children with SSCD usually present with auditory symptoms first, although they share some similarities with adults in clinical manifestations of SSCD. Our study shows that SSCD syndrome, a well-accepted clinical entity, exists in the pediatric population. Conservative management is preferred for children with SSCD; nevertheless, surgical intervention is necessary for those with disabling vestibular symptoms. To date, this is the first clinical case series of symptomatic pediatric patients with SSCD.

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Section: Discussionmentioning

confidence: 99%

Clinical experience in diagnosis and management of superior semicircular canal dehiscence in children

et al. 2011

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“…The association of CM 1 with hearing loss has been showed in pediatric age group previously. 5 Aside from that study, there are very limited reported studies and case reports on this topic. 3,4,6Y8 Our study seems to be the first to evaluate the association between cerebellar tonsil descent along with CM 1 with SHL.…”

Section: Discussionmentioning

confidence: 90%

“…Simons et al 5 had performed a retrospective evaluation of 113 children with SHL and found 6 patients having Chiari malformation (5.3%) that was much more common than the general population, in which the incidence of CM 1 has been cited to be between 0.56% and 0.77%. 4,5 Besides, there are only a few case reports on the association between hearing loss and CM 1 published in the literature. Although a few aforementioned reports are present on this subject, to the best of our knowledge, ectopia of cerebellar tonsils along with CM 1 in SHL has not been studied previously.…”

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confidence: 99%

Association of Chiari I Malformation and Cerebellar Ectopia With Sensorineural Hearing Loss

Haktanır¹,

Yücedağ²,

Kaçar³

et al. 2013

Journal of Craniofacial Surgery

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We aimed to examine the prevalence of cerebellar tonsil ectopia and Chiari 1 malformation in sensorineural hearing loss (SHL) that has, to the best of our knowledge, not been studied previously. Magnetic resonance imaging records of 166 subjects with SHL and 50 controls without known otologic disturbances were included in the study. A tonsils descent more than 2 mm was assumed as cerebellar ectopia, and a descent equal to or more than 5 mm was assumed as Chiari 1 malformation. A tonsil descent group was also formed by summation of both groups. Transverse diameters of bilateral intracranial vertebral arteries and transverse sinuses were also measured, and all parameters were analyzed using appropriate statistics. A significant difference of frequencies of Chiari 1, ectopia, and tonsil descent was detected between patients and controls. In comparison of cerebellar ectopia and Chiari 1 groups, SHL did not show any significant difference. The left lateral sinus diameter showed positive correlation with tonsil descent. There was no significant correlation for the diameters of other vessels. A powerful correlation was detected between SHL and age. In addition, right and vertebral artery diameters showed positive correlations with age. Chiari 1 malformation and cerebellar ectopia showed an association with SHL. These patients should also be evaluated for otologic disturbances. Further high-resolution magnetic resonance imaging studies to explain the exact cause of this currently unknown association seems required.

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“…Chiari I malformations and intracranial tumors are rare etiologies of UHL, but are an impetus for using MRI to image the temporal bones. 22,26 In children, acquired UHL from intrauterine infections (including cytomegalovirus [CMV]), meningitis, head trauma, otologic surgery, and ototoxic medications are well known, but is much less likely to result from removal of acoustic tumors than in adults. 27 Sudden SNHL is often unilateral and includes enlarged vestibular aqueduct, endolymphatic hydrops, trauma, perilymph fistula, 28 autoimmune, and idiopathic etiologies.…”

Section: Causes Of Uhl In Childrenmentioning

confidence: 99%

Children with Unilateral Hearing Loss

Lieu

2010

Semin Hear

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Sensorineural Hearing Impairment in Children with Chiari I Malformation

Abstract: Chiari I malformation may be associated with SNHI. Central nervous system imaging for the evaluation of children with SNHI may be indicated, particularly in individuals with negative results on temporal bone computed tomography and genetic testing.

Cited by 10 publications

References 13 publications

Clinical experience in diagnosis and management of superior semicircular canal dehiscence in children

Clinical experience in diagnosis and management of superior semicircular canal dehiscence in children

Association of Chiari I Malformation and Cerebellar Ectopia With Sensorineural Hearing Loss

Children with Unilateral Hearing Loss

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