Long-term analysis of ABI auditory performance in patients with neurofibromatosis type 2-related schwannomatosis.

PURPOSE: This retrospective monocentric study aimed to evaluate long-term auditory brainstem implant (ABI) function in patients with neurofibromatosis type 2, and to investigate the prognostic factors for ABI use. METHODS: Between 1997 and 2022, 27 patients with at least five years of follow-up underwent implantation with 32 ABIs. At 1- and 5-years post-implantation and at last follow-up, ABIs were classified as used or non-used and the size of the ipsilateral tumor was recorded. For patients who used their ABIs, we assessed speech perception (disyllabic words, MBAA sentences) in quiet conditions with the ABI only, by lip-reading (LR), and with a combination of the two (ABI + LR). Hearing improvement was calculated as Δ ABI = (ABI + LR)-LR scores. Predictive factors for ABI use were analyzed. RESULTS: One year post-implantation, 74% patients were ABI-users and 66% of the ABIs were used. Two of these patients were non-users at five years, and another two at last follow-up (14 ± 5.2 years); 54% of the patients were ABI-users at last follow-up. Δ ABI revealed a hearing improvement of 32-41% (disyllabic words) and 28-37% (MBAA sentences). Among 16 ABIs with at least LR improvement at 1-year post-implantation, 4 decreased their performance, coinciding with a large growing ipsilateral tumor in 3/4 ABIs. We identified no significant prognostic factors for ABI use. CONCLUSIONS: ABIs are indicated in case of bilateral deafness with a non-functional cochlear nerve. Half the patients with ABIs used their implants and auditory performance remained stable over time, except in cases of ipsilateral tumor growth.

Hearing recovery after surgical resection of non-vestibular schwannoma cerebellopontine angle tumors.

PURPOSE: Post-operative outcomes for hearing after resection surgery to remove cerebellopontine angle (CPA) tumors other than vestibular schwannomas (VS) are not well understood. This study presents a series of patients with significant post-operative hearing recovery, trying to define the incidence among all patients operated on for removal of non-VS CPA tumors. METHODS: This is a retrospective observational case series of 8 patients among 69 operated on for removal of non-VS CPA tumors between 2012 and 2020. All patients had pre- and post-operative hearing measurement with pure-tone average (PTA) and speech discrimination score (SDS), according to the American Academy of Otolaryngology-Head and Neck Surgery recommendations, auditory brainstem response (ABR) measurements and imaging. RESULTS: Six meningiomas and two lower cranial nerve schwannomas operated on with a retrosigmoid approach were included for analysis. The mean pre-operative PTA and SDS were 58 ± 20.7 dB and 13 ± 17.5%, respectively. All patients had pre-operative class D hearing and asynchronous ABRs. They all showed significant hearing recovery, with an improvement of 36 ± 22.2 dB (p = 0.0025) and 85 ± 16.9% (p = 0.0001) in PTA and SDS, respectively, with mean follow-up of 21 ± 23.5 months. Seven patients recovered to a class A hearing level and one patient to class B. The ABRs became synchronous for three patients. The incidence of auditory recovery was 13% for patients operated on with a conservative approach (n = 60). CONCLUSION: A significant post-operative improvement in hearing could be a reasonable expectation in non-VS tumors extending into the CPA and a retrosigmoid approach should always be considered regardless of pre-operative hearing status.

Use of granules of biphasic ceramic in rehabilitation of canal wall down mastoidectomy.

The objective of this study is to evaluate the anatomical and functional results of rehabilitation of canal wall down (CWD) mastoidectomy using granules of biphasic ceramic. This is a study design retrospective in a tertiary referral centre Fifty-seven patients (59 ears) operated on between 2006 and 2010 of mastoid obliteration with granules of biphasic ceramic (TricOs, Maurepas, France) have been included (55 revisions and 4 first surgeries). Forty-six patients presented already a CWD mastoidectomy. The mean pre-operative bone conduction (BC) was 29 ± 3.4 dB (mean ± SEM) and mean air conduction (AC) was 57 ± 3.2 dB. Cholesteatoma was found in 33 cases. All but seven cases had post-operative otoscopy examination at 1, 3, 6 months, and 1 year postoperative with a CT scan and pure tone audiometry. Mean follow-up was 14 ± 1.8 months (3-35). At one-year follow-up (n = 52), 47 cases (90 %) presented well-healed external auditory canal. Five cases (10 %) of uncovered granules without sign of infection of external auditory canal skin were observed. Mean post-operative threshold was 25 ± 1.8 and 46 ± 1.9 dB for BC and AC , respectively (n = 47). CT scan (n = 42) showed no opacity suggesting residual disease within or behind obliteration. Mastoid obliteration with granules of biphasic ceramic is a safe and effective procedure that allows restoration of a near normal external auditory canal.

Management and Outcomes of Sporadic Vestibular Schwannoma: A Longitudinal Study Over 12 Years.

OBJECTIVES: To evaluate the management of sporadic vestibular schwannomas (VS) with a 12-year follow-up. STUDY DESIGN: Retrospective study of all VS patients initially treated in 2005 in a tertiary referent center. METHODS: Initial decision making for microsurgical resection (MSR) or wait-and-scan (WaS) was according to VS size and hearing; subsequently, MSR or stereotactic radiosurgery (SRS) was proposed dependent on VS growth and size, hearing, and patient's age or willingness. RESULTS: Two hundred and one sporadic VS were included. The first management apportionment was 120 WaS (61.5%), 72 MSR (37%), three SRS (1.5%), and six others refused MSR and were lost to follow-up (LFU). Within 1 year, 95 (47%) VS were surgically removed; 17 (8.5%) were treated by SRS; and 35 (17.5%) were LFU. The proportions for SRS and LFU were virtually unchanged for the following years, and the proportion under MSR increased slightly within 3 years and then remained stable. Finally, at 12 years, 104 (51.5%) cases had been operated on, 21 (10.5%) treated by SRS, 23 (11.5%) still under WaS, and 53 (26.5%) LFU, which were mainly intracanalicular. The initially and subsequently operated cases presented similar hearing preservation rates and good facial nerve function outcomes. CONCLUSION: This longitudinal study of a large number of VS, which were diagnosed over a short period of time and followed for 12 years, provides new information on both the natural history of these benign tumors and individual patient concerns. This study recommends use of the WaS policy for small and mid-sized VS before active therapeutic decision making. LEVEL OF EVIDENCE: 3 Laryngoscope, 131:E970-E976, 2021.

Management of sporadic vestibular schwannoma with contralateral nonserviceable hearing.

OBJECTIVES/HYPOTHESIS: To outline a possible decision-making process for sporadic vestibular schwannoma (VS) with contralateral nonserviceable hearing at diagnosis. STUDY DESIGN: Retrospective case series. METHODS: Diagnosed VS was studied in a tertiary referral center from 1995 to 2013. RESULTS: Twenty-eight patients were included, with a mean follow-up of 6.9 years (range = 0.5-20 years). Ten were stage 1, 10 were stage 2, five were stage 3, and three were stage 4. Ipsilateral hearing levels were A (n = 3), B (n = 10), C (n = 7) and D (n = 8) American Academy of Otolaryngology-Head and Neck Surgery classification. Contralateral hearing levels were C (n = 11) and D (n = 17). Initial VS management included surveillance (n = 12) or surgery (n = 16), and four patients were later treated with surgery (n = 3) or stereotactic irradiation (n = 1). Hearing was improved by hearing aids and/or etiological treatment (n = 8), restored by contralateral (n = 15) or ipsilateral (n = 4) cochlear implants (CIs), or ipsilateral auditory brainstem implants (ABIs) (n = 3). Finally, 18 CIs were active daily; 14 of them presented high or moderate benefit with mean open-set dissyllabic word scores (WRS) of 58.1% and sentence recognition scores (SRS) of 69.7%, but only one ABI was still active (WRS of 70% and SRS of 87% with lip reading). CONCLUSIONS: When early removal of VS was not necessary, contralateral CI or etiological treatment for hearing loss might be recommended initially. Ipsilateral CI is proposed, whereas VS should be operated on if previous hearing restoration was not successful. ABI should be reserved for the rare cases where a contralateral CI could not be implanted or the cochlear nerve was sectioned during VS removal. LEVEL OF EVIDENCE: 3 Laryngoscope, 130:E407-E415, 2020.

NF2-Related Intravestibular Schwannomas: Long-Term Outcomes of Cochlear Implantation.

OBJECTIVE: Intravestibular schwannomas (IVS) are uncommon tumors in Neurofibromatosis type 2 (NF2) and are mainly associated with multiple internal auditory meatus (IAM) and cerebellopontine angle (CPA) tumors. They usually induce profound hearing loss which can be rehabilitated by cochlear implantation (CI). The aim of this study was to analyze the long-term outcomes of CI during the unpredictable evolution of NF2 disease. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Three adults with neurofibromatosis type 2 and intravestibular schwannomas, and who were cochlear implant recipients. INTERVENTIONS: Periodic radiologic follow-up, tumor resection, and hearing rehabilitation. MAIN OUTCOME MEASURES: Audiological evolution, tumor evolution, surgical outcome, cochlear implant outcome. RESULTS: Three NF2 patients (mean age at diagnosis, 26.3 ±â€Š3.2 yr) were identified with IVS in the period between 2000 and 2017. IVS were first observed by serial MRI and profound hearing loss occurred in this ear after 4 ±â€Š1.5 years of follow-up. IVS were removed via a translabyrinthine approach, and ipsilateral cochlear implantations were simultaneously performed. In two patients, large contralateral CPA tumors had previously been removed without hearing preservation, whereas in the third patient, a small, growing contralateral VS was excised via a retrosigmoid approach 6 months after IVS removal/cochlear implantation with serviceable hearing preservation. In all cases, CI provided good hearing outcomes. In two cases, hearing outcomes were even better for more than 5 years when ipsilateral intracanalicular vestibular schwannomas were removed in either the same or subsequent procedures. CONCLUSIONS: Rehabilitation of hearing with CI provides a favorable long-term outcome in patients with NF2-related IVS which could be altered by the occurrence of other intracanalicular and/or CPA NF2-related tumors.

Oral mobility reflects rate of progression in advanced Friedreich's ataxia.

Our objective was to identify a sensitive marker of disease progression in Friedreich's ataxia. We prospectively evaluated speech, voice, and oromotor function in 40 patients at two timepoints. The mean disease duration was 20.8 ± 9.8 years and mean SARA score 23.7 ± 8.6 at baseline. Oral motor mobility, assessed by a combination of movements of the face, eyes, cheeks, lips, and tongue, decreased significantly after 1 year (P < 0.0001). The standardized response mean over 12 months was considered as large for oral mobility (1.26) but small for SARA (0.12). Oral mobility could therefore be a sensitive marker in therapeutic trials.

Diode laser in otosclerosis surgery: first clinical results.

OBJECTIVE: To evaluate the functional results of otosclerosis surgery using diode laser. STUDY DESIGN: Retrospective cohort analysis. PATIENTS: One hundred seven patients operated on for otosclerosis with a diode laser (119 ears, all primary cases) and 141 patients operated on with a conventional technique (141 ears, all primary cases). Revision cases using the diode laser were also described. METHODS: Preoperative tomographic computed scan findings and intraoperative observations were collected. Pure-tone and vocal audiometry was performed preoperatively and postoperatively (at 3 mo and 1 yr). RESULTS: In the laser group, the air-bone gap was 29 +/- 0.8 dB (n= 112) preoperatively and 9 +/- 0.6 dB (n = 58) at 1 year. Air conduction was improved by 22 +/- 1.7 dB at 1 year (n = 58). In the conventional group, the air-bone gap was 32 +/- 0.9 dB (n=127) preoperatively and 10 +/- 0.6 dB (n = 127) at 1 year. Air conduction was improved by 25 +/- 1.1 dB (n = 127) at 1 year. No difference of hearing gain was observed between the 2 groups at 1 year. A decreased rate of footplate fracture was observed with the diode laser (3.6%) compared with the conventional technique (21.3%). CONCLUSION: Diode laser is a reliable and safe device for otosclerosis surgery. The functional results were similar to those reported in other series.

Role of electrophysiology in guiding near-total resection for preservation of facial nerve function in the surgical treatment of large vestibular schwannomas.

OBJECTIVE In large vestibular schwannoma (VS) surgery, the facial nerve (FN) is at high risk of injury. Near-total resection has been advocated in the case of difficult facial nerve dissection, but the amount of residual tumor that should be left and when dissection should be stopped remain controversial factors. The objective of this study was to report FN outcome and radiological results in patients undergoing near-total VS resection guided by electromyographic supramaximal stimulation of the FN at the brainstem. METHODS This study was a retrospective analysis of a prospectively maintained database. Inclusion criteria were surgical treatment of a large VS during 2014, normal preoperative FN function, and an incomplete resection due to the strong adherence of the tumor to the FN and the loss of around 50% of the response of supramaximal stimulation of the proximal FN at 2 mA. Facial nerve function and the amount and evolution of the residual tumor were evaluated by clinical examination and by MRI at a mean of 5 days postoperatively and at 1 year postoperatively. RESULTS Twenty-five patients met the inclusion criteria and were included in the study. Good FN function (Grade I or II) was observed in 16 (64%) and 21 (84%) of the 25 patients at Day 8 and at 1 year postoperatively, respectively. At the 1-year follow-up evaluation (n = 23), 15 patients (65%) did not show growth of the residual tumor, 6 patients (26%) had regression of the residual tumor, and only 2 patients (9%) presented with tumor progression. CONCLUSIONS Near-total resection guided by electrophysiology represents a safe option in cases of difficult dissection of the facial nerve from the tumor. This seems to offer a good compromise between the goals of preserving facial nerve function and achieving maximum safe resection.

Multivariate Analysis of Factors Influencing Facial Nerve Outcome following Microsurgical Resection of Vestibular Schwannoma.

Objective To assess through multivariate analysis the clinical pre- and intraoperative factors of facial nerve outcomes at day 8 and 1-year recovery of facial palsy, as compared with day 8 status among patients who underwent total resection of unilateral vestibular schwannoma. Study Design Case series with chart review. Setting Tertiary referral center. Subjects and Methods This study included 229 patients with preoperative normal facial function and anatomic preservation of the facial nerve. Clinical, radiologic, and intraoperative factors were assessed according to facial nerve function at day 8 and 1 year. Results We observed that 74% and 84% of patients had good facial function (House-Brackmann [HB] I-II) at day 8 and 1 year, respectively. Of 60 patients, 26 (43%) who had impaired facial function (HB III-VI) at day 8 recovered good facial function (HB I-II) 1 year after surgery. A structured equation model showed that advanced tumor stage and strong facial nerve adhesion were independently associated with facial nerve conduction block at day 8. No predictive factor of impaired facial function recovery was seen at 1 year. In terms of the extracanalicular diameter of the tumor, the cutoff point to minimize the risk of impaired facial function was 16 mm. Conclusion At day 8 after vestibular schwannoma resection, facial function was impaired in the case of large tumors or strong facial nerve adhesion to the tumor. After 1 year, less than half of the patients recovered good facial function, and no predictive factor was found to be associated with this possible recovery.

Internal Auditory Canal Decompression for Hearing Maintenance in Neurofibromatosis Type 2 Patients.

BACKGROUND: In neurofibromatosis type 2 (NF2), multiple therapeutic options are available to prevent bilateral hearing loss that significantly affects the quality of life of patients. OBJECTIVE: To evaluate the morbidity and functional results of internal auditory canal (IAC) decompression in NF2 patients with an only hearing ear. METHODS: Twenty-one NF2 patients operated on for IAC decompression in a 3-year period with a minimum follow-up of 1 year were included in this retrospective study. They presented unilateral deafness due to previous contralateral vestibular schwannoma removal in 16 patients or contralateral hearing loss due to the tumor in 5 patients. Hearing level was of class A (American Academy of Otolaryngology-Head and Neck Surgery classification) in 7 patients, B in 8 patients, C in 1 patient, and D in 5 patients. Pure-tone average and speech discrimination score evaluations were performed at 6 days, 1 year, and during the follow-up. Eight patients had postoperative chemotherapy. RESULTS: No case of facial nerve palsy was observed. In the early postoperative period; all patients maintained the hearing class of the preoperative period. At 1-year follow-up, all but 3 patients maintained their hearing scores; at last follow-up (mean follow-up, 23 ± 8 months; range, 12-44 months), hearing classes remained stable with only 1 patient worsening from class B to C and 1 patient improving from class D to B. CONCLUSION: Decompression of IAC seems to be a useful procedure for hearing maintenance in NF2 patients, with very low morbidity. Ideal timing and association with chemotherapy should be evaluated in the future. ABBREVIATIONS: FN, facial nerveIAC, internal auditory canalNF2, neurofibromatosis type 2PTA, pure tone averageSDS, speech discrimination scoreVS, vestibular schwannoma.

Four-channel electromyography of the facial nerve in vestibular schwannoma surgery: sensitivity and prognostic value for short-term facial function outcome.

OBJECTIVE: To evaluate the short-term facial prognostic value of a four-channel facial electromyographic device in vestibular schwannoma surgery. STUDY DESIGN: Eighty-nine vestibular schwannomas operated on and intraoperatively monitored by a four-channel facial electromyographic device between October 2002 and September 2003 were included in this prospective study. Detection was performed in frontal, orbicularis oculi, orbicularis oris, and platysma muscles. MAIN OUTCOME MEASURE: Facial function grading at postoperative Days 1, 8, 30, and 180 (House-Brackmann classification). SETTING: Tertiary referral center. RESULTS: Postoperative facial function at Day 180 was assessed as Grade 1 or 2 in 80%, as Grade 3 or 4 in 16%, and as Grade 5 or 6 in 4% (n=80). The postoperative facial function was related to the intraoperative nerve stimuli thresholds (range, 0.01-3 mA for a response >100 microV) near the brainstem and the proximal-to-distal ratio of the stimulation threshold. A proximal threshold between 0.01 and 0.04 mA had a positive predictive value of 94% for good facial function (Grade 1 or 2). The proximal threshold was lower in patients with improving or stable facial function in comparison with those with a delayed deterioration between Days 8 and 30. The stimulation threshold at the adhesion zone was related to the immediate facial function outcome. The maximal electromyographic response was detected in the frontal muscle or the platysma in 27% of cases and in orbicularis oris and oculi in 73% of cases. CONCLUSION: A four-channel device may enhance electromyographic sensitivity. Determination of stimulation threshold below 0.05 mA yields facial prognostic information.

Middle ear and mastoid obliteration for cochlear implant in adults: indications and anatomical results.

AIM OF THE STUDY: To review indications, anatomical results, and complications of cochlear implant (CI) surgery in adults for which middle ear and mastoid obliterations were performed. PATIENTS AND METHODS: Thirty cases (26 patients, 4 bilaterally implanted) of 837 CI surgeries (3.5%) performed between January 2009 and December 2013 have been included in this retrospective study. The mean follow-up was 21 ± 18 months (mean ± SD, range 3-58). There were 11 males and 15 females. The mean age was 59 ± 19 years (range 35-82). All surgeries were performed with a single-stage technique including a canal wall down mastoidectomy with external auditory canal closure and mastoid obliteration with fat. A postoperative CT scan was performed in all cases. RESULTS: Etiologies of hearing loss were mainly chronic otitis with or without cholesteatoma in 24 cases. Other etiologies were meningitis with cochlear ossification in one case, progressive hearing loss in two cases, enlarged vestibular aqueduct in one case, temporal bone fracture with CSF leak in one case, and congenital aural atresia in one case. Four of those 30 cases were revision CI surgery for electrode array misplacement (one case with cochlear ossification) or extrusion from an open cavity (one case) and recurrent cholesteatomas (two cases). All surgeries were uneventful and performed in a single stage. The electrode array was inserted in the basal turn (29 cases) or in the middle turn (one case) of the cochlea. No complications were observed. Two cases of postoperative abdominal hematoma were drained under local anesthesia. A major failure of the CI device occurred 5 months after surgery. CONCLUSION: CI with mastoid and middle ear obliteration is a safe and effective technique for selected cases of cochlear implantation. Mastoid obliteration prevents from recurrent disease and lowering the facial ridge allows more space to manage extensive cochlear ossification or malformation.

Tolerance and osteointegration of TricOs(TM)/MBCP(®) in association with fibrin sealant in mastoid obliteration after canal wall-down technique for cholesteatoma.

CONCLUSION: The TricOs™/MBCP(®) and fibrin sealant composite was a convenient, effective, and well-tolerated material for mastoid cavity filling and immediate reconstruction of the external auditory meatus after cholesteatoma surgery with canal wall down (CWD). OBJECTIVE: To assess the tolerance and osteointegration of a bone graft substitute, TricOs™/MBCP(®), in association with fibrin sealant for filling the mastoid cavity after cholesteatoma surgery using the CWD technique. METHODS: In this prospective observational study 57 patients with cholesteatoma suitable for CWD were recruited from April 2006 to April 2008 and followed up for 1 year. The mastoid cavity was filled with TricOs™/MBCP(®) followed by immediate reconstruction of the external auditory meatus covered with fascia temporalis and/or cartilage. The main outcome was skin tolerance assessed by a novel weighted score emphasizing long-term results. The typical weighted reference score was 1.67; skin tolerance was considered acceptable if 75% of patients had a score ≤ 1.67. Secondary outcomes were otorrhea and/or otalgia, hearing, and osteointegration assessed through computed tomography scanning at 12 months. RESULTS: Forty-one patients had a complete follow-up; 34 (82.3%) patients achieved the main end point with scores ≤ 1.67. Otorrhea decreased postoperatively. No otalgia interfering with daily tasks was reported. Ossicular reconstruction was carried out in 29 patients. Absence of cochlear toxicity was confirmed by unimpaired bone conduction. Preoperative and postoperative speech audiometry results were similar. No serious adverse events were observed. Osteointegration was satisfactory with hyperdensity or intermediate density in 95% of patients at 12 months.

Diagnostic value of auditory brainstem responses in cerebellopontine angle tumours.

CONCLUSION: Auditory brainstem responses (ABRs) associated with other audio-vestibular examinations and a thorough clinical examination should allow detection of the majority of cerebellopontine angle (CPA) lesions (99.2-100%). OBJECTIVE: The increasing quality of MRI in the detection of CPA lesions, and the reports of false negative ABRs have raised issues concerning the value of ABR in the diagnosis and preoperative assessment of CPA lesions. The aim of this work was to assess the value of the ABR in the diagnosis of vestibular schwannomas (VS) and other CPA lesions. PATIENTS AND METHODS: This retrospective study included 676 solitary VS (548 operated on and 128 followed up) and 70 other CPA tumours (72% meningiomas, 11% cholesteatomas, 3% ependymomas, 15% miscellaneous) managed between 1990 and 2001. All patients underwent clinical examination, audiometry, ABR, vestibular caloric tests and MRI. RESULTS: ABRs were normal in 4.8% of VS. Association of normal ABR, vestibular caloric tests and audiometry (AAO-HNS class A) represented only 0.7% of VS. In other CPA lesions, ABR were normal in 15% of cases and the association of the three above-mentioned examinations was encountered in 10%. However, in the latter cases the clinical examination showed an abnormality in all cases.