The Gruppo Otologico experience of endolymphatic sac tumor

Objective

Endolymphatic sac tumor (ELST) is a rare low grade adenocarcinoma of the skull base. During the past decade the number of the reported cases has increased. This study exposes our experience in the management of ELST with a review of the literature.

Study design

Retrospective study of patients with ELST at a quaternary referral otology and skull base center.

Methods

A review of the records from the Gruppo Otologico revealed 7 patients treated for ELST. All papers containing series of three or more cases of ELST published in the English literature were selected for analysis.

Results

Hearing loss and tinnitus were present in almost all our cases. All of them were evaluated with audiometric tests, computed tomography and magnetic resonance imaging. All the patients were treated surgically with preservation of the facial nerve and preoperative embolization was performed in 5 patients. Genetic study was performed on all our cases and revealed the presence of von Hippel–Lindau syndrome in one patient who had the tumor as the initial manifestation of his syndrome. None of the patients received postoperative radiotherapy and one of them had recurrence of the tumor 13 years following surgery.

Conclusions

Complete surgical resection with preoperative embolization of large tumors is the mainstay treatment for ELST. The facial nerve should not be sacrificed unless it is totally invaded by the tumor. A long term follow up is recommended and the role of postoperative adjunctive radiotherapy is still controversial.     

Inner ear extension of vestibular schwannomas

OBJECTIVE: Inner ear extension of vestibular schwannomas (VSs) is a rare finding but has important clinical implications. This report reviews the treatment options and presents the experience of the Gruppo Otologico, Piacenza, Italy, in this field. STUDY DESIGN: Case report and literature review. METHODS: Five cases of VSs with inner ear extension were surgically removed. In all of them, the cochlea was partially or completely invaded by the lesion. RESULTS: In 4 cases, the inner ear extension was preoperatively identified on magnetic resonance imaging, and the surgical removal was planned through a transotic approach. In the last case, the cochlear invasion was not detected preoperatively, and the lesion was removed during a second surgery performed to seal a cerebrospinal fluid fistula. CONCLUSIONS: VSs with inner ear extension should be distinguished from pure intralabyrinthine schwannomas because of differences in clinical significance. Cochlear involvement is more frequent than vestibular involvement and is often accompanied by a dead ear. Dead ear caused by small VSs should alert the surgeon to the possibility of a cochlear extension. The presence of an intracochlear involvement requires the adoption of an approach that allows control of the cochlear turns, and we found the transotic approach to be the most suitable. Undetected cochlear extensions that are left in place may grow with time.     

Third branchial cleft sinus route of infection in deep neck abscesses

Several recent articles have described a third branchial cleft sinus as a route of infection in deep neck abscess. This article describes two children whose presentation and clinical course were secondary to a third branchial cleft sinus.     

Transapical extension in difficult cerebellopontine angle tumors

The transapical extension of the enlarged translabyrinthine approach is aimed at making the removal of certain tumors of the cerebellopontine angle simpler and with less morbidity. The extension is classified into 2 types--type I and type II--based on the extent of bone drilling around the internal auditory canal. Type I extension involves drilling for 300 degrees to 320 degrees around the canal and is indicated for large or giant vestibular schwannomas and vestibular schwannomas with significant extension anterior to the internal auditory canal. Type II extension entails complete removal of the bone around the canal for 360 degrees and is indicated for meningiomas of the posterior surface of the petrous bone extending anterior to the internal auditory canal. The extension allows better control over the anterior pole of the tumor, the displaced facial nerve, the prepontine cistern, and the venous vascularity of the cerebellopontine angle. The aim of this report is to present our experience with this extension.     

Pulsatile tinnitus as a rare presenting symptom of residual cholesteatoma

Pulsatile tinnitus is an uncommon otological symptom, which often presents a diagnostic and management dilemma to the otolaryngologist. This symptom always deserves a thorough evaluation to avoid disastrous consequences from potentially life-threatening associated pathology. In most of the patients a treatable underlying aetiology can be identified. Frequent causes mentioned in the literature responsible for pulsatile tinnitus are benign intracranial hypertension syndrome, temporal bone paragangliomas and arteriovenous fistulae. Pulsatile tinnitus as a consequence of sigmoid sinus compression by a cholesteatoma has not been reported previously in the literature. Here a case of residual cholesteatoma with pulsatile tinnitus is presented, nine years after the first surgery.     

Rapidly growing cystic vestibular schwannoma with sudden onset facial palsy,ten years after subtotal excision

An elderly male patient diagnosed with a right‐sided cystic vestibular schwannoma (CVS) at our center underwent a translabyrinthine approach with a subtotal excision to preserve the facial nerve (FN). The tumor grew slowly for the first 9 years but in the subsequent 2 years grew rapidly, with the patient developing a FN paralysis. Using the previous approach, a second surgery was done and the tumor was excised, leaving behind a sheath of tumor on the facial and lower cranial nerves. This case demonstrates that CVSs show unpredictable growth patterns and need to be followed up for a longer period of time. Laryngoscope, 128:1649–1652, 2018     

An unusual cause of obstructive sleep apnoea presenting during pregnancy

We describe a case of lingual thyroid (LT) with primary hypothyroidism, presenting during pregnancy and continuing beyond it with oropharyngeal obstructive symptoms and sleep apnoea syndrome (SAS) of mixed type. Although SAS of a combined obstructive and central type should not be too surprising in a case of LT with hypothyroidism, we were unable to find such a documentation previously. Only four weeks of L-thyroxin treatment resulted in a dramatic improvement in dysphagia, disturbed phonation, haemoptysis, arterial desaturation, sleep apnoea and overall sleep efficiency, in conjunction with a regression in the size of the lingual mass. This case highlights the vagaries confronted in the management of such a case and focuses on efforts towards accurate diagnosis and treatment.     

Petrous bone cholesteatoma

Petrous bone cholesteatoma is a rare pathologic entity and may be a difficult surgical challenge because of potential involvement of the facial nerve, carotid artery, dura mater, otic capsule, and risk of cerebrospinal fluid leak. The objective of this article is to present a personal classification of petrous bone cholesteatomas, a survey of recent surgical attitudes, and our present surgical strategy based on our experience with 54 operations between 1978 and 1990. Radical petromastoid exenteration with marsupialization and the middle cranial fossa approach were used only for small pure infra- or supralabyrinthine cholesteatomas, respectively. The enlarged transcochlear approach with closure of the external auditory canal was used for infralabyrinthine, infralabyrinthine-apical, and massive petrous bone cholesteatomas. Five cases with petrous bone cholesteatomas in different locations are described in detail to present the signs and symptoms together with the management.     

The system of modified transcochlear approaches for intradural skull base lesions

The modified transcochlear approach was used to manage 27 patients with intradural tumours of petroclival region and prepontine cistern. Total tumour removal was possible in 21 cases. Planned subtotal removal was done in 2 elderly patients; one was a chordoma with involvement of the cavernous sinus and extending upto the optic chiasma while the other was a petroclival meningioma involving the cavernous sinus with normal abducent nerve function. The remaining 4 cases are awaiting their second stage. The modified transcochlear approach is systematized and further classified into types A, B, C and D depending upon its extensions. The basic surgical technique with its extensions, details of the classification, indications of each type and results are presented in this report.