Citations

ObjectiveSeveral studies have demonstrated small but statistically significant differences in quality‐of‐life (QOL) scores among vestibular schwannoma (VS) treatment modalities. However, does a several‐point difference on a 100‐point scale really matter? The minimal clinically important difference (MCID)—defined as the smallest difference in scores that patients perceive as important and that could lead to a change in management—was developed to answer this important question. While the MCID has been determined for QOL measures used in other diseases, it remains undefined in the VS literature.Study DesignDistribution‐ and anchor‐based techniques were utilized to define the MCID for the Penn Acoustic Neuroma Quality of Life (PANQOL) and 36‐Item Short Form Health Survey (SF‐36).SettingTwo academic referral centers.PatientsPatients with VS (N = 538).InterventionCross‐sectional postal survey.Main Outcome MeasuresMCID for PANQOL domains and total score and SF‐36 Physical and Mental Health Component Summary scores.ResultsThe MCID (median, interquartile range) for the PANQOL total score was 11 points (10‐12); the MCIDs for individual domains were as follows: hearing, 6 (5‐8); balance, 16 (14‐19); facial, 10 (no interquartile range); pain, 11 (10‐13); energy, 13 (10‐17); anxiety, 11 (5‐22); and general, 15 (11‐19). The MCID was 7 points (6‐11) for the SF‐36 Mental Health Component Summary score and 8 points (6‐10) for the Physical Health Component Summary score.ConclusionsThe MCIDs determined in the current study generally exceed differences reported in previous prospective studies, in which conclusions about QOL benefit (or harm) among VS treatment modalities were based on statistical significance alone. Moving forward, these MCIDs should be considered when interpreting results of VS QOL studies.

Objectives/HypothesisInverting papillomas (IPs) are benign locally invasive tumors that most commonly present within the sinonasal cavity. Temporal bone involvement is exceedingly rare, with fewer than 30 cases reported within the English literature to date.Study DesignCase series and systematic review of the literature.MethodsFour consecutive subjects with temporal bone inverting papilloma (TBIP) were treated, and an additional 28 previously published cases were identified in the literature. Main outcome measures were disease presentation, diagnostic evaluation, management strategy, and outcome.ResultsA total of 32 cases were analyzed. The median age at diagnosis was 54 years (mean 54.1; range 19–81 years). Nineteen (59%) patients had synchronous or metachronous sinonasal IP, whereas 13 (41%) had isolated temporal bone disease without sinus involvement. Over half of the patients undergoing microsurgical resection experienced at least one recurrence. Compared to patients with a history of sinus IP, subjects with primary TBIP were younger at time of presentation (44 vs. 58 years; P = 0.012); were more commonly female (62% vs. 32%; P = 0.15); and were less likely to have intracranial spread (8% vs. 26%; P = 0.36), cranial neuropathy (8% vs. 26%; P = 0.36), human papillomavirus positivity (11% vs. 57%; P = 0.11), or associated carcinoma (0% vs. 47%; P = 0.004).ConclusionsInverting papilloma of the lateral skull base is rare and can pose a significant therapeutic challenge. Primary lesions of the temporal bone appear to follow a less aggressive clinical course when compared to those arising in association with sinonasal disease. Gross total resection is the preferred method of treatment, when feasible, given the high rate of recurrence with subtotal resection and risk of associated malignancy.Level of Evidence4. Laryngoscope, 125:2576–2583, 2015

Objectives: This case presents a previously undescribed clinical scenario of spontaneous cerebrospinal fluid (CSF) leaks secondary to a lateral sphenoid sinus recess skull base dehiscence and contralateral Sternberg’s canal. This case report aims to characterize the presentation and successful management of these lesions. Methods: The electronic medical record was used to collect information pertaining to the patient’s clinical history. Results: The patient was a middle-aged, obese female with persistent clear rhinorrhea as her only presenting symptom. Neuroradiologic studies localized the defect to the lateral sphenoid sinus recess. CSF opening pressures were within normal limits, but radiographic findings were consistent with elevated intracranial pressure. After an endoscopic transnasal transsphenoidal approach failed to resolve the CSF leak, a transpterygoid approach facilitated CSF leak resolution. The patient then did well for the following 2 years, but later developed a CSF leak through a contralateral Sternberg’s canal. An endoscopic suprapterygoid procedure and ventriculoperitoneal shunt placement led to CSF leak resolution. Conclusion: This case demonstrates one of the only published examples of a sphenoid sinus CSF leak secondary to Sternberg’s canal as it was originally described in the literature. Wide endoscopic surgical exposure and intracranial pressure management ultimately led to CSF leak resolution.

OBJECT The optimal treatment for sporadic vestibular schwannoma (VS) is highly controversial. To date, the majority of studies comparing treatment modalities have focused on a narrow scope of technical outcomes including facial function, hearing status, and tumor control. Very few publications have investigated health-related quality of life (HRQOL) differences between individual treatment groups, and none have used a disease-specific HRQOL instrument. METHODS All patients with sporadic small- to medium-sized VSs who underwent primary microsurgery, stereotactic radiosurgery (SRS), or observation between 1998 and 2008 were identified. Subjects were surveyed via postal questionnaire using the 36-Item Short Form Health Survey (SF-36), the 10-item Patient-Reported Outcomes Measurement Information System short form (PROMIS-10), the Glasgow Benefit Inventory (GBI), and the Penn Acoustic Neuroma Quality-of-Life (PANQOL) scale. Additionally, a pool of general population adults was surveyed, providing a nontumor control group for comparison. RESULTS A total of 642 respondents were analyzed. The overall response rate for patients with VS was 79%, and the mean time interval between treatment and survey was 7.7 years. Using multivariate regression, there were no statistically significant differences between management groups with respect to the PROMIS-10 physical or mental health dimensions, the SF-36 Physical or Mental Component Summary scores, or the PANQOL general, anxiety, hearing, or energy subdomains. Patients who underwent SRS or observation reported a better total PANQOL score and higher PANQOL facial, balance, and pain subdomain scores than the microsurgical cohort (p < 0.02). The differences in scores between the nontumor control group and patients with VS were greater than differences observed between individual treatment groups for the majority of measures. CONCLUSIONS The differences in HRQOL outcomes following SRS, observation, and microsurgery for VS are small. Notably, the diagnosis of VS rather than treatment strategy most significantly impacts quality of life. Understanding that a large number of VSs do not grow following discovery, and that intervention does not confer a long-term HRQOL advantage, small- and medium-sized VS should be initially observed, while intervention should be reserved for patients with unequivocal tumor growth or intractable symptoms that are amenable to treatment. Future studies assessing HRQOL in VS patients should prioritize use of validated disease-specific measures, such as the PANQOL, given the significant limitations of generic instruments in distinguishing between treatment groups and tumor versus nontumor subjects.

Objectives/HypothesisTo investigate the influence of posttreatment audiovestibular symptoms, facial neuropathy, and headache on long‐term quality‐of‐life outcomes in patients with sporadic vestibular schwannoma (VS) utilizing the Short Form 36 (SF‐36) Health Survey and the Penn Acoustic Neuroma Quality of Life (PANQOL) scale.Study DesignCross‐sectional observation study.MethodsPatients with sporadic small‐ or medium‐sized VS (< 3.0 cm) who were evaluated between 1998 and 2008 at two independent tertiary academic referral centers were surveyed. Multivariable associations with the PANQOL total score and the SF‐36 physical and mental component scores evaluated using regression analysis.ResultsA total of 538 surveyed patients returned a completed questionnaire, providing a response rate of 79%. Two hundred forty‐seven (46%) patients underwent stereotactic radiosurgery, 143 (27%) microsurgery, and 148 (28%) observation. Multivariable regression analysis revealed that ongoing dizziness was associated with the greatest reduction in PANQOL total score, followed by headache. After adjusting for all examined features, ongoing dizziness and ongoing headache were the only two variables that were associated with both the SF‐36 physical and mental component scores. Patient sex and treatment modality did not significantly influence PANQOL or SF‐36 scores.ConclusionsOngoing dizziness and headache are the strongest predictors of long‐term quality‐of‐life reduction in patients with sporadic VS, while the impact of hearing loss, facial nerve function, and tinnitus are less by comparison. This information may be valuable for patient counseling, refinement of VS quality‐of‐life assessment instruments, and determining high‐yield targets for therapy in efforts to further improve patient outcomes.Level of Evidence4. Laryngoscope, 125:1697–1702, 2015

Objectives/HypothesisFibrous dysplasia is a condition of nonmalignant osseous change and may occur in a monostotic or polyostotic pattern, the latter potentially being associated with McCune‐Albright syndrome. Symptoms are highly variable and dependent upon lesion location and size.Study DesignRetrospective review.MethodsConsecutive subjects with fibrous dysplasia of the temporal bone were evaluated between 2000 and 2013 at two tertiary academic referral centers. Main outcome measures included disease presentation, diagnostic evaluation, management strategy, and outcome.ResultsSixty‐six patients with fibrous dysplasia of the skull were found to have involvement of the temporal bone. The mean age at diagnosis was 25 years, 39 (59%) were female, and the mean duration of follow‐up was 48 months. Six (11%) patients had monostotic disease, with the remaining 60 (89%) patients having the polyostotic form; 16 (24%) patients had McCune‐Albright syndrome. The most common presenting complaint was headache (59%), followed by hearing loss (29%). The most common exam finding was cosmetic deformity (50%). Cholesteatoma (3%) and spontaneous cerebrospinal fluid fistula (1.5%) were found in a small percentage. No patients had evidence of motor cranial neuropathy by history or physical examination.ConclusionsThe clinical presentation of fibrous dysplasia involving the temporal bone is variable. A growing number of patients are diagnosed incidentally through imaging, and since most patients experience a benign course, the majority can be followed clinically without need for intervention.Level of Evidence4. Laryngoscope, 125:1438–1443, 2015

Objective (1) To characterize long‐term dizziness following observation, microsurgery, and stereotactic radiosurgery (SRS) for small to medium‐sized vestibular schwannoma (VS) using a validated self‐assessment inventory; and (2) to identify clinical variables associated with long‐term dizziness handicap. Study Design Cross‐sectional observational study. Setting Two independent tertiary academic referral centers: one located in the United States and one in Norway. Subjects and Methods All patients with sporadic VS of less than 3 cm who underwent primary microsurgery, SRS, or observation between 1998 and 2008 were identified. Subjects were surveyed via a postal questionnaire using the Dizziness Handicap Inventory (DHI) and a VS symptom questionnaire. Results The overall survey response rate was 79%. A total of 538 respondents (mean age, 64 years; 56% female) were analyzed, and the mean time interval between treatment and survey was 7.7 years. Pretreatment variables associated with greater dizziness handicap included female sex, older age, larger tumor size, preexisting diagnosis of headache or migraine, and symptoms of dizziness predating treatment. Significant posttreatment features strongly associated with poor long‐term DHI scores included frequency and severity of ongoing headache. On multivariable analysis, treatment modality did not influence long‐term dizziness handicap. Conclusion At a mean of approximately 8 years following treatment, over half of patients with VS reported ongoing dizziness. The authors have identified several baseline features that may help predict the risk of lasting dizziness. Treatment modality does not appear to influence long‐term DHI score. We found a strong association between posttreatment headache and poor dizziness handicap. Future study is needed to further define this relationship.

Objectives/HypothesisApplication to otolaryngology–head and neck surgery residency is highly competitive, and the interview process strives to select qualified applicants with a high aptitude for the specialty. Commonly employed criteria for applicant selection have failed to show correlation with proficiency during residency training. We evaluate the correlation between the results of a surgical aptitude test administered to otolaryngology resident applicants and their performance during residency.Study DesignRetrospective study at an academic otolaryngology–head and neck surgery residency program.MethodsBetween 2007 and 2013, 224 resident applicants participated in a previously described surgical aptitude test administered at a microvascular surgical station. The composite score and attitudinal scores for 24 consecutive residents who matched at our institution were recorded, and their residency performance was analyzed by faculty survey on a five‐point scale. The composite and attitudinal scores were analyzed for correlation with residency performance score by regression analysis.ResultsTwenty‐four residents were evaluated for overall quality as a clinician by eight faculty members who were blinded to the results of surgical aptitude testing. The results of these surveys showed good inter‐rater reliability. Both the overall aptitude test scores and the subset attitudinal score showed reliability in predicting performance during residency training.ConclusionsThe goal of the residency selection process is to evaluate the candidate's potential for success in residency and beyond. The results of this study suggest that a simple‐to‐administer clinical skills test may have predictive value for success in residency and clinician quality.Level of Evidence4 Laryngoscope, 125:E57–E61, 2015

Object The aim in this study was to describe the clinical presentation, differential diagnosis, and risk for neurofibromatosis Type 2 (NF2) in pediatric patients presenting with cerebellopontine angle (CPA) and internal auditory canal (IAC) tumors. Methods The authors conducted a retrospective study at a tertiary care academic referral center. All patients with an age ≤ 18 years who had presented with an extraaxial CPA or IAC tumor between 1987 and 2012 were included in the study cohort. Data regarding symptoms, diagnosis, tumor characteristics, and NF2 status were collected and analyzed. Results Sixty patients (55% female, 45% male) harboring 87 tumors were identified. The mean age at diagnosis was 12.8 years (median 14.0 years, range 0.9–18.9 years). Schwannomas were the most commonly identified lesions (57 of 87 tumors, including 52 vestibular, 3 facial, and 2 trigeminal schwannomas), followed by meningiomas (5 of 87) and epidermoid cysts (4 of 87). Six malignant tumors were diagnosed, including small-cell sarcoma, squamous cell carcinoma, malignant meningioma, atypical rhabdoid-teratoid tumor, endolymphatic sac tumor, and malignant ganglioglioma. Headache, followed by hearing loss and imbalance, was the most common presenting symptom, whereas dysphagia, otalgia, and facial pain were uncommon. Neurofibromatosis Type 2 was diagnosed in 20 (61%) of 33 patients with vestibular schwannoma (VS), while the other 13 patients (39%) had sporadic tumors. Nineteen of the 20 patients with NF2 met the diagnostic criteria for that disorder on initial presentation, and 15 of them presented with bilateral VS. At the last follow-up, 19 of the 20 patients subsequently diagnosed with NF2 demonstrated bilateral VSs, whereas 1 patient with a unilateral VS and multiple other NF2-associated tumors has yet to demonstrate a contralateral VS to date. Only 1 patient presenting with an isolated unilateral VS and no family history of NF2 demonstrated a contralateral VS on subsequent radiological screening. Conclusions Cerebellopontine angle and IAC tumors in the pediatric population are rare. There are several noteworthy differences between the adult and pediatric populations harboring these lesions. While VS is the most common pathology in both age groups, the lesion was found in only 60% of the pediatric patients in the present study. Unlike in adults, VSs in the pediatric population were associated with NF2 in over one-half of all cases. The majority of pediatric patients with NF2 fulfilled the diagnostic criteria at initial presentation; however, approximately 7% of patients presenting with a seemingly sporadic (no family history of NF2) unilateral VS will meet the criteria for NF2 later in life. Finally, malignancies account for a significantly higher percentage (10%) of cases among pediatric patients. These findings underscore the importance of early screening and close radiological follow-up and may be helpful in patient counseling.