Endoscopic Ossiculoplasty for the Management of Isolated Congenital Ossicular Chain Malformation: Surgical Results in 16 Ears.

OBJECTIVE: The aim of this study is to describe the clinical characteristics and intraoperative findings and further evaluate the efficacy of endoscopic ossiculoplasty for the management of isolated congenital ossicular chain malformation. METHODS: A retrospective study was performed on 16 ears (15 patients) with the isolated congenital ossicular chain malformation who underwent endoscopic ossiculoplasty in our department from May 2017 to January 2019. Endoscopic exploratory tympanotomy was conducted to check the ossicular chain; at the same time, endoscopic ossiculoplasty was performed depending on intraoperative findings. Air-conduction thresholds, bone-conduction thresholds, and air-bone gaps (ABGs) were measured before and after surgery, and the hearing outcome was assessed at 6 months postoperatively. RESULTS: The most common malformations of ossicular chain were the missing of the incus long process and stapes suprastructure. A serial assessment of the hearing status was conducted before and 6 months after surgery. It showed the mean postoperative pure-tone average (PTA) was significantly reduced, and the mean postoperative ABG was obviously closed, respectively (P < .001). The mean PTA gain was 36.3 ± 8.6 dB, and the ABG closure was 35.1 ± 8.3 dB; ABG closure to 20 dB or less and ABG closure to 10 dB or less were achieved in 14 cases (87.5%) and 5 cases (31.3%), respectively. No differences were observed in postoperative hearing outcome between type Ⅲ cases and type Ⅳ cases; however, cases with partial ossicular replacement prosthesis implantation showed a larger hearing gain (P = .049) and a higher proportion of postoperative ABG less than 10 dB (P = .021). No facial palsy and significant sensorineural hearing loss occurred; all patients completed the surgery without the need of canalplasty, and the chorda tympani nerve was preserved in all patients. CONCLUSIONS: This research showed endoscopic surgery was effective in the diagnosis and management of isolated congenital ossicular chain malformation; the endoscopic ossiculoplasty provides an alternative method to manage congenital ossicular chain malformation, with comfortable hearing outcome and the advantage of excellent vision and less invasion.

Surgical management of cochlear implant in chronic otitis media patients: safe and sound.

INTRODUCTION: Cochlear implantation (CI) in cases with chronic otitis media (COM) was previously contraindicated but recent advances have made it possible. OBJECTIVE: To review surgical and audiological outcomes of COM patients that underwent CI. MATERIAL AND METHODS: Retrospective review of patients above 18 years old. RESULTS: Ten patients with complete data were included. Patients were aged 24-69 years old. Tympanoplasty and mastoidectomy were performed before CI. Imaging was performed to rule out ossifications. Eight patients underwent a standard canal wall up with either cochleostomy or round window approach. One patient had additional canalplasty and tympanoplasty and another one had blind sac procedure respectively. Analysis of the hearing aided level with CI and hearing aid showed significant benefit provided by the CI (Z = 2.803, p = .005). DISCUSSION: Creating a dry and safe ear is important prior to CI. Definite hearing improvement is seen in all our cases that helped them to become independent again in their daily life. Hearing aid usage pre-CI might not be important as the hearing aids may continue to cause discharging ears and the benefits of hearing aids in severe to profound hearing loss are very minimal. CONCLUSIONS: Cochlear implant is safe and effective in COM patients.

The learning curve for endoscopic tympanoplasties: a single-institution experience, in Birmingham, UK.

OBJECTIVE: This study aimed to assess whether increasing operative experience results in better surgical outcomes in endoscopic middle-ear surgery. METHODS: A retrospective single-institution cohort study was performed. Patients underwent endoscopic tympanoplasty between May 2013 and April 2019 performed by the senior surgeon or a trainee surgeon under direct supervision from the senior surgeon. Following data collection, statistical analysis compared success rates between early (learning curve) surgical procedures and later (experienced) tympanoplasties. RESULTS: In total, 157 patients (86 male, 71 female), with a mean age of 41.6 years, were included. The patients were followed up for an average of 43.2 weeks. The overall primary closure rate was 90.0 per cent. CONCLUSION: This study demonstrates an early learning curve for endoscopic ear surgery that improves with surgical experience. Adoption of the endoscopic technique did not impair the success rates of tympanoplasty.

Endoscopic type 1 cartilage tympanoplasty in children.

OBJECTIVES: Endoscopic type 1 tympanoplasty using cartilage grafts for repair of chronic tympanic membrane perforation is increasing. The aim of this study was to evaluate the results of endoscopic type 1 cartilage tympanoplasty in children. MATERIALS AND METHODS: Patients under 18 years of age who underwent type 1 cartilage tympanoplasty between January 2013 and February 2019 were retrospectively evaluated. Anatomic success rate was calculated according to the intact status of the graft. Air conduction (AC), bone conduction (BC), air-bone gap (ABG) and hearing gain were calculated using pure tone audiometry tests at pre-operative, and 6th month postoperative period. Patients with postoperative ABG ≤20 dB were considered as functionally successful. RESULTS: The study included 56 patients (6 bilateral) and 62 ears. The anatomic success rate of our study was 91.9% (57/62). Preoperative AC was 36.4 ± 6.5 (21-50) dB, BC was 7.8 ± 3.7 (5-25) dB, and ABG was 28.6 ± 6.9 (10-41) dB. Postoperative AC was 24.5 ± 8.8 (7-45) dB, BC was 7.6 ± 3.4 (5-19) dB, and ABG was 16.9 ± 7.4 (2-32) dB. There was no change in postoperative BC (p: 0.683), whereas AC, and ABG significantly decreased (p < 0.001). Mean hearing gain was 12.1 ± 6.2 (3-26) dB and the functional success rate was 72.5% (45/62). CONCLUSION: Endoscopic type 1 cartilage tympanoplasty, a minimally invasive surgical procedure, provided successful functional and anatomical results at the pediatric population.

A novel computed tomography guided, transcutaneous approach to treat refractory autophony in patients with a patulous Eustachian tube - a case series.

BACKGROUND: Patulous Eustachian tube is a benign but notoriously difficult condition to treat successfully. Symptoms include autophony of voice and breathing, and aural fullness. METHODS: This paper presents a series of 8 patients (12 ears) for whom a novel computed tomography guided injection of silicone elastomer suspension implant (Vox) was used to treat patulous Eustachian tube. This is the largest and only series in the current literature using this technique. RESULTS: The combined complete and partial symptom resolution rate was 91 per cent. Complications related to the procedure are described. The pros and cons of this novel approach are also discussed in relation to traditional endoscopic transnasal techniques. CONCLUSION: Computed tomography guided injection of Vox for the treatment of patulous Eustachian tube is suggested to be a feasible alternative to endoscopic transnasal approaches, particularly for refractory cases.

Prognostic Factors for Hearing Outcomes in Children with Cleft Lip and Palate.

BACKGROUND: This study was performed to investigate the prognostic factors that influence hearing outcomes of children with cleft lip and palate after ventilation tube insertion. METHODS: The authors retrospectively reviewed the hearing thresholds of 90 children with cleft lip and palate and performed univariate and multivariate analyses of five prognostic factors for hearing outcomes: age at palatoplasty, age at first ventilation tube insertion, timing of ventilation tube insertion, frequency of ventilation tube insertion, and type of cleft palate. RESULTS: On univariate analysis, the authors found that older age at palatoplasty (p = 0.002), older age at first ventilation tube insertion (p = 0.025), and increased frequency of ventilation tube insertion (p = 0.048) were significant prognostic factors for poorer hearing outcomes in children with cleft lip and palate. Multiple logistic regression analysis showed that late palatoplasty (age older than 1 year) was the most influential factor for poor hearing outcome (OR, 2.83), followed by two or more ventilation tube insertions (OR, 1.94). CONCLUSION: The age at palatoplasty and frequency of ventilation tube insertion were found to be significant prognostic factors influencing hearing outcomes in children with cleft lip and palate. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Mechanical Effects of Cochlear Implant on Acoustic Hearing.

Residual hearing loss in cochlear implant users is investigated using the mechanical-human-cochlear model. Hearing loss due to stiffening of the round window increases significantly as input frequencies decrease from 3 kHz to 1 kHz but remains constant at lower frequencies, whereas loss due to the presence of an electrode insert becomes significantly higher at lower frequencies ([Formula: see text] kHz). The latter also shifts the characteristic frequency map toward the basal end of the cochlea. In the region away from the end of the electrode insert, cochlear function recovers, but the user still suffers from hearing loss caused by round window stiffening.

Oculostapedial synkinesis following idiopathic facial palsy: something to listen out for.

OBJECTIVE: This paper presents a rare case of oculostapedial synkinesis.Case reportAfter partial resolution of an idiopathic facial palsy, a male patient presented with persistent distortion of hearing when blinking and closing his eye. Audiometry findings were unremarkable, and cross-sectional imaging of the facial nerve revealed no abnormalities apart from an incidental contralateral meningioma. Initial conservative management, with referral to a specialist physiotherapist, failed to resolve the symptoms. The patient subsequently opted for surgical intervention, and underwent a transmeatal tympanotomy and transection of the stapedial tendon. Following this, he had complete resolution of symptoms. CONCLUSION: Oculostapedial synkinesis is a rare complication of facial palsy, but is recognised in the literature. Given its unusual presentation, it can be overlooked, especially by more junior team members. This case highlights the need to pay careful attention to patients' symptoms and listen out for the description of hearing distortion on facial movement.

The Modified Rambo Transcanal Approach for Cochlear Implantation in CHARGE Syndrome.

OBJECTIVE: CHARGE syndrome is associated with a variety of temporal bone anomalies and deafness. The lack of surgical landmarks and facial nerve irregularities make cochlear implantation in this population a challenging endeavor. This study aims to describe a safe and efficacious transcanal approach for cochlear implantation that obviates the need to perform a mastoidectomy and facial recess. PATIENTS: Three children with profound hearing loss secondary to CHARGE syndrome. INTERVENTION: Transcanal cochlear implantation with closure of the ear canal via a modified Rambo meatoplasty. MAIN OUTCOME MEASURE(S): Retrospective chart review of temporal bone anomalies associated with CHARGE syndrome, technical nuances of this transcanal approach, and cochlear implant outcomes. RESULTS: The mean patient age was 2.5 years (range 1.5-3.8 yr). Two were male and two were left ears. All patients had a hypoplastic mastoid, semicircular canal aplasia, and had some degree of cochlear dysplasia. A full cochlear implant insertion was achieved in all cases, even in the presence of grossly abnormal middle ear and facial nerve anatomy. There were no intraoperative or postoperative complications. The mean follow-up was 12.4 months (range, 3.9-25.2 mo). All three patients use their device daily. Their guardians report improved vocalization and environmental awareness. CONCLUSIONS: The modified Rambo transcanal approach provides a safe corridor for cochlear implantation in patients with CHARGE syndrome. This approach minimizes the anatomical variations associated with the syndrome and may reduce the risk of electrode extrusion. Implant outcomes in this patient population remain highly variable based on the patient's global cognitive capacity.

Advances in translational inner ear stem cell research.

Stem cell research is expanding our understanding of developmental biology as well as promising the development of new therapies for a range of different diseases. Within hearing research, the use of stem cells has focused mainly on cell replacement. Stem cells however have a broad range of other potential applications that are just beginning to be explored in the ear. Mesenchymal stem cells are an adult derived stem cell population that have been shown to produce growth factors, modulate the immune system and can differentiate into a wide variety of tissue types. Potential advantages of mesenchymal/adult stem cells are that they have no ethical constraints on their use. However, appropriate regulatory oversight seems necessary in order to protect patients from side effects. Disadvantages may be the lack of efficacy in many preclinical studies. But if proven safe and efficacious, they are easily translatable to clinical trials. The current review will focus on the potential application on mesenchymal stem cells for the treatment of inner ear disorders.

Investigating the use of a Gammatone filterbank for a cochlear implant coding strategy.

BACKGROUND: Contemporary speech processing strategies in cochlear implants (CIs) such as the Advanced Combination Encoder (ACE) use a standard Fast Fourier Transform (FFT) filterbank to extract envelopes. The assignment of the FFT bins to approximate the frequency resolution of the basilar membrane is only partly based on physiology, especially since the bins are distributed linearly below 1000Hz and logarithmically above 1000Hz. NEW METHOD: A Gammatone filterbank which provides a closer approximation to the bandwidths of filters in the human auditory system could replace the standard FFT filterbank in the ACE strategy. An infinite impulse response (IIR) all-pole design of the Gammatone filterbank was compared to the FFT filterbank with 128, 256 and 512 points resolutions and the effect of the frequency boundaries of the filters was also investigated. RESULTS: Melodic contour identification (MCI) and just noticeable difference (JND) experiments, both involving synthetic clarinet notes in octaves 3 and 4, were conducted with 6 normal hearing (NH) participants using noise vocoded stimuli; and 10 CI recipients just performed the MCI experiment. The MCI results for both NH and CI subjects, showed a significant effect of the filterbank on the percentage correct responses of the participants. COMPARISON WITH EXISTING METHODS: The Gammatone filterbank can better resolve the harmonics of tested synthetic clarinet notes which led to better performances in the MCI experiment. CONCLUSIONS: The total delay of the Gammatone filterbank can be made smaller than the delay of the FFT filterbank with the same frequency resolution at low frequencies.

Postsurgical outcome in patients with auditory auras and drug-resistant epilepsy.

PURPOSE: We assessed whether patients with auditory auras have similar outcomes after epilepsy surgery as patients without auditory auras, and hypothesized that patients with non-dominant hemisphere foci might fare better after temporal lobe surgery than patients with dominant resections. METHODS: In this retrospective study, outcome after temporal resection was assessed for patients with drug-resistant epilepsy. Preoperative demographic data, clinical data, and surgical outcome were prospectively registered in a database from 1986 through 2016. Seizure outcome was classified as either seizure-free or relapsed. RESULTS: Data were available in 1186 patients. Forty five patients (3.8%) reported auditory auras; 42 patients (93%) had temporal lobe epilepsy (TLE), and three patients (7%) had extratemporal epilepsy. Since most patients with auditory auras had TLE and in order to have comparable groups, we selected 41 patients with auditory auras and compared them with patients without auditory auras who had temporal lobe resections (767 patients). There were no significant demographic or clinical differences between TLE patients with auditory auras and those without. Patients who had auditory auras were more likely to relapse after temporal lobe surgery than those without (p=0.03). Among patients who had auditory auras and temporal lobe surgery, side of surgery was not related to postoperative outcome (p=0.3). CONCLUSION: Auditory auras are rare among patients with drug-resistant TLE. The presence of an auditory aura in a patient with drug-resistant TLE carries a worse prognosis for a postoperative seizure free outcome and this is not related to the side of surgery.

Impact of the Degree of the Mastoid Pneumatization on Cartilage Type 1 Tympanoplasty Success.

OBJECTIVES: To evaluate the effect of the degree of the mastoid pneumatization on the success rate of cartilage type 1 tympanoplasty. STUDY DESIGN: A retrospective clinical chart review. METHODS: In total, 90 patients (44 females and 46 males; average age, 38.40 ±â€Š11.12 years; age range: 21-65 years) who underwent cartilage type 1 tympanoplasty were included. Demographics and anatomical outcomes were recorded. The patients were divided into 2 groups in terms of anatomical success. In group A, all patients had intact grafts without perforation, retraction, or lateralization, and a dry ear, at 6 months postoperatively. In group B, reperforation of the tympanic membrane was evident 6 months postoperatively. The mastoid air cell volumes of each groups were compared. RESULTS: The mean mastoid pneumatization in groups A and B was 5.32 ±â€Š1.96  and 5.06 ±â€Š2.12 cm, respectively. The mastoid pneumatization of diseased ears did not differ between the groups (P > 0.05). The mastoid pneumatization of diseased ears did not differ between males and females (P > 0.05). The mastoid pneumatization of normal ears did not differ between the groups (P > 0.05). The mastoid pneumatization of normal ears did not differ between males and females (P > 0.05). CONCLUSIONS: The degree of mastoid pneumatization did not affect the success rate of cartilage type 1 tympanoplasty. Further studies with larger numbers of patients are needed to evaluate the relationship between the degree of the mastoid pneumatization and anatomical outcomes after placement of various graft types.

Contemporary surgical issues in paediatric cochlear implantation.

OBJECTIVE: To review the contemporary surgical issues in paediatric cochlear implantation (CI) based on published evidence. DESIGN: Narrative literature review. RESULTS: Surgical challenges in paediatric CI are discussed, with respect to post meningitic labyrinthitis ossificans; cochlear malformation; cochlear implantation in infants; auditory neuropathy and cochlear nerve deficiency; bilateral cochlear implantation; hearing preservation; otitis media; and device failure. CONCLUSION: Early CI is recommended if bacterial meningitis causes profound sensorineural hearing loss (SNHL). CI in cochleovestibular malformation requires pre-operative imaging to plan surgical technique, choice of electrode, and to anticipate complication. Children with congenital severe to profound SNHL should undergo early bilateral simultaneous implantation, preferably before 12 months of age, except those with auditory neuropathy spectrum disorder who should be implanted after one year. Soft surgical technique should be deployed in an attempt to preserve any residual hearing. Otitis media with effusion is not a contraindication to cochlear implantation, but active or recurrent acute otitis media requires resolution of infection with grommet insertion pre-operatively. Device failure in CI recipients requires a stepwise audiological, medical, radiological, and device integrity assessment to determine the need for reimplantation.

Barriers to early cochlear implantation.

OBJECTIVE: Identify variables associated with paediatric access to cochlear implants (CIs). DESIGN: Part 1. Trends over time for age at CI surgery (N = 802) and age at hearing aid (HA) fitting (n = 487) were examined with regard to periods before, during, and after newborn hearing screening (NHS). Part 2. Demographic factors were explored for 417 children implanted under 3 years of age. Part 3. Pre-implant steps for the first 20 children to receive CIs under 12 months were examined. RESULTS: Part 1. Age at HA fitting and CI surgery reduced over time, and were associated with NHS implementation. Part 2. For children implanted under 3 years, earlier age at HA fitting and higher family socio-economic status were associated with earlier CI. Progressive hearing loss was associated with later CIs. Children with a Connexin 26 diagnosis received CIs earlier than children with a premature / low birth weight history. Part 3. The longest pre-CI steps were Step 1: Birth to diagnosis/identification of hearing loss (mean 16.43 weeks), and Step 11: MRI scans to implant surgery (mean 15.05 weeks) for the first 20 infants with CIs under 12 months. CONCLUSION: NHS implementation was associated with reductions in age at device intervention in this cohort.

A practical guide to cochlear implantation in adults with long durations of monaural sound deprivation.

OBJECTIVE: This paper aims to summarize published findings by the authors and integrate these within current literature to support clinical guidelines when choosing an ear for cochlear implantation in adults with long-term monaural sound deprivation. STUDY SAMPLE: Four retrospective cohort studies based on data collected in five cochlear implantation centres with adults with bilateral hearing loss who used a single hearing aid for at least 15 years prior to unilateral or bilateral cochlear implantation. DESIGN: Review, integration and interpretation of retrospective cohort studies to support clinical recommendations. RESULTS: In this population, the prelingual nature of the hearing loss and the duration of bilateral significant hearing loss were the most reliable predictors of cochlear implantation outcomes. Importantly, the duration of sound deprivation in the ear to be implanted was not a significant predictor of speech recognition scores after cochlear implantation and should carry less weight in making recommendations. CONCLUSIONS: In most adults with postlingual hearing loss and long-term monaural sound deprivation, the sound-deprived sound deprivation ear should be preferred for implantation. For adults with prelingual deafness and monaural sound deprivation, the decision should weigh the risks of obtaining poorer results with the cochlear implant compared to the benefits of accessing binaural hearing.

Clinical evaluation of the xDP output compression strategy for cochlear implants.

Technological advances in the domain of digital signal processing adapted to cochlear implants (CI) are partially responsible for the ever-improving outcomes observed with this neural prosthesis. The goal of the present study was to evaluate audiometric outcomes with a new signal processing strategy implemented in Oticon Medical-Neurelec cochlear implant systems, the xDP strategy. The core of this approach is a preset-based back-end output compression system, modulating a multi-channel transfer function depending on the intensity and information content of input sounds. Twenty adult CI patients, matched for age and CI experience, were included in this study. Pure-tone thresholds and vocal audiometry scores were measured with their former signal processing strategy and with xDP. Speech perception was assessed using dissyllabic words presented in quiet, at different intensity levels: 40, 55, 70, and 85 dB SPL, and in a cocktail party noise at a signal-to-noise ratio of +10 dB. Results with the xDP strategy showed, as awaited, no major modification of pure-tone thresholds. A global increase of speech perception scores was observed after a 1-month habituation period, with significant improvements for speech perception in quiet for moderate (55 dB SPL), loud speech sounds (85 dB SPL), and speech-in-noise comprehension. Subjective signal quality assessment showed a preference for Crystalis(xDP) over the former strategy. These results allow the quantification of improvements provided by the xDP signal processing strategy.

Trans-tympanic catheter insertion for treatment of patulous eustachian tube.

OBJECTIVES: To evaluate the safety and therapeutic efficacy of trans-tympanic catheter insertion (TCI) in patients with refractory patulous eustachian tube (PET). METHODS: TCI was attempted in thirty-six ears of twenty-nine patients with chronic PET refractory to conservative treatment. The catheter was inserted under local anesthesia in an operating room through the bony orifice of the eustachian tube (ET) to occlude the isthmus of the tube via a myringotomy site on the tympanic membrane. Patients were evaluated postoperatively by nasal endoscopy and by interview to document symptoms. Successful treatment was defined as complete relief or significant improvement plus satisfaction with treatment. Patients had no concurrent disease and did not undergo any additional surgical procedure. RESULTS: TCI was performed in all except one ear, in which it failed because of an abnormally narrow tympanic ET orifice. Follow-up durations ranged from 6 to 37 months, with an average of 19.3 months. Successful treatment of subjective autophony was achieved in twenty-nine (82.4%) of the thirty-five ears. Ventilation tube (VT) placement was performed in the two ears because of otitis media with effusion (OME) after TCI. In one ear, the inserted catheter was finally removed due to additional unilateral mastoiditis after VT extrusion. CONCLUSION: TCI seems to be a minimally invasive and was used successfully to treat PET. The procedure had a good overall success rate and complications were rare in the long-term.