[Hearing rehabilitation in postmeningitis deafness in the context of the time factor]. / Agyhártyagyulladás okozta súlyos fokú sensorineuralis halláscsökkenés rehabilitációs esélyei az idofaktor tekintetében.

INTRODUCTION: Postmeningitis deafness appears in 0-11% of the meningitis cases. Cochlear ossification can develop in these patients, which may make the hearing rehabilitation impossible with cochlear implantation. Due to ossification, it is critical to refer patients to the implant centre without any delay. OBJECTIVE: The aim of this study was to examine the time factor between the appearance of deafness and the first examination in a cochlear implant centre, the possibilities and effectivity of hearing rehabilitation. METHOD: In our tertial referral centre, postmeningitis deafened patients were examined between 2014 and 2022 retrospectively. Hearing results, imaging, possibilities of rehabilitation, complications of cochlear implantations and the hearing results were investigated. RESULTS: 8 patients (3 children, 5 adults) were investigated. The time between the start of deafness and the first appearance varied between 3 weeks to 9 years. Bilateral profound hearing loss was measured in all patients. In 6 cases, cochlear ossification was observed (4 patients bilateral). Cochlear implantation was conducted in 5 patients (4 bilateral, 1 unilateral). In 3 cases, implantation was impossible due to severe ossification. Hearing results showed good hearing levels with poor speech perception in all cases. DISCUSSION: The rehabilitation of severe hearing loss caused by meningitis can present many challenges to clinicians. A critical point in the care is the urgent referral of patients to a cochlear implantation centre as soon as possible after the life-threatening condition has passed. The implementation of further diagnostic and the earliest possible implantation is the responsibility of the implantation centre itself. CONCLUSION: It is recommended to develop a new protocol with the involvement of allied professions to clear patient pathways for an effective treatment strategy. Orv Hetil. 2023; 164(19): 729-738.

Changes in the cochlear and retrocochlear parts of the auditory system in 19-39 and 40-60 years old patients with type 1 diabetes mellitus.

Pathophysiological alterations in the cochlea and functional tests of the auditory pathway support that in diabetes both vasculopathy and neural changes could be present. The aim of our research was to study the differential effect of type 1 diabetes mellitus (T1DM) on two different age groups. Audiological investigation was carried out in 42 patients and 25 controls at the same age groups. Investigation of the conductive and sensorineural part of the hearing system by pure tone audiometry, distortion product otoacoustic emission measurement and acoustically evoked brainstem response registration were evaluated. Among the 19-39-year-old people the incidence of hearing impairment was not different in the diabetes and control groups. Among the 40-60-year-old people hearing impairment was more common in the diabetes group (75%) than in the control group (15,4%). Among patients with type 1 diabetes, the mean threshold values were higher in both age groups at all frequencies although significant difference was in 19-39 years old group: 500-4000Hz right ear, 4000Hz left ear, in 40-60 years old group: 4000-8000 Hz both ears. In the 19-39 years old diabetes group only at 8000 Hertz on the left side was a significant (p<0,05) difference in otoacoustic emissions. In the 40-60 years old diabetes group significantly less otoacoustic emissions at 8000 Hz on the right side (p<0,01) and at 4000-6000-8000 Hertz on the left side, (p<0,05, p<0,01, p<0,05 respectively) was present compared to the control group. According to ABR (auditory brainstem response) latencies and wave morphologies, a possible retrocochlear lesion arose in 15% of the 19-39 years old and 25% of the 40-60 years old diabetes group. According to our results, T1DM affects negatively the cochlear function and the neural part of the hearing system. The alterations are more and more detectable with aging.

[Recognition and complex diagnostics of functional hearing loss]. / A funkcionális halláscsökkenés felismerése és komplex diagnosztikája.

INTRODUCTION: Hearing loss is a sensory impairment that impairs speech understanding, communication and therefore the quality of life. Sometimes the patient's perceived loss of function is exaggerated; subjective and objective test results are inconsistent, the subjectively reported hearing loss is more significant, and in these cases functional hearing loss is considered. OBJECTIVE: Our aim was to collect and retrospectively analyze cases with the diagnosis of functional hearing loss, in order to draw conclusions about the characteristics of functional hearing loss, the signs and conditions that may be of attention and the consideration of appropriate rehabilitation. METHODS: Subjective tests were performed with pure-tone auditory threshold, speech understanding and communication tests, which were compared with the results obtained with objective impedance measurements, stapedial reflex tests, otoacoustic emission measurements, and brainstem evoked response recordings. Imaging studies, psychologist, psychiatrist, neurologist, neurologist and other co-specialists were involved as needed. We excluded cases of deception deliberately intended to obtain financial or other benefits. RESULTS: Between 2007 and 2022, 19 patients were diagnosed with functional hearing loss. The majority (17 cases) were female, the complaints were prevalent at a young age (10-41 years); the average age in the study population was 19.6 years, and the majority of patients (13 cases) were children aged 10-17 years. No organic cause was found in 11 cases, and in the remaining cases no detectable organic abnormality explained the extent of the hearing loss experienced by the patient. The degree of functional hearing loss varied (35-120 dB), with an average of 60,2 dB. CONCLUSION: Recognizing and diagnosing functional hearing loss is very difficult and requires a complex series of tests and professional cooperation. Without recognition, the patient may receive unjustified, even harmful and financially burdensome care, which may lead to the deterioration of his condition. Orv Hetil. 2023; 164(8): 283-292.

Universal newborn hearing screening with automated auditory brainstem response (AABR) in Hungary: 5-year experience in diagnostics and influence on the early intervention.

PURPOSE: In 2015 a new regulation and guidelines for the universal newborn hearing screening by AABR measurement have been implemented in Hungary. The aim of our study was to analyse (1) the past 5 years of data from our diagnostic centre about the incidence and types of congenital hearing losses, and (2) the first experiences with the National Newborn Hearing Screening Registry, started in 2019, and (3) the influence of the screening on the pediatric cochlear implant program. METHODS: 1269 children referred to our diagnostic centre between 2017 and 2021 were investigated. A third AABR measurement and full audiological evaluation were performed. Furthermore, one-year period data of the screening registry, and the number of implanted children at or under the age of 3 were analysed using the national databases. RESULTS: Altogether 276 newborns (22% of the referred cases after the two-stage screening) had hearing loss, 134 (49%) out of them was conductive origin, almost twice frequent in male as in female. Permanent sensorineural hearing impairment was found in 142 (51%), 58 (40%) of them had bilateral, severe to profound hearing loss, occurring more frequently in male as in female. The national digital registration of the screening data within 12 months concerned 68%. The number of early cochlear implantation in one year increased from 1 to 23 children in the past 15 years. CONCLUSION: A third AABR after the two-stage screening increased the efficiency and filtered the 78% false-positive cases. The audiological diagnostics verified and typed the hearing losses ensuring the early intervention.

[Verification results of objective newborn hearing screening]. / Az újszülöttkori objektív hallásszurés utánvizsgálatának eredményei.

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.

[Etiological factors of sensorineural hearing loss in children after cochlear implantation]. / Halláscsökkenést okozó etiológiai tényezok cochlearis implantáción átesett gyermekekben.

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 ß-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.

Cochlear implantation under local anesthesia: a possible alternative for elderly patients.

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.

Analysis of GJB2 mutations and the clinical manifestation in a large Hungarian cohort.

PURPOSE: Pathogenic variants of the gap junction beta 2 (GJB2) gene are responsible for about 50% of hereditary non-syndromic sensorineural hearing loss (NSHL). In this study, we report mutation frequency and phenotype comparison of different GJB2 gene alterations in Hungarian NSHL patients. METHODS: The total coding region of the GJB2 gene was analyzed with Sanger or NGS sequencing for 239 patients with NSHL and 160 controls. RESULTS: Homozygous and compound heterozygous GJB2 mutations were associated with early onset serious clinical phenotype in 28 patients. In 24 patients, two deletion or nonsense mutations were detected in individuals with mainly prelingual NSHL. In compound heterozygous cases, a combination of deletion and missense mutations associated with milder postlingual NSHL. A further 25 cases harbored single heterozygous GJB2 mutations mainly associated with later onset, milder clinical phenotype. The most common mutation was the c.35delG deletion, with 12.6% allele frequency. The hearing loss was more severe in the prelingual groups. CONCLUSION: 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.

Cochlear implantation using posterior suprameatal approach.

When cochlear implantation was first introduced, the mastoidectomy and posterior tympanotomy approach was the most frequently used technique to gain access to the middle ear and the cochlea. Since 2000, several authors have routinely used a non-mastoidectomy nonposterior tympanotomy technique, which has undergone several modifications. Alternative surgical techniques for cochlear implantation have recently been introduced, such as endomeatal-alone or suprameatal-alone and combined posterior tympanotomy/endomeatal approaches. The goal of this study was to describe another modification of this less invasive technique to perform cochlear implantation. Cochlear implantations were performed between January 1, 2002, and December 31, 2012, in 220 patients through the posterior suprameatal approach. In reviewing our experiences, we have concluded that this approach, which eliminates the need for mastoidectomy, is considered safe, time-efficient, and minimally invasive. The possible pitfalls and the microsurgical relevance of anatomic structures of this technique are discussed in detail. Using this technique, not all classical anatomic orientation points are identified. However, certain landmarks predict the depth and the three-dimensional location of invisible anatomic structures.

How does cochlear implantation affect the contralateral vestibular system?

Cochlear implantation has been performed for 16 years by investigators at Semmelweis University. During this period, different types of cochlear implants have been used and, in 30% of cases, hearing was observed to be restored in the nonimplanted ear. In addition to contralateral hearing improvement, significant improvement was observed in the caloric responsiveness of the nonoperated labyrinth. The preoperative median value of the average slow-phase velocity of the caloric test increased, and the increase was statistically significant on the contralateral side. The reason for this caloric response improvement is unclear, although possible explanations are brain plasticity or presently obscure trophic influence on the vestibular system. Whereas the role of brainstem function in the improvement of the contralateral ear's caloric response remains unclear it is also possible that hearing impulses affect the labyrinth. Clearly, the influence of cochlear implants on vestibular function requires further investigation to explain the improvement of contralateral vestibular responsiveness.