Cochleovestibular Anomalies in Children With Cholesteatoma

Propst, Evan J.; Blasér, Susan; Trimble, Keith; James, Adrian L.; Friedberg, Jacob; Papsin, Blake C.

doi:10.1097/mlg.0b013e31815d8e55

Cited by 11 publications

(9 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This number is similar to the incidence of congenital cholesteatoma among all cholesteatoma in the general population ranging from 2 to 24% found in the literature (Friedberg, 1994). A correlation between the formation of congenital cholesteatoma and abnormal cochleo-vestibular anatomy has been reported previously (Propst et al, 2008). Because those with abnormal cochleo-vestibular anatomy often have significant SNHL, a relationship has also been suggested between profound SNHL and the presence of congenital cholesteatomas (Jackler et al, 1987).…”

Section: Discussionsupporting

confidence: 85%

Congenital cholesteatoma and cochlear implantation: Implications for management

Chung

Cushing

James

et al. 2013

Cochlear Implants International

View full text Add to dashboard Cite

The 0.25% incidence of congenital cholesteatoma in our population of CI patients is higher than expected of this rare condition. It is surprisingly common given the absence of any cases of primary acquired cholesteatoma, which is considerably more common even in the pediatric population. Both patients likely had an inherited form of hearing loss and a genetic contribution to the presence of congenital cholesteatoma cannot be excluded. The presence of congenital cholesteatoma has implications for the algorithm currently employed for the assessment of CI. We consider that surgery should be staged to ensure complete removal of the cholesteatoma before implantation. Thus bilateral CI should be provided sequentially rather than simultaneously in the presence of unilateral cholesteatoma.

show abstract

Section: Discussionsupporting

confidence: 85%

Congenital cholesteatoma and cochlear implantation: Implications for management

Chung

Cushing

James

et al. 2013

Cochlear Implants International

View full text Add to dashboard Cite

show abstract

“…Our case, as well as both reported by Chung et al of congenital cholesteatoma patients, most likely had an inherited form of hearing loss and, as they suggested, genetic contribution to the presence of congenital cholesteatoma cannot be excluded [ 4 ]. A correlation between the formation of congenital cholesteatoma and abnormal cochleovestibular anatomy and SNHL have also been reported by Propst et al and Jackler et al [ 15 , 16 ].…”

Section: Discussionsupporting

confidence: 55%

Cochlear implant and congenital cholesteatoma

Mierzwiński

Grochowski

et al. 2016

Journal of Otolaryngology - Head & Neck Surgery

View full text Add to dashboard Cite

BackgroundThe occurence of cholesteatoma and cochlear implant is rare. Secondary cholesteatomas may develop as a result of cochlear implant surgery. Primarily acquired cholesteatoma is not typically associated with congenital sensorineural hearing loss or cochlear implant in children. The occurrence of congenital cholesteatoma during cochlear implant surgery has never been reported before, partly because all patients are preoperatively submitted to imaging studies which can theoretically exclude the disease.Case presentationWe have reported a rare case of congenital cholesteatoma, found during sequential second side cochlear implantation in a 3-year-old child. The child underwent a computed tomography (CT) scan and magnetic resonance imaging (MRI) at 12 months of age, before the first cochlear implant surgery, which excluded middle ear pathology. The mass was removed as an intact pearl, without visible or microscopic violation of the cholesteatoma capsule. All the areas where middle ear structures were touching the cholesteatoma were vaporized with a laser and the cochlear implant was inserted uneventfully. Further follow-up excluded residual disease.ConclusionWe believe that primary, single stage placement of a cochlear implant (CI) with simultaneous removal of the congenital cholesteatoma can be performed safely. However, to prevent recurrence, the capsule of the cholesteatoma must not be damaged and complete laser ablation of the surface, where suspicious epithelial cells could remain, is recommended. In our opinion, cholesteatoma removal and cochlear implantation should be staged if these conditions are not met, and/or the disease is at a more advanced stage. It is suspected, that the incidence of congenital cholesteatoma in pediatric CI candidates is much higher that in average pediatric population.

show abstract

“…Given the more frequent use of imaging in the setting of UHL, we more recently have begun to appreciate the relatively high prevalence (50%) of bCNC stenosis as the underlying etiology in children presenting with profound UHL . Recent work has demonstrated that both cochlear and vestibular apparati appear to be affected bilaterally in the majority of patients with cochlear anomalies, which may be due to abnormal signaling in the first few weeks’ of embryogenesis . Such a hypothesis may be supported by evidence that anatomic abnormalities such as bCNC occur on a spectrum that might range from bilateral symmetric deficits, to asymmetric deficits, and finally deficits that affect only a single ear.…”

Section: Discussionmentioning

confidence: 99%

“…experienced in temporal bone measurements used the measurement tools provided by the GE PACS system to analyze studies. The specific methods for measurements are detailed in Table and are consistent with those methods previously utilized and reported by our group for several temporal bone measurement studies . In summary, 26 measurements were performed on each cochleovestibular apparatus using direct measurements recorded in millimeters (rounded to two decimal points) and direct visualization and categorization.…”

Section: Methodsmentioning

confidence: 98%

“…Precise measurement of temporal bone structures has improved detection of previously overlooked anomalies . More specifically, both cochlear and vestibular apparati appear to be affected bilaterally in the majority of patients with cochlear anomalies, which is likely due to abnormal signalling in the first few weeks’ of embryogenesis . The purpose of the present study was to evaluate the entire vestibulocochlear apparatus in children with unilateral bCNC stenosis to determine if the ipsilateral and/or contralateral vestibulocochlear apparatus are affected.…”

Section: Introductionmentioning

confidence: 98%

See 1 more Smart Citation

Children with unilateral cochlear nerve canal stenosis have bilateral cochleovestibular anomalies

et al. 2018

Self Cite

View full text Add to dashboard Cite

Objectives/Hypothesis To investigate the cochleovestibular apparatus bilaterally in children with isolated unilateral bony cochlear nerve canal (bCNC) stenosis. Study Design Retrospective review. Methods Imaging studies of children with unilateral bCNC stenosis (<1.0 mm) on computed tomography imaging (N = 36) were compared with controls imaged due to trauma without temporal bone injury (N = 32). Twenty‐six measurements were obtained in each ear, assessing the bony internal auditory canal (IAC), cochlea, and vestibular end‐organs, and were analyzed using one‐way analysis of variance for intersubject comparisons and paired t tests for intrasubject comparisons with a Bonferroni adjustment for multiple comparisons (P = .0006). Results Patients with bCNC stenosis had a smaller IAC (P < .000) and cochlea (P < .000) on the stenotic side as compared with controls. Although the vestibular end‐organ was also smaller in bCNC ears, this difference was not significant. The contralateral ear also had a smaller bCNC (P < .000) and cochlea (P < .000) as compared with controls, although to a lesser degree than the stenotic side. Conclusions Children with unilateral bCNC stenosis have abnormal biometry of both the cochlea and the vestibular end‐organ in the affected and the normal contralateral ear as compared with controls. Level of Evidence 3b Laryngoscope, 129:2403–2408, 2019

show abstract

Cochleovestibular Anomalies in Children With Cholesteatoma

Cited by 11 publications

References 7 publications

Congenital cholesteatoma and cochlear implantation: Implications for management

Congenital cholesteatoma and cochlear implantation: Implications for management

Cochlear implant and congenital cholesteatoma

Children with unilateral cochlear nerve canal stenosis have bilateral cochleovestibular anomalies

Contact Info

Product

Resources

About