Neurofibromatosis 2 Invasion of the Internal Auditory Canal Wall

Doherty, Joni K.; Go, John L.; Linthicum, Fred H.

doi:10.1097/mao.0000000000000494

Cited by 6 publications

(6 citation statements)

References 16 publications

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“…Cerebellopontine angle (CPA) lesions are uncommon in children. Tumor mimickers in this region have been described, especially in cases of neurofibromatosis type 2, but often involve one type of tumor mistaken for another [ 1 , 2 ]. Here, we describe a patient with left 6th cranial nerve (CN) palsy whose imaging workup revealed a right CPA encephalocele emanating from the middle cerebellar peduncle that was later identified as containing heterotopic cerebellar tissue.…”

Section: Introductionmentioning

confidence: 99%

Developmentally anomalous cerebellar encephalocele arising within the cerebellopontine angle and extending into the adjacent skull base in a pediatric patient

et al. 2021

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Lesions of the cerebellopontine angle (CPA) in young children are rare, with the most common being arachnoid cysts and epidermoid inclusion cysts. The authors report a case of an encephalocele containing heterotopic cerebellar tissue arising from the right middle cerebellar peduncle and filling the right internal acoustic canal in a 2-year-old female patient. Her initial presentation included a focal left 6th nerve palsy. Magnetic resonance imaging was suggestive of a high-grade tumor of the right CPA. The lesion was removed via a retrosigmoid approach, and histopathologic analysis revealed heterotopic atrophic cerebellar tissue. This report is the first description of a heterotopic cerebellar encephalocele within the CPA and temporal skull base of a pediatric patient.

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Section: Introductionmentioning

confidence: 99%

Developmentally anomalous cerebellar encephalocele arising within the cerebellopontine angle and extending into the adjacent skull base in a pediatric patient

et al. 2021

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“…It is well established that the risk of damage to the vestibular, cochlear, and facial nerves is higher in patients with NF2-related VS. 31,32,35,39 VS in patients with NF2 are often larger than sporadic VS 34,37 and have also been found to be both more invasive into the nerve and surrounding bone as well as more adherent to adjacent structures. 40,41 In addition, the risk of recurrence is also higher in NF2-related VS due to the more aggressive nature of these tumors, larger tumor size, and involvement of adjacent structures, which results in the need for incomplete excision more often than in sporadic VS. 31,37,41,42 All of these factors may explain our worse facial nerve outcomes in patients with NF2, even after accounting for tumor size alone.…”

Section: Discussionmentioning

confidence: 99%

Facial Nerve Outcomes After Vestibular Schwannoma Microsurgical Resection in Neurofibromatosis Type 2

Sobieski

Killeen

Barnett

et al. 2020

Otolaryngol.--head neck surg.

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Objective The aim of this study is to investigate facial nerve outcomes after microsurgical resection in neurofibromatosis type 2 (NF2) compared to sporadic tumors. Study Design Single institutional retrospective chart review. Setting Tertiary referral center. Methods All adult patients with NF2 vestibular schwannoma (VS) or sporadic VS who underwent microsurgical resection from 2008 to 2019 with preoperative magnetic resonance imaging (MRI) and 1 year of postsurgical follow-up were included. The primary outcome measure was postoperative House-Brackmann (HB) facial nerve score measured at first postoperative visit and after at least 10 months. Results In total, 161 sporadic VSs and 14 NF2 VSs met inclusion criteria. Both median tumor diameter (NF2, 33.5 mm vs sporadic, 24 mm, P = .0011) and median tumor volume (NF2, 12.4 cm3 vs sporadic, 2.9 cm3, P = .0005) were significantly greater in patients with NF2. The median follow-up was 24.9 months (range, 12-130.1). Median facial nerve function after 1 year for patients with NF2 was HB 3 (range, 1-6) compared to HB 1 (range, 1-6) for sporadic VS ( P = .001). With multivariate logistic regression, NF2 tumors (odds ratio [OR] = 13.9, P = .001) and tumor volume ≥3 cm3 (OR = 3.6, P = .025) were significantly associated with HB ≥3 when controlling for age, sex, extent of tumor resection, translabyrinthine approach, and prior radiation. Conclusion Tumor volume >3 cm3 and NF2 tumors are associated with poorer facial nerve outcomes 1 year following microsurgical resection.

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“…The infiltrative nature of the tumor in the IAC and its tendency to remodel bone are other factors that may affect hearing and warrant study over time in patients with CVSs. 13,15-17…”

Section: Discussionmentioning

confidence: 99%

Audiologic Natural History of Small Volume Cochleovestibular Schwannomas in Neurofibromatosis Type 2

DeTorres

Brewer

Zalewski

et al. 2018

Otology &Amp; Neurotology

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Neurofibromatosis 2 Invasion of the Internal Auditory Canal Wall

Cited by 6 publications

References 16 publications

Developmentally anomalous cerebellar encephalocele arising within the cerebellopontine angle and extending into the adjacent skull base in a pediatric patient

Developmentally anomalous cerebellar encephalocele arising within the cerebellopontine angle and extending into the adjacent skull base in a pediatric patient

Facial Nerve Outcomes After Vestibular Schwannoma Microsurgical Resection in Neurofibromatosis Type 2

Audiologic Natural History of Small Volume Cochleovestibular Schwannomas in Neurofibromatosis Type 2

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