Carlson, Matthew L.

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.

ObjectivesTo elucidate the clinical behavior, treatment, and outcomes of tenosynovial giant cell tumors (TGCT) involving the temporomandibular joint (TMJ) and adjacent temporal bone.Study DesignRetrospective case series with histopathologic review.MethodsA retrospective chart review was performed identifying and collecting data from all cases of TGCT involving the TMJ and adjacent temporal bone that were treated at the authors' center between January 1960 and December 2015.ResultsEleven histopathologically confirmed cases met inclusion criteria. The median age at diagnosis was 49 years, eight patients were men, and the median follow‐up was 116 months. Computed tomographic (CT) imaging revealed a lytic expansile mass centered on the TMJ. Magnetic resonance imaging (MRI) most commonly exhibited hypointense signal on precontrast T1‐ and T2‐weighted sequences and variable postcontrast enhancement. The median delay in diagnosis was 24 months, and the most common presenting symptoms were hearing loss and pain. All patients underwent surgical resection, eight receiving gross total removal, one receiving near total removal, and two patients from early in the series receiving subtotal resection with neoadjuvant or adjuvant radiation. Histopathological review of surgical specimens revealed chondroid metaplasia in seven tumors. Eight of nine cases receiving gross total or near total resection have no evidence of recurrence to date.ConclusionsTGCT of the TMJ and temporal bone are rare and locally aggressive tumors that commonly present with nonspecific symptoms. A careful review of CT and MRI followed by early biopsy is critical in establishing an accurate diagnosis and facilitating appropriate treatment. TGCT of the TMJ more commonly contain chondroid metaplasia when compared to TGCT at other anatomic locations. Gross total resection is achievable in most cases and offers long‐term cure. Radiation may be considered for recurrent disease or adjuvant therapy following subtotal resection.Level of Evidence4. Laryngoscope, 127:2340–2346, 2017

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).

ObjectivePatients with incidental or minimally symptomatic superior semicircular canal dehiscence (SSCD) are usually observed, without surgical repair. However, it remains unknown whether a labyrinthine fistula of the superior semicircular canal is associated with progressive conductive or sensorineural hearing loss over time.Study DesignRetrospective review at two tertiary care academic referral centers.MethodsAdults analyzed were diagnosed with SSCD by high‐resolution temporal bone computed tomography and vestibular evoked myogenic potential testing and observed with a minimum of two sequential audiograms. Patients with other potential causes of hearing impairment were excluded.ResultsA total of 40 ears in 30 adult patients (median age: 59 years; 63% female) were analyzed. Median audiometric follow‐up was 23 months (range 1–136 months). None experienced a sudden hearing loss over the follow‐up period. In patients with audiometric follow‐up of at least 20 months (median 34 months), the median change in air‐conduction pure tone average and air–bone gap was 0.9 decibels (dB) per year (interquartile range [IQR] 0–2.1) and 0.7 dB per year (IQR 0–2.0), respectively. Speech discrimination scores did not differ when comparing median initial (100%) and median final (98%) scores (P = 0.77). There was no statistically significant change in bone‐conduction thresholds at 0.5, 1, 2, and 4 kHz over the period of observation.ConclusionThe risk of progressive hearing loss with observed SSCD is low during short‐ and intermediate‐term follow‐up. Further studies are necessary to determine whether late hearing loss occurs. Such information may be critical toward patient counseling regarding the need for and timing of surgery.Level of Evidence4. Laryngoscope, 127:1181–1186, 2017

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

Objective: To report hemifacial spasm (HFS) as a rare presenting symptom of a benign parotid neoplasm involving the stylomastoid foramen. Methods: Case report with literature review. Results: An 18-year-old woman presented with a 1-year history of a progressively enlarging right infra-auricular mass with new onset ipsilateral severe HFS. Clinical vetting unveiled a palpable 2 cm, firm, minimally mobile mass medial to the angle of the mandible. Imaging revealed a heterogeneous intraparotid mass with tumor extension into the stylomastoid foramen. Diagnostic consideration was given to neurogenic tumors and neoplasms of parotid origin. The patient ultimately underwent superficial parotidectomy and the tumor was found superficial and extrinsic to the proximal facial nerve trunk but extended into the stylomastoid foramen. The tumor was removed after performing mastoidectomy with mastoid segment facial nerve decompression. Frozen section pathology was consistent with pleomorphic adenoma. Postoperatively the patient had normal facial nerve function with immediate and complete resolution of HFS. To date there are only three other published reports of benign parotid tumors presenting with HFS and this is the first illustrating intratemporal tumor extension. Conclusions: HFS is a rare presenting symptom associated with benign parotid neoplasms with scarce documentation in the literature. In this index case, tumor extirpation with mastoid segment facial nerve decompression provided immediate and durable resolution of symptoms.

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.

Objective/HypothesisEosinophilic otitis media (EOM) is a variant of chronic otitis media that is characterized by the development of thick mucoid middle ear effusion, adult onset bronchial asthma, sinonasal polyposis, and aspirin sensitivity. EOM is typically refractory to corticosteroid therapy and surgical intervention. Pegylated interferon (PEG‐IFN) has effectively treated hypereosinophilic syndrome in clinical trials; however, the efficacy of this medication for EOM treatment remains undefined.Study DesignRetrospective, case series, tertiary academic center.MethodsA retrospective chart review was performed on EOM patients from 2008–2014. A total of 32 patients met the clinical criteria for EOM according to established diagnostic guidelines. Outcomes of all patients with severe, refractory EOM who initiated PEG‐IFN therapy are reported.ResultsEight patients were treated with pegylated interferon‐α 2a or 2b for refractory EOM. Half of the patients had significant side effects with interferon treatment. Three of these were able to continue at a reduced dosage without side effect reoccurrence, and one patient stopped the medication permanently. Four of eight (50%) patients had a complete clinical response with total resolution of otorrhea and normalization of middle ear mucosa, and were able to discontinue corticosteroid treatment. Two patients attempted to stop PEG‐IFN therapy after prolonged symptom remission and had recurrent otorrhea. Both patients had symptom resolution after PEG‐IFN reinitiation.ConclusionsThese data demonstrate that pegylated interferon‐α 2a and 2b therapy may benefit patients with severe, refractory EOM. Further larger studies with long‐term follow‐up are required to validate these early but promising results.Level of Evidence4. Laryngoscope, 127:1208–1216, 2017