Introduction As average life-expectancy increases, a sufficient hearing rehabilitation for elderly patients with severe-to-profound sensorineural hearing loss becomes more important. Cochlear implantation is a relatively safe surgical procedure also for elderly patients, the higher risk is caused by general anesthesia. We report on four patients who underwent cochlear implantation under local anesthesia. Methods After detailed preoperative examinations (audiological tests, imaging, genetic tests, evaluation of motivation and compliance of the patient), four patient with severe-to-profound hearing loss were selected for cochlear implantation under local anesthesia. For the electrode insertion, we used the posterior suprameatal approach technique. Pre- and postoperative pure tone audiometry and speech-perception tests were conducted to prove the success of the procedure. Results The mentioned technique was applied; the average length of the operation was 52 min. The intraoperative measurements showed normal impedance and normal neuronal response telemetry, all the patients had sound experience during the intraoperative examination of the engineer. No complications were observed. The postoperative audiological tests showed a significant increase in the hearing perception. Conclusion Cochlear implantation under local anesthesia is a safe and fast procedure for elderly patients. The intraoperative sound experience can give an extra motivation in the postoperative rehabilitation. Our results prove that by carefully selected elderly patients cochlear implantation can assure a significant increase in speech perception. We can establish that the new posterior suprameatal approach technique combined with local anesthesia presents a viable future option for those patients who were inoperable beforehand because of high risks of general anesthesia.
The mutation frequency of GJB2 in the investigated cohort is lower than in other European cohorts. The most serious cases were associated with homozygous and compound heterozygous mutations. In our cohort the hearing impairment and age of onset was not altered between in cases with only one heterozygous GJB2 mutation and wild type genotype, which may exclude the possibility of autosomal dominant inheritance. In early onset, severe to profound hearing loss cases, if the GJB2 analysis results in only one heterozygous alteration further next generation sequencing is highly recommended.
Introduction: Congenital sensorineural hearing loss is one of the most common sensory defects affecting 1–3 children per 1000 newborns. There are a lot of causes which result in congenital hearing loss, the most common is the genetic origin, but infection, cochlear malformation or other acquired causes can be reasons as well. Aim: The aim of this study was to establish the etiological factors of congenital profound sensorineural hearing loss in children who underwent cochlear implantation. Results: Our results show that the origin of the hearing loss was discovered in 62.9% of our patients. The most common etiological factor was the c.35delG mutation of the gap junction protein β-2 gene, the allele frequency was 38.7% in our cohort. Infection constituted to 10.1%, and meningitis and cytomegalovirus infection were the second most common cause. 79.9% of our patients received sufficient hearing rehabilitation before the end of the speech development’s period (6 years old), but 11.2% of our cases were still diagnosed late. Conclusions: Based on our data we can state that genetic evaluation is crucial in the diagnostic process of congenital profound sensorineural hearing loss. Sufficient hearing rehabilitation affects the whole life of the child, and by late cochlear implantation the speech development falls behind. We can decrease the ratio of the late implantation with the new protocol of newborn hearing screening, and with sufficient information provided to the colleagues, so the children may be referred to the proper center for rehabilitation without delay. Orv Hetil. 2019; 160(21): 822–828.
Extra-and intracranial complications of cholesteatoma are rare in the new era of antibiotics. The rarity of the severe complications and the atypical symptoms cause difficulties to set up proper diagnosis and treatment. We report about a 76-year old patient with external auditory canal cholesteatoma causing Bezold abscess and sigmoid sinus thrombosis. The patient underwent canal wall up mastoidectomy and mastoid obliteration with abdominal fat and Bezold abscess drainage. Systemic antibiotic and anticoagulant therapy were applied. The treatment resulted in fast improvement in the general condition of the patient, and the control magnetic resonance imaging (MRI) showed no recurrence of the disease.
Sufficient hearing is the cornerstone of the development of children’s complex sensory perception, sound recognition, speech development and optimal communication skills. Hearing screening of newborns is necessary to detect congenital hearing disorders. Compulsory objective hearing screening in Hungary is a significant improvement in early diagnosis. The Audiological Department of Otorhinolaryngology, Head and Neck Surgery Clinic at Semmelweis University serves as a verification center for children identified via the compulsory objective newborn hearing screening and necessitates more detailed assessment. The goal of this study was to summarize the verification results of the year 2018. Case history, ENT examination, electric response measurement, impedance tests, otoacoustic emission measurement, surdopedagogical examination, and genetic examination are the basics of the diagnosis and the therapy as well. Altogether 261 newborns were examined in 2018 and 164 were subjected to audiological tests during the analyzed seven-month period. Normal hearing was detected in both ears in 77% of the cases, while hearing loss has been verified in 37 patients (23% of cases). Permanent hearing loss has been diagnosed in 19 cases, 4 unilateral and 15 bilateral. Hearing loss of sensorineural origin was confirmed in 17, conductive in 2 children. Temporary hearing loss caused by otitis media with effusion was found in further 18 children which healed spontaneously in most cases. Organized neonatal objective hearing examination has been established in Hungary in 2015. As a result, we can diagnose and provide care for children with hearing loss at the earliest stage. The National Newborn Hearing Screening Registry ensures a well-coordinated and smooth process. Orv Hetil. 2019; 160(47): 1850–1855.
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