Stephan R. Wolf

Cranial Nerve and Hearing Function after Combined-Approach Surgery for Glomus Jugulare Tumors

In this retrospective study, oncologic and functional results of 46 patients treated for glomus jugulare tumor are reported. The standard surgical approach was the combined transmastoid-transcervical approach, modified according to the individual tumor growth, and eventually combined with a transtemporal or a suboccipital approach. Complete tumor removal resulted in a cure rate of 90%. New-onset cranial nerve palsies developed in less than 22% of patients. In 54% of cases it was possible to retain middle ear function. From a total of 12 patients with incomplete tumor removal and postoperative irradiation, progressive tumor growth was noted in 4 patients, and was controlled by salvage irradiation or surgery. Radical tumor removal by ablative surgery can be modified by efforts to reduce mutilating resections. In their place, individually tailored and combined multidirectional surgical approaches may allow total tumor removal with lower morbidity.

Die idiopathische Fazialisparese

ZusammenfassungDie akute „idiopathische” Fazialisparese kann heute, wegen besserer Diagnostik, häufiger entzündlichen Ursachen zugeordnet werden als noch vor wenigen Jahren. Das Herpes simplex Virus Typ I und Borrelia burgdorferi sind in die Differentialdiagnose und in therapeutische Überlegungen einzubeziehen. Die klinische Untersuchung birgt, vor allem im Kindesalter, das Risiko einer Verkennung des Schweregrads einer Lähmung. Auch der „inkomplette Lidschluß” ist kein zuverlässiger Indikator einer Restfunktion des Nervs. Im Zweifelsfall ist bei jeder stärker ausgeprägten Fazialisparese eine elektromyographische Untersuchung und Verlaufskontrolle angezeigt. Die Elektroneuronographie spiegelt den „Denervierungsgrad” nicht zuverlässig, weshalb die Nadel-Elektromyographie bevorzugt wird.Die Therapie der Fazialisparese unklarer Genese ist noch immer nicht wissenschaftlich befriedigend abgesichert. Zumindest bei einer ausgeprägten Lähmung sollte trotzdem frühzeitig eine Kombinationstherapie mit Cortison, Virostatikum und hämorheologischer Therapie, eventuell auch Antibiotika eingesetzt werden. Eine chirurgische Dekompression des N. facialis bleibt höchstens in besonderen Ausnahmesituationen als ultima ratio.Individuelle Anleitungen zu mimischen Übungen und lockernden Gesichtsmassagen können die Rehabilitation unterstützen und bei richtigem Einsatz möglicherweise die Ausbildung von pathologischen Mitbewegungen reduzieren. Eine Elektrostimulationsbehandlung ist nicht notwendig. Eine Vielzahl von Möglichkeiten stehen heute zur Milderung einer Defektheilung zu Verfügung, wie z.B. die Injektion von Botulinum-Toxin. Die entscheidenden Weichen für den Verlauf einer Parese werden aber in der Frühphase gestellt, so daß intensive Diagnostik und Therapie nicht gescheut werden dürfen. Eine Verbesserung der Rehabilitation und vergleichende, multizentische Therapiestudien sollten in Zukunft intensiviert werden.SummaryAlthough acute idiopathic facial paresis is often laballed ”Bell’s palsy”, historical studies show that Nicolaus Anton Friedreich (1761– 1836) from Würzburg was the first physician to describe the typical symptoms of the disorder in 1797, approximately 24 years prior to the paper published by Sir Charles Bell. Diagnostics has now improved to the extent that acute idiopathic facial palsy can more frequently be assigned to etiologies caused by inflammatory disorders. Herpes simplex virus type I and Borrelia burgdorferi are particularly relevant. Underestimation of the degree of paresis is, particularly in children, a drawback of the clinical examination. ”Incomplete eyelid closure” is not a reliable indicator of remaining nerve function. For this reason complete electromyography (EMG) is recommended in all cases of severe facial paresis. Since electroneurography does not reliably reflect the degree of denervation present, needle EMG is preferred. The therapy of the facial palsy of unclear etiology is still not well defined. Nevertheless, we recommend that a combined treatment should be used early, at least in patients with disfiguring pareses. Combinations may consist of cortisone, virostatic agents and hemorrheologic substances and possibly antibiotics. Surgical decompression of the facial nerve remains controversial, since positive surgical results lack statistical support. Individual instructions for facial exercises, massage and muscle relaxation can support rehabilitation and possibly reduce the production of pathological synkinesia. Electrical stimulation should not be used. There are a number of possibilities available to reduce the effects of misdirected reinnervation, especially the use of botulinum-A-toxin. However, intensive diagnosis and therapy in the early phase of paresis are decisive in obtaining a favorable outcome. Further refinements in rehabilitation and comparative multicenter controlled studies are still required for future improvements in affected patients.

A threshold-like measure for the assessment of olfactory sensitivity : the random procedure

Abstract Many tests of olfactory dysfunction are either too complex, too expensive, or too time-consuming to be of use in routine clinical testing. Thus, the present multi-center study was undertaken to investigate a new approach, the so-called “random” test. In this test different concentrations of citronellal and phenyl ethyl alcohol are applied according to a pre-established order; patients are asked to identify the odor if possible. The test score is the sum of correctly identified odors. Test administration takes about 10 min. Two studies were performed. Basic characteristics of the test were explored in experiment 1 in 176 healthy subjects (76 male, 100 female; age 12–85 years, mean age 30 years), namely test–retest reliability, correlation with other measures of olfactory sensitivity, and sensitivity of the test to differences in age and gender. In the second experiment the test was tried in 97 patients (45 male, 52 female; age 19–78 years, mean age 47 years) in a clinical environment to investigate its usefulness in diagnosing olfactory loss. The “random”-test was found (1) to exhibit a test–retest reliability similar to that reported for established measures of olfactory function (r = 0.71; P < 0.001), (2) to correlate with other measures of olfactory sensitivity (0.82 > r > 0.60; P < 0.001), (3) to differentiate between expected differences in olfactory sensitivity in relation to gender (t > 2.602, P < 0.011), and (4) to discriminate between different degrees of olfactory loss (F > 36.6, P < 0.001). Based on these data, and the fact that the new test requires little time and is easy to use, this approach can be expected to suit clinical needs.

Clinical Survey of Meniere's Disease: 574 cases

Only 27% out of 574 patients suffering from Meniéres disease initially started with the typical Meniéres triad. A great advantage in order to diagnose Meniéres disease can be established with the telemetric-ENG. With this equipment it is possible to verify a nystagmus outside the clinic (at home, at work) at any time in patients suffering from vertigo attacks. Most of our patients showed an omnifrequent sensorineural hearing loss on the affected side. Many of our patients with Meniéres disease suffered from internal diseases (e.g. hypotonia, hyperlipidemia, diabetes mellitus), allergy or an affection of the paranasal sinuses, which may constitute co-factors triggering Meniéres disease.

Experimental Sensorineural Hearing Loss Following Drill-induced Ossicular Chain Injury

In a guinea pig model, a standardized drill-induced injury to the body of the incus was applied, and the effects on hearing were characterized by electrocochleography. Drilling resulted in a threshold shift within seconds, and after 15 min it averaged 35.7 dB for clicks, 35 dB nHL for 4 kHz bursts, 36.7 dB nHL for 6 kHz bursts and 39 dB nHL for 8 kHz bursts. The deterioration of the threshold shift remained stable throughout the 5-week post-operative observation period. In five animals a disarticulation of the incudostapedial joint was performed prior to drilling, but this did not reduce the threshold shift. Caution is mandatory during drilling around an intact ossicular chain to avoid a permanent sensorineural hearing loss, and disarticulation of the incudostapedial joint prior to drilling has no protective value.

Endonasal endoscopic surgery for rhinogen intraorbital abscess : a report of six cases

Intraorbital abscess is a serious complication of sinusitis with the danger of permanent loss of vision and even the danger of life‐threatening progress. The recommended surgical procedure in the literature for drainage of an intraorbital abscess is the external approach. We report on successful functional endonasal endoscopic surgery in a series of six sequential cases with intraorbital abscesses following sinusitis. The main advantages of this approach are the simultaneous treatment of causative disorders with surgery following the pathogenic route of the abscess formation and lack of trauma to further structures. The endoscope with 25‐ or 70‐degree angled axis of vision enables the surgeon to explore and drain the abscess cavity, which often is located behind the bulbus, with minimal trauma. For the trained surgeon the field of vision is favorable as compared with the external approach when the abscess is located right in the axis of vision and one has to cut through healthy tissue and the intact skin, which, especially in children, can lead to long‐lasting visible scars.

Funktionserhaltende Chirurgie von Glomus-jugulare-Tumoren Ein realisierbares Ziel?

ZusammenfassungEine radikale Entfernung ausgedehnter Glomus-jugulare-Tumoren hinterläßt häufig schwere Einschränkungen durch Lähmungen der kaudalen Hirnnerven und die Obliteration des Mittelohrs. Die Operationsstrategie der Erlanger HNO-Klinik setzte sich zum Ziel, die postoperative Morbidität zu senken, und gleichzeitig die Sicherheit der Tumorkontrolle zu erreichen. Die vorliegende Untersuchung stellt die onkologischen und funktionellen Resultate retrospektiv an 24 Patienten mit Glomus-jugulare-Tumoren der Größen C und D dar. Der mittlere Nachbeobachtungszeitraum betrug 3,7 Jahre. Als Standardmethode der Operation diente der transmastoidale-transzervikale Zugangsweg, der je nach Wachstum des Tumors modifiziert und gegebenenfalls mit einer transtemporalen Freilegung des Felsenbeins oder mit Hilfe eines retrosigmoidalen Zugangs kombiniert wurde. Präoperativ wurde eine superselektive Embolisation, soweit möglich, ausgeführt. Eine Ergänzung mit postoperativer Strahlentherapie erfolgte in 7 Fällen. Eine Heilungsrate von 100% wurde nach vollständiger Entfernung des Tumors mit Hilfe eines multidirektionalen Vorgehens erzielt. Bei 38% dieser Patienten traten postoperativ neue Hirnnervenlähmungen auf. In 54% der Fälle konnten ein normal konturierter äußerer Gehörgang und das Mittelohr entweder erhalten oder rekonstruiert werden. Residualtumore mußten in 2 Fällen nach inkompletter Resektion und postoperativer Radiotherapie beobachtet werden. Die individuelle Anpassung des chirurgischen Vorgehens erlaubt eine funktionsschonende Therapie ausgedehnter Glomus-jugulare-Tumore. Die entsprechend der Tumorausbreitung gewählten Zugangswege und operativen Schritte gestatten vollständige Tumorresektion mit minimierter Morbidität.SummaryIn this retrospective study, oncologic and functional results of 24 patients treated for glomus jugulare tumor stages C and D are reported. The surgical approach used was a combined transmastoid-transcervical approach, which was modified according to individual tumor growth. As needed, this was combined with a transtemporal or retrosigmoid approach. Complete tumor removal resulted in a cure rate of 100%. Surgically induced cranial nerve palsies developed in 38% of the patients. In 54% of cases it was possible to retain middle ear function. Seven patients had incomplete tumor removal requiring postoperative irradiation, with tumor progression occurring in 2 patients. Radical tumor removal was modified by efforts to reduce mutilating resections. These results show that individually tailored and combined multidirectional surgical approaches can allow total tumor removal to be performed with lower morbidity.

Preservation of hearing in bilateral acoustic neurinomas by deliberate partial resection.

With the aim of preserving hearing, 20 acoustic neurinomas in 17 patients with neurofibromatosis 2 were intentionally submitted to an incomplete (80%) tumour removal. In 12 cases this was an operation on the last hearing ear with total deafness of the contralateral ear. If an auditus existed in both ears the better hearing ear was selected for the primary intervention. Early audiological controls evidenced residual hearing in 19 of the 20 cases operated on by the enlarged middle fossa approach, which was utilized inspite of the tumour diameters being between 1 and 6 cm in the cerebello-pontine angle. The oncologic and functional follow-up over 1 to 7 years showed different patterns of slow progression of hearing loss and of persistent auditory function over 2 to 7 years. Facial nerve function was excellent in 16 of the 18 controlled cases. Continued CT or MRT imaging revealed no signaling in 2 cases, constant tumour sizes in 10 cases and slow progression in 3 cases. With regard to the importance of an auditory communication in the younger adult, the described treatment modality appears to be the first choice method.

The Value of Methylprednisolone in the Treatment of an Experimental Sensorineural Hearing Loss Following Drill-induced Ossicular Chain Injury: A Randomized, Blinded Study in Guinea-Pigs

In a previously established animal model a standardized drill-induced injury to the body of the incus was applied, and the effects on hearing were characterized by electrocochleography. In a placebo-controlled, randomized, blinded study methylprednisolone showed no protective effect in reducing or improving the auditory threshold shifts, which occurred within seconds after drilling and remained stable throughout the 5-week observation period. Therefore the otologic surgeon must pay close attention to avoiding any contact of a rotating burr with an ossicle in an intact ossicular chain.

On the site of transcranial magnetic stimulation of the facial nerve: electrophysiological observations in two patients after transection of the facial nerve during neuroma removal

The site of stimulation of the facial nerve after transcranial temporo-occipital magnetic stimulation is being controversially discussed, particularly whether the nerve is stimulated in the root exit zone in the cerebellopontine angle or whether stimulation originates within the bony canal of the facial nerve. In two case reports, the neurophysiological findings after the surgical transection of the facial nerve during the extirpation of a large acoustic and a facial nerve neuroma are presented. In both cases, transcranial magnetic stimulation of the facial nerve produced compound muscle action potential 4 and 2 days after the dissection of the facial nerve at the internal auditory canal and in the supralabyrinthine portion. These findings indicate that the site of stimulation in transcranial magnetic stimulation can be located to the course of the facial nerve within its bony petrosal canal distal to the external genu.