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Usman A. Khan, Jillian H. Plonsker, Rick A. Friedman, and Marc S. Schwartz

The natural history of neurofibromatosis type 2 (NF2) is profound bilateral hearing loss. The decision to pursue microsurgery may be more complicated in NF2 than with sporadic tumors. Schwannomas in NF2 often occur with other skull base tumors. Treatment should be tailored to preserve auditory perception for as long as possible. The authors present the case of a man with NF2 and a vestibular schwannoma who has poor hearing on the same side as a large petrous apex meningioma, both opposite to a well-hearing ear. This case highlights surgical decision-making and technical nuances during resection of collision tumors in NF2.

The video can be found here: https://stream.cadmore.media/r10.3171/2021.7.FOCVID21130

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William H. Slattery III, Laurel M. Fisher, William Hitselberger, Rick A. Friedman, and Derald E. Brackmann

Object

The authors reviewed the proportion of pediatric patients with neurofibromatosis Type 2 (NF2) in whom hearing was preserved after middle fossa resection of vestibular schwannoma (VS).

Methods

In this retrospective chart review the authors examined the cases of 35 children with NF2 who had undergone middle fossa resection (47 surgeries) between 1992 and 2004 in a neurotological tertiary care center. Surgical outcome was assessed using pure-tone average (PTA) thresholds obtained before and immediately after resection. Speech discrimination scores (SDSs) and pre- and postfacial nerve grades were also recorded. In 55% of surgeries, hearing of less than or equal to 70 dB PTA was maintained postoperatively. The American Academy of Otolaryngology–Head and Neck Surgery Class A hearing (PTA ≤ 30 dB and SDS ≥70%) was preserved in 47.7%. Facial nerve function was good (House–Brackmann Grades I or II) in 81% of the patients. Twelve patients had bilateral middle fossa resections; in nine (75%) of these patients hearing was maintained postoperatively in both ears.

Conclusions

More than half of the children with NF2 in the authors' cohort experienced hearing preservation after middle fossa resection was performed for VS. The authors recommend this approach for preserving hearing in children with NF2.

Free access

nlm-article

Robert C. Rennert, Danielle M. Levy, Jillian Plonsker, Jeffrey A. Steinberg, Rick A. Friedman, John R. Crawford, and Michael L. Levy

Pediatric cerebellopontine angle (CPA) meningiomas are extremely rare and are usually treated with a retrosigmoid surgical approach or radiation. The authors present the use of a middle fossa approach for the treatment of a symptomatic CPA meningioma in a 22-month-old female. The patient initially presented at 17 months with isolated progressive, long-standing right-sided facial weakness. MRI demonstrated a 5.0 × 5.0–mm right CPA lesion just superior to the cisternal segment of cranial nerve (CN) VII, which demonstrated growth on interval imaging. At 22 months of age she underwent a successful middle fossa craniotomy, including wide exposure of the porus acusticus, allowing for a gross-total resection with preservation of CNs VII and VIII. Pathological analysis revealed a WHO grade I meningioma. The patient remained neurologically stable on follow-up. The middle fossa approach can be used to safely access the CPA in properly selected pediatric patients.

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nlm-article

Robert C. Rennert, Michael G. Brandel, Jeffrey A. Steinberg, David D. Gonda, Rick A. Friedman, Takanori Fukushima, John D. Day, Alexander A. Khalessi, and Michael L. Levy

OBJECTIVE

The middle fossa transpetrosal approach to the petroclival and posterior cavernous sinus regions includes removal of the anterior petrous apex (APA), an area well studied in adults but not in children. To this end, the authors performed a morphometric analysis of the APA region during pediatric maturation.

METHODS

Measurements of the distance from the clivus to the internal auditory canal (IAC; C-IAC), the distance of the petrous segment of the internal carotid artery (petrous carotid; PC) to the mesial petrous bone (MPB; PC-MPB), the distance of the PC to the mesial petrous apex (MPA; PC-MPA), and the IAC depth from the middle fossa floor (IAC-D) were made on thin-cut CT scans from 60 patients (distributed across ages 0–3, 4–7, 8–11, 12–15, 16–18, and > 18 years). The APA volume was calculated as a cylinder using C-IAC (length) and PC-MPB (diameter). APA pneumatization was noted. Data were analyzed by laterality, sex, and age.

RESULTS

APA parameters did not differ by laterality or sex. APA pneumatization was seen on 20 of 60 scans (33.3%) in patients ≥ 4 years. The majority of the APA region growth occurred by ages 8–11 years, with PC-MPA and PC-MPB increasing 15.9% (from 9.4 to 10.9 mm, p = 0.08) and 23.5% (from 8.9 to 11.0 mm, p < 0.01) between ages 0–3 and 8–11 years, and C-IAC increasing 20.7% (from 13.0 to 15.7 mm, p < 0.01) between ages 0–3 and 4–7 years. APA volume increased 79.6% from ages 0–3 to 8–11 years (from 834.3 to 1499.2 mm3, p < 0.01). None of these parameters displayed further significant growth. Finally, IAC-D increased 51.1% (from 4.3 to 6.5 mm, p < 0.01) between ages 0–3 and adult, without significant differences between successive age groups.

CONCLUSIONS

APA development is largely complete by the ages of 8–11 years. Knowledge of APA growth patterns may aid approach selection and APA removal in pediatric patients.