Surgery in cholesteatoma: ten years follow-up

Abstract Background: During last decades, many otologists attempted to make a long standing dry ear, resulting in better hearing after cholesteatoma surgery. This study evaluated the prognosis of two common methods of cholesteatoma ear surgery considering ten years follow-up. Methods: This retrospective analysis was done using medical records of 108 adult patients with cholesteatoma. After ten years of operation, in these patients we compared the results of the two major trends of canal wall up (CWU) and canal wall down (CWD). Results: Among these patients, 72 (67%) and 36 (33%) underwent the CWD and CWU method respectively. During follow- up, 8% of patients with CWD and 21% of those with CWU had undergone at least one revision operations. Ten yrs after primary surgery, 93% of the ears were dry, 90% with intact tympanic membrane, with 42% of CWD and 52% of CWU groups having fair hearing. One deaf individual was found in each group. The recurrence rate was reported in 6% of CWD and 17% CWU patients, and independent of the size of cholesteatoma, mastoid status or foot plate erosion. The major predisposing factors for recurrence were retraction and discharge, especially in combination. Intact tympanic membrane was more frequent in CWD group, although there was no significant difference between the recurrence rates of these two groups. Conclusion: The result of this study indicated a need for improving surgical techniques in order to lower the recurrence rate and improvement of hearing, especially with the help of endoscopes

Complications in a series of 4400 paediatric cochlear implantation

Objectives: The purpose of this study is to retrospectively review the complications of paediatric patients undergoing cochlear implantation at four major Iranian cochlear implant centres. Methods: A retrospective analysis was performed of all patients who underwent primary cochlear implantation from January 1991 to December 2013. The patients were reviewed for demographic information, and complications including cerebrospinal fluid leak, meningitis, facial palsy, and wound infection. Results: 4400 records were reviewed. Fifty-four patients were lost to follow-up; therefore, 4346 records were analysed. The most common aetiology of hearing loss was non-syndromic genetic sensori-neural hearing loss (69). Other less common aetiologies of hearing loss included TORCH (Toxoplasmosis, Other infections, Rubella, Cytomegalovirus, Herpes) (11), syndromic hearing loss (7), ototoxicity (5), and autoimmune inner-ear disease (4). The most common major complications were CSF leak (0.4), skin necrosis (0.2), meningitis (0.1), facial paralysis (0.07) and massive haemorrhage (0.05). Conclusion: Cochlear implantation continues to be reliable and safe in experienced hands, with a very low percentage of severe complications. © 2015 Elsevier Ireland Ltd

The frequency of congenital long QT syndrome based on new formula in children with sensori-neural hearing loss

Introduction: Long QT syndrome (LQTS) is a repolarization cardiac disorder that can lead to syncope, cardiac arrest and sudden death in apparently healthy individuals. The congenital type can be accompanied with congenital sensory-neural deafness (Jervell-Lang-Nielsen syndrome). Although there are limited studies assessed the frequency of LQTS in these children in developed countries, regarding introducing the new formula, it is necessary to re-evaluate the frequency of this syndrome. Materials and Methods: This cross-sectional and descriptive study was done on 203 patients with congenital sensory-neural hearing loss (SNHL) that had cochlear implant surgery in Baqiyatallah cochlear implant center from 2008 to 2012. Corrected QT was calculated with this formula: QTC = QT + 1.75 (heart rate-60) Patients with QTC > 460 ms, were categorized in four groups: Long QT: QT > 460, Borderline: 440 470 and very markedly: >500. Also, cardiac arrhythmias or arrest were evaluated in patients during cochlear implant surgery and in the postoperative recovery period. Result: Prevalence of LQTS in patients was 12.32 (25 patients). Prevalence of markedly long QT and very markedly long QT were 8.87 (18 patients) and 2.46 (5 patients) respectively. The prevalence of borderline group was 14.29 (29 patients). None of the patients during or after surgery were affected by cardiac arrhythmias or arrest. Conclusion: This study showed higher prevalence of LQTS in patients with SNHL than the normal population, and we suggest that all patients with congenital deafness should be screen for LQTS

Cochlear implantation in children with inner ear malformation: A multicenter study on auditory performance and speech production outcomes

Objectives: This multicenter study evaluated the auditory performance and speech production outcomes of cochlear implantation in children with inner ear anomaly and compared the outcomes of patients with different kinds of malformation. Methods: Cochlear implantation was performed in 107 children with inner ear malformation at four tertiary academic centers. The categories of auditory performance (CAP) and speech intelligibility rating (SIR) scores were evaluated preoperatively and one year and two years postoperatively. Results: Types of inner ear malformation and their frequencies were: incomplete partition type-I, 19 (17.8) patients; incomplete partition type-II, 31 (29), common cavity, 17 (15.9), cochlear hypoplasia, 17 (15.9), and isolated enlarged vestibular aqueduct (isolated EVA), 23 (21.5) patients. EVA was the coexisting anomaly in 27(25.2) subjects. The median CAP and SIR scores improved significantly during the first two years after cochlear implantation in all groups (p-values <0.001 and < 0.001, respectively). No significant difference was seen in CAP and SIR scores of children with different inner ear malformations (p-value = 0.147 and 0.570, respectively) or in patients with isolated EVA compared to coexisting EVA (p-value = 0.538 and 0.075, respectively). Conclusion: The median CAP and SIR scores two years after surgery were 5 (Understanding of common phrases without lip-reading) IQR: 4�6, and 3 (Connected speech is intelligible to a listener who concentrates and lip-reads within a known context) IQR: 3�4, respectively. Auditory performance and speech production were significantly improved in all inner ear malformation patient groups, and no significant difference was observed between the scores of patients with different types of anomaly. © 2020 Elsevier B.V

Cochlear implantation in children with auditory neuropathy spectrum disorder: A multicenter study on auditory performance and speech production outcomes

Objectives: To evaluate the auditory performance and speech production outcome in children with auditory neuropathy spectrum disorder (ANSD). The effect of age on the outcomes of the surgery at the time of implantation was also evaluated. Methods: Cochlear implantation was performed in 136 children with bilateral severe-to- profound hearing loss due to ANSD, at four tertiary academic centers. The patients were divided into two groups based on the age at the time of implantation; Group I: Children �24 months, and Group II: subjects >24 months. The categories of auditory performance (CAP) and speech intelligibility rating (SIR) scores were evaluated after the first and second years of implantation. The differences between the CAP and SIR scores in the two groups were assessed. Results: The median CAP scores improved significantly after the cochlear implantation in all the patients (p value < 0.001). The improvement in the CAP scores during the first year in Group II was greater than Group I (p value: 0.007), but the improvement in CAP scores tended to be significantly higher in patients who were implanted at �24 months (p value < 0.001). There was no significant difference between two groups in SIR scores at first-year and second-year follow-ups. The evaluation of the SIR improvement revealed significantly higher values for Group I during the second-year follow-up (p value: 0.003). Conclusion: The auditory performance and speech production skills of the children with ANSD improved significantly after cochlear implantation, and this improvement was affected by age at the time of implantation. © 2018 Elsevier B.V

Cochlear implantation in children with auditory neuropathy spectrum disorder: A multicenter study on auditory performance and speech production outcomes

Objectives: To evaluate the auditory performance and speech production outcome in children with auditory neuropathy spectrum disorder (ANSD). The effect of age on the outcomes of the surgery at the time of implantation was also evaluated. Methods: Cochlear implantation was performed in 136 children with bilateral severe-to- profound hearing loss due to ANSD, at four tertiary academic centers. The patients were divided into two groups based on the age at the time of implantation; Group I: Children �24 months, and Group II: subjects >24 months. The categories of auditory performance (CAP) and speech intelligibility rating (SIR) scores were evaluated after the first and second years of implantation. The differences between the CAP and SIR scores in the two groups were assessed. Results: The median CAP scores improved significantly after the cochlear implantation in all the patients (p value < 0.001). The improvement in the CAP scores during the first year in Group II was greater than Group I (p value: 0.007), but the improvement in CAP scores tended to be significantly higher in patients who were implanted at �24 months (p value < 0.001). There was no significant difference between two groups in SIR scores at first-year and second-year follow-ups. The evaluation of the SIR improvement revealed significantly higher values for Group I during the second-year follow-up (p value: 0.003). Conclusion: The auditory performance and speech production skills of the children with ANSD improved significantly after cochlear implantation, and this improvement was affected by age at the time of implantation. © 2018 Elsevier B.V