The endolymphatic sac tumor: challenges in the eradication of a localized disease.

OBJECTIVE: Identify the critical points that lead to recurrences and lack of radicality in endolymphatic sac tumors (ELSTs). STUDY DESIGN: Retrospective case study and review of the literature. SETTING: Tertiary referral center. PATIENTS: Thirteen cases of ELST were included in the study and their preoperative, intraoperative and postoperative data were analyzed and compared to a review of the literature. INTERVENTION(S): Therapeutical. MAIN OUTCOME MEASURE(S): Prevalence of recurrent and residual tumors, comparison to the literature and analysis of ELST characteristics. RESULTS: Diagnosis was made 26 ± 17 months after the onset of symptomatology, and an ELST was preoperatively suspected in only six cases. At the time of surgery, 10 patients suffered from hearing loss. Preoperative symptoms or audiometry could not predict labyrinth infiltration, although speech discrimination scores were significantly associated with labyrinth infiltration (p = 0.0413). The labyrinth was infiltrated in 8 cases (57.1%), and in 7 cases (46.7%) the tumor eroded the carotid canal, whereas 6 cases (40%) presented an intradural extension. A gross total resection was achieved in 11 cases. There were two residual tumors, one of which because of profuse bleeding, and one recurrence (23.1%). A mean of 22.8% of recurrent or residual tumors are described in the literature based on 242 published cases, in more than half of the cases as a consequence of subtotal tumor resection (STR). CONCLUSIONS: Recurrence derives mostly from the difficulty to identify the extension of the tumor due to the extensive bone infiltration. Accurate diagnosis and correct preoperative planning, with embolization when possible, will facilitate surgery and avoid STR due to intraoperative bleeding. Long follow-ups are important in order to avoid insidious recurrences.

Decision Making in the Wait-and-Scan Approach for Vestibular Schwannomas: Is There a Price to Pay in Terms of Hearing, Facial Nerve, and Overall Outcomes?

BACKGROUND: The wait-and-scan modality has emerged as an important strategy in the management of vestibular schwannoma (VS) as it has been demonstrated that many tumors grow slowly or do not show any growth over long periods. OBJECTIVE: To analyze long-term outcomes of wait-and-scan in the treatment of patients with VS, discuss the factors contributing to the decision making, determine the inherent risks of the policy, and compare our results with literature. METHODS: In total, 576 patients with sporadic unilateral VS who were managed with wait-and-scan were reviewed retrospectively. Of these, a subset of 154 patients with 5-yr follow-up was separately analyzed. The tumor characteristics including patterns of growth, rate of growth, hearing outcomes, and likely factors affecting the above parameters were analyzed. RESULTS: The mean period of follow-up was 36.9 ± 30.2 mo. The mean age was 59.2 ± 11.6 yr. Thirteen different patterns of tumor growth were observed. Eighty-four (54.5%) of 154 tumors with 5-yr follow-up showed no growth throughout 5 yr. Fifty-six (36.4%) tumors showed mixed growth rates. Only 57 (37%) patients had serviceable hearing at the start of follow-up, but 32 (56.1%) maintained it at the end of follow-up. One hundred fifty (26%) of the 576 patients who failed wait-and-scan had to be taken up for surgery. CONCLUSION: While there may be no price to pay in wait-and-scan as far as hearing is concerned, this may not be the case for facial nerve outcomes, wherein the results may be better if the patients are taken earlier for surgery.

Is endoscopic ear surgery an alternative to the modified Bondy technique for limited epitympanic cholesteatoma?

The objective of this study is to evaluate the outcomes of the modified Bondy's technique performed at our center and for limited epitympanic cholesteatomas and to debate the purported benefits of endoscopic surgery for the same indication. This is a retrospective study. 269 ears of 258 patients with a minimum of 5-year follow-up that were operated for limited epitympanic cholesteatoma using the modified Bondy's technique were included in the study. All patients had primary acquired cholesteatoma with good preoperative hearing in the affected ear and an intact ossicular chain. The outcomes of were analyzed and the results were compared with a literature review of outcomes of endoscopic ear surgery for the same indication. The mean follow-up was 81.63 months. The mean preoperative air-bone gap was 13.6 ± 7 dB. Intraoperatively, the ossicular chain was preserved in all patients. Postoperatively, there was no significant change from preoperative levels in mean air conduction, mean bone conduction and the air-bone gap. There were no recurrent cholesteatomas in our series. A residual pearl-like cholesteatoma was found lateral to the tympanic membrane in 8.1 % of ears, which was removed in the outpatient clinic. Three patients (1.2 %) developed stenosis of the meatoplasty. Eight (3.1 %) ears exhibited retraction pockets involving the attic. Postoperative ear discharge was observed in 1.5 % cases. The modified Bondy technique, which provides excellent postoperative outcomes, is the surgery of choice for limited epitympanic cholesteatomas. The endoscope, despite its better visualization of hidden areas does not provide a distinct overall technical advantage or better results over the microscope.

Hyperventilation-induced nystagmus in vestibular schwannoma and unilateral sensorineural hearing loss.

We evaluated the incidence and characteristics of hyperventilation-induced nystagmus (HVN) in 49 patients with gadolinium-enhanced magnetic resonance imaging evidence of vestibular schwannoma and 53 patients with idiopathic unilateral sensorineural hearing loss and normal radiological findings. The sensitivity and specificity of the hyperventilation test were compared with other audio-vestibular diagnostic tests (bedside examination of eye movements, caloric test, auditory brainstem responses) in the two groups of patients. The hyperventilation test scored the highest diagnostic efficiency (sensitivity 65.3 %; specificity 98.1 %) of the four tests in the differential diagnosis of vestibular schwannoma and idiopathic unilateral sensorineural hearing loss. Small tumors with a normal caloric response or caloric paresis were associated with ipsilateral HVN and larger tumors and severe caloric deficits with contralateral HVN. These results confirm that the hyperventilation test is a useful diagnostic test for predicting vestibular schwannoma in patients with unilateral sensorineural hearing loss.

Cystic vestibular schwannoma: classification, management, and facial nerve outcomes.

OBJECTIVE: Review of postoperative morbidity and facial nerve outcomes of cystic vestibular schwannoma (CVS) patients compared with solid vestibular schwannoma (SVS) patients and a proposal for a new CVS classification system. STUDY DESIGN: Retrospective review. SETTING: Tertiary care facility. PATIENTS: Ninety-six patients with surgically treated CVS (1998-2008). Outcomes were assessed in a subpopulation of 57 patients with greater than or equal to 1-year follow-up compared with 57 SVS patients. INTERVENTION: Fifty-six CVS patients underwent the enlarged translabyrinthine approach with transapical extension (Type I), and 1 patient underwent a transcochlear/transzygomatic approach. MAIN OUTCOME MEASURE: Preoperative and postoperative (at least 1 yr) House-Brackmann facial nerve (HBFN) grade evaluation. RESULTS: Favorable HBFN grades (I-III) were observed in 46 (81%) CVS patients, and unfavorable HBFN grades (IV-VI) were seen in 11 (19%) CVS patients. Comparison of tumor size and 1-year HBFN grades showed significant, moderate to strong, Pearson correlation (0.38). Comparison of long-term facial nerve outcomes with a sample of 57 matched SVS patients showed no significant difference (p = 0.74). When the tumor was adherent to the facial nerve and a dissection plane could not be developed between the cyst wall and the nerve, only subtotal resection could offer the CVS patients a normal facial nerve outcome. CONCLUSION: In most CVS cases, complete resection should be foreseen. Central and thick-walled tumors can be removed in almost all cases. However, when peripheral thin-walled, adherent, cystic tumors are confronted and the cysts are medially or anteriorly located, we recommend subtotal resection, leaving portions of the cyst walls on neurovascular structures and on the facial nerve. This surgical strategy allows us to improve facial nerve outcomes and to reduce complications.

Recurrent otitis media with effusion in preterm infants with histologic chorioamnionitis--a 3 years follow-up study.

BACKGROUND: Recurrent otitis media with effusion (OME) is a leading cause of acquired hearing loss in childhood. Histological chorioamnionitis (HCA) is an important cause of preterm delivery and neonatal morbidity and mortality. Here, we tested the hypothesis of an association between recurrent OME during the first 3 years of life and HCA in very low birth weight (VLBW) infants. METHODS: A total of 110 randomly selected VLBW preterm newborns with HCA and 135 gestational age and gender-matched, HCA-negative VLBW infants were evaluated prospectively during the first 3 years of life for the presence of OME, as diagnosed on the basis of otoscopy, type B or C tympanogram, ipsilateral absence of transient evoked otoacoustic emissions responses, and ipsilaterally increased threshold at diagnostic auditory brain responses evaluation. Potential risk factors for OME were also examined in the two groups. RESULTS: The HCA-positive infants showed a approximately six times higher frequency of recurrent OME (P<0.0001), increased frequency (>5/yr) of clinical otitis media episodes (P=0.000020), approximately five times higher frequency of adenoid hypertrophy (P<0.00001), a significant seasonal pattern of birth with autumn predominance (P<0.00001), and the first OME occurred earlier (P<0.0001), as compared to the HCA-negative counterparts. Recurrent OME was significantly associated with HCA (O.R.=17.76, 95% CI: 8.98-35.13, P<0.00001), adenoid hypertrophy (O.R.=9.96, 95% CI: 5.17-19.18, P<0.00001), frequency of acute otitis episodes >5/yr (O.R.=8.91, 95% CI: 1.96-40.41, P=0.0005), and birth in autumn (O.R.=5.58, 95% CI: 2.79-11.12, P<0.00001). CONCLUSIONS: These findings indicate that HCA is a previously unrecognized risk factor for the development of recurrent bilateral OME in VLBW preterm infants during the first 3 years of life.