Driscoll, Colin L. W.

ObjectiveTo evaluate the use of monosyllabic word recognition versus sentence recognition to determine candidacy and long‐term benefit for cochlear implantation.Study DesignProspective multi‐center single‐subject design.MethodsA total of 21 adults aged 18 years and older with bilateral moderate to profound sensorineural hearing loss and low monosyllabic word scores received unilateral cochlear implantation. The consonant‐nucleus‐consonant (CNC) word test was the central measure of pre‐ and postoperative performance. Additional speech understanding tests included the Hearing in Noise Test sentences in quiet and AzBio sentences in +5 dB signal‐to‐noise ratio (SNR). Quality of life (QoL) was measured using the Abbreviated Profile of Hearing Aid Benefit and Health Utilities Index.ResultsPerformance on sentence recognition reached the ceiling of the test after only 3 months of implant use. In contrast, none of the participants in this study reached a score of 80% on CNC word recognition, even at the 12‐month postoperative test interval. Measures of QoL related to hearing were also significantly improved following implantation.ConclusionResults of this study demonstrate that monosyllabic words are appropriate for determining preoperative candidate and measuring long‐term postoperative speech recognition performance.Level of Evidence2c. Laryngoscope, 127:2368–2374, 2017

OBJECTIVEThe aim of this study was to evaluate the incidence, presentation, and treatment outcomes of trigeminal nerve–mediated symptoms secondary to large vestibular schwannomas (VSs) with trigeminal nerve contact. Specifically, the symptomatic results of pain, paresthesias, and numbness after microsurgical resection or stereotactic radiosurgery (SRS) were examined.METHODSThe authors conducted a retrospective review of a database for concomitant diagnosis of trigeminal neuralgia (TN) or trigeminal neuropathy and VS between 1994 and 2014 at a tertiary academic center. All patients with VS with TN or neuropathy were included, with the exception of those patients with neurofibromatosis Type 2 and patients who elected observation. Patient demographic data, symptom evolution, and treatment outcomes were collected. Population data were summarized, and outcome comparisons between microsurgery and SRS were analyzed at last follow-up.RESULTSSixty (2.2%) of 2771 total patients who had large VSs and either TN or neuropathy symptoms met inclusion criteria. The average age of trigeminal symptom onset was 53.6 years (range 24–79 years), the average age at VS diagnosis was 54.4 years (range 25–79 years), and the average follow-up for the microsurgery and SRS groups was 30 and 59 months, respectively (range 3–132 months). Of these patients, 50 (83%) had facial numbness, 16 (27%) had TN pain, and 13 (22%) had paresthesias (i.e., burning or tingling). Subsequently, 50 (83%) patients underwent resection and 10 (17%) patients received SRS.Treatment of VS with SRS did not improve trigeminal symptoms in any patient. This included 2 subjects with unimproved facial numbness and 4 patients with worsened numbness. Similarly, SRS worsened TN pain and paresthesias in 5 patients and failed to improve pain in 2 additional patients. The Barrow Neurological Institute neuralgia and hypesthesia scale scores were significantly worse for patients undergoing SRS compared with microsurgery.Resection alleviated facial numbness in 22 (50%) patients, paresthesias in 5 (42%) patients, and TN in 7 (70%) patients. In several patients, surgery was not successful in relieving facial numbness, which failed to improve in 17 (39%) cases and became worse in 5 (11%) cases. Also, surgery did not change the intensity of facial paresthesias or neuralgia in 6 (50%) and 3 (25%) patients, respectively. Microsurgery exacerbated facial paresthesias in 1 (8%) patient but, notably, did not aggravate TN in any patient.CONCLUSIONSOverall, resection of large VSs provided improved outcomes for patients with concomitant TN, facial paresthesia, and numbness compared with SRS. However, caution should be used when counseling surgical candidates because a number of patients did not experience improvement. This was especially true in patients with preoperative facial numbness and paresthesias, who frequently reported that these symptoms were unchanged following surgery.

OBJECTIVEPatient-reported outcomes are increasingly used in studies of vestibular schwannoma (VS); however, few studies have examined self-evaluated facial nerve function and its relation to physician-reported outcomes. The primary objective of this study was to compare patient self-evaluations of facial disability with physician-evaluated facial nerve status and with self-evaluations of a healthy control group. The second objective was to provide insight into the controversial subject of the optimal initial management of small- and medium-sized VSs; consequently, the authors compared patient-reported facial nerve disability following treatment via observation (OBS), Gamma Knife surgery (GKS), or microsurgery (MS). Lastly, the authors sought to identify risk factors for facial nerve dysfunction following treatment for small- and medium-sized VSs.METHODSAll patients with a VS 3 cm or smaller that was singly treated with OBS, GKS, or MS at either of 2 independent treatment centers between 1998 and 2008 were retrospectively identified. Longitudinal facial nerve measures and clinical data, including facial nerve evaluation according to the House-Brackmann (HB) grading system, were extracted from existing VS databases. Supplementing the objective data were Facial Disability Index (FDI) scores, which were obtained via survey of patients a mean of 7.7 years after initial treatment.RESULTSThe response rate among the 682 eligible patients was 79%; thus, data from a total of 539 patients were analyzed. One hundred forty-eight patients had been managed by OBS, 247 with GKS, and 144 with MS. Patients who underwent microsurgery had larger tumors and were younger than those who underwent OBS or GKS. Overall, facial nerve outcomes were satisfactory following treatment, with more than 90% of patients having HB Grade I function at the last clinical follow-up. Treatment was the major risk factor for facial nerve dysfunction. Almost one-fifth of the patients treated with MS had an objective decline in facial nerve function, whereas only 2% in the GKS group and 0% in the OBS cohort had a decline. The physical subscale of the FDI in the VS patients was highly associated with HB grade; however, the social/well-being subscale of the FDI was not. Thus, any social disability caused by facial palsy was not detectable by use of this questionnaire.CONCLUSIONSThe majority of patients with small- and medium-sized VSs attain excellent long-term facial nerve function and low facial nerve disability regardless of treatment modality. Tumor size and microsurgical treatment are risk factors for facial nerve dysfunction and self-reported disability. The FDI questionnaire is sensitive to the physical but not the social impairment associated with facial dysfunction.

OBJECTIVEOver the last 30 years, stereotactic radiosurgery (SRS) has become an established noninvasive treatment alternative for small- to medium-sized vestibular schwannoma (VS). This study aims to further define long-term SRS tumor control in patients with documented pretreatment tumor growth for whom conservative observation failed.METHODSA prospective clinical database was queried, and patients with sporadic VS who elected initial observation and subsequently underwent SRS after documented tumor growth between 2004 and 2014 were identified. Posttreatment tumor growth or shrinkage was determined by a ≥ 2-mm increase or decrease in maximum linear dimension, respectively.RESULTSSixty-eight patients met study inclusion criteria. The median pre- and posttreatment observation periods were 16 and 43.5 months, respectively. The median dose to the tumor margin was 13 Gy (range 12–14 Gy), and the median maximum dose was 26 Gy (range 24–28 Gy). At the time of treatment, 59 tumors exhibited extracanalicular (EC) extension, and 9 were intracanalicular (IC). Of the 59 EC VSs, 50 (85%) remained stable or decreased in size following treatment, and 9 (15%) enlarged by > 2 mm. Among EC tumors, the median pretreatment tumor growth rate was 2.08 mm/year for tumors that decreased or were stable, compared with 3.26 mm/year for tumors that grew following SRS (p = 0.009). Patients who demonstrated a pretreatment growth rate of < 2.5 mm/year exhibited a 97% tumor control rate, compared with 69% for those demonstrating ≥ 2.5 mm/year of growth prior to SRS (p = 0.007). No other analyzed variables were found to predict tumor growth following SRS.CONCLUSIONSOverall, SRS administered using a marginal dose between 12–14 Gy is highly effective in treating VSs in which initial observation fails. Tumor control is achieved in 97% of VSs that exhibit slow (< 2.5 mm/year) pretreatment growth; however, SRS is less successful in treating tumors exhibiting rapid growth (≥ 2.5 mm/year).

Objectives/HypothesisThis study was designed to examine speech recognition and self‐perceived health‐related quality of life (HRQoL) received from cochlear implantation among a cohort of adults and children with a short duration of unilateral hearing loss greater than 6 months, but less than 2 years.Study DesignSingle‐subject repeated measures prospective study.MethodsThis study assessed changes in speech recognition and self‐perceived quality of life by prospectively analyzing data at the preoperative evaluation and at the 3‐month and 6‐month postactivation intervals. Measurement tools included Medical Outcomes Study Questionnaire Short Form 36, Nijmegen Cochlear Implant Questionnaire, Speech Spatial and Qualities of Hearing–Comparative, and speech recognition measures in quiet and in noise.ResultsResults indicated significant improvement in speech recognition, both in quiet and noise. Quality‐of‐life measures showed a significant increase in self‐perceived benefit with disease‐specific instruments, but remained constant with a generic HRQoL instrument.ConclusionsCochlear implantation was a successful intervention for improved hearing in quiet and noise, and a self‐perceived benefit for this group of adults and children with a short duration of unilateral hearing loss.Level of Evidence4 Laryngoscope, 127:1683–1688, 2017

ObjectiveDemonstrate the association of radiographic dural enhancement with increased tumor adherence at the porus acusticus, which may influence completeness of resection and facial nerve outcome.Study DesignCase series with chart review.SettingAcademic referral center.Subjects and MethodsA total of 205 consecutive patients with histopathologically confirmed vestibular schwannoma (VS) were evaluated. Patients with tumors exhibiting dural enhancement on postgadolinium T1‐weighted magnetic resonance imaging were identified and compared with matched controls. Completeness of resection, intraoperative details, and facial nerve outcomes were analyzed.ResultsExcluding strictly intracanalicular tumors (n = 32, 16%) and those with NF2 (n = 10, 4.9%), the presence of dural enhancement was radiographically confirmed in 16 (9.8%) cases. Paired analysis did not reveal significant baseline differences between cases and controls. Subtotal resection was performed in 5 (31%) of the 16 patients with tumors exhibiting dural enhancement, in contrast to 1 (3%) of the matched controls (P =. 01). Four (25%) demonstrated increased tumor adherence at the porus acusticus intraoperatively, compared with 1 control (3%, P =. 04). Long‐term facial nerve function was similar between cases and controls (81% vs 84% House‐Brackmann I‐II function, P = 1.00).ConclusionDural enhancement is present in approximately 10% of extracanalicular VS. Dural enhancement at the porus acusticus may represent hypervascularity, dural reaction, or infiltration, and portends increased tumor adherence and greater likelihood of subtotal resection to preserve facial nerve function. To our knowledge, this is the first series that reports the prevalence of this phenomenon in VS and the potential surgical implications. Recognition preoperatively may be valuable toward patient counseling.

Purpose The purpose of this research note is to identify and prioritize diseases important for detection in adult hearing health care delivery systems. Method Through literature review and expert consultation, the authors identified 195 diseases likely to occur in adults complaining of hearing loss. Five neurotologists rated the importance of disease on 3 dimensions related to the necessity of detection prior to adult hearing aid fitting. Results Ratings of adverse health consequences, diagnostic difficulty, and presence of nonotologic symptoms associated with these diseases resulted in the identification of 104 diseases potentially important for detection prior to adult hearing aid fitting. Conclusions Current and evolving health care delivery systems, including direct-to-consumer sales, involve inconsistent means of disease detection vigilance prior to device fitting. The first steps in determining the safety of these different delivery methods are to identify and prioritize which diseases present the greatest risk for poor health outcomes and, thus, should be detected in hearing health care delivery systems. Here the authors have developed a novel multidimensional rating system to rank disease importance. The rankings can be used to evaluate the effectiveness of alternative detection methods and to inform public health policy. The authors are currently using this information to validate a consumer questionnaire designed to accurately identify when pre- fitting medical evaluations should be required for hearing aid patients.