Cochlear Implantation: Long-Term Effect of Early Activation on Electrode Impedance.

Objectives: The growing adoption of cochlear implants (CIs) necessitates understanding the factors influencing long-term performance and improved outcomes. This work investigated the long-term effect of early activation of CIs on electrode impedance in a large sample of CI users at different time points. Methods: A retrospective study on 915 ears from CI patients who were implanted between 2015 and 2020. According to their CI audio processor activation time, the patients were categorized into early activation (activated 1 day after surgery, n = 481) and classical activation (activated 4 weeks after surgery, n = 434) groups. Then, the impact of the activation times on the electrode impedance values, along the electrode array contacts, at different time points up to two years was studied and analyzed. Results: The early activation group demonstrated lower impedance values across all the electrode array sections compared to the classical activation at 1 month, 1 year, and 2 years post-implantation. At 1 month, early activation was associated with a reduction of 0.34 kΩ, 0.46 kΩ, and 0.37 kΩ in the apical, middle, and basal sections, respectively. These differences persisted at subsequent intervals. Conclusions: Early activation leads to sustained reductions in the electrode impedance compared to classical activation (CA), suggesting that earlier activation might positively affect long-term CI outcomes.

Efficacy of vibrant sound bridge in congenital aural atresia: an updated systematic review.

PURPOSE: The indications of Vibrant Soundbridge (VSB) have been expanded to include patients with conductive and mixed hearing loss due to congenital aural atresia (CAA). However, the current evidence supporting the auditory outcomes of VSB is based mainly on case reports and retrospective chart reviews. Therefore, the present systematic review aims to summarize and critically appraise the current evidence regarding the safety and effectiveness of VSB in children and adult patients with CAA. METHODS: A systematic literature search retrieved studies that evaluated the outcomes of unilateral or bilateral implantation of VSB in patients with CAA. The bibliographic search was conducted in PubMed, Scopus, EBSCO, and Cochrane Central Register of Controlled Trials (CENTRAL) databases from January 2000 to December 2022. RESULTS: Twenty-seven studies were included in the present systematic review. Overall, the speech perception after VSB was good, with a mean word recognition score (WRS) score ranging from 60 to 96.7%. The mean postoperative speech recognition threshold (SRT) after implantation ranged from 20.8 to 50 dB. The effective gain was reported in 15 studies, ranging from 31.3 to 45.5 dB. In terms of user satisfaction with VSB, the included studies showed significant improvements in the patient-reported outcomes, such as the Speech Spatial and Qualities of Hearing scale and Glasgow Hearing Aid Benefit Profile. The VSB implantation was generally safe with low incidence of postoperative complications. CONCLUSION: VSB provides significant benefits to individuals with hearing loss owing to CAA, with very good subjective outcomes and a low risk of complications.

A novel coupling quality index to estimate the coupling efficiency in Vibrant Soundbridge.

BACKGROUND: The objective-based methods for intraoperative monitoring have been suggested to assess the coupling and the outcomes of Vibrant Soundbridge (VSB). Although several techniques were proposed, they have not been widely adopted due to their complexity and invasiveness. PURPOSE: This study aimed to investigate the accuracy of a new coupling quality index using an intraoperative ABR threshold via AcoustiAP and its correlation with the perioperative measures. METHODS: This is a prospective study conducted at a tertiary center. The medical records were retrieved for all patients who underwent VSB implantation and had an intraoperative objective assessment for the coupling efficiency. AcoustiAP was used to evaluate the intraoperative ABR thresholds, which were assessed directly after the floating mass transducer (FMT) placement using acoustic CE-Chirp signals. The Vibrogram was used for the postoperative audiological evaluation. A new coupling quality index was calculated based on the intraoperative ABR thresholds. RESULTS: Ten patients were eligible for the present study. The ABR thresholds for good coupling ranged from 35 to 60 dBnHL. The loose coupling thresholds ranged considerably from 40 to 100 dBnHL. Overall, the median intraoperative ABR threshold at good coupling was 42.5 (40-60) dBnHL and 60 (40-100) dBnHL at loose coupling. The analysis showed that there was a significant change in the coupling quality index at the good and loose coupling points (24.3 ± 14 vs 38.8 ± 18.2, respectively, p < 0.001). At a cut-off value of 22.6 dB, the coupling quality index had a sensitivity of 70%  and specificity of 90% for discriminating good and loose coupling. CONCLUSION: This study provides evidence for the utility of intraoperative ABR measurements in predicting the coupling efficiency in patients with VSB. Our results showed that the coupling quality index had an acceptable accuracy in discriminating between good and poor coupling, which can help clinicians optimize the fitting process for individuals and may ultimately lead to improved patient outcomes.

Risk factors and management strategies of inadvertent facial nerve stimulation in cochlear implant recipients: A systematic review.

Objectives: To systematically review the prevalence and risk factors of inadvertent facial nerve stimulation (FNS) after cochlear implant (CI) surgery. And to report the different management strategies used for reducing and resolving FNS. Data Source: Web of Science, Scopus, PubMed, Cochrane Library, and Virtual Health Library (VHL) of the World Health Organization (WHO). Review Methods: A systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) on studies that reported FNS as a complication after CI. A comprehensive electronic search strategy was used to identify the relevant articles. We extracted the data on the prevalence of FNS after CI activation, the reported grades, and the management strategies. The number of associated electrodes; cause of deafness; co-anomalies; and duration of hearing loss and their relationships with FNS were also studied. Results: Twenty-one relevant articles were included in this review. The prevalence of FNS among the CI populations was 5.29% (175/3306 patients). Among those whose ages were reported, 58.3% (95/163) were adults, and 41.7% (68/163) were pediatrics. Modifying the different fitting parameters was the most used strategy, as it successfully resolved FNS in 85.5% of the patients (142/166). The second commonly used management strategy was surgical intervention (reimplantation or explantation), which was reported in seven studies for 23 patients. Conclusion: FNS after CI activation could be controlled and resolved with many advances that range from readjusting the fitting parameters to surgical intervention. However, further studies are required to validate the efficacy of each management strategy and its impact on patients' performance. Our findings demonstrate that CI recipients with FNS could still benefit from the CI devices and their FNS could be controlled.

Can Electrocochleography Help Preserve Hearing After Cochlear Implantation With Full Electrode Insertion?

OBJECTIVES: To evaluate the utility of intracochlear electrocochleography (ECochG) monitoring during cochlear implant (CI) surgery on postoperative hearing preservation. STUDY DESIGN: Prospective, randomized clinical trial. SETTING: Ten high-volume, tertiary care CI centers. PATIENTS: Adult patients with sensorineural hearing loss meeting the CI criteria who selected an Advanced Bionics CI. METHODS: Patients were randomized to CI surgery either with audible ECochG monitoring available to the surgeon during electrode insertion or without ECochG monitoring. Hearing preservation was determined by comparing preoperative unaided low-frequency (125-, 250-, and 500-Hz) pure-tone average (LF-PTA) to postoperative LF-PTA at CI activation. Pre- and post-CI computed tomography was used to determine electrode scalar location and electrode translocation. RESULTS: Eighty-five adult CI candidates were enrolled. The mean (standard deviation [SD]) unaided preoperative LF-PTA across the sample was 54 (17) dB HL. For the whole sample, hearing preservation was "good" (i.e., LF-PTA change 0-15 dB) in 34.5%, "fair" (i.e., LF-PTA change >15-29 dB) in 22.5%, and "poor" (i.e., LF-PTA change ≥30 dB) in 43%. For patients randomized to ECochG "on," mean (SD) LF-PTA change was 27 (20) dB compared with 27 (23) dB for patients randomized to ECochG "off" ( p = 0.89). Seven percent of patients, all of whom were randomized to ECochG off, showed electrode translocation from the scala tympani into the scala vestibuli. CONCLUSIONS: Although intracochlear ECochG during CI surgery has important prognostic utility, our data did not show significantly better hearing preservation in patients randomized to ECochG "on" compared with ECochG "off."

Osseointegrated device placement with minimally invasive surgery: Experience and audiological outcome.

OBJECTIVES: To evaluate the clinical and audiological outcomes of percutaneous bone conduction device placement by minimally invasive Ponto surgery (MIPS). METHODS: This was a retrospective descriptive study of patients who underwent MIPS from March-November 2019 at King Abdullah Ear Specialist Center, Collage of Medicine, King Saud University, Riyadh, Saudi Arabia. We reviewed all the clinical data of patients, including preoperative data, postoperative surgical results, and audiological performance (aided and unaided pure tone audiometry and aided and unaided speech tests). RESULTS: A total of 9 patients with 10 implants were enrolled in this study. One patient underwent revision surgery because of infection and loss of the abutment. We followed the patients from 1-2 years, with a mean of 16.8 months. A significant difference was found between the unaided air conduction pure tone average, with a mean of 72.6±28.4 decibel (dB), and the postoperative aided threshold, with a mean of 20.8±12.2 dB/hectoliter (p=0.008), indicating a functional gain of 51.8 dB. The mean unaided speech discrimination at 65 dB sound pressure level was 34.7±24.8, which was significantly improved to 88.4±11.7 after implantation (p=0.007). CONCLUSION: minimally invasive Ponto surgery is a suitable minimally invasive surgical method for bone-anchored implant placement. This technique has an advantage in terms of skin sensitivity, cosmetic outcomes, and operative duration.

Mucosal melanocytic lesion in the middle ear extending to the inner ear and nasopharynx.

Benign dendritic melanocytic proliferation is usually observed in the skin. We report an extremely rare case of a melanocytic lesion in the middle ear mucosa. Only 3 cases of melanocytic lesions in the middle ear have been reported. Our report is the only one that describes an adult with a melanocytic lesion in the middle ear that extended to the inner ear and nasopharyngeal mucosa. A 23-year-old female presented with profound sensory neural hearing loss and recurrent discharge from the right ear. Examination revealed a blue right tympanic membrane with a small perforation. Computed tomography scans and magnetic resonance imaging were performed. After surgical exploration and histopathological examination, the patient was found to have a benign melanocytic lesion in the right middle ear. Melanocytic lesions are commonly found in the skin. There are no previous reports have described adult patients with melanocytic lesions in the middle ear that extend to the inner ear and nasopharynx, which cause profound hearing loss with recurrent ear discharge. These patients require regular follow-up to assess the progression of the lesion and to watch for any malignant behavior.

Cochlear implantation versus auditory brainstem implantation in children with auditory nerve deficiencies.

BACKGROUND: Cochlear nerve deficiency is one of the known causes of congenital sensorineural hearing loss. Management of hearing loss in children with cochlear nerve deficiency poses a multidimensional challenge. The absent or hypoplastic cochlear nerve may prevent electrical stimulation from reaching the brainstem and the auditory cortex. A deficient cochlear nerve can be associated with other inner ear malformations, which may diminish the success of cochlear implantation in those children. Promising results in adults after auditory brainstem implantation led to the expansion of candidacy to include the pediatric populations who were contraindicated for CIs. OBJECTIVE: To review the outcomes of cochlear implantation versus that of auditory brainstem implantation in children with various conditions of the auditory nerve. METHODS: This retrospective chart review study comprised two pediatric groups. The first group consisted of seven ABI recipients with cochlear nerve aplasia and the second group consisted of another seven children with cochlear nerve deficiencies who underwent CI surgery. The participants' auditory skills and speech outcomes were assessed using different tests selected from the Evaluation of Auditory Responses to Speech (EARS) test battery. RESULTS: There were some individual variations in outcomes depending on the status of the auditory nerve. The mean CAP score of the ABI group was 2.87, while the mean SIR score was 0.62. On the other hand, the mean CAP score of the CI group was 1.29, while the mean SIR score was 0.42. CONCLUSION: Our results are in good agreement with the reported auditory perception and speech and language development outcomes of pediatric auditory brainstem implantation. We added to the growing body of literature on the importance of verifying and identifying the status of the cochlear nerve in the decision-making process of the surgical management of those pediatric groups.

Cochlear implantation in common cavity deformity: a systematic review.

PURPOSE: Cochlear implantation became a valid hearing rehabilitation option in common cavity deformity. This study aimed to assess the audiological and speech outcomes of cochlear implantation in common cavity deformity patients and to address the surgical aspect used in this population. METHODS: A comprehensive systematic literature review based on preferred reporting items for systematic reviews and meta-analyses (PRISMA) guideline from database inception through April 2020. Eighteen published articles including 138 patients with common cavity deformity met the inclusion criteria. These articles studied the surgical techniques and the audiological outcomes of cochlear implantation in patients with common cavity deformity in English language. RESULTS: Trans-mastoid labyrinthotomy was the common surgical approach in these patients. The average speech intelligibility rating and categories of auditory performance scores in common cavity deformity were lower than in normal cochlea subjects (p < 0.05), and non-significant (p > 0.05) compared with other types of inner ear malformations. CONCLUSION: Patients with common cavity deformity who underwent cochlear implantation showed a beneficial audiological and speech outcome. However, their performance is highly variable. Therefore, pre-operative counseling of the parents is necessary. The surgical approaches should be individualized according to clinical, radiological, and surgical findings.

Clinical profile and management of revision cochlear implant surgeries.

OBJECTIVES: To discuss our experience with managing cochlear implant cases that required revision surgery. METHODS: A retrospective case series study including data from 922 cochlear implant patients at an academic tertiary center was evaluated retrospectively. All patients who underwent revision cochlear implant (CI) surgery between January 2011 and July 2017 were included. The following data were collected: patient demographic data, details on the first implant, reasons for the revision, duration from initial implantation to revision, type of device, and management. RESULTS: Out of 922 CI patients, 37 (4%) underwent revision surgery, comprising 33 children and 4 adults. The most common reason for revision surgery, at 28/37 cases (75.6%), was device failure. Surgical and medical aetiologies were responsible for 9/37 (24.3%) revisions. The mean duration from the initial implantation to the revision surgery was 29 months. CONCLUSION: Revision CI surgery is not uncommon after initial implantation. Cochlear implant programs must implement long-term follow-up processes for CI users. Whenever a patient's rehabilitated performance regresses, the cause should be investigated to determine whether subsequent reimplantation is necessary.

Evaluation of computed tomography parameters in patients with facial nerve stimulation post-cochlear implantation.

PURPOSE: To compare the preoperative computed tomography (CT) parameters, including the thickness and density of the bone separating the upper basal turn of the cochlea (UBTC) and the labyrinthine segment of the facial nerve (LSFN), in patients with and without facial nerve stimulation (FNS) in post-cochlear implants (CI). METHODS: A retrospective case review of 1700 CI recipients in a tertiary referral center between January 2010 and January 2020 was performed; out of the 35 recipients who were found to have FNS, 29 were included in the study. The control group comprised the same number of randomly selected patients. CT parameters of the patients were measured independently by three fellowship-trained neuro-otologists blinded to the postoperative status of the patients. Thickness in axial and coronal views and density of the bone separating the UBTC and the LSFN were measured. RESULT: There was satisfactory agreement between the readings of the three reviewers. The distances (in mm) between the UBTC and LSFN obtained from the coronal (0.43 ± 0.24 vs. 0.63 ± 0.2) and axial (0.42 ± 0.25 vs. 0.6 ± 0.18) views were statistically lower in the FNS group (p = 0.001 and 0.005, respectively). The density (in HU) of the bony partition was also statistically lower in the FNS group (1038 ± 821 vs. 1409 ± 519; p = 0.029). CONCLUSION: Patients who experienced FNS postoperatively had significantly lower distance and bone density between the UBTC and the LSFN. This finding can help surgeons in preoperative planning in an attempt to decrease the occurrence of FNS.

Effect of early activation of cochlear implant on electrode impedance in pediatric population.

OBJECTIVES: To assess the evolution of electrode impedance after the early fitting of audio processors (activation after one-day) and classical fitting (activation after one-month) over an up-to-one year after cochlear implant (CI) surgery. METHODS: A retrospective cohort study on Fifty-two CI recipients divided into two groups. The study group included 24 recipients (40 ears) who underwent early fitting, whereas the control group contained 28 recipients (40 ears) who underwent classical fitting. The electrode impedance was recorded during the surgery, switch-on session and at one, three, six, and twelve-months after the surgery. RESULTS: In the study group, electrode impedance values obtained intraoperatively and at switch-on and one, three, six, and twelve-months were 4.89, 3.69, 6.52, 6.24, 6.05, and 5.81 KΩ, respectively, and only the switch-on and one-month values were significantly different (p < 0.0001). In the control group, electrode impedance values obtained intraoperatively and at switch-on and one, three, six, and twelve-months were 4.71, 7.19, 6.40, 6.05, and 5.73 KΩ, respectively. Thus, the electrode impedance value at switch-on was 52.65% (p < 0.001) greater than it intraoperatively. For both groups, the electrode impedance value at twelve-months was significantly higher than the respective intraoperative values (study group: 18.6% higher, P = 0.04; control group: 21.65% higher, P = 0.0001). CONCLUSION: Electrode impedance was significantly lower in the study group compared to the control group until one month after the surgery. However, the electrode impedance at twelve-months after the CI was similar in both groups.

Middle Ear Implant in a Patient With Fibrous Dysplasia: An Alternative for Hearing Restoration.

We propose middle ear implants (MEI) as alternative hearing restoration option for patients with fibrous dysplasia of the temporal bone (FDTB). A 60-year-old man presented with unilateral right-sided conductive hearing loss (CHL) and FDTB. The Vibrant Soundbridge (VSB) MEI was implanted in the right ear. Preoperatively, the right side had a pure tone average (PTA) threshold of 67 dB, speech reception threshold (SRT) = 75 dB HL, and speech discrimination score (SDS) = 54% at 80 dB HL presentation level. Postoperative aided PTA threshold of the right ear was 28 db, aided SRT = 30 db, and SDS = 96% at 65dB HL. After excluding cholesteatoma, VSB provides an alternative hearing restoration technique for adults with FDTB and CHL due to stenosis of the external auditory canal and/or ossicular crowding.

Performance of cochlear implant recipients fitted with triphasic pulse patterns.

PURPOSE: To assess the effect of triphasic pulse pattern stimulation strategy on the audiological performance of cochlear implant recipients with unintended facial nerve stimulation (FNS), and to compare the audiological and speech outcomes before and after switching to triphasic stimulation. METHODS: A retrospective study of patients who have changed their fitting maps from biphasic to triphasic pulse pattern stimulation because of FNS after cochlear implantation (CI). All identified patients with FNS after CI from 2017 to 2019 were included in this study. The medical records of 11 patients (16 ears) were queried for demographic and radiological data, pure tone audiometry, speech reception thresholds, speech discrimination score at 65 dB, maximum comfortable levels, thresholds, and dynamic range. Then, these parameters were compared in the two conditions, biphasic and triphasic. RESULTS: Using triphasic pulse stimulation only or combined with switch-off of few channels, complete resolution of FNS was achieved. Triphasic pulse pattern stimulation was associated with better speech discrimination scores (75.25 ± 26.13%) compared to the biphasic pulse (58.25 ± 26.13%). This triphasic strategy also showed higher maximum comfortable levels (36.62 ± 1.63 qu) than biphasic strategy (31.58 ± 2.5 qu). Moreover, the dynamic range was wider using triphasic pulse strategy. In general, the triphasic pulse pattern resulted in successful suppression of facial nerve stimulation with suitable maximum comfortable levels and better speech discrimination. CONCLUSION: Triphasic pulse pattern stimulation is an appropriate tool in controlling FNS following cochlear implantation with wider dynamic range. We recommend that all patients with facial nerve stimulation after CI surgery be switched to a triphasic pulse program prior to considering further surgery.

Cochlear Implantation in Children with Otitis Media.

One of the concerns during the cochlear implant candidacy process is the presence of chronic otitis media which could delay the implantation process. The aim of this study was to evaluate the surgical difficulties and the long-term complications in children with otitis media and to examine whether it is necessary to delay the implantation until the infection is resolved. The study used a comparative retrospective design based on chart review of all patients who received their implant(s) from January to December of 2012. A total of 200 patients were identified and were followed for 4 years post surgery. Patients were divided into three groups based on their history of otitis media (non-otitis media, chronic otitis media with effusion, and acute otitis media). Data included long-term complications, operative time and duration from first clinical visit to the time until implantation was received. None of study participants had long-term complications during the study period. The operative time was longer in the acute otitis media group with a difference of 45 min. The average delay in cochlear implantation due to the presence of otitis media in chronic group was more than 5 months. Pediatric patients with otitis media could be implanted in one stage safely and effectively.

Complications of post-cochlear implantation in 1027 adults and children.

BACKGROUND: The number of cochlear implant (CI) recipients is increasing so there needs to be greater awareness of possible CI complications. OBJECTIVES: Describe complications of CI. DESIGN: Retrospective, descriptional. SETTINGS: Tertiary health care center. PATIENTS AND METHODS: Data was collected by retrospective chart review on complications for recipients implanted for the period from January 2006 to June 2017. MAIN OUTCOME MEASURES: Major and minor post cochlear implantation complications. SAMPLE SIZE: 1027 cochlear implant recipients. RESULTS: Post-operative complications were reported in 105 patients (10.2%). Minor complications were most often encountered (9.5%). Swelling (wound seroma or hematoma) was the most common complication. The major complication rate was only 7/105, 0.7%. Meningitis did not occur and cholesteatoma occurred in only one patient. CONCLUSION: CI is a safe surgical procedure at our center. We believe that this is probably due to the preoperative protocol, the surgical technique used, and the postoperative care conducted for all our patients. Despite this, it is important that both physicians and patients have knowledge of the possible consequences and risks posed by CI, especially in view of the fact that these patients require lifetime follow-up. LIMITATIONS: Further studies are needed, and should include more ENT centers across all regions of Saudi Arabia. CONFLICT OF INTEREST: None.

Value of Routine Magnetic Resonance Imaging for the Preoperative Assessment of Cochlear Implant Candidates.

Background The selection of an appropriate imaging technique for assessment before cochlear implantation is critical for precise diagnosis and management. While magnetic resonance imaging (MRI) is used for the diagnosis of several conditions, such as labyrinthitis ossificans, cochlear nerve deficiency, and neoplasms, high-resolution computed tomography (HRCT) provides excellent details of the temporal bone. However, it remains unclear whether routine MRI provides any additional benefits over HRCT. Objectives To assess the added value of MRI as a screening tool for temporal bone abnormalities in cochlear implant candidates through comparisons of its findings with those of HRCT. Materials and method It is a retrospective analysis of preoperative HRCT and MR images in a tertiary referral center. A total of 308 patients who underwent MRI and HRCT examinations before cochlear implantation between 2013 and 2015 were included. Preoperative HRCT and MR images were screened for temporal bone abnormalities by a senior neurotologist and a neuroradiologist. Results HRCT detected inner ear deformities in 51 of the 308 (16.6%) subjects, whereas MRI revealed abnormalities in only 18 (5.8%) of subjects. HRCT detected the same inner abnormalities in 16 of the 18 (88.9%) subjects diagnosed by MRI, whereas it showed normal results for the remaining two subjects. MRI detected cochlear nerve aplasia/hypoplasia in 13 subjects, 11 of whom had associated inner ear deformities that were detected by HRCT. The MR images of nine subjects showed cochlear fibrosis, which was confirmed by HRCT in all nine subjects. Conclusion In this study, MRI did not exhibit significant additional benefits over HRCT, and its routine use for the preoperative assessment of CI candidates was not justified. However, MRI is warranted for subjects at an increased risk of cochlear nerve aplasia due to an inner ear deformity or a narrow internal auditory canal. The establishment of criteria that facilitate the performance of MRI only when absolutely needed will reduce healthcare costs, prevent unnecessary exposure to the risks associated with general anesthesia, and shorten delays before cochlear implantation.

Comparison of cochlear duct length between the Saudi and non-Saudi populations.

BACKGROUND: There are no data on cochlear duct lengths (CDL) among Middle East populations. OBJECTIVES: The main aims of this study were to estimate the average CDL in the Saudi population and to compare it with the reported CDL in other regions/ethnic groups outside the Middle East. DESIGN: Retrospective study. SETTING: Tertiary otolaryngology head and neck surgery center. SUBJECTS AND METHODS: Temporal bone CT scans were reviewed to determine CDL. We excluded any CT scan of an ear with a congenital inner ear anomaly or acquired pathology. MAIN OUTCOME MEASURES: CDL. SAMPLE SIZE: 441 temporal bone CT scans. RESULTS: The overall CDL mean was 31.9 mm (range 20.3-37.7 mm). The cochleae of males was significantly longer than of females and cochleae from the left side were significantly longer than of the right side. No significant difference was found between children and adults. Inter-study comparison revealed a significant difference in CDL between the Saudi population in our study and European and Australian studies, but not between the present study and North American studies. CONCLUSIONS: The CDL differed significantly according to side of the cochlea and sex, but not by age. Geographically and ethnically, the mean CDL for Saudis was significantly different from the CDL of subjects of some ethnic backgrounds, but not others. Due to this diversity, we recommend that the CDL be measured before cochlear implant surgery. LIMITATIONS: All the measurements were done by one person, and the subjects' physical measurements, such as height or head circumference, were not included. CONFLICT OF INTEREST: None.

The outcome of cochlear implantation among children with genetic syndromes.

OBJECTIVE: To assess the outcome and efficacy of cochlear implantation in children with genetic syndromes. METHOD: Study design: case-control study. SETTING: A cochlear implantation tertiary referral center. PATIENTS: All pediatric cochlear implantation recipients with Waardenburg syndrome, Usher syndrome, Dandy-Walker syndrome, or albinism. A control group was appropriately matched to the syndromic group with regard to age at implantation and duration of device use. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Subjects' auditory abilities, speech intelligibility, and pure tone thresholds were compared between the syndromic and non-syndromic group. RESULTS: A total of 25 subjects (13 syndromic and 12 non-syndromic) participated in the study. Neither auditory ability nor speech intelligibility scores differed significantly by group. The final PTA of both the groups showed normal-to-mild hearing loss: 26 dB HL in the syndromic group and 23 dB HL for the control group. CONCLUSIONS: Cochlear implant recipients with genetic syndromes achieved similar levels auditory perception and speech intelligibility as their peers with a genetic syndrome. The presence of any of the genetic syndromes described herein should not be a contraindication to cochlear implant provision, as it would have a positive impact on the patients' sensory perception and lifestyle.

Effectiveness of stapedotomy in improving hearing sensitivity for 53 otosclerotic patients: retrospective review.

BACKGROUND: Otosclerosis is a common cause of progressive hearing impairment that causes fixation of the stapes. Surgical intervention is the preferred treatment approach to ameliorate the conductive hearing loss associated with stapedial otosclerosis. However, given that it is a difficult and delicate procedure, the surgery may fail for a number of reasons. Therefore, it is very important to evaluate the success rate of the surgical approach used in each regional center. OBJECTIVE: To examine the effectiveness of stapedotomy in improving hearing sensitivity for otosclerotic patients at King Abdul Aziz University Hospital in Riyadh. DESIGN: Retrospective chart review with an analysis of pre- and postoperative surgical treatment. SETTING: Tertiary referral otolaryngology clinic. PATIENTS AND METHODS: All cases who underwent stapedotomy between 1997 and 2009 were retrospectively reviewed. Preoperative and postoperative audiometric assessments were conducted using conventional pure tone audiometry. Differences were analyzed by two-way repeated measures ANOVA. MAIN OUTCOME MEASURE(S): Pre- and postoperative pure tone thresholds for air and bone conduction. RESULT: Fifty-three patients underwent stapedotomy. Stapedotomy yielded significant improvements in mean (SD) postoperative air-conduction thresholds of about 18.7 (11.7) dB (P < .0001) and mean (SD) post.operative bone-conduction thresholds of about 2 (7.2) dB (P < .05). Additionally, a significant correlation was found between improvement in air-conduction thresholds and the size of preoperatve air-bone gap (P < .01) About 70%of patients achieved an air-bone gap of 20 dB or better. None of the cases examined in this study exhibited sensorineural hearing loss or adverse complications following stapedotomy. CONCLUSIONS: Stapedotomy is a safe and effective treatment option for patients with otosclerosis. Given that the majority of participants in this study exhibited mixed hearing loss preoperatively, the results further suggest that stapedotomy can also be effective in improving thresholds for these patients. LIMITATIONS: The sample size was relatively small.