The prevalence of autoimmune diseases has been increasing over the last 20 years. The clinical presentation of this large and heterogeneous group of disorders depends on whether the involvement is organ-specific or non-organ-specific. Dizziness, vertigo, and disequilibrium are common symptoms reported by patients with vestibulocochlear involvement. The association of vertigo and autoimmune diseases has been largely documented, suggesting that autoimmune disorders could be overrepresented in patients with vertigo in comparison to the general population. The aim of this review is to present the recent literature findings in the field of autoimmune-mediated diseases with cochleovestibular involvement, focusing on the clinical presentation, diagnosis, and treatment of immune-mediated inner ear diseases including autoimmune inner ear disease (AIED), Meniere's disease, and bilateral vestibulopathy, as well as of systemic autoimmune diseases with audiovestibular disorders, namely, Behçet's disease, Cogan's syndrome, sarcoidosis, autoimmune thyroid disease, Vogt-Koyanagi-Harada syndrome, relapsing polychondritis, systemic lupus erythematosus, antiphospholipid syndrome, IgG4-related disease, and ANCA-associated vasculitides.

SUMMARY Disregarding the widely used division of skull base into anterior and lateral, since the skull base should be conceived as a single anatomic structure, it was to our convenience to group all those approaches that run from the antero-lateral, pure lateral and postero-lateral side of the skull base as “Surgery of the lateral skull base”. “50 years of endeavour” points to the great effort which has been made over the last decades, when more and more difficult surgeries were performed by reducing morbidity. The principle of lateral skull base surgery, “remove skull base bone to approach the base itself and the adjacent sites of the endo-esocranium”, was then combined with function preservation and with tailoring surgery to the pathology. The concept that histology dictates the extent of resection, balancing the intrinsic morbidity of each approach was the object of the first section of the present report. The main surgical approaches were described in the second section and were conceived not as a step-by-step description of technique, but as the highlighthening of the surgical principles. The third section was centered on open issues related to the tumor and its treatment. The topic of vestibular schwannoma was investigated with the current debate on observation, hearing preservation surgery, hearing rehabilitation, radiotherapy and the recent efforts to detect biological markers able to predict tumor growth. Jugular foramen paragangliomas were treated in the frame of radical or partial surgery, radiotherapy, partial “tailored” surgery and observation. Surgery on meningioma was debated from the point of view of the neurosurgeon and of the otologist. Endolymphatic sac tumors and malignant tumors of the external auditory canal were also treated, as well as chordomas, chondrosarcomas and petrous bone cholesteatomas. Finally, the fourth section focused on free-choice topics which were assigned to aknowledged experts. The aim of this work was attempting to report the state of the art of the lateral skull base surgery after 50 years of hard work and, above all, to raise questions on those issues which still need an answer, as to allow progress in knowledge through sharing of various experiences. At the end of the reading, if more doubts remain rather than certainties, the aim of this work will probably be achieved.

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Il carcinoma del sacco endolinfatico è un tumore molto raro come evidente dai dati presenti in letteratura ove anche centri di esperienza riferiscono tuttavia casistiche limitate. E’ un tumore maligno a lenta crescita, con tendenza all’invasione locale e scarsa tendenza alla disseminazione metastatica. L’insorgenza spesso tardiva dei sintomi e la difficoltà ad eseguire indagini bioptiche a livello della sede di origine, parete posteriore della rocca, ha reso la diagnosi di questo tumore spesso difficile, nonostante si riconoscano attualmente dati radiologici patognomonici della neoplasia. Patterns tipici di presentazione sono evidenziabili alla RM con mezzo di contrasto e alla TC per osso a strato sottile, rendendo nella maggior parte dei casi possibile la diagnosi radiologica. L’incidenza del tumore del sacco endolinfatico è maggiore nei pazienti affetti da sindrome di von Hippel Lindau (VHL), con una frequenza del 25% che fa parte del quadro sindromico. Negli anni dal 2012 al 2015 abbiamo osservato 7 casi, uno di essi con manifestazione della patologia bilaterale, tutti affetti da VHL. Quattro tra questi sono stati sottoposti a chirurgia presso il nostro centro per un totale di 5 procedure chirurgiche. Ogni caso è stato descritto dettagliatamente analizzando sintomi, intervallo tra comparsa dei sintomi, diagnosi e terapia. Non vi è stata morbidità post-operatoria aggiuntiva nei pazienti in cui la diagnosi e la terapia sono state precoci, mentre la gestione di tumori localmente avanzati è stata associata a deficit neurologici postoperatori, in particolare del VII, IX e X nervo cranico. I siti anatomici critici di coinvolgimento della malattia che hanno coinciso con un pianificato rischio di danno neuronale sono risultati essere il canale di Falloppio, il forame giugulare, l’apice della rocca petrosa. L’estensione intradurale nella fossa cranica posteriore è stato un altro elemento caratterizzante i tumori in stadio avanzato. I tumori del sacco endolinfatico che lo screening permette di evidenziare precocemente nei pazienti VHL, hanno buona prognosi quando affrontati precocemente, compatibilmente con le esigenze terapeutiche della malattia di base.

No abstract