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Objective: The goal of this study was to determine open field stapedius reflex thresholds (oSRTs) in CI patients with fittings based on subjective loudness ratings. A further objective was to compare these oSRTs and those of eSRT-based fittings that are similar to the oSRTs of normal hearing. Design: Impedance measurements of the ear drum were taken while subjects were wearing their audio processors. The stapedius reflex was elicited by electrical stimulation transmitted through the activated CI system in response to an acoustic stimulus presented in the free sound field. Study sample: Subjects were 50 experienced CI users (n = 57 ears) with CI fittings based on subjective loudness scaling. Results: A reference range for the oSRTs was defined that was identified in CI patients with eSRT-based fittings. Sound levels for stapedius reflex detection were inside the reference target range in 70% of the cases, below the reference range (i.e. down to 40 dB HL) in 20% of the cases, and above the reference range in 10% of the cases. Conclusion: Stapedius reflex detection in a free sound field may help detect fittings with too high or too low stimulation levels that might reduce audiological performance. [ABSTRACT FROM AUTHOR]

Objective: The study aimed to compare ipsilateral and contralateral electrically evoked stapedial reflex thresholds in children with a unilateral cochlear implant surgically implanted either through Veria or posterior tympanotomy approaches. Methods: Forty-nine children using cochlear implants were studied, of whom 27 underwent the Veria approach and 22 underwent the posterior tympanotomy approach. The electrically evoked stapedius reflex thresholds were measured ipsilaterally and contralaterally by stimulating four equally spaced electrodes. Results: The ipsilateral electrically evoked stapedius reflex threshold was absent in all four electrodes in the children implanted using the Veria approach. However, the ipsilateral electrically evoked stapedius reflex threshold was present in 70 per cent of the children implanted using the posterior tympanotomy approach. The contralateral electrically evoked stapedius reflex threshold was present in most of the children for both surgical approaches. Conclusion: The presence of the ipsilateral electrically evoked stapedius reflex threshold varies depending on the surgical technique used for cochlear implantation. However, contralateral reflexes are present in the majority of children using cochlear implants, irrespective of the surgical approach. [ABSTRACT FROM AUTHOR]

A persistent stapedial artery is a congenital vascular malformation that can cause tinnitus and/or conductive hearing loss. Although rare, this case highlights the importance of recognizing aberrant anatomy as a potential cause of patients' symptoms. It also demonstrates how to recognize and treat patients with a symptomatic persistent stapedial artery. [ABSTRACT FROM AUTHOR]

Objective: This study sought to evaluate the demographics as well as clinical, audiometric, and surgical outcomes of patients who underwent endoscopic and microscopic stapedotomies. Methods: This study enrolled 87 otosclerosis patients who underwent endoscopic and microscopic stapedotomies. Results: In total, 44 and 43 surgeries were performed using an endoscope and a microscope, respectively. No significant difference was noted in the air--bone gaps of the cohort before or after the procedures (P = .611). Both procedures resulted in similar audiometric outcomes. The mean operative time was more significant in the last 22 patients of the endoscopic group than in the first 22 patients and patients who underwent microscopic stapedotomy (P = .027 and P = .018, respectively). Conclusions: Endoscopic stapedotomy exhibits a learning curve similar to that of other procedures. Patient outcomes were similar between the endoscopic and microscopic procedures. [ABSTRACT FROM AUTHOR]

Background and purpose: Retrofacial approach (RFA) is an access route to sinus tympani (ST) and it is used in cholesteatoma surgery, especially when type C ST is encountered. It may also be used to gain an access to stapedius muscle to assess the evoked stapedius reflex threshold. The primary object of this study was to evaluate the morphology of sinus tympani and its relationship to facial nerve (FN) and posterior semicircular canal (PSC) in context of planning retrofacial approach in pneumatized temporal bones. Methods: CBCT of 130 adults were reviewed. The type of sinus tympani was assessed according to Marchioni's classification. Width of entrance to sinus tympani (STW), depth of ST (STD), distance between the posterior semicircular canal and facial nerve (F-PSC), distance between the latter plane to the floor of ST at the right angle (P-ST) were measured at level of round window (RW) and pyramidal ridge (PR). Results: All of the bones were well-aerated and classified in Dexian Tan pneumatization group 3 or 4. Type B of ST is dominant (70.8%) in adult population with no history of inflammatory otologic diseases, followed by type C (22.7%) and then type A (6.5%). The depth of ST (STD) presented significant deviations (ANOVA, p < 0.05) among all three types. STW reaches greater values on the level of PR. F-PSC does not correlate with type of ST. In over 75% of examined type C sinus tympani the distance P-ST was less than 1 mm. Conclusions: The qualitative classification of the sinus tympani into types A, B and C, introduced by Marchioni is justified by statistically significant differences of depth between individual types of tympanic sinuses. The STW distance reaches greater values inferiorly—it may suggest that RFA should be performed in infero-superior manner rather than opposite direction. Preoperative assessment of temporal bones CT scans gives very important information about size of sinus tympani and distance between FN and PSC. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to evaluate the long-term hearing outcomes in stapedotomy surgery using skeeter oto-drill and to assess safety in difficult situations. Method: A retrospective study was conducted with 944 patients who underwent 1007 stapedotomy procedures over 16 years, performed by a single surgeon using a trans-canal approach and a self-retaining ear canal retractor. Hearing thresholds were calculated over four frequencies. Air–bone conduction hearing thresholds were obtained at 1, 5 and 10 years post-operatively and compared to the pre-operative records. Results: Out of 1007 operated ears with one year follow up, 98.61 per cent of cases showed a negligible air–bone gap of equal to or less than 5 dB, 1.19 per cent of cases showed an air–bone gap equal to or more than 5 dB but less than 10 dB, and only 0.2 per cent of cases showed an air–bone gap of more than 10 dB. Conclusion: In this study, using skeeter drill with a 0.6 mm diamond burr to make the fenestra was constant in all the cases and one of the safest techniques, showing persistent long-term hearing results. [ABSTRACT FROM AUTHOR]

High-resolution computed tomography (HRCT) is often used to diagnose otosclerosis. To investigate whether CT values change over time and correlate with hearing in the non-surgical ears of otosclerosis patients. 32 patients with bilateral otosclerosis who had undergone unilateral stapedectomy were enrolled in the study. HRCT examination and pure tone audiometry were performed before and after the surgery. CT values of different regions of interest (ROIs) were measured manually and the data were collected for analysis. The CT value of anterior to the inner auditory meatus (AIAM) decreased by 88.5 HU (p <.05), and the changes in CT values in other ROIs showed no statistical difference. Regarding hearing, the value of air bone gap (ABG) increased by 3.6 dB (p =.020), and the average hearing deterioration rate of ABG was 3.1 dB/year. The CT value of the mid-point of the stapes footplate (MPSF) showed a certain correlation with the change of ABG (p <.05). The CT value of AIAM may change over time. The overall hearing of non-surgical ears in otosclerosis patients shows a deteriorating trend. Moreover, the changes in the CT value of MPSF may be related to the hearing, which needs further research. [ABSTRACT FROM AUTHOR]

Background: Otosclerosis is a disease of the osseous labyrinth. The disease causes 5% to 9% of all cases of hearing loss and 18% to 22% of conductive hearing loss. The treatment of choice is a surgery. The hearing improvement after the operation is determined by various factors. Aims/Objectives: The aim of the analysis is to determinate changes in hearing after stapedoplasty in view of surgery side in the patients operated on otosclerosis by right-handed surgeons. Material and Methods: The analysis involved patients hospitalized and operated on otosclerosis between 2012 and 2018. Only patients with their first middle ear surgery due to otosclerosis were included in the study. The patients were operated by 2 right-handed surgeons who used the same surgical technique and had similar experience in otosclerosis surgery. The study included patients who were divided into 2 groups: with self-tightening prosthesis and with manually tightening prosthesis. Results: The procedure performed by right-handed operators on the left side using prostheses requiring manual fixation on the incus was associated with poorer audiometric results compared to the results of surgeries on the right side. In patients with the self-tightening prostheses, the audiometric improvement of hearing was bilaterally comparable independently from operation side. Conclusion: (1) The dependence of hearing improvement on the surgery side was demonstrated in cases of surgeries performed on the left ear by right-handed surgeons, particularly with manually tightening prosthesis. (2) Self-tightening prostheses in stapedotomy limit the human factor, reducing the risk of complications after otosclerosis surgery and provide repeatable hearing improvement. [ABSTRACT FROM AUTHOR]

Purpose: Sinus tympani is the space in the retrotympanum, with variable morphology. Computed tomography is a common tool to investigate sinus tympani anatomy. During cochlear implantation or tympanoplasty, electrocochleography can be used for hearing monitoring. In such a surgical strategy the electrode is placed in the round window's region throughout posterior tympanotomy. Common accessible needle-shaped electrodes using is difficult in achieving intraoperative stabilization. The aim of the study is to assess the dimensions and shape of sinus tympani, basing on the micro computed tomography scans for purposes of establishing the possible new electrocochleography electrode shape. Materials and methods: Sixteen fresh frozen cadaveric temporal bones were dissected. MicroCT measurements included the depth and the width of sinus tympani, width of facial canal with stapedius muscle chamber. Obtained data were analyzed statistically with the use of RStudio 1.3.959 software. Results: The highest average width of sinus tympani amounted for 2.68 mm, depth measured at the round window plane for 3.19 mm. Width of facial canal with stapedius muscle chamber highest average values at the round window plane- 3.32 mm. The lowest average minimum and maximum values were calculated at the 1 mm above the round window plane. The highest average posterior tympanotomy width was 2.91 mm. Conclusions: The shape of the tympanic sinus is like a trough with the narrowest and deepest dimensions in the middle part. The ST shape and dimensions should be taken into account in constructing the ECochG electrode, designed for optimal placement through posterior tympanotomy approach. [ABSTRACT FROM AUTHOR]

Higher probe tone frequencies have been shown to increase the elicitation rates in electrically evoked stapedius reflex threshold (eSRT) measurements. To determine the optimal probe tone frequency for contralateral eSRT measurements at individual electrodes in children with unilateral cochlear implants and to assess the relationship between eSRTs for this frequency and most comfortable levels (M-levels). Contralateral eSRT measurements with three probe tone frequencies (226, 678, and 1000 Hz) at individual electrodes were performed on 26 paediatric Advanced Bionics cochlear implant recipients. The elicitation rates of eSRTs for 226, 678, and 1000 Hz probe tones were 73.08% (57/78), 88.46% (69/78), and 88.46% (69/78), respectively. The average eSRT for the 1000 Hz probe tone was significantly lower than those for 226 and 678 Hz probe tones (p<.001 and p=.009, respectively). ESRTs for the 1000 Hz probe tone and M-levels were significantly correlated at all tested electrodes (all p<.001). The optimal probe tone frequency for contralateral eSRT measurement at individual electrodes in children with unilateral cochlear implants is 1000 Hz. ESRTs for the 1000 Hz probe tone are significantly correlated with M-levels and can be used to guide the M-levels setting in these children. [ABSTRACT FROM AUTHOR]

To determine the relation between stapedius reflex thresholds in cochlear implant users evoked once through direct electric stimulation on single channels (ESRT) and once through acoustic stimulation in free sound field. For comparison, stapedius reflex thresholds were obtained in free sound field in a normal-hearing control group. For each participant a new ESRT-based fitting was created. Stapedius reflex thresholds were obtained for this new fitting in free sound field for different loudness adjustments. Acoustic stimuli for eliciting the stapedius reflex were narrow band noise signals covering the audiometric frequency range. N = 29 experienced CI users (34 ears) and N = 10 normal hearing listeners. ESRT-based fitting resulted in different stapedius reflex behaviour compared to normal-hearing listeners. A frequency dependence was observed. Stapedius reflex thresholds decreased with increasing centre frequencies of acoustic narrow band noise stimuli. A linear relation between upper stimulation levels on the implant channels and corresponding stapedius reflex thresholds evoked in free sound field was found. The found correlation may be a guideline for adjusting the electrical dynamic range during cochlear implant fitting. This allows the implant system to mimic the natural reflex behaviour in the best possible way and potentially avoid overstimulation. [ABSTRACT FROM AUTHOR]

In the present study, hearing sensitivity in children with speech sound disorder (SSD) is scrutinized. Middle ear function (wideband tympanometry and acoustic stapedial reflexes, ASR) and inner ear function (audiometric thresholds in the conventional1-8 kHz and extended10-16 kHz high frequency (EHF) range, and distortion product otoacoustic emissions (DPOAEs2-10 kHz) were investigated. Hearing results were analyzed in relation to speech discrimination of phonemic contrasts (quiet and in noise conditions) and reproduction. Thirty-two children with SSD and 41 children with typical development (TD) ages 4–5 years participated. Children with SSD exhibited significantly less sensitive hearing compared to children with TD. This was demonstrated as more absent contralateral ASR (right ear SSD 43.7%; TD 22.0%), a higher prevalence of minimal hearing loss (MHL, > 15 dB HL at one or more frequencies or ears1-8 kHz and PTA ≤ 20 dB HL, SSD 53.1%; TD 24.3%) and EHF hearing impairment (EHF HI, > 20 dB HL at one or more frequencies or ears10-16 kHz, SSD 31.3%; TD 24.3%). At 2 kHz bilaterally, children with SSD showed significantly higher hearing thresholds than children with TD (mean difference, left ear 3.4 dB: right ear 4.3 dB), together with a significantly lower SNR in DPOAEs at 2.2 kHz (left ear 5.1 dB mean difference between groups). In all children, audiometric thresholds at the key-frequencies for speech, 2 and 4 kHz and DPOAEs within similar spectral regions, predicted 7–12% of the variance in phonemic discrimination and reproduction. Overall, these results suggest that hearing should be more fully investigated in children with SSD. [ABSTRACT FROM AUTHOR]

Mondini dysplasia with spontaneous cerebrospinal fluid (CSF) leak is rare in cochlear implantation (CI) candidates but lethal. Detailed evaluation and surgical intervention are needed for these patients. To report our findings of the peristapedial bulb in computer tomography (CT) as diagnostic evidence of spontaneous CSF leak and discuss its clinical value to direct the surgical plan for patients with profound sensorineural hearing loss (SNHL) and meningitis. A retrospective review was conducted, including patients' demographic features, radiographic examination, operation records, auditory/speech evaluation. The patients presented with a peristapedial bulb were included. In 2775 CI recipients, 7 out of 219 (3.2%) patients with Mondini anomaly had detectable peristapedial bulbs in HRCT, among whom 6 patients have a history of meningitis. Surgical exploration verified the radiographic findings in 6 out of 7 patients. All patients received CI and vestibular obliteration. Control of meningitis was acceptable. The threshold of hearing significantly decreased from 74.1 dB ± 6.9 dB to 37.1 ± 4.8 dB after CI and the word recognition score elevated from 21.4 ± 14.9% to 78.6 ± 9.3%. Peristapedial bulb is an indicator of spontaneous CSF leak in patients with profound SHNL and Mondini anomaly. Patients will benefit from CI and surgical intervention [ABSTRACT FROM AUTHOR]

The study is aimed to assess the scope of endoscopic stapedotomy in overcoming technical challenges faced during conventional stapedotomy using operating microscope. Sixty-four patients with clinical and audiological diagnosis of otosclerosis were randomly assigned into one of the 2 groups—one underwent conventional stapedotomy using operating microscope, while the other group underwent endoscopic stapedotomy, the operating surgeon being the same for both groups, for all cases. The 2 groups were observed in terms of extent of the postero-superior canal bone curettage/drilling, chorda tympani repositioning, visualization of footplate area, surgical time from first incision to ear packing, post-operative morbidity in terms of post-operative pain, vertigo, hearing outcome, and changes in taste sensation. It was observed that irrespective of the width of the external auditory canal, endoscopic approach offered better access to the footplate area requiring lesser bone removal and chorda tympani repositioning. The operating time, post-operative pain, and changes in taste sensation were significantly less in the endoscopic group. However, no difference was noted in terms of the post-operative hearing outcome and incidence of vertigo. Endoscopic stapedotomy has clear advantages in terms of the technicality and accessibility to the working area as well as faster recovery. [ABSTRACT FROM AUTHOR]

Background: In otosclerosis mixed hearing loss is the most frequent symptom and arises when the focus involves the stapes footplate. Surgeons usually prefer to wait a minimum air-bone gap of 25 – 35 dB before surgery. Objectives: To evaluate the outcome of microdrill stapedotomy for otosclerosis in patients with a preoperative air-bone gap (ABG) <25 dB versus patients with a preoperative gap ≥ 25 dB. Material and methods: For this retrospective study, the outcomes and complications after microdrill stapedotomy were compared between adult patients with a preoperative small ABG (n = 127, ABG <25 dB) and those with a large ABG (n = 254, ABG ≥25 dB). Results: The postoperative ABG was significantly smaller than the preoperative ABG (p <.05) in both groups; there were no differences in complications rates (severe sensorineural hearing loss, footplate fracture or early postoperative vertigo) between the two groups. Conclusions: Our findings show that microdrill stapedotomy is safe and can be performed even in patients with a preoperative small ABG without increasing the risk of sensorineural hearing loss due to inner ear damage. [ABSTRACT FROM AUTHOR]

This study aimed to establish an extended morphometric dataset regarding the stapedius muscle for anatomists and otologists. The tympanic cavity of ten cadavers (five females, five males) aged with 75.70 ± 13.75 years was bilaterally dissected. Morphometric properties of the stapedius muscle (i.e., its muscular belly and tendon) and its relationship with the neighborhood structures including the facial nerve was evaluated. The length of the entire stapedius muscle was found as 4.80 ± 1.13 mm. The depth between the entrance of the external auditory canal and stapedius muscle was measured as 18.23 ± 2.30 mm. The incudostapedial joint and stapedial tendon were found to be 1.66 ± 0.25 mm and 1.18 ± 0.19 mm away from the facial nerve, respectively. The stapedial tendon length was standardized as five types: Type 1, extremely short tendon (under 0.5 mm), 5% of cases; Type 2, short tendon (between 0.5 and 1 mm), 30% of cases; Type 3, normal tendon (between 1 and 2 mm), 55% of cases; Type 4, long tendon (between 2 and 2.5 mm), 10% of cases; and Type 5, extremely long tendon (above 2.5 mm), no cases. Our findings showed that the stapedius tendon size in adults was quite similar to fetuses. Therefore, probably entire muscle dimension does not alter after birth. Considering the concordance between direct anatomic (our findings) and radiologic measurements (literature findings) of the stapedius muscle, preoperative radiological evaluation may be important for otologists in terms of the choice of surgical tools such as diamond burr sizes. Due to the lack of standardization regarding the evaluation of the stapedius tendon size (i.e., extremely short or extremely long), we defined the tendons below 0.5 mm as extremely short (Type 1) and above 2.5 mm as extremely long (Type 5). [ABSTRACT FROM AUTHOR]

Background: Detailed investigations of the stapedial muscle (SM) in congenital aural atresia (CAA) patients have yet to be adequately conducted. Objectives: To assess image variations in the mastoid segment of the facial nerve (FN) and SM in CAA. Materials and methods: A total of nine patients comprising of 9 ears with unilateral CAA were studied. The courses of the FN and SM were evaluated from the basic point to 1 mm intervals between the mastoid portion of FN, and measured from the mean X and Y values in each group. Results: The atresia side of FN among the Y values showed significant differences compared to the contralateral side. In terms of the SM, there were no significant differences in both the X and Y values. The stapedial muscle of the CAA patients was located medially to the FN. Conversely, the distance from the PSC to the FN revealed no significant differences with regard to the X and Y values for each group. Conclusion: The current observations revealed that the SM is located more posterior to the FN in CAA patients, and this is mainly attributed to the laterally and anteriorly displaced FN. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The aim of this study was to evaluate the morphology of the stapedius muscle and its tendon with the use of microCT and to describe their anatomic relationship with facial nerve and incudostapedial joint. The study was performed on 16 fresh cadaveric temporal bones scanned in microtomography (microCT). Stapedius muscle and its tendon were identified in each set of images. The length of the medial and lateral border of the stapedius tendon (STL-med, STL-lat), width at the insertion to stapes (STW-s), at the point it emerges from the pyramidal eminence (STW-p) and in the half way from the pyramidal eminence to stapes (STW-m), and the length and the width of the belly of stapedius muscle (BSML and BSMW) were measured in modified axial plane. The shortest distance between the facial canal and incudostapedial joint (FN-isj), and between the facial canal and stapedius tendon (FN-st) were measured in the Pöschl plane. The average values of all distances measured were: STL-lat 1.29 ± 0.50 mm, STL-med 1.27 ± 0.44 mm, BSML 2.98 ± 0.51 mm, STW-s 0.47 ± 0.10 mm, STW-p 0.46 ± 0.12 mm, STW-m 0.35 ± 0.12 mm, BSMW 1.26 ± 0.29 mm, FN-isj 1.72 ± 0.33 mm, FN-st 1.35 ± 0.30 mm. The stapedius muscle complex consists of the tendon and the belly, and the border between them in microCT scans is not always evident. The distance between the facial nerve and the incudostapedial joint is greater than the distance between the facial nerve and the stapedius muscle tendon. [ABSTRACT FROM AUTHOR]

Purpose: The main objective of the study was to investigate the morphometric properties of the stapedial tendon (ST) for pediatric otosurgeons and anatomists. Methods: The present study was placed on 15 fetuses (8 females, 7 males) aged from 20 to 30 weeks of gestation (at mean, 24.27 ± 3.24 weeks) using the collection of the Anatomy Department of Medicine Faculty, Mersin University. All measurements were obtained with a digital image analysis software. Results: In terms of male/female or right/left comparisons, no statistically significant difference was found in relation with the numerical data of ST. The surface area, length, and width of ST were detected as follows: 0.61 ± 0.15 mm2, 1.27 ± 0.30 mm, and 0.45 ± 0.08 mm, respectively. The absence of ST was observed in two fetuses with and without severe malformations. In another fetus with cleft lip and polydactyly, multiple abnormalities were bilaterally identified in the middle ear: (1) the absence of the incudostapedial joint and (2) the presence of an abnormal tissue attaching to the stapes. The abnormal tissue was determined to be irregular dense connective tissue using light microscope and electron microscope. Conclusion: Our findings showed that ST did not proportionally grow according to increasing gestational weeks. In the light of the numerical data, we thought that similar to stapes, ST attains the adult size in the fetal period. As ST anomalies may accompany severe malformations (e.g., cleft lip, polydactyly or syndactyly) that can be easily detected on observation by clinicians, we suggest that the detailed examination of middle ear in newborns should be taken into account for early diagnosis of conductive hearing loss to prevent any management delays. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the quality of perceived sound after stapedotomy over a 1-year follow-up period focussing on incidence of dysacusis, particularly distorted sound perception (DSP). DSP was assessed by (i) determination of the frequencies and hearing level that such perceptions are elicited by pure tones (pure-tone-evoked distorted sound perception, PTE-DSP), a novel psychoacoustic measurement introduced in this paper, and (ii) assessment of patient-reported occurrence of DSP using the Amsterdam Post Operative Sound Evaluation (APOSE) questionnaire (APOSE-DSP). Design: Prospective study. Study sample: Patients (n = 23) with otosclerosis undergoing stapedotomy. Results: An air-bone gap of <20 dB was achieved in 100% of the patients. Three weeks postoperatively, 48% of the patients reported measured PTE-DSP and 39% of the patients experienced APOSE-DSP. The PTE-DSP significantly decreased during the 1-year follow-up period (p = 0.03). Postoperatively, APOSE-DSP was associated with a smaller benefit (improvement in air conduction; p = 0.03), yet, a lower bone conduction pure-tone average was associated with PTE-DSP (p = 0.006). Conclusions: DSP after stapedotomy is associated with a smaller benefit 3 months after stapedotomy. DSP commonly occur after stapedotomy, but decrease over time. This is important information to be included in patient counselling before stapedotomy. [ABSTRACT FROM AUTHOR]

Stapedius muscle even though being the smallest skeletal muscle in human body, it has a major role in otology. As many of the distinguished books in otology missed to explain much about stapedius muscle, and also considering the need for the anatomy based visit to this small muscle we felt it was important to have a exercise like this. In the dissection hall of our institution we dissected 32 cadaveric temporal bones and delineated stapedius muscle as a part of PG teaching programme to have a clear idea of the anatomy of stapedius muscle, its origin, attachment, extension, size (all dimensions). Length of the stapedius muscle varied between 9 and 11 mm. Stapedial tendon measured about 2 mm. The muscle had a classical sickle shape with tendon looking like the handle of the sickle. It has a bulky belly with a maximum breadth of 2-3 mm. It was found to be medial to midportion of vertical limb of facial nerve. All of our temporal bones measured size varied from 9 to 11 mm in length excluding stapedial tendon. Stapedial tendon measured almost 2 mm. Muscle is classical sickle shaped with tendon acting like the handle of the sickle. It has a bulky tummy which forms the maximum breadth of 1-3 mm. Why to have a clear idea about the anatomy of stapedius muscle is that, unless the anatomy is clear there is chance of confusing the muscle with that of facial nerve while doing facial nerve grafting and also while drilling for facial nerve decompression in experienced hands may get confused and decompress the muscle. Stapedius muscle said to be the smallest muscle in the body, but its not as small as its been described. Detailed awareness of the anatomy of stapedius muscle is needed so as to avoid confusion while facial nerve grafting and while drilling. [ABSTRACT FROM AUTHOR]

Unilateral anterior crossbite (UAC) has been demonstrated to cause masseter hyperactivity via the periodontal trigeminal mesencephalic nucleus (Vme)–trigeminal motor nucleus circuit. Here, we studied activation of motor neurons of the facial nucleus (VII), hypoglossal nucleus (XII), nucleus ambiguus (Amb), and spinal nucleus of the accessory nerve (SNA) in rats with UAC via their similar connections with Vme. An anterograde tracer, biotinylated dextran amine (BDA), was injected into the Vme to identify the central axon terminals around the motor neurons of VII, XII, Amb, and SNA. The expression of vesicular glutamate transporter 1 (VGLUT1) in neurons of VII, XII, Amb, and SNA, and the expression of acetylcholinesterase (AChE) were measured in the stapedius, lingualis, palatopharyngeal, and sternocleidomastoid muscles. In BDA‐treated rats, many BDA‐labeled cell bodies in the Vme and terminals in VII, XII, Amb, and SNA were identified. Compared with control rats, rats with UAC showed higher expression of VGLUT1 in these nuclei, and statistically significantly higher expression of AChE in the stapedius, lingualis, and sternocleidomastoid muscles, but not in the palatopharyngeal muscle. These findings suggest that UAC activates orofacial, head, and cervical multimotor behaviors via connections between the Vme and the corresponding motor nuclei. [ABSTRACT FROM AUTHOR]

Objective: This prospective study involved a longitudinal analysis of the progression of hearing thresholds in patients with osteogenesis imperfecta. Methods: Audiometric results from 36 osteogenesis imperfecta patients (age range, 6–79 years) were compared between two test times with an average interval of 4 years. Audiometric evaluation included acoustic admittance measurements, acoustic stapedial reflex measurements, pure tone audiometry and otoacoustic emissions testing. Results: Air conduction pure tone average, corrected for sex and age, and bone conduction pure tone average increased significantly in the study population (p < 0.05 and p < 0.001, respectively). In 14.3 per cent of the evaluated ears, an alteration in type and/or severity of hearing loss was observed. Conclusion: After an average time interval of four years, significant changes in hearing status occurred in a population of osteogenesis imperfecta patients. These findings highlight the importance of regular audiological follow up in osteogenesis imperfecta patients, including audiometry, and measurements of acoustic admittance, acoustic stapedial reflexes and otoacoustic emissions. [ABSTRACT FROM AUTHOR]

Background: Totally endoscopic ear surgery and endoscope-assisted microsurgery are still new concepts, with relatively few centres in the UK performing them. Advantages include better visualisation of difficult to reach areas, such as the sinus tympani, and limited external incisions. This paper reports our short-term outcomes for endoscopic middle-ear surgery. Methods: A prospective review was conducted of the first 103 consecutive patients undergoing totally endoscopic ear surgery or endoscope-assisted microsurgery in 1 centre performed by 1 operator. The outcomes assessed were: tympanic membrane healing, audiological data and complications. Results: Twenty-five patients underwent endoscope-assisted microsurgery, while 78 had totally endoscopic ear surgery. There were no reported cases of dead ear or permanent facial nerve palsy. The average air–bone gap following stapedectomy was 7.38 dB. The tympanic membrane healing rate was 89 per cent. Conclusion: Our results confirm that endoscopic middle-ear surgery is safe, and the short-term outcomes are comparable with conventional surgery. [ABSTRACT FROM AUTHOR]

Objective measures, such as the electrically evoked stapedial reflex threshold (ESRT), are critical components of optimal cochlear implant (CI) programming. Recent data suggest, however, that ESRT measurements are not commonly used by audiologists when programming CIs (Hemmingson & Messersmith, 2017). Potential reasons for limited use of these measures may be limited knowledge of the procedure for completing the measures and limited knowledge of how to apply results to programming. Clinically, ESRT is observed as a change in the static admittance of the ear canal in response to electrical stimulation through the CI; this deflection can be observed using the decay setting on an immitance bridge. ESRT is defined as the lowest amount of electrical stimulation that results in a 0.02 deflection from baseline (Gelfand, 2002). ESRT has been found to align closely to behaviorally measured upper comfort levels (e.g., C/M levels in the manufacturer software) and, as such, can assist in determining appropriate C/M levels during programming (Lira de Andrade et al., 2014; Stephan & Welzl-Müller, 2000). The purpose of this article is to describe a procedure for completing and applying ESRT measures, an objective measure that correlates to C/M levels, to CI programming. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: The electrically evoked stapedial reflex threshold (ESRT) has been shown to be a good predictor of upper stimulation level for cochlear implant recipients. Previous research has shown that the ESRT may be recorded at lower stimulation levels and with a higher incidence of success with the use of higher frequency probe tones (e.g., 678 and 1000 Hz) relative to the use of the conventional 226-Hz probe tone. Research has also shown that the acoustic reflex may be recorded at lower stimulus levels with the use of wideband reflectance when compared to the acoustic reflex threshold recorded with a conventional acoustic immittance measurement. Purpose: The objective of this study was to compare the ESRT recorded with acoustic immittance and wideband reflectance measurements. Research Design: A repeated measures design was used to evaluate potential differences in ESRTs with stimulation at an apical, middle, and basal electrode contact with the use of two different techniques, acoustic immittance measurement and wideband reflectance. Study Sample: Twelve users of Cochlear Nucleus cochlear implants were included in the study. Data Collection and Analysis: Participants' ESRTs were evaluated in response to simulation at three different electrode contact sites (i.e., an apical, middle, and basal electrode contact) with the use of two different middle ear measurement techniques, acoustic immittance with the use of a 226-Hz probe tone and wideband reflectance with the use of a chirp stimulus. Results: The mean ESRT recorded with wideband reflectance measurement was significantly lower when compared to the ESRT recorded with acoustic immittance. For one participant, the ESRT was not recorded with acoustic immittance before reaching the participant's loudness discomfort threshold, but it was successfully recorded with the use of wideband reflectance Conclusions: The ESRT may potentially be recorded at lower presentation levels with the use of wideband reflectance measures relative to the use of acoustic immittance with a 226-Hz probe tone. This may allow for the ESRT to be obtained at levels that are more comfortable for the cochlear implant recipient, which may also allow for a higher incidence in the successful recording of the ESRT. [ABSTRACT FROM AUTHOR]

Background: One of the most important steps for good user performance with a cochlear implant (CI) is activation and programming, aimed at determining the dynamic range. In adults, current levels are determined by psychophysical measures. In babies, small children, or individuals with multiple disorders, this procedure requires techniques that may provide inconsistent responses because of auditory inexperience or the age of the child, making it a very difficult process that demands the collaboration of both the patient and the family. Purpose: To study the relationship between the electrically evoked stapedius reflex threshold (ESRT) and maximum comfort level for stimulating electrodes (C-level) in postoperative CI users. Research Design: Cross-sectional analytical observational case series study. Study Sample: We assessed 24 patients of both sexes, aged between 18 and 68 yr, submitted to CI surgery. Intervention: Otoscopy and immittance. Next, an implant speech processor connected to an Itautec® computer containing the manufacturer's software (custom sound Ep 3-2) was used, as well as an AT 235h probe inserted into the ear contralateral to the CI to capture the stapedius reflex, obtaining electrically evoked stapedius reflex thresholds. Data Collection and Analysis: Data from the last programming, defining C-levels for each electrode studied, were extracted from the databank of each patient. The manual decay function of the AT 235h middle ear analyzer was used to observe ESRT response in a same window for a longer response capture time. Electrodes 22, 16, 11, 6, and 1 were tested when active, with the aim of using electrodes over the entire length of the CI, and ESRT was considered present when compliance was ≥0.05 ml. Stimuli, in current units, were always initiated at 20 cu above the C-level. The analysis of variance parametric test, Tukey's honest significant difference test, the t-test, Wilcoxon nonparametric test, and the Kolmogorov-Smirnov test examined whether significant relationships existed between these other factors. Results: The results demonstrate that all the electrodes selected for the study exhibited higher mean reflex threshold values than their mean C-level counterparts. However, there was no significant difference between them, for electrodes 1, 6, 11, and 16. The data provided allow the use of ESRT to define C-level values and make it possible to stipulate a correction factor ranging between 6 and 25.6 electrical units. Conclusion: The use of electrically evoked stapedius reflex thresholds can help the team in charge of programming CIs, making the process faster and safer, mainly for infants, small children, or individuals with multiple disorders. [ABSTRACT FROM AUTHOR]

The aim of this study was to investigate the possible role of cochlear outer hair cell function with TEOAE and DPOAE tests in patients with normal hearing and tinnitus. 25 tinnitus patients with normal hearing sensitivity selected as study group. Control group consist of 50 normal hearing subjects without tinnitus. All subjects had thresholds below 25 dBHL at frequencies 250–8,000 Hz, tympanogram type A and normal acoustic reflex thresholds. TEOAE were recorded with click stimulus at 80 dB SPL at 1,000, 2,000, 3,000 and 4000 Hz. DPOAE were measured at frequencies 1,000–8,000 Hz and intensity of L1 55 dB SPL and L2 65 dB SPL. Amplitude of DPOAE and TEOAE were decreased in all frequencies in study group. There was significant difference regarding prevalence abnormal TEOAE and DPOAE between study group and control group. There was relationship between dysfunction of outer hair cells and tinnitus in subjects with normal hearing. [ABSTRACT FROM AUTHOR]

Background: Chronic suppurative otitis media (CSOM) is a rampant clinical entity in India, and hearing loss is a very debilitating condition which is treatable. Hence, ossiculoplasty is a frequently combined operation with myringoplasty to reconstruct the hearing apparatus of a CSOM patient. Ossiculoplasty can be accomplished through autograft, homograft, and allograft materials. The glass ionomer cement (GIC) included under bone cement is biocompatible and easily available, for restoring the ossicular continuity, more specifically, the incudostapedial joint. Objective: The purpose of this study is to evaluate hearing improvement after repair of ossicular discontinuity between the incus and stapes with GIC. Materials and Methods: This prospective clinical study was conducted in a tertiary care hospital. CSOM patients found to have incudostapedial joint discontinuity intraoperative underwent ossiculoplasty with GIC. Postoperatively, hearing was evaluated at 1 month and 3 months. Audiometric pure-tone threshold by air conduction (AC) was recorded at 0.5, 1, 2, 3, 4, 6, and 8 kHz and by bone conduction at 0.5, 1, 2, 3, and 4 kHz. As per the AAO-CHE guidelines, thresholds at 0.5, 1, 2, and 3 kHz were used to calculate the Pure Tone Average (PTA). The air-bone gap (ABG) was calculated for each patient and the results were tabulated. Results: At the end of each predesignated PTA evaluation, postoperatively, the patients showed improvement in AC thresholds and narrowing of ABG, which was statistically significant. In our study, after applying GIC, the mean AC was 30.89 dB at the end of 3rd month which was significant improvement. Eighteen patients (66.67%) had closure of ABG <20 dB. Interpretation and Conclusion: The current study reveals that tympano-ossicular reconstruction using GIC is a simple, cost-effective method which gives definite good postoperative hearing improvement. Other advantages are more physiological continuity between the incus and stapes, technically easier application, nonextrusion. [ABSTRACT FROM AUTHOR]

Counter, S. Allen, Leo H. Buchanan, Fernando Ortega, Anthony B. Jacobs, and Göran Laurell. Assessment of the brainstem-mediated stapedius muscle reflex in Andean children living at high altitudes. High Alt Med Biol. 18:37-45, 2017.-This study examined the physiological thresholds, amplitude growth, and contraction duration of the acoustic stapedius reflex (ASR) in Andean children aged 2-17 years living at altitudes of 2850 m (Altitude I Group) and 3973 m (Altitude II Group) as part of a general medical assessment of the health status of the children. The brainstem-mediated ASR reveals the integrity of the neuronal components of the auditory reflex arc, including the cochlea receptors, eight cranial nerves, and brainstem neural projections to the cochlear nuclei, bilateral superior olivary nuclei, facial nerve nuclei, and facial nerve and its stapedius branch. Uncrossed (ipsilateral) and crossed (contralateral) ASR thresholds (ASRT), ASR amplitude growth (ASRG) function, and ASR muscle contraction duration (decay/fatigue) (ASRD) were measured noninvasively with 500, 1000 Hz and broadband (bandwidth = 125-4000 Hz) noise stimulus activators using a middle ear immittance system. Oxygen saturation (SaO2) level and heart rate were measured in a subsample of the study group. Statistical analyses revealed that the Altitude I and Altitude II groups had ASRT, ASRG function, and ASRD rates comparable to children at sea level and that the two groups were not significantly different for any of the ASR measures. No significant association was found between SaO2 or heart rate and ASRT, growth, and muscle fatigue rate. In conclusion, the assessment of the ASR in children in the high-altitude groups revealed normal function. Furthermore, the results indicate no adverse oto-physiological effects of altitude on the brainstem-mediated ASR at elevations between 2850 and 4000 m and suggest normal middle ear and auditory brainstem function. [ABSTRACT FROM AUTHOR]

When Capacitive Micromachined Ultrasonic Transducers (CMUTs) are coupled with water, they show high front-face acoustic reflectivity, due to the impedance mismatch between the transducer substrate material, typically based on silicon, and the propagation medium. During pulse-echo operation, surface reflectivity is responsible for multiple reflections of the received acoustic signals, which result in a set of unwanted echoes. In ultrasound imaging applications, this signal reverberation creates artifacts and reduces the image contrast. In this paper, a method to reduce front-face reflectivity is proposed, and a Reverberation Level (RL) index is introduced in order to quantify the unwanted reverberation of the signal returned to the transducer surface. The proposed method combines the increase of the bias voltage, the application of an optimized resistive load and the addition of a low-impedance acoustic backing to CMUTs realized by Reverse Fabrication Process (RFP). In this way, the mechanical energy conversion and transmission to the backing, as well as the electrical energy dissipation, are improved, thus reducing the energy reflection into the medium. The proposed method is analyzed by means of Finite Element simulations and is experimentally validated by characterizing single-element RFP-CMUTs, provided with different backing materials and electrical loads. In the analyzed prototypes, a RL reduction of 8.6 dB is obtained. [ABSTRACT FROM AUTHOR]

• Tensor tympani and stapedius muscles were pulled in human cadaver temporal bones. • Muscle pulls reduced low-frequency middle-ear sound transmission and group delay. • Tensor tympani pull changed the tympanic membrane shape and umbo position. • Modulations of middle-ear delay by the muscles could aid sound localization. • Middle-ear muscles could help the brain align head-centered auditory and ocular-centered visual representations of the environment. The three-bone flexible ossicular chain in mammals may allow independent alterations of middle-ear (ME) sound transmission via its two attached muscles, for both acoustic and non-acoustic stimuli. The tensor tympani (TT) muscle, which has its insertion on the malleus neck, is thought to increase tension of the tympanic membrane (TM). The stapedius (St) muscle, which has its insertion on the stapes posterior crus, is known to stiffen the stapes annular ligament. We produced ME changes in human cadaveric temporal bones by statically pulling on the TT and St muscles. The 3D static TM shape and sound-induced umbo motions from 20 Hz to 10 kHz were measured with optical coherence tomography (OCT); stapes motion was measured using laser-Doppler vibrometry (LDV). TT pulls made the TM shape more conical and moved the umbo medially, while St pulls moved the umbo laterally. In response to sound below about 1 kHz, stapes-velocity magnitudes generally decreased by about 10 dB due to TT pulls and 5 dB due to St pulls. In the 250 to 500 Hz region, the group delay calculated from stapes-velocity phase showed a decrease in transmission delay of about 150 µs by TT pulls and 60 µs by St pulls. Our interpretation of these results is that ME-muscle activity may provide a way of mechanically changing interaural time- and level-difference cues. These effects could help the brain align head-centered auditory and ocular-centered visual representations of the environment. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

This study aims to compare the outcomes of patients with Meniere's disease submitted to either endolymphatic mastoid shunt (ES) or tenotomy of the stapedius and tensor tympani muscles (TSTM). This is a retrospective chart review of patients treated with ES or TSTM between 2000 and 2010 and followed up for at least 12 months. The main outcomes were represented by: (1) vertigo class, hearing stage and functional level according to the American Academy of Otolaryngology-Head and Neck Surgery criteria; (2) adjustment of dizziness handicap inventory (DHI) and (3) complete and substantial vertigo control using the Kaplan-Meier survival method. Sixty-three patients met the inclusion criteria: 34 underwent ES and 29 TSTM. The baseline demographic characteristics, the hearing stage, the functional level, the DHI and hearing levels were not different between the two groups. No significant difference in vertigo class was demonstrated: 66 % of TSTM patients attained class A compared to 44 % in the ES group ( p = 0.14). Kaplan-Meier survival curves specific to class A showed significant differences, favoring TSTM (log-rank test, p = 0.022). TSTM patients demonstrated significantly improved functional level ( p = 0.0004) and improved DHI scores ( p = 0.001). Eight ES patients (25 %) demanded a second surgical attempt compared to none in the TSTM. Aural fullness was significantly improved in TSTM group ( p = 0.01), while the difference in tinnitus improvement was non-significant. Hearing preservation was significantly better in TSTM group ( p = 0.001). TSTM is a safe surgical procedure, with significant vertigo control rates, and important hearing preservation rates. More patients and longer follow-up are needed to support our preliminary findings. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Key Clinical Message We assessed the value of stapedius reflex testing as an objective measurement of striated muscle function in four patients with Pompe disease. Common tests of disease severity and efficacy of enzyme replacement therapy ( ERT) yield highly variable results that depend on patient cooperation and day-to-day performance, whereas stapedius reflex thresholds improved in seven of eight ears after 2 years of ERT. [ABSTRACT FROM AUTHOR]