Long term surgical and hearing outcomes in the management of tympanomastoid paragangliomas.

OBJECTIVE: To analyze the long term outcomes after surgery in tympanomastoid paragangliomas. STUDY DESIGN: Retrospective study. METHODS: The charts of 145 patients with tympanomastoid paragangliomas managed between 1988 and 2013 were reviewed. The clinical features, audiological data, pre- and postoperative notes were noted. The tumors were staged according to the modified Fish and Mattox classification. The surgical approaches for all patients were formulated according to the surgical algorithm developed at our center. RESULTS: 34 (23.5%), 46 (31.7%), 22 (15.2%), 18 (12.4%) and 25 (17.2%) patients were diagnosed to have TMP class A1, A2, B1, B2 and B3 tumors respectively. Gross tumor resection was achieved in 141 (97.2%) patients. The facial nerve was uncovered in four patients and infiltrated in three. The cochlea was found eroded in seven cases. The mean follow-up was 48.4 months. Recurrence was seen in one patient (0.7%). In the cases where the facial nerve was preserved (n=143), the nerve function was graded as HB grade 1 in 138 patients (97%). Postoperatively, the mean AC showed an improvement in all categories except in class B2 and B3, which corresponds to the classes that include patients who underwent subtotal petrosectomy. CONCLUSION: We report the long term surgical outcomes in tympanomastoid paragangliomas in the largest series published till date. It is possible to completely eradicate all types of tympanomastoid paragangliomas with minimum sequelae by choosing the correct surgical approach to achieve adequate exposure for individual tumor classes as described in our classification and algorithm. LEVEL OF EVIDENCE: IIb.

Intermediate nerve schwannoma: a rare tumour.

The intermediate nerve (IN), also called the nerve of Wrisberg, is a relatively small nerve with a variable anatomical course. Currently, this nerve is not addressed well in the pertinent literature. Pre-operative diagnosis of IN tumours is clinically challenging due to the lack of related symptoms. Symptoms are typically caused by the functional deterioration of neighbouring anatomical structures. In this report, we describe a patient with a schwannoma that arose at the porus acusticus of the internal auditory canal; during surgery, this proved to originate from the IN. We discuss our findings in the context of previously reported cases of IN schwannomas and analyse the vague characteristics and symptoms of this rare tumour.

[Emotional aspects and quality of life of patients after acoustic neuroma surgery]. / Aspetti emotivi e qualità della vita in pazienti operati di neurinoma del nervo acustico.

UNLABELLED: Acoustic neuromas are rare lesions arising in the internal auditory canal (IAC) and extending in the posterior cranial fossa. Surgical removal is the treatment of choice; peri and postoperative complications are directly related to the size of the tumour. Some Authors think the surgical treatment and related temporary or permanent neurological deficits could change the QoL and the psychological status. PURPOSE: The aim of this analysis is to ascertain if the surgical procedure could significantly worsen the QoL of this population 12 months after the discharge from surgical department. METHODS: By using a questionnaires of Quality of Life aims to evaluate the impact that the disease and the surgical procedure have on this population. SUBJECTS: We have evaluated a sample of 150 subjects one year after the surgical removal of a acoustic neuroma. Data have been collected through questionnaires the Whoqol-Brief, the BDI, the STAI Y1 and Y2 and specific item to assess effects of postoperative dysfunctions. RESULTS: Data reveal a negative effect on QoL, 94% of the subjects show lower score at Whoquol-Brief and in anxiety with scores higher than normative score sample (state-anxiety (t = 5.602 p = .000; trait-anxiety: t = 4.354 p = .000). Higher incidence of depressive disorders have not been found. CONCLUSION: Psychological support to subjects after discharge from acoustic neuroma surgical removal should be tailored to improve quality of life and for a better management of anxiety.

Internal auditory canal metastasis.

This report deals with 3 cases of internal auditory canal metastasis, an extremely rare lesion, few cases having been reported in the international literature. Since pre-operative diagnosis is fundamental in the planning of a correct therapeutic strategy, it is important that the neurotologist be aware of the possibility of their occurrence in this particular area. Metastasis can occur unilaterally as well as bilaterally; the latter being the case in 1 of the patients described herein. Correct pre-operative diagnosis is particularly difficult in patients in whom the primary tumour has not been detected at the time of identification of the lesion in the internal auditory canal. The only characteristic, specific of metastasis, is the presence of multifocal cerebral lesions. However, these were detected in only 1 of the present cases. On the contrary, in cases of a single metastasis, both magnetic resonance imaging and computed tomography usually fail to show any distinctive feature when compared to the most common tumours of the internal auditory canal (vestibular schwannomas and meningiomas). Bilateral metastases can also be misdiagnosed as neurofibromatosis type 2. Clinical data that should alert the clinician are: rapidly progressive sensorineural hearing loss, followed by onset of progressive facial nerve weakness. Radiotherapy and/or chemotherapy are the two main treatment modalities, while surgical removal is reserved for selected cases of a single metastasis. Albeit, due to the paucity of specific radiological and clinical characteristics, surgical removal is often necessary to reach the correct diagnosis, as occurred in 2 of the present patients.

[Preoperative imaging in chronic otitis surgery]. / Utilità della radiologia preoperatoria nella chirurgia dell'otite media cronica.

High resolution computed tomography (CT) is presently the most accurate technique to study the temporal bone. Nevertheless, there is no general agreement about its usefulness in pre-operative evaluation of chronic otitis media. Indeed, if we rule out some exceptions, CT is not fundamental for diagnosis which can often be obtained through an accurate otomicroscopy. The Otology Group in Piacenza applies the following absolute indications for pre-operative CT in chronic otitis media: 1) difficult otomicroscopy evaluation; 2) suspected petrous bone cholesteatoma; 3) dubious diagnosis; 4) suspect of malformations; 5) review of cases that had previously undergone mastoidectomy; 6) suspected intracranial complications and/or meningoencephalic herniation (in this case also a magnetic resonance imaging must be performed). With the exception of these specific conditions, pre-operative CT is useless in cases of simple chronic otitis. However, when a cholesteatoma is suspected, CT can provide the surgeon, particularly when inexperienced, useful, but not indispensable, informations. Pre-operative knowledge of these informations can allow a more accurate evaluation of the case, with a better planning of the surgical procedure, in order to ensure a more specific informed consent. Finally, the Authors point out the fact that surgeon must be able to interpret by his own the CT data to have a real advantage by this examination.

Electromyographic evaluation of facial nerve damage in acoustic neuroma surgery.

OBJECTIVE: This study aimed to determine whether postoperative facial nerve paralysis or surgical manipulation causing paralysis could be predicted on train responses during intraoperative facial nerve monitoring in acoustic neuroma surgery. STUDY DESIGN AND SETTING: This was a prospective study performed at a tertiary referral center. PATIENTS AND METHODS: Train responses were recorded on a floppy disk and compared with postoperative facial nerve function in 51 patients who underwent enlarged translabyrinthine acoustic neuroma surgery. MAIN OUTCOME MEASURES: The number, duration, frequency, and peak-to-peak amplitude of train responses were analyzed and compared with postoperative facial nerve function. RESULTS: Trains were observed in 42 of 51 patients. Six of seven patients with high-amplitude trains more than 250 microV, and three of five patients with bomber-type high-frequency trains elicited during tumor dissection from the facial nerve or stretching the nerve, showed severe facial nerve dysfunction. On the other hand, seven of the nine patients with no trains also showed severe facial nerve dysfunction. CONCLUSIONS: The presence of high-amplitude or high-frequency trains elicited by surgical manipulation to the facial nerve seems to indicate a critical situation for the facial nerve. However, certain types of mechanical trauma resulting in severe facial nerve paralysis cannot be identified by train responses.

Meningiomas intrinsic to the geniculate ganglion.

Geniculate ganglion meningiomas are extremely rare lesions-only 14 cases have been reported in the literature. Two new cases of these tumors are described. On computed tomography and magnetic resonance imaging, both lesions appeared centered on the area of the geniculate ganglion, extending to the tympanic cleft and eroding the middle cranial fossa floor. The first case was treated through a middle cranial fossa approach. Because the tumor was so large in the second case, a subtotal petrosectomy was used. The authors review the literature to clarify the clinical and radiological characteristics of these tumors and their surgical treatment.

[Treatment of residual acoustic neurinomas]. / Il trattamento dei neurinomi dell'acustico residui.

Generally the main objective of acoustic neurinoma (AN) surgery is to totally remove the tumor associated with minimum morbidity. Nevertheless, in some cases residual tumor fragments are intentionally or accidentally left in place. These residues can lead to new growth. The present study provides a retrospective analysis of 14 cases of residual AN have undergone surgery at the Otology Group in Piacenza from 1987 to 1999. All these patients had previously undergone at least one retrosigmoidal exeresis although only one had been performed at the Otology Group. All patients except 1 were affected by anacusia at the time of surgery. The list of post-operative deficits included 2 hemipareses, 3 irreversible facial paralysis with consequent corneal opacity in 2 cases, 1 dysmetria and 1 paralysis of the abducent nerve. The patients had also undergone the following additional treatments: 1 emergency revision to drain a cerebellar hematoma, 3 ventricle-peritoneal derivations, 1 double application of stereotactic radiotherapy and 2 surgical procedures for facial plasty. Ten cases underwent the revision surgery at the Otologic Group using a translabyrinthine approach and 4 using a transcochlear approach. Tumor removal was deemed complete in all cases. The sole post-operative complications were a subcutaneous hematoma at the point where abdominal fat was removed and a temporary paralysis of the abducent nerve. Post-operative hospitalization was an average of 6.9 days. Analysis of the results showed that AN must be operated at selected centers in order to reduce the post-operative neurological deficit and the percentage of residual tumor. It also indicated that the retrosigmoid approach has a higher risk of accidentally leaving tumor residues than the other approaches. Finally, in the presence of a residual AN, the translabyrinthine approach offers the greatest advantages.

Fast-growing vestibular schwannoma.

A case of a Jehovah's witness affected by an intracanalicular vestibular schwannoma with an extremely fast growth rate is presented. Nine months after presentation, the tumor reached 23 mm in the cerebellopontine angle. A partial removal through a retrosigmoid approach was planned. Because of the presence of a dominant high jugular bulb masquering the internal auditory canal, the intracanalicular portion of the tumor was left in place. The residual tumor grew 12 mm in 2 months. Even after a gross total removal through a middle cranial fossa approach, the tumor recurred, reaching the size of 30 mm in 17 months. A modified transcochlear approach was then performed, and the patient was free of disease at the last radiologic follow-up, 8 months after the surgery. We illustrate our strategy in treating this aggressive benign lesion with unusual behavior.

[Facial nerve identification in the translabyrinthine approach: an alternative method]. / Identificazione del nervo facciale nella via translabirintica: un metodo alternativo.

Since the abrupt drop in the mortality rate as a result of the introduction of microsurgical dissection techniques in the treatment of acoustic neuromas, surgeons have concentrated their efforts on preserving hearing and facial nerve function. In the translabyrinthine approach, identification of the facial nerve at the fundus of the internal auditory canal is an important step for subsequent dissection. However, the identification techniques available to date carry with them some potential risk of facial nerve injury when performed by inexperienced surgeons. In addition, they are time-consuming procedures. The authors present an alternative method for identification of the facial nerve at the fundus of the internal auditory canal during the translabyrinthine approach. The superior ampullary nerve is interrupted at the superior cribrosa area where it is not in intimate relationship with the facial nerve. Medial reflection of the superior ampullary nerve and the superior vestibular nerve facilitates identification of the facial nerve and preparation of a vestibulo-facial dissection plane.

[Intrapetrous carotid artery aneurysm]. / Gli aneurismi della carotide intrapetrosa.

Intratemporal carotid artery aneurysms are rare lesions, with only 54 cases reported in the literature. Their most common symptoms are pulsatile tinnitus, hearing loss and signs of Eustachian tube obstruction. In case of aneurysm rupture, bleeding may be so profuse as to require emergency legation of the common carotid in the neck. Arteriography is the diagnostic gold standard for this disorder. Successful treatment usually involves selective aneurysm embolization or carotid closure with detachable balloons. The authors report a new case of intratemporal carotid artery aneurysm previously treated with selective embolization. To avoid the risk of aneurysm recanalization and/or infection through the external auditory canal, middle ear obliteration and blind-sac closure of the external canal were performed in this case.

[Mastoid osteomas: review of literature and presentation of 2 clinical cases]. / Gli osteomi della mastoide: revisione della letteratura e presentazione di due casi clinici.

The authors report two cases of mastoid osteoma in patients at the "S. Filippo Neri" Hospital in Rome, Italy. The literature on this topic is critically reviewed. This review includes the 48 cases between 1875 and 1955 reported by Kecht, as well as the 92 cases Probst reported in 1991 in a review running from 1861. The literature published since 1991 has presented eight cases which, when added to the two presented here and the 92 indicated by Probst, total 102 cases of mastoid osteoma. The most widely accepted theories on the etiopathogenesis of mastoid osteoma are presented. These include: embryogenesis, metaplasia, inflammation, and trauma. The conclusions underline the complications of this disorder which are mainly linked to osteoma growth, and the importance of the differential diagnosis with other mastoid lesions for which prognosis is poorer.

[Mucocele of sphenoid sinus: presentation of 3 cases]. / Il mucocele del seno sfenoidale: presentazione di tre casi clinici.

Sphenoidal mucoceles are equally distributed between males and females occur rarely and have an incidence of 1%. Their low incidence is certainly to be ascribed to their deep position with respect to the superior respiratory tract as well as to the characteristics of the mucosa coating of the sinus (where the scarce muciparous component does not cause drainage problems). During endocranial manifestation, it is important to make a differential diagnosis between tumors at the base of the skull and adenomas of the hypophysis. The most feasible etiopathogenetic hypotheses are insufficient drainage of the sinus, cystic degeneration of the ghiandolar epithelium, presence of embryonal remnants and previous surgery. The Authors report three cases of mucoceles located in the sphenoid, two of which with destruction of the sellar floor and upward invasion, one with posterior invasion and involvement of the clivus. Sphenoidal mucoceles generally tend to spread more frequently in an anterior-inferior fashion with invasion of the etmoid, the nasal fossae and the rhinopharynx. In some cases we observe upward invasion with destruction of the sellar floor, as in the first two patients. There may by an invasion in the orbital cavity when spreading occurs sideways. More rarely, the intermediate cranial fossa is invaded through the lateral wall and the posterior cranial fossa through the posterior wall. Therefore, it is evident that an early diagnosis is always desirable because it may spare the patient the neurological manifestations resulting from the evolution of the disease. The treatment for a sphenoidal mucocele is surgical. The Authors report a synthesis of the surgical techniques most frequently used by otorhinolaryngologist and neurosurgeons, and present a critical analysis of the cases presented.

[Rare manifestation of nasal polyposis in the Peutz-Jeghers syndrome]. / Rare manifestazioni della poliposi nasale nella sindrome di Peutz-Jeghers.

We are relating a case of the Peutz-Jeghers syndrome, a disease characterized by hamartomatous polyposis and by mucocutaneous melanic pigmentation, in a rare variant identified by the presence of nasal polyposis. At the objective E.N.T. examination, the patient's nasal fossae were completely obstructed by translucid masses of a polypoid appearance and, moreover, a melanic pigmentation of the cutis, of the perilabial mucosa and of the oral cavity was encountered. At the X-ray examination and, later, during the operation, also the right maxillary sinus appeared completely obstructed by polypoid masses. At the histologic examination, such polypoid neoformations were quite similar to common polyps of an inflammatory nature and, so, absolutely without any peculiar structural features. The nasal polyposis in patients suffering from the Peutz-Jeghers syndrome has been mentioned by Peutz since his first observation but it has been seldom described in the subsequent publications, even in relation to the presentation of rare causes to Congresses and Magazines of an exclusively gastroenterological interest, in which the main concern was that of identifying both the clinical and histological aspects of gastrointestinal hamartomatous polyposis and the correlation between the syndrome and the formation of malignant neoplasias in other organs to the prejudice of the secondary manifestations of the disease. Nevertheless, the nasal polyposis and, at any rate, the tendency to the formation of polyps in other districts (urinary tract, bronchi) are described among the less important features also in gastroenterological treatises of most recent publication. Clinical observations, even occasional, are completely missing in the E.N.T. ambit.(ABSTRACT TRUNCATED AT 250 WORDS)