Kraniyal sinir flivannomlar›n›n efllik etti¤i tip 2 nörofibromatozis: Olgu sunumu

On altı yaşında erkek hasta, bir ay önce başlayan sağ taraflı kulak çınlaması ve hafif sağırlık yakın- malarıyla başvurdu. Aile öyküsünde nörofibromato- zis tip 2 bulunan hasta bir yıl önce sol vestibüler şivannom nedeniyle ameliyet geçirmişti. Otonörolo- jik muayenede orta derecede sensorinöral işitme kaybı saptandı. Manyetik rezonans görüntülemede, iki taraflı hipoglossal sinirler, sağ ve sol vestibüler sinirler, sağ trigeminal, sol okulomotor ve sağ ab- dusens sinirlerin komşuluğunda ekstra-aksiyel yer- leşim gösteren, kontrast tutan birçok kitle saptandı. Kitleler kraniyal sinir şivannomu olarak değerlendi- rildi. Bulgular, eşlik eden herhangi bir santral sinir sistemi anormalliğinin bulunmadığı, nörofibromato- zis tip 2’nin nadir görülen bir tablosu olarak kabul edildi. Semptomatik medikal tedaviye başlanan hasta beyin cerrahisi kliniğine sevk edildi

Neurofibromatosis type 2 associated with multiple cranial nerve schwannomas: a case report

A 16-year-old male patient complained of right-sided tinnitus and mild deafness of one-month history. He also had a family history of neurofibromatosis type 2 and a history of a prior operation for left vestibular schwannoma a year ago. Otoneurologic examination revealed moderate sensorineural hearing loss.Magnetic resonance imaging demonstrated multiple extra-axial enhancing masses in the vicinity of both hypoglossal nerves, the right vestibular nerve, the left vestibular nerve, the right trigeminal, the left occulo- motor and the right abducens nerves. These findings were evaluated as multiple cranial nerve schwanno- mas. The case was considered a rare manifestation of neurofibromatosis type 2 without any concomitant abnormality in the central nervous system.Symptomatic medical treatment was initiated and the patient was referred to the neurosurgery department.

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