Surgical management of middle cranial fossa bone defects: meningoencephalic herniation and cerebrospinal fluid leaks.

Along the medical practice of an Otologist he/she will face middle cranial fossa (MCF) bone defects. The purpose of this study is to contribute to the understanding of this possible life threatening condition, and to share and discuss our management approach. A literature review is also presented. STUDY DESIGN: Retrospective case series at García-Ibáñez Otology and Skull base private center referral. METHODS: This study is based on the analysis of data collected from 19 cases of temporal bone meningoencephalic herniations surgically treated from 2006 to 2018. The follow-up ranged from 18 to 162 months with a mean average of 44.5 months. MAIN FINDINGS: Meningoencephalic herniations were divided into four etiologic groups: spontaneous (24.8%), secondary to chronic otitis media (21.8%), iatrogenic (45.9%), and posttraumatic (7.5%). Different surgical techniques were used as treatment: transmastoid (TM) approach (27.8%), MCF approach (27.8%), combined technique (transmastoid plus minicraniotomy, 3%), and middle ear obliteration with blind sac closure of the external auditory canal (41.4%). CONCLUSIONS: Variables like bilateral hearing level, size and location of the bone defect and existence of CSF leak should be analyzed to select the safest and most effective closing surgical approach.

Tumor growth rate: A new prognostic indicator of hearing preservation in vestibular schwannoma surgery.

BACKGROUND: The management of small- to medium-size (< 20 mm) sporadic vestibular schwannomas (VSs) continues to inspire debate. Preoperative pure tone thresholds and tumor size are recognized prognostic features of hearing preservation after surgery. OBJECTIVE: To investigate what preoperative characteristics were associated with nonserviceable hearing after surgery for VSs. METHODS: We retrospectively reviewed the audiological results of 92 patients treated with the middle cranial fossa (MCF) approach for resection of VSs (< 20 mm). Sex, age, symptoms, tumor site, tumor size, growth rate, and hearing class according to American Academy of Otolaryngology-Head and Neck Surgery guidelines were evaluated. Negative outcome was progression after surgery to nonserviceable hearing according to Gardner-Robertson classification. We included only patients with preoperative serviceable hearing. RESULTS: After surgery, 48 patients (52.2%) had nonserviceable hearing. At univariate analysis, sex age, symptoms, tumor site, and size were not associated to hearing outcome. Tumor growth rate ≥ 2.16 mm/year (P = 0.02, odds ratio 8.5) and preoperative hearing class B (P = 0.03, OR 5.89) were statistically associated to nonserviceable hearing after VSs resection. Tumor growth rate ≥ 2.16 mm/year was also significantly associated to preoperative hearing class B (P = 0.01). At multivariate analysis, the only independent prognostic factor of progression to nonserviceable hearing in operated VSs was tumor growth rate ≥ 2.16 mm/year (P = 0.01, OR = 4.15). CONCLUSION: We found a new prognostic indicator of hearing preservation after VS surgery with the MCF approach: the tumor growth rate. This feature should be further investigated before being considered in the decision-making process of VS treatment. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:2378-2383, 2019.

One-Year Results for Patients with Unilateral Hearing Loss and Accompanying Severe Tinnitus and Hyperacusis Treated with a Cochlear Implant.

OBJECTIVE: To show that patients with unilateral hearing loss (UHL), with one ear fulfilling cochlear implant (CI) indication criteria, and an additional severe tinnitus handicap can be treated effectively with a CI. METHOD: A prospective multi-centre study was conducted in five Spanish centres. Sixteen adult patients with UHL and a mean Tinnitus Handicap Inventory (THI) score of at least 58 were implanted. The study design included repeated within-subject measures of quality of life (Health Utility Index Mark 3 [HUI3]), tinnitus (THI, Visual Analogue Scale [VAS] on tinnitus loudness), hearing (Speech, Spatial, and Qualities of Hearing Scale- [SSQ]), and hyperacusis (Test de Hipersensibilidad al Sonido [THS]) up to 12 months after the initial CI fitting. RESULTS: Group data showed significant subjective benefit from CI treatment: the preoperative HUI3 total utility score of 0.45 went up to 0.57 at 6 months and 0.63 at 12 months; the preoperative THI total score of 75 decreased to 40 at 6 months and 35 at 12 months. The preoperative tinnitus loudness VAS score of 8.2 decreased to 2.4 at 6 months and 2.2 at 12 months with the implant "On" and to 6.7 at 6 months and 6.5 at 12 months with the implant "Off." The preoperative THS total score of 26 decreased to 17 at 12 months. The preoperative SSQ total score of 4.2 increased to 5.1 at 6 months and 6.3 at 12 months. No unanticipated adverse events were reported during the study period. At 12 months after CI activation all subjects (except 1 subject who used the device 6 days a week) wore their devices all day and every day. The primary reason for CI use was split evenly between tinnitus suppression (n = 6) and both hearing and tinnitus (n = 6). CONCLUSION: A CI should be considered as a treatment option in patients with UHL and a concomitant severe tinnitus handicap. However, appropriate counselling of candidates on the anticipated risks, benefits, and limitations that are inherent to cochlear implantation is imperative.

Cochlear implants as a treatment option for unilateral hearing loss, severe tinnitus and hyperacusis.

Tinnitus is an incapacitating condition commonly affecting cochlear implant (CI) candidates. The aim of this clinical study is to assess the long-term effects of CI treatment in patients with severe-to-profound, sensorineural, unilateral hearing loss (UHL) and incapacitating tinnitus. We performed a prospective Cochlear™ company-sponsored multicentre study in five Spanish centres. Sixteen patients with UHL and incapacitating tinnitus, which was indicated by a Tinnitus Handicap Inventory (THI) score >58%, received a Nucleus® CI in their deaf ear. The study design includes repeated within-subject measures on hearing, tinnitus, hyperacusis and quality of life up to 12 months after initial CI fitting. In addition to hearing loss and tinnitus, all patients suffered from hyperacusis. Most patients had a sudden hearing loss and received a CI within 2 years after their hearing loss. Preliminary 6-month, post-CI activation data of 13 subjects showed that the majority of patients perceived a subjective benefit from CI treatment, which was assessed using the THI, a Visual Analogue Scale of tinnitus loudness/annoyance and the Speech, Spatial and Qualities of Hearing Scale. Preliminary 12-month data of 7 subjects showed that most patients also perceived a degree of relief from their hyperacusis. One patient showed no improvements in any of the applied scales, which could be explained by partial insertion of the electrode due to obstruction of the cochlea by otosclerosis. In conclusion, CI can successfully be used in the treatment of UHL patients with accompanying severe tinnitus and hyperacusis. Implantation resulted in hearing benefits and a durable relief from tinnitus and hyperacusis in the majority of patients. These findings support the hypothesis that pathophysiological mechanisms after peripheral sensorineural hearing loss are at least partly reversible when hearing is restored with a CI.

Evaluation of Neural Response Telemetry (NRT™) with focus on long-term rate adaptation over a wide range of stimulation rates.

Custom Sound EP™ (CSEP) is an advanced flexible software tool dedicated to recording of electrically evoked compound action potentials (ECAPs) in Nucleus® recipients using Neural Response Telemetry™ (NRT™). European multi-centre studies of the Freedom™ cochlear implant system confirmed that CSEP offers tools to effectively record ECAP thresholds, amplitude growth functions, recovery functions, spread of excitation functions, and rate adaptation functions and an automated algorithm (AutoNRT™) to measure threshold profiles. This paper reports on rate adaptation measurements. Rate adaptation of ECAP amplitudes can successfully be measured up to rates of 495 pulses per second (pps) by repeating conventional ECAP measurements and over a wide range of rates up to 8000 pps using the masked response extraction technique. Rate adaptation did not show a predictable relationship with speech perception and coding strategy channel rate preference. The masked response extraction method offers opportunities to study long-term rate adaptation with well-defined and controlled stimulation paradigms.

Contralateral hearing aid use in cochlear implanted patients: multicenter study of bimodal benefit.

CONCLUSION: The use of a hearing aid (HA) in combination with a cochlear implant (CI) significantly improved performance for speech perception in quiet, in noise, and for localization compared with monaural conditions. No significant differences in functional performance were observed following optimization of HA fitting. OBJECTIVES: To evaluate the binaural benefits derived from using a contralateral HA in conjunction with a CI in subjects with significant functional hearing in the nonimplanted ear and the effects of HA fitting optimization. METHODS: Fifteen adult CI users, intra-subject controls, were enrolled in a prospective repeated-measure multicenter study. Evaluation of performance for speech understanding, localization, and subjective impressions was conducted before and following HA fitting optimization for CI alone, HA alone, and CI + HA. RESULTS: For speech testing in quiet, bimodal scores were significantly better than for HA alone and CI alone conditions (p < 0.01). For speech and noise (S0N0) at 0° azimuth the scores were significantly better in the bimodal condition than for CI alone (p = 0.01), indicating binaural summation. When noise was presented to the HA side (S0NHA) bimodal scores were significantly better than for CI alone (p < 0.01 and p < 0.05, respectively), suggesting a significant binaural squelch effect. Sound localization ability was significantly improved in the bimodal condition compared with the CI alone condition (p = 0.002).

Prevalencia de test afisiológicos de posturografía dinámica en pacientes laborales / Prevalence of aphysiologic performance on dynamic posturography in work-related patients

Introducción y objetivos: El vértigo y la inestabilidad tienen una importante implicación médico-legal en el paciente laboral. La posturografía dinámica computarizada (PDC) aporta información adicional a las pruebas vestibulares estándar, pero además se han descrito patrones de control postural compatibles con escasa colaboración o falta de sinceridad al esfuerzo, denominados afisiológicos. El objetivo de este trabajo es determinar la prevalencia de testafisiológicos en estudios de PDC sobre pacientes laborales con vértigo y/o inestabilidad. Material y métodos: Estudio retrospectivo de pacientes remitidos por mutuas laborales para estudio de vértigo y/o inestabilidad. Se realizó un estudio vestibular estándar y PDC. Los resultados de la prueba de organización sensorial (sensory organization test [SOT]) se valoraron como normales, vestibulares o afisiológicos usando el método de cálculo publicado por Cevette et al. en 1995. Resultados: El patrón afisiológico en el SOT, definido por los índices de Cevette, se observó en el 31% de los casos estudiados. Se ha encontrado una relación estadísticamente significativa (p = 0,01) entre los valores bajos de la puntuación compuesta (composite score) y el resultado afisiológico. La videonistagmografía (VNG) presentaba alteraciones en 14 de los 31 casos con SOT afisiológico. Conclusiones: La prevalencia de casi un tercio de los resultados de PDC afisiológicos entre pacientes laborales con vértigo y/o inestabilidad en nuestro medio, es relativamente elevada en comparación con el 25% publicado por Longridge y Mallinson en 2005. El patrón afisiológico no necesariamente significa simulación o exageración, siendo frecuente la presencia concomitante de signos videonistagmográficos de vestibulopatía en estos casos(AU)

Introduction and objectives: Medical-legal implications of dizziness and imbalance in work-related patients are important. In these cases, computerized dynamic posturography (CDP)adds information to standard vestibular tests and aphysiologic patterns have been described. The objective is to assess the prevalence of aphysiologic performance on CDP in work-related patients complaining of dizziness/imbalance. Material and Methods: Retrospective review of patients referred by the workers’ compensation board for assessment of dizziness, imbalance or both. Standard vestibular assessment including CDP was carried out in all patients. The sensory organization test (SOT) summaries were scored as normal, aphysiologic or vestibular using the scoring method published by Cevette et al. in1995.Results: Aphysiologic performance in SOT, evaluated with the Cevette formula, was found in 31out of 100 cases. Low composite score results and aphysiologic SOT results had a statistically significant association (P=.01). Videonystagmography (VNG) was altered in 14 out of 31 cases with aphysiologic SOT. Conclusion: The 31% prevalence of aphysiologic results on CDP among work-related patients complaining of dizziness/imbalance is relatively high in comparison with the 25% published by Longridge and Mallinson in 2005. However, aphysiologic performance should not necessarily be related to malingering or exaggeration and altered vestibular tests are found in some of these cases(AU)

[Prevalence of aphysiologic performance on dynamic posturography in work-related patients]. / Prevalencia de test afisiológicos de posturografía dinámica en pacientes laborales.

INTRODUCTION AND OBJECTIVES: Medical-legal implications of dizziness and imbalance in work-related patients are important. In these cases, computerized dynamic posturography (CDP) adds information to standard vestibular tests and aphysiologic patterns have been described. The objective is to assess the prevalence of aphysiologic performance on CDP in work-related patients complaining of dizziness/imbalance. MATERIAL AND METHODS: Retrospective review of patients referred by the workers' compensation board for assessment of dizziness, imbalance or both. Standard vestibular assessment including CDP was carried out in all patients. The sensory organization test (SOT) summaries were scored as normal, aphysiologic or vestibular using the scoring method published by Cevette et al. in 1995. RESULTS: Aphysiologic performance in SOT, evaluated with the Cevette formula, was found in 31 out of 100 cases. Low composite score results and aphysiologic SOT results had a statistically-significant association (P=.01). Videonystagmography (VNG) was altered in 14 out of 31 cases with aphysiologic SOT. CONCLUSION: The 31% prevalence of aphysiologic results on CDP among work-related patients complaining of dizziness/imbalance is relatively high in comparison with the 25% published by Longridge and Mallinson in 2005. However, aphysiologic performance should not necessarily be related to malingering or exaggeration and altered vestibular tests are found in some of these cases.

Clinical evaluation of cochlear implant sound coding taking into account conjectural masking functions, MP3000™.

Efficacy of the SPEAK and ACE coding strategies was compared with that of a new strategy, MP3000™, by 37 European implant centers including 221 subjects. The SPEAK and ACE strategies are based on selection of 8-10 spectral components with the highest levels, while MP3000 is based on the selection of only 4-6 components, with the highest levels relative to an estimate of the spread of masking. The pulse rate per component was fixed. No significant difference was found for the speech scores and for coding preference between the SPEAK/ACE and MP3000 strategies. Battery life was 24% longer for the MP3000 strategy. With MP3000 the best results were found for a selection of six components. In addition, the best results were found for a masking function with a low-frequency slope of 50 dB/Bark and a high-frequency slope of 37 dB/Bark (50/37) as compared to the other combinations examined of 40/30 and 20/15 dB/Bark. The best results found for the steepest slopes do not seem to agree with current estimates of the spread of masking in electrical stimulation. Future research might reveal if performance with respect to SPEAK/ACE can be enhanced by increasing the number of channels in MP3000 beyond 4-6 and it should shed more light on the optimum steepness of the slopes of the masking functions applied in MP3000.

Effect of changing pulse rate on profile parameters of perceptual thresholds and loudness comfort levels and relation to ECAP thresholds in recipients of the Nucleus CI24RE device.

The Nucleus CI24RE 'Freedom' device offers higher stimulation rates and lower noise levels in action potential measurements (ECAPs) than previous devices. A study including ten European implant teams showed that the effect of changes in rate from 250 to 3500 pulses per second on tilt and curvature of the T and C profiles is insignificant. When changing rate one may change the levels at all electrodes by the same amount. Using an automated procedure ECAPs could be measured quickly and reliably at a noise level of only 1 microV, this did not result in improved correlations between the tilt and curvature parameters of the ECAP profiles and those of the T and C profiles. Average C levels appear to differ markedly among implant centers; a better assessment protocol is required. When increasing stimulus rate one should take into account that this requires higher pulse charges per second and more power consumption.

An evaluation of the preservation of residual hearing with the Nucleus Contour Advance electrode.

CONCLUSION: Our study results confirm that it is possible to preserve preoperative hearing levels in the majority of subjects when using the Nucleus 24 Contour Advance provided that there is adherence to the major principles of 'soft surgery'. Our study group demonstrated that 71-86% of subjects showed preservation of preoperative hearing thresholds at 6 months to varying degree. OBJECTIVES: The aim of the study was to assess the degree of residual hearing preserved postoperatively in a group of standard cochlear implant (CI) candidates following implantation via soft surgery with a Nucleus 24 Contour Advance CI. Surgical technique variations from the soft surgery guidelines provided were assessed and their potential impact upon the conservation of residual hearing was examined. SUBJECTS AND METHODS: A prospective multicentre study involving a within-subject repeated measures design with each subject acting as their own control was performed. Pure-tone audiometric thresholds were assessed and compared in both implanted and contralateral ears for each subject preoperatively as baseline measures and at 6 months postoperatively. Surgeons were asked to complete a questionnaire to capture various aspects of the surgical technique used for each subject. Variations in the surgical technique performed were examined for potential correlation with conservation of residual hearing. Twenty-eight adult subjects, with a severe to profound hearing impairment, were enrolled in the study across eight implant clinics in four countries. RESULTS: In all, 36% of subjects demonstrated preservation of thresholds to within 10 dB of preoperative thresholds across the frequency range (0.25, 0.5, 1.0, 2.0 and 4.0 KHz) and for the low frequency range (0.25-1.0 KHz). Approximately two-thirds of subjects demonstrated preservation of preoperative thresholds to within 20 dB. Preservation of low frequency thresholds post-implant was shown to correlate moderately with cochleostomy site, being more likely for subjects with a site anterior-inferior to the round window but also possible with inferior locations; weakly with cochleostomy size, being more likely when smaller than 1.2 mm; and also with the use of Healon as a sealant and lubricant. Preservation of hearing thresholds across up to 4000 Hz was shown to correlate weakly with the use of suction following opening of the endostium and with bone dust contamination, both having a negative effect upon preservation, while no correlation was observed with the preservation of thresholds for low frequencies alone.

Speech perception with the ACE and the SPEAK speech coding strategies for children implanted with the Nucleus cochlear implant.

OBJECTIVE: The aim of this study is to determine whether implanted children using the ACE speech coding strategy demonstrate superior performances compared to implanted children using the SPEAK speech coding strategy over time. METHODS: Cochlear implanted children with prelinguistic sensorineural bilateral deafness of profound degree, using either the ACE or SPEAK coding strategy, were evaluated and compared. Both groups of children used one of the speech coding strategies continuously from the initial programming session and for a period of 2 years post-switch-on. One group comprised children who were retrospectively implanted and had received the SPEAK speech coding strategy (n=32) and the second group consisted of prospectively implanted children who received the ACE speech coding strategy (n=26). Both populations were homogenous as far as age of implantation, degree of hearing loss, anatomy of the cochlea, depth of electrode insertion, and educational and rehabilitative support provided. Children were assessed at 6, 12 and 24 months post switch-on via pure-tone audiometry and for speech perception tests. Children using the ACE speech coding strategy were additionally evaluated using the MAIS and MUSS language scales. RESULTS: Satisfactory benefits in speech perception were demonstrated by both groups of implanted children. No significant difference between the mean pure tone thresholds was observed postoperatively between the groups. Two years post switch-on the group using the ACE speech coding strategy demonstrated superior results for vowel discrimination in comparison to children using the SPEAK coding strategy. No significant difference was observed between the groups for performance on discrimination of syllable patterns (ESP) or for disyllablic word recognition tests. Additionally, the group of ACE users demonstrated maximum performance on MAIS and MUSS scales, 2 years post switch-on. CONCLUSIONS: The results clearly demonstrate significant benefit of cochlear implantation in prelinguistically deafened children for speech perception ability when using either the SPEAK or ACE speech coding strategies. Children using the ACE speech coding strategy demonstrate more rapid progress in improved speech perception ability initially, however 2 years post switch-on, no significant difference in performance on open-set speech recognition tests can be noted irrespective of the strategy in use.