Haynes, David S.

Objective: To report and discuss the effectiveness of stereotactic radiosurgery (SRS) or fractionated radiotherapy (FRT) for tumor control following surgical resection of endolymphatic sac tumors (ELST). Study Design: Retrospective case series. Setting: Multi-institutional academic referral centers. Patients: Patients undergoing surgical resection for ELST followed by SRS or FRT. Intervention(s): Surgical resection followed by radiotherapy. Main Outcome Measures: Local tumor control. Results: Two of the five patients experienced tumor recurrence after gross total microsurgical at 78 and 11 months, respectively. The former patient received salvage 3D conformal radiotherapy (3D-CRT) and while the latter patient underwent three courses of salvage SRS for recurrence, two of which were in-field and was disease-free at last follow up. Two additional patients underwent subtotal tumor resection (STR) followed by intensity-modulated radiation therapy (IMRT) and are currently without disease. One patient underwent STR followed by proton-beam therapy (PBT) and was free of disease at most recent follow-up. Conclusion: SRS/FRT remains a useful adjuvant for treatment of residual or recurrent ELSTs, where the risk of revision microsurgical resection is high.

Objective: To date, prediction models for estimating risk of acquiring non-serviceable hearing in subjects with observed vestibular schwannoma (VS) have evaluated outcomes primarily based on features at initial diagnosis. Herein, we evaluate the association of rate of hearing decline during the initial period of observation with time to non-serviceable hearing. If significant, rate of hearing decline may inform decision making after an introductory period of observation. Setting: Two tertiary care centers. Patients: VS patients with serviceable hearing who underwent at least three audiograms and two magnetic resonance imaging (MRI) studies before intervention or being lost to follow-up. The rate of change in pure-tone average (PTA) and word recognition score (WRS) was calculated as the score from the second audiogram minus the score from the first audiogram, divided by the duration in months between the two. Main Outcome Measure(s): Serviceable hearing, defined as PTA ≤50 dB HL and WRS ≥50%. Results: Among 266 patients meeting inclusion criteria, 52 developed non-serviceable hearing at last follow-up. Kaplan–Meier estimated rates of maintaining serviceable hearing (95% CI; number still at risk) at 1, 3, 5, 7, and 10 years were 97% (95–100; 206), 78% (72–85; 98), 68% (60–77; 39), 60% (50–73; 17), and 44% (29–67; 2), respectively. In a univariable setting, each 1 dB increase per month in the rate of initial PTA change was associated with a 96% increased likelihood of acquiring non-serviceable hearing (hazard ratio [HR] 1.96; 95% CI 1.44–2.68; p < 0.001). Each 1% increase per month in the rate of initial WRS change was associated with a decreased likelihood of acquiring non-serviceable hearing (hazard ratio [HR] 0.79; 95% confidence interval [CI] 0.66–0.94; p = 0.009). After multivariable adjustment, both rate of PTA change (HR 2.42; 95% CI 1.72–3.41; p < 0.001) and rate of WRS change (HR 0.81; 95% CI 0.67–0.99; p = 0.043) remained statistically significantly associated with time to non-serviceable hearing. Conclusion: Rate of early PTA and WRS decline during the initial period of observation are significantly associated with time to development of non-serviceable hearing. This information may facilitate accurate patient counseling and inform decision-making regarding prospective disease management.

Objective: To characterize the risk of progression to nonserviceable hearing in patients with sporadic vestibular schwannomas (VS) who elect initial observation. Study Design: Retrospective case series. Setting: Two tertiary care centers. Patients: VS patients with serviceable hearing who underwent at least two audiograms and two MRI studies before intervention or loss to follow-up. Main outcome measure(s): Serviceable hearing, defined as the pure tone average ≤ 50 dB HL and word recognition score ≥ 50%. Results: Four-hundred sixty-six patients (median age of 57 yr and median tumor diameter of 7.3 mm) had serviceable hearing at presentation and were followed for a median of 2.3 years (IQR 1.0 – 4.0). Kaplan–Meier estimated rates of maintaining serviceable hearing (95% CI; number still at risk) at 1, 3, 5, 7, and 10 years following diagnosis were 94% (91–96; 357), 77% (73–82; 172), 66% (60–73; 81), 56% (49–65; 31), and 44% (33–59; 10), respectively. Each 10-dB increase in pure-tone averages at diagnosis was associated with a 2-fold increased likelihood of developing nonserviceable hearing (hazard ratio 2.07; p < 0.001). Each 10% decrease in word recognition score was associated with a 1.5-fold increased likelihood of developing nonserviceable hearing (hazard ratio 1.48; p < 0.001). Conclusions: Among patients with sporadic VS, good baseline word recognition score and low pure-tone average are jointly associated with maintenance of serviceable hearing. These data may be used to guide patient counseling and optimize management.

Objective: To date, less than 150 cases of middle ear adenomatous neuroendocrine tumors (MEANTs) have been reported in the English literature. The objective of this study was to provide a contemporary analysis of these rare lesions and develop a consensus-driven staging system. Study Design: Multi-institutional retrospective histopathologic, radiologic, and clinical review. Setting: Six tertiary referral centers. Patients: Thirty-two patients with pathologically confirmed MEANT. Intervention: Surgical resection, adjuvant therapy. Main Outcome Measures: Clinical manifestations, outcomes, staging system. Results: Patients commonly presented with progressive conductive or mixed hearing loss (90%), aural fullness (50%), and tonal tinnitus (46%). Pulsatile tinnitus (16%), carcinoid syndrome (4%), and facial nerve paresis (4%) were less commonly observed. MEANTs frequently mimicked temporal bone paraganglioma (31%) and cholesteatoma (15%) at presentation. According to a novel T/N/M/S staging system (S = secretory tumor) proposed herein, there were 6 (19%) T1, 19 (59%) T2, and 7 (22%) T3 MEANTs. T3 tumors were significantly more likely to undergo subtotal or near total resection compared with lower staged tumors secondary to adherence to critical neurovascular structures such as the petrous internal carotid artery and facial nerve (p = 0.027). Patients with T3 MEANTs were more likely to experience multiple recurrences, require adjuvant therapy with a somatostatin analogue, or develop permanent facial nerve paresis compared with lower staged tumors. At last follow up, no patients with T1 MEANTs had developed recurrence, whereas 37% (7/19) of patients with T2 MEANT and 57% (4/7) of patients with T3 MEANT experienced either disease recurrence after gross total resection (GTR) or regrowth of known residual tumor requiring additional surgery at a median duration of 72 months (95% CI, 24–84). Conclusions: Patients with MEANTs can present with nonspecific symptomatology that overlaps with more commonly encountered middle ear lesions. MEANT exhibits a proclivity for recurrence according to T-stage and long-term clinical follow up is necessary, particularly for advanced stage tumors.

Objectives: Dizziness, vertigo, and unsteadiness are common complaints of patients who present to primary care providers. These patients often are referred to otology for assessment and management. Unfortunately, there are a small number of specialists to manage these patients. However, there are several dizziness disorders that can be successfully managed by primary care providers if the disorder is properly identified. To assist in the identification of several of the most common dizziness disorders, we developed the dizziness symptom profile (DSP). The DSP is a self-report questionnaire designed to generate one or more differential diagnoses that can be combined with the patient’s case history and physical examination. Design: This report describes three investigations. Investigations 1 and 2 (i.e., exploratory and confirmatory investigations, N = 514) describe the development of the DSP. Investigation 3 (N = 195) is a validation study that describes the level of agreement between the DSP completed by the patient, and, the differential diagnosis of the otologist. Results: The final version of the DSP consists of 31 items. Preliminary findings suggest that the DSP is in agreement with the differential diagnoses of ear specialists for Meniere’s disease (100% agreement), vestibular migraine (95% agreement), and benign paroxysmal positional vertigo (82% agreement). Conclusions: Early results suggest that DSP may be useful in the creation of differential diagnoses for dizzy patients that can be evaluated and managed locally. This has the potential to reduce the burden on primary care providers and reduce delays in the diagnosis of common dizziness and vertigo disorders.

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

Objectives: This study characterizes primary petroclival meningioma growth rates, before intervention, using volumetric analysis. In addition, predictors of growth are analyzed. Methods: Patients with previously untreated petroclival meningiomas were retrospectively reviewed (1999–2015). Image analysis software was used to perform volumetric analyses of tumor size and growth. Three-dimensional segmentation volumetric analyses were compared with volumes estimated utilizing three orthogonal dimensions. Tumor growth was defined as a 15% increase in volume. Results: Thirty-four patients who underwent at least two magnetic resonance imaging (MRI) studies before intervention were included. The mean age was 55.2 years, and 65.7% were women. The mean tumor volume at presentation was 5.6 cm 3 (range, 0.1–25.8 cm 3 ) as determined from segmentation volumetric analysis. At a mean follow-up of 44.5 months (range, 3.7–125.1 mo), 88.2% of tumors grew. The mean annual volumetric growth rate was 2.38 cm 3 /yr (−0.63 to 25.9 cm 3 /yr). Tumor volume, T2 hyperintensity within the tumor, peritumoral edema, and ataxia and/or cerebellar symptoms at presentation were all significantly associated with greater rates of tumor growth. Ultimately, 10 (29.4%) patients underwent treatment during the follow-up period. Conclusion: Our experience demonstrates that the vast majority (88%) of untreated petroclival meningiomas grow; the mean volumetric growth rate was noted to be 2.38 cm 3 /yr. We found a significant association between increased growth rate and larger tumor size at diagnosis, T2 hyperintensity within the tumor, peritumoral edema, and the presence of ataxia and/or cerebellar symptoms.

Objectives/HypothesisTo highlight the phenomenon of cholesteatoma arising from the vascular strip following tympanoplasty and tympanomastoidectomy.Study DesignMulti‐institutional retrospective chart review.MethodsConsecutive adult and pediatric patients evaluated between 2000 and 2015 with acquired cholesteatoma arising from the skin of a prior vascular strip were identified. Patients with evidence of residual or recurrent cholesteatoma elsewhere in the middle ear, mastoid, or ear canal were excluded.ResultsSeventeen cases (71% female, 53% right‐sided) were identified, and the mean age at presentation was 39.2 years. Patients presented on average 12.9 years following prior otologic surgery, which was most commonly tympanoplasty without mastoidectomy (59%). However, younger patients presented with symptoms sooner following prior surgery (r = 0.61, r2 = 0.37, P = 0.0095). The most common presenting symptom was otorrhea (76%). All patients were found to have mastoid cholesteatoma with bony erosion, and three patients exhibited additional tegmen or posterior fossa bone defects. The middle ear was not involved with cholesteatoma in any case. A canal wall down procedure was performed due to extensive bony canal erosion in 29% of cases.ConclusionThe vascular strip is an uncommon source of iatrogenic cholesteatoma that can present years following an otherwise uncomplicated otologic surgery. The findings presented herein highlight the importance of careful vascular strip orientation at the conclusion of otologic surgery.Level of Evidence4. Laryngoscope, 127:698–701, 2017

ObjectiveTo describe the clinical presentation and management of patients with cholesterol granulomas (CGs) that develop following temporal bone surgery.Study DesignCase series with chart review.SettingTwo independent tertiary academic referral centers.Subjects and MethodsA multicenter retrospective review was performed to identify all patients between 2001 and 2014 who were diagnosed with a CG that developed following temporal bone surgery. Patients with a history of idiopathic petrous apex CGs were excluded, as were those with <6 months of follow‐up after diagnosis. Demographic and clinical data were recorded at presentation, and the main outcome measure was symptom evolution over time.ResultsA total of 20 patients met inclusion criteria (median age, 55.0 years; 70.0% female), and their cases were analyzed. Diagnosis was made, on average, 149.5 months (median, 94.5; range, 13.0‐480.0) following a temporal bone operation, which was for chronic ear disease in 75.0% of cases. The most common presenting symptoms were otalgia (55.0%) and otorrhea (40.0%). In patients initially managed with observation, 71.4% required no surgical intervention through a mean of 56.8 months (median, 30; range, 12.0‐178.0) of follow‐up. For those who initially underwent surgical excision, durable symptom relief was achieved in 66.7% of cases.ConclusionPostoperative CG formation in the temporal bone is a rare occurrence that can be encountered years following surgery. The present study suggests that conservative management can be appropriate for many cases in which patients are relatively asymptomatic.

ObjectivesTo analyze factors that influence hearing preservation over time in cochlear implant recipients with conventional‐length electrode arrays located entirely within the scala tympani.Study DesignCase series with planned chart review.SettingSingle tertiary academic referral center.Subjects and MethodsA retrospective review was performed to analyze a subgroup of cochlear implant recipients with residual acoustic hearing. Patients were included in the study only if their electrode arrays remained fully in the scala tympani after insertion and serviceable acoustic hearing (≤80 dB at 250 Hz) was preserved. Electrode array location was verified through a validated radiographic assessment tool. Patients with <6 months of audiologic follow‐up were excluded. The main outcome measure was change in acoustic hearing thresholds from implant activation to the last available follow‐up.ResultsA total of 16 cases met inclusion criteria (median age, 70.6 years; range, 29.4‐82.2; 50% female). The average follow‐up was 18.0 months (median, 16.1; range, 6.2‐36.4). Patients with a lateral wall electrode array were more likely to have stable acoustic thresholds over time (P <. 05). Positive correlations were seen between continued hearing loss following activation and larger initial postoperative acoustic threshold shifts, though statistical significance was not achieved. Age, sex, and noise exposure had no significant influence on continued hearing preservation over time.ConclusionsTo control for hearing loss associated with interscalar excursion during cochlear implantation, the present study evaluated patients only with conventional electrode arrays located entirely within the scala tympani. In this group, the style of electrode array may influence residual hearing preservation over time.