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.

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.

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.