Intralabyrinthine schwannomas : Surgical management and hearing rehabilitation with cochlear implants. 2017

S K Plontke, and T Rahne, and M Pfister, and G Götze, and C Heider, and N Pazaitis, and C Strauss, and P Caye-Thomasen, and S Kösling
Department of Otorhinolaryngology, Head & Neck Surgery, Martin Luther University Halle-Wittenberg, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. stefan.plontke@uk-halle.de.

Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss and vertigo. In an own case series of 12 patients, 6 tumors showed an intracochlear, 3 an intravestibular, 1 a transmodiolar including the cerebellopontine angle (CPA), 1a transotic including the CPA, and 1 a multilocular location. The tumors were removed surgically in 9 patients, whereas 3 patients decided for a "wait-and-test-and-scan" strategy. Of the surgical patients, 3 underwent labyrinthectomy and cochlear implant (CI) surgery in a single-stage procedure; 1 patient had extended cochleostomy with CI surgery; 3 underwent partial or subtotal cochleoectomy, with partial cochlear reconstruction and CI surgery (n = 1) or implantation of electrode dummies for possible later CI after repeated MRI follow-up (n = 2); and in 2 patients, the tumors of the internal auditory canal and cerebellopontine angle exhibiting transmodiolar or transmacular growth were removed by combined translabyrinthine-transotic resection. For the intracochlear tumors, vestibular function could mostly be preserved after surgery. In all cases with CI surgery, hearing rehabilitation was successful, although speech discrimination was limited for the case with subtotal cochleoectomy. Surgical removal of intracochlear schwannomas via partial or subtotal cochleoectomy is, in principle, possible with preservation of vestibular function. In the authors' opinion, radiotherapy of ILS is only indicated in isolated cases. Cochlear implantation during or after tumor resection (i. e., as synchronous or staged surgeries) is an option for hearing rehabilitation in cartain cases and represents a therapeutic approach in contrast to a "wait-and-test-and-scan" strategy.

UI MeSH Term Description Entries
D007758 Ear, Inner The essential part of the hearing organ consists of two labyrinthine compartments: the bony labyrinthine and the membranous labyrinth. The bony labyrinth is a complex of three interconnecting cavities or spaces (COCHLEA; VESTIBULAR LABYRINTH; and SEMICIRCULAR CANALS) in the TEMPORAL BONE. Within the bony labyrinth lies the membranous labyrinth which is a complex of sacs and tubules (COCHLEAR DUCT; SACCULE AND UTRICLE; and SEMICIRCULAR DUCTS) forming a continuous space enclosed by EPITHELIUM and connective tissue. These spaces are filled with LABYRINTHINE FLUIDS of various compositions. Labyrinth,Bony Labyrinth,Ear, Internal,Inner Ear,Membranous Labyrinth,Bony Labyrinths,Ears, Inner,Ears, Internal,Inner Ears,Internal Ear,Internal Ears,Labyrinth, Bony,Labyrinth, Membranous,Labyrinths,Labyrinths, Bony,Labyrinths, Membranous,Membranous Labyrinths
D007759 Labyrinth Diseases Pathological processes of the inner ear (LABYRINTH) which contains the essential apparatus of hearing (COCHLEA) and balance (SEMICIRCULAR CANALS). Inner Ear Disease,Ear Disease, Inner,Ear Diseases, Inner,Inner Ear Diseases,Labyrinth Disease
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D008297 Male Males
D008575 Meniere Disease A disease of the inner ear (LABYRINTH) that is characterized by fluctuating SENSORINEURAL HEARING LOSS; TINNITUS; episodic VERTIGO; and aural fullness. It is the most common form of endolymphatic hydrops. Meniere's Disease,Meniere's Syndrome,Vertigo, Aural,Auditory Vertigo,Aural Vertigo,Ménière Disease,Ménière's Disease,Ménière's Vertigo,Otogenic Vertigo,Auditory Vertigos,Disease, Meniere,Disease, Meniere's,Disease, Ménière,Disease, Ménière's,Diseases, Ménière,Diseases, Ménière's,Meniere Syndrome,Menieres Disease,Menieres Syndrome,Ménière Diseases,Ménière Vertigo,Ménière's Diseases,Ménière's Vertigos,Ménières Disease,Ménières Vertigo,Otogenic Vertigos,Syndrome, Meniere's,Vertigo, Auditory,Vertigo, Ménière's,Vertigo, Otogenic,Vertigos, Auditory,Vertigos, Ménière's,Vertigos, Otogenic
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009464 Neuroma, Acoustic A benign SCHWANNOMA of the eighth cranial nerve (VESTIBULOCOCHLEAR NERVE), mostly arising from the vestibular branch (VESTIBULAR NERVE) during the fifth or sixth decade of life. Clinical manifestations include HEARING LOSS; HEADACHE; VERTIGO; TINNITUS; and FACIAL PAIN. Bilateral acoustic neuromas are associated with NEUROFIBROMATOSIS 2. (From Adams et al., Principles of Neurology, 6th ed, p673) Acoustic Neuroma,Melanocytic Vestibular Schwannoma,Schwannoma, Acoustic,Schwannoma, Vestibular,Acoustic Neuroma, Cerebellopontine Angle,Acoustic Tumor,Angle Tumor,Cerebellopontine Angle Acoustic Neuroma,Cerebellopontine Angle Tumor,Neurilemmoma, Acoustic,Neurilemoma, Acoustic,Neurinoma of the Acoustic Nerve,Neurinoma, Acoustic,Neuroma, Acoustic, Unilateral,Vestibular Schwannoma,Acoustic Neurilemmoma,Acoustic Neurilemmomas,Acoustic Neurilemoma,Acoustic Neurilemomas,Acoustic Neurinoma,Acoustic Neurinomas,Acoustic Neuromas,Acoustic Schwannoma,Acoustic Schwannomas,Acoustic Tumors,Angle Tumor, Cerebellopontine,Angle Tumors,Angle Tumors, Cerebellopontine,Cerebellopontine Angle Tumors,Melanocytic Vestibular Schwannomas,Neurilemmomas, Acoustic,Neurilemomas, Acoustic,Neurinomas, Acoustic,Neuromas, Acoustic,Schwannoma, Melanocytic Vestibular,Schwannomas, Acoustic,Schwannomas, Melanocytic Vestibular,Schwannomas, Vestibular,Tumor, Acoustic,Tumor, Angle,Tumor, Cerebellopontine Angle,Tumors, Acoustic,Tumors, Angle,Tumors, Cerebellopontine Angle,Vestibular Schwannoma, Melanocytic,Vestibular Schwannomas,Vestibular Schwannomas, Melanocytic
D003051 Cochlea The part of the inner ear (LABYRINTH) that is concerned with hearing. It forms the anterior part of the labyrinth, as a snail-like structure that is situated almost horizontally anterior to the VESTIBULAR LABYRINTH. Cochleas
D003054 Cochlear Implants Electronic hearing devices typically used for patients with normal outer and middle ear function, but defective inner ear function. In the COCHLEA, the hair cells (HAIR CELLS, VESTIBULAR) may be absent or damaged but there are residual nerve fibers. The device electrically stimulates the COCHLEAR NERVE to create sound sensation. Auditory Prosthesis,Cochlear Prosthesis,Implants, Cochlear,Auditory Prostheses,Cochlear Implant,Cochlear Prostheses,Implant, Cochlear,Prostheses, Auditory,Prostheses, Cochlear,Prosthesis, Auditory,Prosthesis, Cochlear
D003639 Hearing Loss, Sudden Sensorineural hearing loss which develops suddenly over a period of hours or a few days. It varies in severity from mild to total deafness. Sudden deafness can be due to head trauma, vascular diseases, infections, or can appear without obvious cause or warning. Deafness, Sudden,Sudden Deafness,Sudden Hearing Loss

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