[Acoustic neurinoma with preserved inner ear function. Interesting audiologic constellation of findings].
Neurofibromatosis (NF) is a syndrome with multiple neuromas. With the central form or NF2 very often bilateral acoustic neuromas are found. Tumours derive from Schwann cells of the eighth cranial nerve; symptoms are tinnitus, hearing loss and vertigo. Therapy is surgical, if possible with the protection of remaining hearing function. We report the case of a 28 year old female patient with neurofibromatosis type 2. After complete resection of the left AN and partial resection of the right AN the patient suffered from bilateral complete deafness, although almost normal DPOAE can be recorded on one side. Therefore we find the classical neural deafness with intact function of outer haircells (OHC).
Hesse
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G
Tinnitus-Klinik Arolsen, Grosse Allee 1-3, 34454 Bad Arolsen.
Laubert
A
A
D000328:Adult; D003054:Cochlear Implants; D017626:Cochlear Nucleus / Q000503:physiopathology; D003638:Deafness / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D018072:Hair Cells, Auditory, Outer / Q000503:physiopathology; D006801:Humans; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D009464:Neuroma, Acoustic / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D017084:Otoacoustic Emissions, Spontaneous / Q000502:physiology*; D011183:Postoperative Complications / Q000175:diagnosis / Q000503:physiopathology*