Wait-and-scan management in sporadic Koos grade 4 vestibular schwannomas: A longitudinal volumetric study.

Background: Volumetric natural history studies specifically on large vestibular schwannomas (VSs), commonly classified as Koos grade 4, are lacking. The aim of the current study is to present the volumetric tumor evolution in sporadic Koos grade 4 VSs and possible predictors for tumor growth. Methods: Volumetric tumor measurements and tumor evolution patterns from serial MRI studies were analyzed from selected consecutive patients with Koos grade 4 VS undergoing initial wait-and-scan management between January 2001 and July 2020. The significant volumetric threshold was defined as a change in volume of ≥10%. Results: Among 215 tumors with a median size (IQR) of 2.7 cm3 (1.8-4.2), 147 tumors (68%) demonstrated growth and 75 tumors (35%) demonstrated shrinkage during follow-up. Growth-free survival rates (95% CI) at 1, 2, 5, and 10 years were 55% (48-61), 36% (29-42), 29% (23-36), and 28% (21-34), respectively and did not significantly differ in tumors> 20 mm (Chi-square = .40; P-value = .53). Four tumor evolution patterns (% of total) were observed: continued growth (60); initial growth then shrinkage (7); continued shrinkage (27); and stability (5). Good hearing (adjusted HR 2.21, 95% CI 1.48-3.30; P < .001) and peritumoral edema (adjusted HR 2.22, 95% CI 1.18-4.13; P = .01) at diagnosis were significantly associated with an increased likelihood of growth. Conclusions: Koos grade 4 VSs show a wide variety in size and growth. Due to variable growth patterns, an initial wait-and-scan strategy with short scan intervals may be an acceptable option in selected tumors, if no significant clinical symptoms of mass effect that warrant treatment are present.

Characteristics and Clinical Management Strategy of Petrous Apex Cholesterol Granulomas.

Purpose: To evaluate the clinical characteristics of petrous apex cholesterol granulomas (PACG) and assess outcomes after different treatment strategies. Method: A consecutive case series of 34 patients with a PACG. Main outcomes were PACG growth, symptoms, and the outcomes of different treatment strategies: wait-and-scan (WS) and surgical drainage. Results: Thirty-four patients were analyzed; mean follow-up time was 7.1 years. Twenty-one patients (61.7%) showed symptoms, mostly more than one. Most symptoms reported were cranial nerve palsy (58.8%) and headache (35.3%). Twenty-one patients (61.8%) received solely wait-and-scan (WS), and thirteen patients (38.2%) underwent surgery, five of whom (38.5%) after an initial WS period. In the solely WS group, one (4.8%) developed new symptoms, and two (9.5%) reported symptom progression despite a stable granuloma size. Two (9.5%) showed granuloma growth on follow-up scans without symptom progression. Surgery consisted of drainage. Eleven (84.6%) of these thirteen patients reported partial recovery; one (7.7%) reported no recovery; and one (7.7%) reported full recovery of reported symptoms related to PACG. Among the patients with cranial nerve involvement, 7.7% showed full recovery after surgery; 84.6% showed partial recovery; and 7.7% did not recover. Adverse events occurred in five out of 13 patients who underwent surgery, all with full recovery. Conclusions: This study confirms that PACG are slow-growing lesions with a low risk of adverse events. Solely using wait-and-scan strategy is a safe option for patients without symptoms, with acceptable symptoms without symptom progression, and with asymptomatic growth. Surgical treatment can be considered in patients with symptom progression or symptomatic growth.

Sensorineural Hearing Impairment Recovery after Transmastoidal Surgery for a Petrous Apex Cholesterol Granuloma: A Case Report.

Cholesterol granulomas are the most common primary lesions of the petrous apex. Although their benign character, impingement of critical neurovascular structures can cause significant symptomatology such as hearing impairment. We present unique results after treatment of a cholesterol granuloma located in the petrous apex causing sensorineural hearing impairment. A transmastoidal approach was performed using an intraoperative computed tomography-guided navigation system. The video, which is included for reference, illustrates clear drainage of the cyst and drain positioning. The hearing improved completely in the lower frequencies at 500 and 1000 Hz and with a 19 dB improvement in overall bone conduction in the affected ear.

Diagnosis, treatment and prognosis of otomastoiditis induced by Fusobacterium necrophorum: A retrospective multicentre cohort study.

OBJECTIVES: Otomastoiditis caused by the anaerobic Fusobacterium necrophorum (F. necrophorum) often induces severe complications, such as meningitis and sinus thrombosis. Early diagnosis is difficult, partly because little is known about specific early signs. Comprehensive research about clinically chosen antimicrobial therapy has not been done yet and prognostic information about otomastoiditis caused by F. necrophorum is scarce. More knowledge about this subject is required. METHODS: In this retrospective cohort study, we included all cases of otomastoiditis caused by F. necrophorum treated in two university medical centres in the Netherlands during the past 10 years. Data was gathered from patient records and analysed using independent sample T-tests and Chi2-tests. RESULTS: This study reveals that otomastoiditis caused by F. necrophorum potentially induces neurological sequelae. Thereby, 80% of all included patients (n = 16) needed readmission within six months due to recurrence or complications of otomastoiditis caused by F. necrophorum. Mean (range) of age, CRP and temperature were 4.5 years (0.9-29.3), 243 mg/L (113-423) and 40 °C (37-41). All patients were hospitalized and treated with antibiotics, mostly metronidazole (n = 13/16) and a ß -lactam (n = 15/16). Additional treatment contained low molecular weight heparin (83%, n = 10/12), dexamethasone (78%, n = 7/9) and/or surgery (80%, n = 12/16, whereof 9/12 mastoidectomy). CONCLUSIONS: Patients and/or their parents need to be informed about this potential unfortunate prognosis when otomastoiditis caused by F. necrophorum is diagnosed. To improve early diagnosis, otomastoiditis caused by F. necrophorum should be suspected and therefore immediately cultured when a) young children present with otomastoiditis, with b) high CRP values, and/or c) vomiting and decreased consciousness.

Clinical characteristics and an evaluation of predictors for a favourable outcome of Mycobacterium abscessus otomastoiditis: a systematic review and meta-analysis of individual participant data.

BACKGROUND: Otomastoiditis caused by Mycobacterium abscessus is rare, but its incidence has increased over the past decades and its optimal treatment remains unknown. This study aims to summarise the clinical and therapeutic features and find characteristics of patients with M. abscessus otomastoiditis associated with favourable treatment outcomes. METHODS: We searched MEDLINE, Embase and Web of Science to identify studies including patients with M. abscessus otomastoiditis. A 1-stage individual patient data (IPD) meta-analysis was conducted. A 2-level mixed-effects linear regression model was provided for antimycobacterial treatment duration. RESULTS: Twenty-three studies reported a total of 85 patients. Children possess a unique clinical profile including a history of ear infections, tympanostomy tube placement and antibiotic treatment. Antimycobacterial treatment was administered for 26 (interquartile range [IQR]: 15-35) weeks. Macrolides were prescribed in 98.8% of the cases. Surgery was performed in 80.5% of the cases, of which, 47.1% required revision surgery. Otalgia was a significant predictor (ß = 9.3; P = .049) of antimycobacterial treatment duration. CONCLUSIONS: Mastoid surgery (regularly requiring revision) and a multidrug regimen for a minimum of 6 months, including a minimum of 3 active agents, are most often needed to attain cure. The presence of otalgia significantly extends the treatment duration of M. abscessus otomastoiditis.

Cochlear Implantation in NF2 Patients Without Intracochlear Schwannoma Removal.

OBJECTIVE: To determine if cochlear implantation without removal of inner ear schwannomas (IES) is an effective treatment option for Neurofibromatosis 2 (NF2) patients. To determine how the presence of an intracochlear schwannoma might impact cochlear implant (CI) outcomes and programming parameters. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary center for cochlear and auditory brainstem implantation. PATIENTS: Of 10 NF2 patients with IES, 8 are reported with no previous tumor removal on the implanted ear. INTERVENTIONS: Cochlear implant without tumor removal. MAIN OUTCOME MEASURES: Performance outcomes with CI at least 1-year post intervention. Programming parameters, including impedances, for patients with IES. RESULTS: All patients had full insertion of the electrode arrays with round window approaches. Performance outcomes ranged from 0 to 100% for Bamford-Kowal-Bench sentences. Impedance measurements for active electrodes for patients with IES were comparable to those measured in patients without vestibular schwannoma (VS). Only patients who had radiation treatment before receiving their implant had elevated threshold requirements for CI programming compared with CI recipients without VS. CONCLUSION: Cochlear implantation without tumor removal is an effective option for treating NF2 patients with IES. The presence of an intracochlear tumor did not have an impact on CI performance or programming requirements compared with patients without VS; however, previous treatment with radiation may be related to elevated current requirements in the CI settings.

Identifying the Need to Discuss Infertility Concerns Affecting Testicular Cancer Patients: An Evaluation (INDICATE Study).

Men with testicular cancer (TC) risk impaired fertility. Fertility is a major concern for TC patients due to diagnosis in almost always reproductive ages and high overall survival. This study assessed counselling in regards to the risk of impaired fertility and sperm cryopreservation. A cross-sectional survey was performed on 566 TC patients diagnosed between 1995-2015. Of the 566 survivors, 201 questionnaires were completed (35.5%). Eighty-eight percent was informed about possible impaired fertility, 9.5% was not informed. The majority (47.3%) preferred the urologist to provide information. Collecting sperm was troublesome but successful for 25.6%, 4.8% did not succeed in collecting sperm. The reasons were high pressure due to disease, pain after surgery and uncomfortable setting. Due to impaired fertility, 19% of the respondents reported grief and 9.3% stated as being less satisfied in life. Sperm cryopreservation was performed by 41.3% (n = 83). One third (n = 63, 31.3%) had children after treatment, of which 11.1% made use of preserved sperm (n = 7). The results of this survey indicate the importance of timely discussion of fertility issues with TC patients. While being discussed with most men, dissatisfaction and grief may occur as a result of impaired fertility and a lack of counselling. Overall, 6.5% made use of cryopreserved sperm (n = 13). Men prefer their urologist providing counselling on fertility.

Surgery, radiotherapy or a combined modality for jugulotympanic paraganglioma of Fisch class C and D.

OBJECTIVES: To identify the risks associated with surgery, radiotherapy or a combined treatment approach for Fisch class C and D jugulotympanic paraganglioma, in order to develop an individualised approach for each patient depending on Fisch class, age, mutation presence, tumour size growth rate and presenting symptoms. DESIGN: A retrospective multicenter cohort study with all patient records of patients with a head and neck paraganglioma in the Radboudumc, Nijmegen and the St. Elisabeth Hospital, Tilburg, the Netherlands. MAIN OUTCOME MEASURES: Local control, cranial nerve damage, complications, function recovery. RESULTS: We found highest local control rates after tumour debulking with postoperative radiotherapy in case of residual tumour growth, referred to as the combined treatment group, (100%; n = 19), which was significantly higher than the surgical group (82%; n = 17; P = 0.00), but did not differ from the radiotherapy group (90%; n = 29). There were significantly less complications in the radiotherapy group, when compared to surgery (63 vs 27%; P = 0.002) and the combined group (44 vs 27%; P = 0.016). Furthermore,: using a logistic regression model, we found that pretreatment tumour growth was a negative predictor for post-treatment cranial nerve function recovery (OR = 50.178, P = 0.001), reducing the chance of symptom recovery (67.3% vs 35.7%) post-treatment. CONCLUSIONS: Radiotherapy should be the treatment of choice for the elderly. For younger patients, tumour debulking should be considered, with potential radiotherapy in case of residual tumour growth.

Feasibility of a wait-and-scan period as initial management strategy for head and neck paraganglioma.

BACKGROUND: The main goal of head and neck paraganglioma (PGL) management is reduction of treatment-induced and tumor-induced complications. In the current study, tumor growth rates and tumor-induced complications during a wait-and-scan period are evaluated. METHODS: This was a retrospective cohort study. Tumor growth was measured in axial plane diameter and tumor volume. RESULTS: Of 59 jugulotympanic tumors, 71 carotid body tumors, and 29 vagal body tumors, 44% were growing (median follow-up of 63.6 months). Median growth rates were 0.41 mm/year (range 0-439 mm), 1.6 mm/year (range 0-23.68 mm), and 1.6 mm/year (range 0-23.68 mm) respectively. Growth was significantly correlated to age at presentation (odds ratio [OR] = 0.974; P < .05). Seventeen tumors induced 20 complications. Six of these tumors were growing, and growth rates were higher than in tumors not inducing complications (P = .016; F = 6.496). CONCLUSION: These results illustrate the feasibility of a wait-and-scan strategy for head and neck PGL. The management strategy could not prevent tumor-induced complications in 16% of nongrowing tumors.

The influence of newborn hearing screening on the age at cochlear implantation in children.

OBJECTIVES/HYPOTHESIS: To evaluate the influence of the introduction of newborn hearing screening programs on the age at cochlear implantation in children. STUDY DESIGN: Retrospective, multicenter cohort study. METHODS: All 1,299 pediatric cochlear implant users who received their implants before the age of 5 years between 1995 and 2011 in the Medical University Hannover, Germany and University Medical Center Utrecht, the Netherlands were enrolled in this study. Age at implantation and the number of children implanted within the first year of life was assessed for each center. RESULTS: Age at cochlear implantation gradually declined over the years in both centers. The introduction of the screening resulted in significant decline in the age at implantation in the Netherlands; simultaneously, the number of children implanted within their first year of life increased significantly. Comparing 4-year epochs immediately before and after introduction of the screening, the mean age decreased from 2.4 to 1.2 years, and the percentage of early implanted children increased from 9% to 37%. In the German population, a similar effect of the introduction of the hearing screening program was absent. CONCLUSIONS: The introduction of the national newborn hearing screening program has reduced the age at cochlear implantation in young children in the Netherlands but not in Germany. Correspondingly, it resulted in an increase in the number of children implanted early in life. The difference between the Dutch and German population might be due to differences in the follow-up and referral after the hearing screening.

Deafness induction in mice.

HYPOTHESIS: How to induce most efficiently severe sensorineural hearing loss in mice using a single coadministration of an aminoglycoside antibiotic and a loop diuretic? BACKGROUND: The coadministration of aminoglycosides and a loop diuretic has been widely used to induce hair cell and spiral ganglion cell loss in guinea pigs. However, the development of new treatment strategies against sensorineural hearing loss, such as tissue engineering techniques, requires the use of mouse models. Previous attempts to induce hearing loss in mice have rendered inconsistent results because of resistance to aminoglycoside-induced ototoxicity. Especially inner hair cells seem to be resistant to aminoglycoside-induced ototoxicity. METHODS: In the present study, we aim to optimize hearing loss in mice, using a single high-dose kanamycin (700 and 1,000 mg/kg) injection followed by a furosemide (100 mg/kg) administration. Although previous studies used intraperitoneal furosemide injections 30 minutes after kanamycin administration, we used intravenous furosemide injections administered within 5 minutes after kanamycin treatment. RESULTS: Auditory brain stem responses illustrated severe threshold shifts, and histologic analysis showed marked outer hair cell destruction as well as spiral ganglion cell loss. The present protocol results in more severe inner hair cell loss when compared with the results of previous researches. CONCLUSION: We conclude that severe sensorineural hearing loss can be induced in mice. Moreover, we found that this mouse model can be augmented via the use of rapid intravenous furosemide administrations to maximize inner hair cell loss.