COVID-19 infection is associated with a variety of neurological manifestations. Since the inner ear is vulnerable to viruses, sensorineural hearing loss (SNHL) has been reported to occur following SARS-CoV-2 infection. We present here two cases of profound SNHL following SARS-CoV-2 infection. Pure-tone audiograms confirmed profound SNHL. The tympanogram and Auditory Brainstem Responses showed no abnormal symptoms. MRIs and CTs identified some changes but no significant anatomical nor physiological manifestations explaining the obvious cause for hearing loss. High doses of oral corticosteroids with additional conservative therapy were given with no therapeutic response, and therefore, cochlear implant surgery was performed. One case was bilaterally treated, and the other one received an implantation on one side. Both surgeries were carried out without intra- nor postoperative complications. Interestingly, in both cases, advanced fibrotic tissue was found during surgery. Both cases reported successful rehabilitation and are satisfied with their new sound perception following cochlear implantation.

Objectives: The presence of a functional cochlear nerve is a key issue in the preoperative evaluation of pediatric candidates for cochlear implants. Correlations between cochlear nerve deficiency (CND) and bony abnormalities of the labyrinth or bony canal of the cochlear nerve are not yet well understood. The aim of this study was to determine whether the width of the bony cochlear canal (BCNC) can serve as a reliable predictive factor for the existence of a CND. Materials and methods: A total of 11 children with a confirmed diagnosis of prelingual, severe sensorineural hearing loss were included in this study. In all patients, indication for CI was confirmed and according to the preoperative protocol, high-resolution CT and MR were performed. Reconstructions at a distance of 0.6 mm of the axial plane and images from the HRCT of temporal bones were used for measuring the width of the BCNC. The cochlear nerves were evaluated on axial and sagittal - oblique T2 - MRI images and classified as normal, hypoplastic or aplastic. Two factors were reviewed retrospectively: the presence of inner ear anomalies and the relationship between BCNC stenosis and the existence of CND. Results: From a total of 22 temporal bones analyzed (22 ears in 11 patients), inner ear malformations were detected in 6 ears from 3 patients (27.27%). All three children had a bilateral malformation, in one it was Michel deformity and in two it was IP2 (incomplete partition 2). The BCNC diameter ranged from 0.1mm to 2.33mm with a mean value of 1.46±0.6mm. CND was recorded in 4 of 22 ears and all were associated with stenosis of the BCNC. In a total of three ears with a stenotic canal, we obtained a normal finding for the cochlear nerve on MR. Conclusion: Children with BCNC stenosis have a high incidence of CND. A narrowed BCNC on CT can be an indicator for the selection of children with sensorineural hearing loss who will need to be additionally referred for MRI in order to definitively assess the status of the cochlear nerve.

BACKGROUND: To date, cCMV represents the most frequent non-genetic congenital cause of permanent sensorineural hearing loss (SNHL) in childhood and the leading infectious cause of developmental and neurologic disabilities. The aim of this paper is to describe the outcome of cochlear implantation in children affected by severe-to-profound sensorineural hearing loss, due to a symptomatic or asymptomatic cCMV infection, particularly comparing their performance results to that of matched mutated Connexin 26 (Cx26) implanted patients.
METHODS: Retrospective case control study. The clinical data of symptomatic cCMV and asymptomatic cCMV patients were collected and compared to those of Cx26 patients matched for age and pre-CI (cochlear implant) linguistic category; all subjects were affected by bilateral severe-to-profound SNHL and were treated by CI and speech therapy rehabilitation. The Speech Perception Category, the language stage and the linguistic level scores, at 6 months, 1 year, and 3-4 years after CI of the three groups (symptomatic cCMV, asymptomatic cCMV and Cx26 mutation) were collected and compared.
RESULTS: Statistical analysis did not show any significant difference in pre-CI perception category and linguistic level among the three groups; the symptomatic cCMV group showed a statistically worse performance of the language stage over time (p = 0.017).
CONCLUSIONS: Our data support that children affected by cCMV have improved language abilities over time, although the symptomatic cCMV group achieved a lower language stage 3-4 years after CI compared to the asymptomatic cCMV and Cx26 mutation groups. Nonetheless, to date, CI supported by speech therapy can be considered an effective intervention for children affected by cCMV-related severe-to-profound hearing loss.

The N3 wave is a vestibular evoked neurogenic potential detected in some patients with profound sensorineural hearing loss (PSNHL) during brainstem auditory evoked potential (BAEP) analysis. In 1998, Kato et al. mentioned two electropositive waves preceding N3, which we named p1-p2, but no further description was given.
We sought to demonstrate the reproducibility of these waves and hypothesize on their anatomic origin.
We used two cohorts of patients with PSNHL. The first cohort comprised 10 patients with N3, allowing us to establish a new test with adequate electrophysiological conditions headed to detect p1-p2 waves (PN3EP). The second cohort consisted of two groups: group A comprised 10 patients in whom N3 was not detected; and group B comprised 20 patients presenting N3. PN3EP was performed in both groups, of which 50% had cervical myogenic vestibular evoked potentials (cVEMPs).
Only group B presented p1-p2. The PN3EP facilitated the identification of p1-p2 over BAEP analysis, and their presence correlated well with cVEMPs.
P1-p2 may be covered due to inadequate BAEP setting conditions, and could be generated in the distal neural path that generates the N3 wave.

Deafness gene variants play a key role in inner ear malformations. However, the relationship between congenital middle ear malformations and common deafness genes (GJB2, SLC26A4, and mtDNA) in profound sensorineural hearing loss (SNHL) child patients remains poorly investigated. Here we showed that there was no statistical significance in the total mutation frequency of the three common deafness genes in the middle ear malformation group (21.2%, 41/193) in comparison with the normal middle ear and inner ear group (21.0%, 116/553) (χ2 = 0.0061, p = 0.940). Moreover, the mutation ratio of GJB2 and SLC26A4 in the middle ear malformation group (18.7%, 36/193; 2.6%, 5/193) was not significantly different from that in the normal middle ear and inner ear group (17.7%, 98/553; 2.4%, 13/553) (χ2 = 0.084, p = 0.772; χ2 = 0.0000, p = 1.000). The mutation ratio of GJB2 235delC and GJB2 79G>A in the middle ear malformation group (8.8%, 17/193; 8.8%, 17/193) was almost the same to that in the normal middle ear and inner ear group (8.6%, 48/553; 6.7%, 37/553) (χ2 = 0.0030, p = 0.957; χ2 = 0.9556, p = 0.328). The high jugular bulb subgroup analysis also showed the same results. Our findings suggested that GJB2, SLC26A4, and mtDNA mutations might not be related to the middle ear malformations in profound SNHL child patients. Anat Rec, 303:594-599, 2020. © 2019 American Association for Anatomy.

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.
Absztrakt: Bevezetés: Veleszületett halláscsökkenés 1000 újszülöttből 1–3 esetben fordul elő. A gyermekkori súlyos fokú percepciós halláscsökkenés hátterében számos ok szerepelhet. Az esetek döntő százalékában genetikai eredet valószínűsíthető, de emellett lehet infekció, fejlődési rendellenesség és egyéb szerzett megbetegedések is. Célkitűzés: Célunk volt a Semmelweis Egyetem Fül-, Orr-, Gégészeti és Fej-, Nyaksebészeti Klinikáján a 18 év alatti, cochlearis implantáción átesett betegek között az etiológiai tényezők előfordulási arányának felmérése. Módszer és eredmények: A halláscsökkenés okát betegeink 62,9%-ában meg tudtuk határozni. A leggyakoribb etiológia a gap junction protein β-2 gén c.35delG patogén mutációja, mely a vizsgált populációban 38,8%-os allélfrekvenciát mutatott. Emellett az infektív eredet (10,1%), a meningitishez, illetve a cytomegalovirusfertőzéshez társuló halláscsökkenés fordult elő nagyobb százalékban. Betegeink 79,7%-a részesült a beszédfejlődés lezáródását megelőzően műtéti rehabilitációban, a veleszületett halláscsökkent gyermekek 11,2%-a azonban továbbra is későn diagnosztizált eset volt. Következtetés: Eredményeink alapján elmondható, hogy a gyermekkori súlyos fokú halláscsökkenés esetén fontos a genetikai eredet tisztázása. Az időben megkezdett rehabilitáció a gyermek egész életére hatással van, késői implantáció esetén a gyermek beszédfejlődése jelentősen elmarad. A késői implantációk magas aránya a 2015-ben bevezetett új újszülöttkori hallásszűrés-protokollal, valamint az orvoskollégák megfelelő tájékoztatásával és a betegeknek a megfelelő centrumba történő irányításával várhatóan csökkenthető. Orv Hetil. 2019; 160(21): 822–828.

Objectives: The Chudley-McCullough Syndrome (CMS) is a rare autosomal-recessively inherited disorder caused by mutations in the GPSM2 gene, characterised by deafness and brain anomalies. The purpose of this paper is to report about a case of cochlear implant (CI) procedure in a subject affected by CMS. Methods: A 31-year-old subject affected by CMS referred to our centre requiring an evaluation for a CI, as the results with her hearing aids, which she had been using since she was 2-years-old, were unsatisfactory. A profound bilateral sensorineural hearing loss was pointed out. Pure tone audiometry in free field with hearing aids and speech perception results were poor. The subject was counselled about the surgical procedure and the surgery was performed with no complications. Results: The cochlear implant was switched on 22 days after surgery and the subject began speech therapy training. After 1 year, hearing and speech perception results were satisfactory. The hearing threshold in free field with the CI was around 30 dB, and the open set speech perception score reached 55% in silence. Conclusions: The reported case demonstrates that CI is a feasible and safe procedure in subjects with CMS. Furthermore, since satisfactory hearing and speech perception results were achieved we recognise that cochlear implant should be considered the best option for hearing restoration in subjects with CMS and profound sensorineural hearing loss.

Profound sensorineural hearing loss (PSHL) is not uncommonly encountered in otology. In clinics, there is a high incidence of otolithic damage in patients with PSHL, but relevant reports are few. Sharing a continuous membranous structure and similar receptor cell ultrastructures, the cochlea and vestibule may be susceptible to the same harmful factors. Disorders of the inner ear may result in a variety of manifestations, including vertigo, spatial disorientation, blurred vision, impaired articulation, and hearing impairment. Considering the diversity of clinical symptoms associated with PSHL with otolithic dysfunction, it may be frequently misdiagnosed, and objective means of testing the function of otolithic organs should be recommended for hearing-impaired patients. Vestibular-evoked myogenic potentials (VEMPs) via air-conducted sound are of great importance for the diagnosis of otolithic function. Hearing devices such as cochlear implants are commonly accepted treatments for PSHL, and early identification and treatment of vestibular disorders may increase the success rate of cochlear implantation. Therefore, it is necessary to increase awareness of otolithic functional states in patients with PSHL.