Neil S. Patel

Symptom-Based Clustering in Chronic Rhinosinusitis Relates to History of Aspirin Sensitivity and Postsurgical Outcomes

BACKGROUND Symptoms burden in chronic rhinosinusitis (CRS) may be assessed by interviews or by means of validated tools such as the 22-item SinoNasal Outcome Test (SNOT-22). However, when only the total SNOT-22 scores are used, the pattern of symptom distribution and heterogeneity in patient symptoms is lost. OBJECTIVES To use a standardized symptom assessment tool (SNOT-22) on preoperative symptoms to understand symptom heterogeneity in CRS and to aid in characterization of distinguishing clinical features between subgroups. METHODS This was a retrospective review of 97 surgical patients with CRS. Symptom-based clusters were derived on the basis of presurgical SNOT-22 scores using unsupervised analysis and network graphs. Comparison between clusters was performed for clinical and demographic parameters, postsurgical symptom scores, and presence or absence of a history of aspirin sensitivity. RESULTS Unsupervised analysis reveals coclustering of specific symptoms in the SNOT-22 tool. Using symptom-based clustering, patients with CRS were stratified into severe overall (mean total score, 90.8), severe sinonasal (score, 62), moderate sinonasal (score, 40), moderate nonsinonasal (score, 37) and mild sinonasal (score, 16) clusters. The last 2 clusters were associated with lack of history of aspirin sensitivity. The first cluster had a rapid relapse in symptoms postoperatively, and the last cluster demonstrated minimal symptomatic improvement after surgery. CONCLUSION Symptom-based clusters in CRS reveal a distinct grouping of symptom burden that may relate to aspirin sensitivity and treatment outcomes.

Surgical management of lateral skull base defects

We sought to analyze key factors that influence the management of lateral skull base defects and implement an algorithm to improve outcomes of reconstruction.

Hearing Preservation in Pediatric Cochlear Implantation

OBJECTIVE Currently, there is a paucity of literature evaluating hearing preservation outcomes in children following cochlear implantation. The objective of the current study is to report pediatric hearing preservation results following cochlear implantation with conventional full-length electrodes. STUDY DESIGN Retrospective review (2000-2016). SETTING Tertiary referral center. PATIENTS All pediatric patients with a ≤ 75 dB preoperative low-frequency pure tone average (LFPTA; 250-500 Hz average), who underwent cochlear implantation with a conventional length electrode. INTERVENTION(S) Cochlear implantation. MAIN OUTCOME MEASURE(S) Complete, partial, minimal, or no hearing preservation following cochlear implantation (Skarzynski et al., 2013); maintenance of functional low frequency hearing (≤85 dB LFPTA). RESULTS A total of 43 ears, in 35 pediatric patients, met inclusion criteria. The mean age at time of implantation was 8.6 years (range, 1.4-17.8 yr), 20 (57.1%) patients were female, and 25 (58.1%) cases were left-sided.The mean preoperative ipsilateral low frequency PTA and conventional four-frequency PTA (500, 1000, 2000, 3000 Hz average) were 54.2 dB (range, 15-75 dB) and 82.2 dB (range, 25-102.5 dB), respectively. The mean low frequency PTA and conventional four-frequency PTA shifts comparing the pre- and first postoperative audiogram were Δ25.2 dB (range, -5 to 92.5 dB) and Δ18.3 dB (range, -8.8 to 100 dB), respectively. Overall, 17 (39.5%) ears demonstrated complete hearing preservation, 19 (44.2%) ears partial hearing preservation, 0 minimal hearing preservation, and 7 (16.3%) exhibited no measurable acoustic hearing after surgery. In total, 28 (65.1%) ears maintained functional low-frequency hearing (i.e., ≤85 dB LFPTA) based on the initial postoperative audiogram. There was no statistically significant difference in the initial low frequency PTA shift comparing lateral wall and perimodiolar electrodes (Δ22.2 versus Δ28.1 respectively; p = 0.44), cochleostomy and round window insertions (Δ25.2 vs. Δ24.7 respectively; p = 0.95), or statistically significant association between age at implantation and low frequency PTA shift (r = 0.174; p = 0.26).In total, 22 ears in 19 patients had serial audiometric data available for review. Over a mean duration of 43.8 months (range, 2.6-108.3 mo) following surgery, the mean low frequency PTA and conventional four-frequency PTA shift comparing the initial postoperative and most recent postoperative audiogram was Δ9.7 dB (range, -27.5 to 57.5 dB) and Δ8.1 dB (range, -18.8 to 31.9 dB), respectively. CONCLUSIONS Varying levels of hearing preservation with conventional length electrodes can be achieved in most pediatric subjects. In the current study, 82% of patients maintained detectable hearing thresholds and 65% maintained functional low-frequency acoustic hearing. These data may be used to guide preoperative counseling in pediatric patients with residual acoustic hearing. Additionally, the favorable rates of hearing preservation achieved in children provide further evidence for the expansion of pediatric cochlear implant candidacy to include patients with greater degrees of residual hearing.

Cervical and Ocular VEMP Testing in Diagnosing Superior Semicircular Canal Dehiscence

Objective To determine the sensitivity and specificity of ocular and cervical vestibular evoked myogenic potentials (VEMPs) in the diagnosis of superior semicircular canal dehiscence (SCD) and to describe the VEMP response characteristics that are most sensitive to SCD and compare the findings to previous reports. Study Design Case series with chart review. Setting Two tertiary neurotologic referral centers. Subjects and Methods Cervical and ocular VEMP peak-to-peak amplitudes and thresholds from 39 adult patients older than 18 years with surgically confirmed SCD were compared with 84 age-matched controls. Results Using receiver operating characteristic (ROC) curves, cervical VEMP (cVEMP) amplitudes, cVEMP thresholds, and ocular VEMP (oVEMP) amplitudes had areas under the curve of 0.731, 0.912, and 0.856, respectively, all of which were statistically significant (P < .0001). For cVEMP thresholds, at the clinical equivalent ≤85-dB normalized hearing level (nHL) threshold, the sensitivity and specificity were 97.3% and 31.3%, respectively. At the ≤70-dB nHL threshold, the sensitivity and specificity were 73.0% and 94.0%, respectively. For oVEMP amplitudes >12.0 µV, the sensitivity and specificity were 78.6% and 81.7%, respectively. Conclusion Data from this multicenter study suggest that both cVEMP thresholds and oVEMP amplitudes remain good diagnostic tests for identifying SCD, with each test dependent on a number of factors. The sensitivity and specificity of these individual tests may vary slightly between centers depending on testing parameters used.

Occult Temporal Bone Facial Nerve Involvement by Parotid Malignancies with Perineural Spread

Objective To characterize disease presentation and outcomes following surgical treatment of parotid malignancies with occult temporal bone facial nerve (FN) involvement. Study Design Case series with chart review. Setting Tertiary academic referral center. Subjects and Methods Thirty consecutive patients (mean age 58 years; 77% men) who underwent surgery for parotid malignancies with occult perineural involvement of the intratemporal FN were included. Primary outcome measures included margin status and recurrence. Results The mean duration of clinical follow-up was 49 months, and the most common presenting symptom was FN paresis (n = 23; 77%) followed by pain (n = 15; 50%). To obtain a proximal FN margin, 27 patients (90%) underwent mastoidectomy, and 3 patients (10%) had lateral temporal bone resection. The intratemporal FN margin was cleared in 26 patients (87%), most commonly in the mastoid segment (60%). Adjuvant therapy was given in 25 patients (83%). Ten patients (33%) experienced locoregional (4; 13%) and/or distant (8; 27%) recurrence at a median of 19 months (mean 26, 2–54 months) following surgery. Locoregional failure was significantly more common in cases with a positive intratemporal FN margin (66% vs 8%; P = .045). Overall 1-, 3-, and 5-year disease-specific survival rates were 83%, 79%, and 72%, respectively. Conclusions Perineural invasion of the intratemporal FN by parotid malignancy is uncommon. Normal preoperative FN function does not preclude histopathologic involvement. Temporal bone FN exploration should be considered when a positive margin is encountered at the stylomastoid foramen, as failure to do so is associated with an increased rate of locoregional recurrence.

An Easy and Reliable Method to Locate the Dehiscence During Middle Fossa Superior Canal Dehiscence Surgery: It is a (C)inch.

Objective: The middle fossa floor lacks reliable surface landmarks. In cases of superior semicircular canal dehiscence (SSCD), multiple skull base defects may be present, further confounding the location of the labyrinth. Misidentification of the SSCD during surgery may lead to treatment failure or sensorineural hearing loss. Anecdotally, the authors have observed the distance from the lateral edge of the craniotomy to the SSCD to be consistently 1 inch. Herein, we present radiologic evidence of this practical and clinically useful relationship. Patients: All patients at two tertiary care academic referral centers with high-resolution temporal bone computed tomography (CT) evidence of SSCD were retrospectively reviewed. Intervention(s): Review of high-resolution temporal bone CT. Main Outcome Measures: The horizontal distance from the outer cortex of the squama temporalis immediately superior to the bony external auditory canal (approximating lateral edge of craniotomy) to the SSCD was measured in the coronal plane by two independent reviewers. Results: A total of 151 adult ears with SSCD were analyzed. A Shapiro-Wilk goodness-of-fit test confirmed that measurements were normally distributed. Pearson inter-rater correlation was 0.95, confirming very strong agreement. The mean distance between the outer cortex of the squama temporalis and SSCD was 25.9 mm, or 1.02 inches. Sixty-eight percent of the SSCD population would fall between 0.92 and 1.12 inches and 95% would lie between 0.83 and 1.21 inches. Conclusions: The horizontal distance from the outer cortex of the squama temporalis to the SSCD consistently approximates 1 inch. This easily remembered distance can aid surgeons in locating or confirming the SSCD during middle fossa surgery.

Virtual Mapping of the Frontal Recess: Guiding Safe and Efficient Frontal Sinus Surgery:

Objective To define relationships between the frontal sinus opening, ostia of other frontal recess cells, and endoscopic landmarks and to develop a clinically useful framework to guide frontal sinus surgery. Study Design Retrospective review. Setting Tertiary care academic referral center. Methods Adult patients with computed tomography (CT) without sinonasal pathology were included. Virtual endoscopy (using OsiriX) and corresponding CT reconstructions were used to identify all visible ostia in the frontal recess and characterize their positions in spaces between the uncinate/agger nasi (U), bulla ethmoidalis (EB), and middle turbinate (MT). Results Two hundred sides in 100 patients (median age 51 years, 62% female) were analyzed. The “center” of each map was defined as the intersection of spaces between U, EB, and MT. The frontal sinus opening was in the “center” in 53% of frontal recesses, lateral to this position in 29%, and anterior in 11%. When the frontal sinus opening was at the “center,” anterior ostia drained frontal Kuhn T cells in 51% and intersinus septal cells in 23%. The skull base attachment of the apical strut of the uncinate process demarcated medial and lateral within the space between U and EB, with the opening to the frontal sinus medial in 68% and lateral in 31%. Left-right asymmetry in frontal sinus openings was noted in 46% of patients. Conclusion Combining preoperative imaging and knowledge of these anatomic relationships may facilitate more efficient frontal outflow tract identification and instrumentation. This represents the first and largest description of ostial configurations relative to endoscopic structural landmarks. Level of Evidence: 4

Risk of progressive hearing loss in untreated superior semicircular canal dehiscence

Patients 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.

Primary Epidermoid Tumors of the Cerebellopontine Angle: A Review of 47 Cases

Objective: To analyze disease presentation, treatment, and clinical course of a consecutive series of patients with primary cerebellopontine angle (CPA) epidermoids. Patients: Forty-seven consecutive patients with previously untreated CPA epidermoids. Intervention(s): Observation and microsurgery. Main Outcome Measures: Disease- and treatment-associated morbidity, recurrence. Results: Forty-seven patients (mean age 39 years; 53% women) were analyzed and the average duration of follow-up was 42 months. The most common presenting symptom was headache (27; 57%); 13 (28%) exhibited preoperative asymmetric sensorineural hearing loss, 3 (6%) facial nerve paresis, and 3 (6%) hemifacial spasm. Thirteen patients (28%) were initially observed over a mean interval of 56 months; however, five experienced disease progression requiring operation. Thirty-nine patients (83%) underwent surgical resection; 18 (46%) received gross total, 5 (13%) near total, and 16 (41%) aggressive subtotal resection. Three patients (8%) recurred at a median of 53 months; two after subtotal and one after gross total resection. Ninety-three percent of patients with useful hearing maintained serviceable hearing following treatment and one patient (3%) experienced mild long-term postoperative facial nerve paresis (HB II/VI). All patients with preoperative facial nerve paresis recovered normal function postoperatively. There were no episodes of stroke or death. Conclusions: Surgical intervention is effective in alleviating symptoms of cranial neuropathy and brainstem compression from CPA epidermoids. Gross total resection is preferred; however, aggressive subtotal removal should be considered with adherent or extensive disease as reoperation rates are low, even in the setting of aggressive subtotal resection. Conservative observation with serial imaging is a viable initial strategy in asymptomatic or minimally symptomatic patients.

Facial Nerve Schwannomas: Review of 80 Cases Over 25 Years at Mayo Clinic

OBJECTIVE To elucidate the long-term clinical behavior, treatment, and outcomes of sporadic facial nerve schwannoma (FNS) in a large cohort of patients managed in the post-magnetic resonance imaging era. PATIENTS AND METHODS Retrospective review at a single tertiary health care system (January 1, 1990, through December 31, 2015), evaluating 80 consecutive patients with sporadic FNS. RESULTS Ninety-eight patients with FNS were identified; 10 with incomplete data and 8 with neurofibromatosis type 2 were excluded. The remaining 80 patients (median age, 47 years; 58% women) were analyzed. Forty-three (54%) patients presented with asymmetrical hearing loss, 33 (41%) reported facial paresis, and 21 (26%) reported facial spasm. Seventeen (21%) exhibited radiologic features mimicking vestibular schwannoma, 14 (18%) presented as a parotid mass, and 5 (6%) were discovered incidentally. Factors predictive of facial nerve paresis or spasm before treatment were female sex and tumor involvement of the labyrinthine/geniculate and tympanic facial nerve segments. The median growth rate among growing FNS was 2.0 mm/y. Details regarding clinical outcome according to treatment modality are described. CONCLUSION In patients with FNS, female sex and involvement of the labyrinthine/geniculate and tympanic segments of the facial nerve predict a higher probability of facial paresis or spasm. When isolated to the posterior fossa or parotid gland, establishing a preoperative diagnosis of FNS is challenging. Treatment should be tailored according to tumor location and size, existing facial nerve function, patient priorities, and age. A management algorithm is presented, prioritizing long-term facial nerve function.