Absent pyramidal eminence and stapedial tendon associated with congenital stapes footplate fixation: Intraoperative and radiographic findings.

OBJECTIVE: Report an association between congenital stapes footplate fixation (CSFF) and radiological absence of the pyramidal eminence and stapedial tendon. PATIENTS: Children and adults with intraoperatively confirmed CSFF and an absent stapedial tendon. INTERVENTIONS: Computed tomography (CT); exploratory tympanotomy with stapedotomy. MAIN OUTCOME MEASURES: Absence of a pyramidal eminence and stapedial tendon aperture identified on preoperative CT that was confirmed intraoperatively. RESULTS: Eight patients with intraoperative confirmation of CSFF and absent stapedial tendon were retrospectively identified. The average preoperative bone conduction and air conduction pure tone averages were 19.6 dB (SD 15.6 dB) and 55.9 dB (SD 23.6 dB), respectively. The average air-bone gap was 36.3 dB (SD 17.9 dB) preoperatively. In the seven patients who underwent preoperative CT, all were consistently identified to have an absent or hypoplastic pyramidal eminence and absent stapedial tendon aperture at the pyramidal eminence. In six cases, the stapedial footplate appeared normal, while in one case the footplate appeared abnormal which correlated with severe facial nerve prolapse observed intraoperatively. All eight cases underwent exploratory tympanotomy and demonstrated intraoperative stapes footplate fixation, absent stapedial tendon and either absent or hypoplastic pyramidal eminence, which correlated with preoperative CT findings. CONCLUSIONS: This study identifies a clinically pragmatic association between an absent pyramidal eminence identified on high-resolution CT and the diagnosis of CSFF. In a condition that otherwise generally lacks distinctive radiological features, the absence of a pyramidal eminence on CT in a patient with nonprogressive, congenital conductive hearing loss may strengthen clinical suspicion for CSFF.

Effect of Number of Electrodes Used to Elicit Electrical Stapedius Reflex Thresholds in Cochlear Implants.

INTRODUCTION: When mapping cochlear implant (CI) patients with limited reporting abilities, the lowest electrical stimulus level that produces a stapedial reflex (i.e., the electrical stapedius reflex threshold [eSRT]) can be measured to estimate the upper bound of stimulation on individual or a subset of CI electrodes. However, eSRTs measured for individual electrodes or a subset of electrodes cannot be used to predict the global adjustment of electrical stimulation levels needed to achieve comfortable loudness sensations that can be readily used in a speech coding strategy. In the present study, eSRTs were measured for 1-, 4-, and 15-electrode stimulation to (1) determine changes in eSRT levels as a function of the electrode stimulation mode and (2) determine which stimulation mode eSRT levels best approximate comfortable loudness levels from patients' clinical maps. METHODS: eSRTs were measured with the 3 different electrical stimulation configurations in 9 CI patients and compared with behaviorally measured, comfortable loudness levels or M-levels from patients' clinical maps. RESULTS: A linear, mixed-effects, repeated-measures analysis revealed significant differences (p < 0.01) between eSRTs measured as a function of the stimulation mode. No significant differences (p = 0.059) were measured between 15-electrode eSRTs and M-levels from patients' clinical maps. The eSRTs measured for 1- and 4-electrode stimulation differed significantly (p < 0.05) from the M-levels on the corresponding electrodes from the patients' clinical map. CONCLUSION: eSRT profiles based on 1- or 4-electrode stimulation can be used to determine comfortable loudness level on either individual or a subset of electrodes, and 15-electrode eSRT profiles can be used to determine the upper bound of electrical stimulation that can be used in a speech coding strategy.

Dyna-CT of the temporal bone for case-specific three-dimensional rendering of the stapedial muscle for planning of electrically evoked stapedius reflex threshold determination during cochlear implantation directly from the stapedius muscle via a retrofacial approach: a pilot study.

PURPOSE: Evaluation of 3D Dyna-CTs to improve cochlear implantation (CI) planning and intraoperative electrically elicited stapedius reflex threshold (ESRT) measurements. METHODS: A prospective observational cohort study was performed. Anonymized data collection of Dyna-CTs and CI surgeries in which a retrofacial approach was implemented to access the stapedius muscle. 3D Dyna-CTs of 30 patients and the intraoperative confirmation of the predication in 5/30 patients during CI surgery were evaluated. Inter-rater reliability was also analyzed along with the predictive value of this evaluation. RESULTS: 36 representative structures of the middle and inner ear and 3D renderings of the Dyna-CTs were evaluated by four otoneurological surgeons. Fleiss' kappa values for the evaluation of the visibility were high (> 0.7) for most of the anatomical structures. The stapedius muscle was visible in 90% of the cases. Using the 3D data, the retrofacial access to the stapedius muscles was estimated as feasible in 86.7%. Fleiss' kappa value of the evaluation of the accessibility was 0.942. The intraoperative exploration of the stapedius muscle confirmed the preoperative prediction in all five selected patients (four patients with predicted accessibility and one patient with predicted inaccessibility). CONCLUSIONS: The use of Dyna-CT and 3D rendering is a helpful tool for preoperative planning of cochlear implantations and ESRT measurements from the stapedius muscle via the retrofacial approach.

Manubrio-incudo-stapedioplasty for reconstruction of Austin-Kartush type B ossicular defects.

OBJECTIVE: Manubrio-incudo-stapedioplasty functional outcomes were compared to those of other methods for reconstructing Austin-Kartush type B ossicular defects. METHODS: Forty-two patients underwent Austin-Kartush type B ossicular defect reconstruction using: manubrio-incudo-stapedioplasty (13 patients), an autologous incus (19 patients) or a titanium ossicular replacement prosthesis (10 patients). For manubrio-incudo-stapedioplasty reconstruction, the malleus head was removed, the manubrium was relocated posteriorly and the incus short process was placed on the mobile footplate. The manubrium was placed on the incus body groove and bone cement was applied to stabilise the manubrium-incus junction. Pre- and post-operative hearing thresholds were assessed. RESULTS: The air-bone gap decreased from 25.9 ± 6.0 dB to 12.3 ± 5.0 dB (p < 0.05) in the manubrio-incudo-stapedioplasty group. The hearing gain was 13.6 ± 5.2 dB for manubrio-incudo-stapedioplasty, 3.4 ± 14.2 dB with the autologous incus, and 3.3 ± 11.07 dB with the titanium ossicular replacement prosthesis. Hearing improvement was greater for manubrio-incudo-stapedioplasty compared to the other reconstruction methods (p < 0.05). CONCLUSION: Manubrio-incudo-stapedioplasty resulted in satisfactory hearing outcomes in patients with Austin-Kartush type B ossicular defects. This technique can be considered a stable, inexpensive and effective method to reconstruct Austin-Kartush type B ossicular defects.

Stapedial reflex threshold predicts individual loudness tolerance for people with autistic spectrum disorders.

People with autism spectrum disorder (ASD) frequently show the symptoms of oversensitivity to sound (hyperacusis). Although the previous studies have investigated methods for quantifying hyperacusis in ASD, appropriate physiological signs for quantifying hyperacusis in ASD remain poorly understood. Here, we investigated the relationship of loudness tolerance with the threshold of the stapedial reflex and with contralateral suppression of the distortion product otoacoustic emissions, which has been suggested to be related to hyperacusis in people without ASD. We tested an ASD group and a neurotypical group. The results revealed that only the stapedial reflex threshold was significantly correlated with loudness tolerance in both groups. In addition to reduced loudness tolerance, people with lower stapedial reflex thresholds also exhibited higher scores on the Social Responsiveness Scale-2.

Tympanometric evaluation of Eustachian tube function in Polish scuba divers.

BACKGROUND: Scuba divers are subjected to relatively high ambient pressures while descending. Equalizing maneuvers (e.g., Valsalva) are necessary to open the Eustachian tube (ET) and allow air into the middle ear (ME) cavity. Insufficient opening of the ET leads to ME barotrauma, which is the most common injury related to scuba diving. The study aims were to assess the incidence of ME barotrauma and to compare tympanometric parameters and stapedial reflexes in scuba divers and non-diving individuals. MATERIAL AND METHODS:: 60 scuba divers participated in the study; control consisted of 90 non-diving volunteers without a history of otolaryngologic problems. All participants were examined with the use of otoscopy and tympanometry with evaluation of ipsilateral stapedial reflexes. The group studied was surveyed regarding occurrence of ME barotrauma and diving competence. RESULTS: 51.7% of the divers experienced ME barotrauma, the most common symptoms being earache and hearing loss. Comparison of the group studied and control revealed significantly lower ME pressure and compliance in scuba divers. In scuba divers with ME barotrauma, longer time from injury correlates directly with greater ME pressure and compliance, indicating tissue recovery. At 4,000Hz 100dB percentage of present stapedial reflexes among scuba divers was significantly lower than in controls; moreover, a greater number of dives correlated inversely with percentage of present stapedial reflexes at 4000Hz 100dB. The reduced thresholds at high intensities suggest a negative effect of scuba diving on hearing. CONCLUSIONS: ME pressure and compliance, however still within the norm, are significantly lower in scuba divers than in non-diving healthy volunteers. This may be attributed to a subclinical form of barotrauma.

Stapedotomy using a 4 mm endoscope: any advantage over a microscope?

OBJECTIVES: To ascertain the feasibility of endoscopic (4 mm) stapedotomy, and compare intra- and post-operative variations with microscopic stapedotomies. METHODS: Forty otosclerosis patients were scheduled for microscopic or endoscopic stapedotomy. Intra-operative variables compared were: incision, canalplasty, canal wall curettage for ossicular assessment, chorda tympani manipulation, ability to perform stapes footplate perforation before its supra-structure removal, and operative time. Post-operative variables compared were ear pain and hearing improvement. RESULTS: Of the 20 microscopy patients, 4 required endaural incision and canalplasty because of canal overhangs, and 7 required canal wall curettage for ossicular assessment. None of the 20 endoscopy patients required these procedures. Chorda tympani was manipulated in 13 and 6 patients in the microscopy and endoscopy groups respectively, while the stapes footplate could be perforated in 5 and 11 patients respectively. Mean operative time was 50.25 and 76.05 minutes in the microscopy and endoscopy groups respectively. In the endoscopy group, mean air-bone gap was 37.12 and 10.73 dB pre- and post-operation respectively; in the microscopy group, these values were 35.95 and 13.81 dB. CONCLUSION: Endoscopic stapedotomy has comparable hearing outcomes. Sinonasal endoscope serves as a better tool for: minimal incision, canalplasty avoidance, less chorda tympani mobilisation, and stapes footplate perforation ability.

Clinically recorded cortical auditory evoked potentials from paediatric cochlear implant users fitted with electrically elicited stapedius reflex thresholds.

OBJECTIVES: This study aimed to objectively evaluate access to soft sounds (55 dB SPL) in paediatric CI users, all wearing MED-EL (Innsbruck, Austria) devices who were fitted with the objective electrically elicited stapedius reflex threshold (eSRT) fitting method, to track their cortical auditory evoked potential (CAEP) presence and latency, and to compare their CAEPs to those of normal-hearing peers. METHODS: Forty-five unilaterally implanted, pre-lingually deafened MED-EL CI users, aged 12-48 months, underwent CAEP testing in the clinic at regular monthly intervals post switch-on. CAEPs were recorded in response to short speech tokens /m/, /g/ and /t/ presented in the free field at 55 dB SPL. Twenty children with normal hearing (NH), similarly aged, underwent CAEP testing once. RESULTS: The proportion of present CAEPs increased and CAEP P1 latencies reduced significantly with post-implantation duration. CAEPs were scored based on their presence and age-appropriate P1 latency. These CAEP scores increased significantly with post-implantation duration. CAEP scores were significantly worse for the /m/ speech token compared to the other two tokens. Compared to the NH group, CAEP scores were significantly smaller for all post-implantation test intervals. CONCLUSIONS: This study provides clinicians with a first step towards typical ranges of CAEP presence, latency, and derived CAEP score over the first months of MED-EL CI use. CAEPs within these typical ranges could validate intervention whereas less than optimum CAEPs could prompt clinicians to seek solutions in a timely manner. CAEPs could clinically validate whether a CI provides adequate access to soft sounds. This approach could form an alternative to behavioural soft sound access verification.

Comparing eSRT and eCAP measurements in pediatric MED-EL cochlear implant users.

INTRODUCTION: Electrically evoked compound action potentials (eCAP) and electrically evoked stapedius reflexes are the most frequently used objective measurements for programming a cochlear implant (CI) audio processor. Objective methods are particularly beneficial for children and CI users that encounter difficulties in providing feedback. In this study, we compared the threshold and the slope of the eCAP amplitude growth function with the electrically evoked stapedius reflex threshold (eSRT) in pediatric CI users. Furthermore, the duration times required to perform eCAP and eSRT recordings were compared. METHODS: During a regular fitting session, 52 pediatric CI users with recordable eSRTs having MED-EL devices (MED-EL GmbH, Innsbruck, Austria) were programmed using the eSRT fitting method. The eCAP thresholds and the slopes of the amplitude growth function were measured across one apical, one medial, and one basal electrode contact. RESULTS: There was a weak to medium correlation between eCAP thresholds and eSRTs. The eCAP threshold profile did not correlate with the eSRT profile. Typically ECAP thresholds were at a lower stimulation charge than eSRTs with only 4/152 being higher. An eCAP threshold was found on 152/156 electrode contacts with eSRTs. On average, the eCAP measurements took 4.2 times longer to record per electrode than eSRT measurements (median durations 35 s vs. 120 s). CONCLUSION: eSRTs were significantly higher than eCAP thresholds and eSRT and eCAP profiles were generally different from each other reducing the clinical relevance of eCAP testing for setting MCLs across the array. Additionally, the eSRT measurements were faster to record than the eCAP threshold and slope determination measurements.

Cilia and Ear.

OBJECTIVE: To investigate the prevalence of otological complications derived from primary ciliary dyskinesia (PCD) in adulthood. METHODS: Twenty-three patients with diagnosed PCD underwent medical history aimed at recording the presence of ear, nose, and throat manifestations (ENT) and any surgical treatments. The ENT objectivity was annotated, and then patients were subjected to audiometric test, tympanometry, registration of otoacoustic emission, and vestibular evaluation. RESULTS: Otitis media with chronic middle ear effusion (OME) during childhood was reported in 52% of the subjects, no patient had undergone ear surgery, and only 2 patients had an episode of otitis in the last year. Eleven of 23 patients showed normal hearing, 11 had a conductive hearing impairment, and 1 showed a severe sensorineural hearing loss unrelated to the syndrome. The bilateral stapedial reflex was only found in all cases of normoacusia and type A tympanogram, distortion product otoacoustic emissions (DPOAE) were present in 8 patients, and no patient had vestibular alterations. CONCLUSION: Our study confirms a very frequent prevalence of OME in PCD during childhood. Careful monitoring of otological complications of the syndrome is always desirable, also given the high presence in adults of other manifestations in the upper airways, such as chronic rhinosinusitis and nasal polyposis.

Acoustic reflex patterns in amyotrophic lateral sclerosis.

The aim of the study is to investigate acoustic reflex testing in amyotrophic lateral sclerosis patients. Amplitude, latency, and rise time of stapedial reflex were recorded for 500 and 1000 Hz contralateral stimulus. Statistical analysis was performed by the Wilcoxon test and the level of significance was set at 5 %. Fifty-one amyotrophic lateral sclerosis patients and ten sex- and age-matched control subjects were studied. Patients were further divided in two groups: amyotrophic lateral sclerosis-bulbar (38 cases, with bulbar signs at evaluation) and amyotrophic lateral sclerosis-spinal (13 cases, without bulbar signs at evaluation). Stapedial reflex was present in all patients. There was a statistically significant difference in the mean amplitude, latency, and rise time between the amyotrophic lateral sclerosis patients as compared with the controls. Amplitude was lower in both the amyotrophic lateral sclerosis-bulbar and the amyotrophic lateral sclerosis-spinal patients than in the controls (p < 0.05) and rise time was longer in both patient groups compared with the controls (p < 0.05). These results confirm the presence of abnormal acoustic reflex patterns in amyotrophic lateral sclerosis cases with bulbar signs and, moreover, suggesting a possible subclinical involvement of the stapedial motor neuron even in amyotrophic lateral sclerosis-spinal patients. Amplitude and rise time seem to be good sensitive parameters for investigating subclinical bulbar involvement.

Stimulation parameters differ between current anti-modiolar and peri-modiolar electrode arrays implanted within the same child.

OBJECTIVE: To compare stimulation parameters of peri-modiolar and anti-modiolar electrode arrays using two surgical approaches. METHODS: Impedance, stimulation thresholds, comfortably loud current levels, electrically evoked compound action potential thresholds and electrically evoked stapedial reflex thresholds were compared between 2 arrays implanted in the same child at 5 time points: surgery, activation/day 1, week 1, and months 1 and 3. The peri-modiolar array was implanted via cochleostomy in all children (n = 64), while the anti-modiolar array was inserted via a cochleostomy in 43 children and via the round window in 21 children. RESULTS: The anti-modiolar array had significantly lower impedance, but required higher current levels to elicit thresholds, comfort, electrically evoked compound action potential thresholds and electrically evoked stapedial reflex thresholds than the peri-modiolar array across all time points, particularly in basal electrodes (p < 0.05). The prevalence of open electrodes was similar in anti-modiolar (n = 5) and peri-modiolar (n = 3) arrays. CONCLUSION: Significant but clinically acceptable differences in stimulation parameters between peri-modiolar and anti-modiolar arrays persisted four months after surgery in children using bilateral cochlear implants. The surgical approach used to insert the anti-modiolar array had no overall effect on outcomes.

Evaluation of the acoustic (stapedius) reflex test in children and adolescents with peripheral facial nerve palsy.

INTRODUCTION: The stapedius nerve is one of the branches of the facial nerve in the temporal bone. It supplies the stapedius muscle, which is responsible for the attenuation reflex that protects the inner ear from loud noises. The stapedius (acoustic) reflex (SR) test is useful in identifying the site of facial nerve injury. The return of the SR (acoustic) to normal after an injury is a good prognostic factor in the treatment of facial nerve palsy. OBJECTIVE: The aim of this study was to evaluate the effect of FNP on the SR (acoustic) response and determine the acoustic reflex threshold (ART) levels on the affected side. MATERIAL AND METHOD: In this study, 70 patients, 3-7 years old, were screened. The study population consisted of 26 boys (37%) and 44 girls (63%). Follow-up tests were performed 3-18 months after the initial tests. RESULTS: Most patients in the study population had a negative SR (acoustic) response on the affected side. In other patients, mean ART values were statistically higher on the affected side. There was no statistically significant relationship between a reflex response and the time from the onset of facial nerve palsy. DISCUSSION: In the available literature, the SR (acoustic) testing is limited in determining whether or not the reflex is present without stimulus frequency or ART measurements. It is estimated that the reflex response is negative with ipsilateral stimulation on the affected side in 35-80% patients. CONCLUSIONS: The SR (acoustic) is absent in most patients on the affected side. The ART value was statistically higher on the affected side. The SR (acoustic) response was statistically time independent.

Hammer sound elicited tinnitus in car body repair worker cured by stapedial tenotomy - A case report.

Abnormal auditory sensations or tinnitus caused by abnormal middle ear muscle contraction are extremely rare and uncomfortable for patients. A 67-year-old man who performed paint and body work for cars presented at our hospital with complaint of an audible and annoying abnormal sound that was synchronous with the striking of his hammer against the metal of the car body during his work. The patient reported that the sound was audible of left ear with a split-second delay after his hammer struck the metal. Preoperative subjective and objective testing failed to reveal any abnormal findings in our case. The patient's symptom was successfully cured by selective transection of the stapedius tendon. The characteristic nature of tinnitus with a split-second delay after striking the metal helped our diagnosis and method of intervention in this case.

Cochlear implantees: Analysis of behavioral and objective measures for a clinical population of various age groups.

INTRODUCTION: As of 2014 more than 1200 patients have received a cochlear implant (CI) at Oslo University Hospital (OUS) and approximately half of them have been children. The data obtained from these patients have been used to develop a comprehensive database for a systematic analysis of several objective measurements and programming measurements. During the past 10 years, we have used an objective measurements protocol for our CI surgeries. Our intra-operative protocol includes: Evoked Compound Action Potentials (ECAP), visually observed Electrically evoked Stapedius Reflex Threshold (ESRT), and electrode impedances. Post-operative (Post-OP) programming sessions typically begin 4-6 weeks after surgery and continue on a scheduled basis. The initial programming data include threshold levels (T-levels) and comfortable levels (C-levels) for the different patient age groups. In this study, we compared initial stimulation levels and stimulation levels after at least 1 year of CI with objective measurements obtained intra-operatively. METHOD: This study focused on the development of a comprehensive database of detailed intra-operative objective measures and post-OP programming measurements from a group of 296 CI patients who received the same type of CI and electrode configuration (Cochlear Corporation CI with Contour electrode). This group included 92 bilateral CI patients. Measurements from 388 CI devices were studied. Patients were divided into 5 different age groups at the age of implantation: 0-2, 2-5, 5-10, 10-20, and above 20 years in order to investigate age-related differences in programming levels and objective measurements. For the comparison analysis we used T- and C-levels obtained after the last day of initial programming and also after at least 1 year implant use. These programming levels were then correlated with some of the intra-operative objective measurements. RESULTS: T-levels were found to be the lowest for the youngest patient group and increased with age. C-levels varied within age groups and frequency range. Patients above 20 years of age had the highest comfort levels in the low to mid-frequencies (electrodes 22-8) and the lowest comfort levels in the high-frequency range (electrodes 1-7). Correlation coefficients between intra-operative objective measurements and programming levels were found to be in the range of no correlation to moderate correlation. Adult patients had the most significant correlation coefficients between ECAP thresholds and T-levels in the low frequencies. The younger patients aged 10-20 years and 5-10 years had more significant correlations in the higher frequency channels compared to the other age groups. Intra-operative visually observed ESRTs and electrode impedances were not significantly correlated with initial or stable programming levels for the children or adults. CONCLUSION: Analyzing initial and follow-up mapping levels from previous patients is very important for a CI Center in terms of quality control. The mean T/C-levels reported in this study can provide guidance to our programming audiologists and help them determine the initial programming levels to be stored in the speech processor, especially for very young patients. Unfortunately intra-operative objective measures in our study, such as ECAP, ESRT, and electrode impedances did not provide statistically significant correlations that may help to predict the programming T- and C-levels for all patients. However, we have observed cases where the intra-operative objective measures of ESRT and TECAP profiles were very similar to an individual's MAP profile. It was not possible, however, to determine why some patients did not have an objective measures profile that was similar to their programming levels profile.

Division of the stapedial tendon results in noise-induced damage to the inner ear.

BACKGROUND: The effect of division of the stapedial tendon on susceptibility to noise-induced inner ear damage has not been previously studied. This study aimed to evaluate the effects of noise exposure following division of the stapedial tendon in guinea pigs. MATERIAL AND METHODS: Ten adult albino guinea pigs were used. The stapedial tendon of each right ear was cut. The stapedial tendon in each left ear was left intact and these ears served as a control group. DPOAEs and ABR tests were performed before and 10 days after noise exposure. The animals were exposed to a 110-dB noise stimulus for 6 h in a silent room a week after surgery. Cochleas of the animals were removed, and inner and outer hair cells were examined under a light microscope. RESULTS: We found that noise exposure adversely affected DPOAE measurements at all frequencies except 2 KHz in experimental ears. Noise exposure also produced significantly elevated ABR thresholds in experimental ears at 2, 4, 8, and 16 KHz. On histopathological examination, we found a significantly greater prevalence of apoptotic cells in the experimental ears. CONCLUSIONS: Based on these findings, we can conclude that after division of the stapedial tendon, noise exposure may cause damage to the inner ear. This is the first study in the English literature that demonstrates the potential protective effect of the stapedial tendon against acoustic damage.

Facial nerve palsy associated with a cystic lesion of the temporal bone.

Facial nerve palsy results in the loss of facial expression and is most commonly caused by a benign, self-limiting inflammatory condition known as Bell palsy. However, there are other conditions that may cause facial paralysis, such as neoplastic conditions of the facial nerve, traumatic nerve injury, and temporal bone lesions. We present a case of facial nerve palsy concurrent with a benign cystic lesion of the temporal bone, adjacent to the tympanic segment of the facial nerve. The patient's symptoms subsided after facial nerve decompression via a transmastoid approach.

Effect of total intravenous anaesthesia on intraoperative monitoring of cochlear implant function in paediatric patients.

UNLABELLED: There is increasing evidence in medical literature which proves that post-operative outcomes in children implanted early are better. Initial setting of these children's stimulation parameters is challenging because of their limited communication abilities. Intraoperative electrical impedance measurements, electrically evoked stapedial reflex threshold (ESRT) and evoked compound action potential (ECAP) are used for implant settings in some centres as no cooperation is required from the child. Aim Evaluate the effects of intravenous anaesthesia on intraoperative monitoring of cochlear implant function in paediatric cochlear implantees at our centre. Method Prospective study from January 2011 to December 2011. COHORT: 29 children. Age - 18 months to 11 yrs. All children had bilateral severe to profound sensorineural hearing loss. Children with compromised neural/cochlear anatomy were excluded. Patients were maintained on an infusion of Fentanyl @ 0.3-0.6 ugm/kg/hr and Propofol @ 4-8 mg/kg/hr intraoperatively. Intraoperative measurements were done after performing the train of four test on the adductor pollicis muscle Results It was observed that ESRT was unaffected by intravenous anaesthesia. Electrical impedance and ECAP were not affected by any technique of anaesthesia. Conclusion Intravenous anaesthesia has little or no effect on the intraoperative auditory thresholds and is therefore recommended for determining these thresholds during cochlear implant surgery.

Transitory stapedial myoclonus in a patient with benign fasciculation syndrome.

OBJECTIVE: We report a previously undescribed association between transitory stapedial myoclonus, objective tinnitus and benign fasciculation syndrome. METHOD: Case report and review of the world literature regarding stapedial myoclonus. RESULTS: A 30-year-old man with a diagnosis of benign fasciculation syndrome abruptly developed severe, low-pitched tinnitus on the right side. Otoscopic examination revealed rhythmic movement of the tympanic membrane, which was synchronous with the tinnitus. No palatal spasm was noted on nasopharyngeal examination. Brain magnetic resonance imaging and pure tone audiometry were unremarkable. Based on these findings, a diagnosis of objective tinnitus due to stapedial myoclonus was made. The objective tinnitus spontaneously disappeared within 48 hours of its appearance, but in the following days the patient suffered frequent, brief episodes of objective tinnitus lasting only a few seconds. CONCLUSION: The occurrence of stapedial myoclonus in this patient indicated the presence of an underlying motor unit hyper-excitability. This case suggests that, in some patients, stapedial myoclonus may represent the clinical expression of diffuse motor unit hyper-excitability.

Absent stapedial reflex: otosclerosis or middle ear tumor?

We present an unusual case in which a patient diagnosed as having otosclerosis on the basis of clinical and audiologic findings actually had a middle ear facial nerve schwannoma. To the best of our knowledge, this is the first reported case in English literature in which a facial nerve schwannoma presented with conductive deafness of gradual onset and absent stapedial reflex with a normally functioning facial nerve. We also include a review of the literature.