[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.

[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.

[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.