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Objectives: Two of the most used objective measures are electrically evoked action potentials (ECAPs) and electrically evoked stapedius reflex thresholds (ESRTs). Although stimuli used for these measures differ considerably, both measures are influenced by subjective loudness percept. We focus on the subjective maximum acceptable loudness (MAL) to investigate if loudness sensitivity varied along the electrode array during ECAP recordings. In addition, we explored how the MAL reached during an ECAP recording related to the postoperative ESRT., Methods: Uni- and bilaterally implanted young CI users ( n  = 15, average age = 9 y, age range 3-18 y) underwent ECAP and ESR recordings using the clinical software MAESTRO (MED-EL, Innsbruck, Austria) and a commercially available immittance instrument (PATH MEDICAL GmbH, Germering, Germany)., Results: Loudness tolerance during ECAP recordings was lowest at the two apical-most electrode contacts (number 1 and 2). There was a moderate correlation between the MAL achieved during ECAP recordings and ESR maximum stimulation amplitudes. (r: 0.44344)., Conclusions: ECAP recordings should commence at basal or medial contacts to increase the users' comfort and loudness tolerance, especially in young CI users. A higher maximum stimulation appears to increase the chance of the automatic determination of ECAP thresholds for all electrode contacts.

Purpose: To assess the role of flat panel computed tomography (FPCT) in the evaluation of cochlear implant (CI) electrode position and its relation to speech perception., Methods: From March 2015 to March 2019, we retrospectively enrolled deaf subjects ≥ 18 years who underwent unilateral CI by one surgeon, imaged with FPCT and assessed with disyllabic words score before CI and at 6 months of follow-up. We calculated the disyllabic score difference before CI and after CI (ΔSDS) and divided the subjects in favorable and unfavorable outcome groups using the median ΔSDS as a cutoff. We compared the demographic, clinical, electrode characteristics, and the CI positioning variables scalar position, surgical insertion depth (SID), linear insertion depth (LID), angular insertion depth (AID) and wrapping factor (WF)., Results: We studied 50 subjects (F/M = 27/23; median age = 60.5 years, IQR: 50-70 years). The median ΔSDS was 80% (interquartile range [IQR]: 60-100%) in quiet and 80% (IQR: 47.5-100%) in noise. Of the subjects 23 demonstrated a favorable outcome and had earlier age at CI (median 52 years; IQR 45-67 years versus median 62 years; IQR: 56-71 years p = 0.032) and a significantly higher SID (median: 4.02 mm IQR: 3.00-5.35 mm versus median: 2.94 mm IQR: 2.06-3.90 mm; p = 0.029). No difference was found for LID (p = 0.977), AID (p = 0.302), and WF (p = 0.224). A logistic regression model built with the age at CI, number of CI electrodes, and the SID was significant χ2 ((df = 3, N = 50) = 14.517, p = 0.002). The model explained 33.7% (Nagelkerke R2) of ΔSDS variance and correctly classified 76% of the cases., Conclusion: The SID measured by FPCT predicts the ΔSDS at 6 months follow-up, alongside with age at implantation and number of CI electrodes., (© 2021. Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Little is known regarding fitting parameters and receptive and expressive language development in cochlear-implanted children (CCI) with profound sensorineural hearing loss (SHL) who are diagnosed with Autism Spectrum Disorder (ASD). The aim of the study was to evaluate a group of ASD CCI users in order to describe their ASD clinical features and CCI outcomes; report on the average electrical charge requirements; and evaluate the possible correlations between electrical and psychophysical outcomes with ASD characteristics., Materials and Methods: A multicentre observational study of 22 ASD children implanted in four cochlear implant (CI) centers. Data concerning profound SHL diagnosis, ASD diagnosis, CI timing and CI compliance were collected. Sound Field (SF) was assessed through repeated behavioural measurements. Categories of Auditory Perception (CAP) and Categories of Language (CL) were used to evaluate speech perception and language skills at short (≤2 yrs), medium (5 yrs) and long term (>10 yrs) follow-up. Fitting parameters such as comfortable thresholds, pulse-width (pw, μsec) and clinical units converted into units of charge/phase were collected. The diagnosis of ASD was acquired by the referral neuropsychiatric department and severity was assessed through the Diagnostic and Statistical Manual of Mental Disorders (DSM-V) and the Childhood Autism Rating Scale (CARS)., Results: At the final follow-up session the median SF threshold for CI outcomes was 30 dB HL (min 15 - max 60). CAP score was extremely variable: 45.5% showed no improvement over time and only 22% of children reached CAP scores of 5-7. CL 45.5% showed no improvement over time and score was 1-2 in the majority of ASD children (72.7%), while only 18.2% reached the highest level of language skills. There were no statistically significant differences at each follow-up between subjects with or without comorbidities. CAP and CL were inversely correlated with DSM-V A and B domains, corresponding to lower speech and language scores in children with more severe ASD symptoms, and maintained their correlation at mid and long follow-ups whilst controlling for age at CI. Electrical charge requirements did not correlate with SF or age at implant but did inversely correlate with ASD severity. With regards to CI compliance: only 13.6% children (3) with severe DSM-V A/B levels and CARS score were partial/intermittent users., Conclusion: The present study is a targeted contribution to the current literature to support clinical procedures for CI fitting and audiological follow-up in children with ASD. The findings indicate that the outcomes of CI use and the fitting procedures are both influenced by the severity of the ASD symptoms rather than the demographic variables or associated disorders., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Purpose Aims of this research were (a) to investigate higher order linguistic and cognitive skills of Italian children with cochlear implants (CIs); (b) to correlate them with the comprehension of irony, which has never been systematically studied in this population; and (c) to identify the factors that facilitate the development of this competence. Method We tested 28 Italian children with CI (mean chronological age = 101 [ SD = 25.60] months, age range: 60-144 months), and two control groups of normal-hearing (NH) peers matched for chronological age and for hearing age, on a series of tests assessing their cognitive abilities (nonverbal intelligence and theory of mind), linguistic skills (morphosyntax and prosody recognition), and irony comprehension. Results Despite having grammatical abilities in line with the group of NH children matched for hearing age, children with CI lag behind both groups of NH peers on the recognition of emotions through prosody and on the comprehension of ironic stories, even if these two abilities were not related. Conclusions This is the first study that targeted irony comprehension in children with CI, and we found that this competence, which is crucial for maintaining good social relationships with peers, is impaired in this population. In line with other studies, we found a correlation between this ability and advanced theory of mind skills, but at the same time, a deeper investigation is needed, to account for the high variability of performance in children with CI.

Objective: To calculate the frequency allocation mismatch in a group of very selected cochlear implant (CI) recipients and to contrast it with the speech perception performances., Study Design: Cross-sectional observational prospective study., Settings: Tertiary Audiological Department, University hospital., Patients: Fifteen adults receiving the same CI array by the same surgeon through a posterior tympanotomy, round window approach., Main Outcome Measures: 1) High definition flat panel computed tomography (FPCT) control of the intracochlear position of each electrode contact, and computation of the relative frequency allocation mismatch; 2) analysis of speech perception outcomes in relation with the mismatch., Results: Despite a consistent and reproducible surgical procedure with the same intracochlear array, significant deviations from the frequency allocation tables (FAT) assigned by default by the manufacturer were observed in this study.Their influences on speech perception performances were negligible in the simple tasks of words or sentences recognition in quiet (and, to a lesser extent also in noise). The greatest effect of a significant mismatch was observed for the vocal-consonant-vocal (VCV) sequences recognition under noise masking, the emotional and the linguistic prosody recognition, and the phonemes discrimination of the Auditory Speech Sound Evaluation (A§E) test., Conclusions: The greatest frequency-to-place occurred at the high frequencies. The effect was rather irrelevant on simple words and sentences recognition, while it negatively impacted on the more complex perceptual tasks.

Currently, there are no studies assessing everyday use of cochlear implant (CI) processors by recipients by means of objective tools. The Nucleus 6 sound processor features a data logging system capable of real-time recording of CI use in different acoustic environments and under various categories of loudness levels. In this study, we report data logged for the different scenes and different loudness levels of 1,366 CI patients, as recorded by SCAN. Monitoring device use in cochlear implant recipients of all ages provides important information about the listening conditions encountered in recipients' daily lives that may support counseling and assist in the further management of their device settings. The findings for this large cohort of active CI users confirm differences between age groups concerning device use and exposure to various noise environments, especially between the youngest and oldest age groups, while similar levels of loudness were observed., (© 2017 S. Karger AG, Basel.)

Objective: To report on the outcomes of cochlear implantation (CI) in a child with cochleovestibular nerves (CVN) hypoplasia., Study Design: Retrospective case review., Setting: Tertiary referral center, University hospital., Patients: An 18-month-old child with profound bilateral congenital hearing loss and bilateral hypoplasia of the CVN at imaging., Intervention: Left CI at age 29 months with a Nucleus Contour device (Cochlear Ltd., Lane Cove, New South Wales, Australia) after unsatisfactory results of hearing aid use for 10 months., Main Outcome Measures: Speech perception tests, behavioral observation, electrophysiologic tests, and cognitive evaluation., Results: Although the child scores poorly in every perceptive category with the CI alone, the device greatly enhances his speech understanding with the hearing aid in the opposite ear. In the bimodal condition, his words and sentences identification, recognition, and comprehension far exceed the monaural figures. The Meaningful Auditory Integration Scale (MAIS) tests reaches a score of 26/40, and the MacArthur's questionnaires confirm the improvement of language production and comprehension. These results became noticeable after 5 to 6 months and continued to improve up to the 10th month. The child's cognitive scores and overall performance competences greatly benefit from the CI, with the mental age overcoming the chronological age., Conclusion: We can confirm the chance of achieving satisfactory results by CI even when the imaging of CVN is doubtful and the electrophysiological tests are disappointing. In our experience, a CI in Type IIb dysplasia of the CVN is a feasible option, provided that the candidate shows some responses at aided audiogram and at least minimal signs of language development. Adequate counseling is necessary for these children because the expected outcome is somewhat lower than that of their deaf peers with normal appearance of the nerves.

The aim of this study was to evaluate audiological characteristics and parents' opinions on hearing device use in children with unilateral sensorineural hearing loss (USNHL) who attended a tertiary-level audiologic center. The medical charts of 70 children aged 6 to 12 years with USNHL were reviewed. In 51.4% of cases, the children were diagnosed with USNHL after the age of 2 years. The main causes of USNHL were congenital cytomegalovirus infection (21.4%) and unilateral cochlear nerve hypoplasia (12.9%). The percentage of patients wearing a hearing device was 45.7% (32/70); of these, 28 (87.5%) wore a conventional hearing aid, 2 (6.3%) a CROS device, and 2 (6.3%) a cochlear implant. Regarding the choice to use a hearing device, no significant differences were found between the subcategories of hearing loss degree (p = 0.55) and audiometric configuration (p = 0.54). Most parents of children with mild-to-severe USNHL observed improved attention (90.9%), and reduced fatigue and restlessness (86.4%) using the hearing aid. These children performed significantly better on all audiological tests (speech perception in quiet and in noise conditions, and sound localization) while wearing the hearing aid (p < 0.001). More efforts should be made to raise awareness among professionals and parents about the negative consequences of uncorrected USNHL. [ABSTRACT FROM AUTHOR]

Autosomal dominant non-syndromic hearing loss (HL) typically occurs when only one dominant allele within the disease gene is sufficient to express the phenotype. Therefore, most patients diagnosed with autosomal dominant non-syndromic HL have a hearing-impaired parent, although de novo mutations should be considered in all cases of negative family history. To date, more than 50 genes and 80 loci have been identified for autosomal dominant non-syndromic HL. DFNA22 (MYO6 gene), DFNA8/12 (TECTA gene), DFNA20/26 (ACTG1 gene), DFNA6/14/38 (WFS1 gene), DFNA15 (POU4F3 gene), DFNA2A (KCNQ4 gene), and DFNA10 (EYA4 gene) are some of the most common forms of autosomal dominant non-syndromic HL. The characteristics of autosomal dominant non-syndromic HL are heterogenous. However, in most cases, HL tends to be bilateral, post-lingual in onset (childhood to early adulthood), high-frequency (sloping audiometric configuration), progressive, and variable in severity (mild to profound degree). DFNA1 (DIAPH1 gene) and DFNA6/14/38 (WFS1 gene) are the most common forms of autosomal dominant non-syndromic HL affecting low frequencies, while DFNA16 (unknown gene) is characterized by fluctuating HL. A long audiological follow-up is of paramount importance to identify hearing threshold deteriorations early and ensure prompt treatment with hearing aids or cochlear implants. [ABSTRACT FROM AUTHOR]

This case report describes a peculiar and innovative fixing procedure with a Poly‐D,L‐lactic acid (PDLLA) polymer in the unusual case of magnet dislodgment and rupture of the cochlear implant (CI) silicone sheath holding the magnet. [ABSTRACT FROM AUTHOR]

The purpose of the study was to examine the subjective and objective potential advantage for speech understanding in noise achieved by cochlear implant (CI) recipients when using the autosensitivity control (ASC) input signal processing in combination with the adaptive dynamic range optimization (ADRO). Eighteen subjects (8 females, 10 males, mean age 17.7 ± 6.7) were enrolled in a prospective open blinded comparative study between the ASC + ADRO condition vs. the ADRO alone; 16 were sequential binaural and 2 were monoaural CI recipients. All patients had been wearing their CI for at least 3 years, had no additional disabilities, had an age-appropriate receptive and expressive language. Word recognition performances in noise (at signal-to-noise ratio +5 dB HL) were significantly better in the ADRO-alone condition than in the ADRO + ASC condition. (p = 0.03) These objective outcomes were in agreement with the subjective reports. No significant difference was found in quiet. Our results, apparently in contrast with other reports in the literature, suggest that the decision of adding the slow-acting automatic reduction in microphone sensitivity provided by ASC should be limited to selected CI recipients. [ABSTRACT FROM AUTHOR]

Objective: To report presentation, diagnostic process, management and outcome of a case of autoimmune inner ear disease (AIED) related with Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), treated with cochlear implantation, and review of relevant literature. Case presentation and management: A retrospective case report of AIED associated with EGPA treated with cochlear implantation was described. A multi-step approach for diagnosis and confirmation of AIED and hearing rehabilitation was conducted, eventually leading to left cochlear implantation. Results: The surgery was without complications and postoperative course was uneventful. Two years after surgery, pure-tone and speech soundfield audiometry with left cochlear implant switched on showed a good improvement in pure-tone threshold and a word recognition score of 50% at 60 dB nHL. Literature review does not report any previous case of AIED EGPA-related. Conclusions: Cochlear implantation in AIED EGPA-related have been shown to be a viable treatment option in a stabilized phase of disease. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to evaluate the effect of cochlear implantation (CI) in advanced Ménière's disease (MD). Design: The initial search on PubMed, EMBASE, and Cochrane databases yielded 171 articles; no language restriction was applied. Study sample: A total of 11 articles met the inclusion criteria and were included in this systematic review. Six articles provided patient-level data on improvement in speech recognition testing after CI. Results: The methodological quality of included studies was assessed by examining the study design, level of evidence, method of measurement and adequacy of outcome reporting. A random-effect model was fitted for calculating weighted means. Post-operative improvement in word recognition score (WCS) was 50.8% (95% confidence interval: 34.6–67.1%); general improvement of vestibular symptoms after CI was found in 67% of the pooled patients; when reported in the studies, quality of life (QoL) and tinnitus were also generally improved after CI. Conclusions: CI in advanced MD is a valid option providing good outcomes in terms of speech performances, regardless of the disease duration, uni- or bilaterality, age at implantation, previous therapeutic procedures and stage of activity of MD. [ABSTRACT FROM AUTHOR]

Objective: To report neuroradiologic findings, surgical strategies and clinical and audiological results in a series of children with CHARGE syndrome (CS) who had been evaluated for cochlear implantation (CI).Study design: Retrospective case series.Setting: Tertiary referral university hospital.Patients: Eight profoundly deafened children with CS were included. Routine audiometric measurements, speech perception categories and speech intelligibility ratings were performed pre- and post-operatively. Neuroradiological and intraoperative findings, surgical planning, and post-operative complications were analyzed.Results: Six children were profoundly deaf from birth and 2 had progressive hearing loss to profound levels. Cochlear nerve deficiency (CND) was noted in 5 out of the 6 patients with congenital sensorineural hearing loss (SNHL). Seven children underwent CI. Surgery was performed using standard transmastoid facial recess approach in 3 ears, subtotal petrosectomy in 3, and transmastoid single-slit labyrinthotomy in one. Temporary facial palsy occurred in one patient. In the group of patients with congenital SNHL, 2 children benefitted from CI and developed spoken language; the remaining 3 children obtained improved access to environmental sounds and used signs and gestures as their main mode of communication. The two patients with progressive SNHL had preoperative verbal language and continued to use verbal language after CI.Conclusions: The constant presence of temporal bone anomalies in children with CS requires surgical expertise in performing non-standard approaches for safe and effective CI. Patients with progressive SNHL and normal cochlear nerves had satisfactory results with CI. Limited benefits have been observed in presence of CND. [ABSTRACT FROM AUTHOR]

Currently, there are no studies assessing everyday use of cochlear implant (CI) processors by recipients by means of objective tools. The Nucleus 6 sound processor features a data logging system capable of real-time recording of CI use in different acoustic environments and under various categories of loudness levels. In this study, we report data logged for the different scenes and different loudness levels of 1,366 CI patients, as recorded by SCAN. Monitoring device use in cochlear implant recipients of all ages provides important information about the listening conditions encountered in recipients' daily lives that may support counseling and assist in the further management of their device settings. The findings for this large cohort of active CI users confirm differences between age groups concerning device use and exposure to various noise environments, especially between the youngest and oldest age groups, while similar levels of loudness were observed. [ABSTRACT FROM AUTHOR]

The number of non-neurofibromatosis type 2 (NF2) indications for auditory brainstem implant (ABI) in the literature is increasing. The objective of this study was to analyze and discuss the indications for ABI. Retrospective chart review and systematic review were conducted at Quaternary referral skull base center and referring centers. Analysis of ABI cases with non-NF2 indications and systematic review presenting non-NF2 ABI cases were performed. Fourteen referred cases with ABI were identified. All cases had unsatisfactory results of ABI and all could have been rehabilitated with a cochlear implant (CI). Of these 14 cases, 9 improved with a cochlear implant, and 2 with a hearing aid, two are still planned for CI, one received bilateral CI, no ABI. In literature, we found 31 articles presenting 144 non-NF2 ABI cases with at least 7 different indications other than NF2. ABI should be restricted to those patients who have no other rehabilitation options. Patency of the cochlea and evidence of an intact cochlear nerve should be examined with imaging and electrophysiologic testing. Sometimes a CI trial should be planned prior to proceeding with ABI. We have shown that in many cases a CI is still possible and CI provided better results than ABI. In vestibular schwannoma in the only hearing ear, cochlear otosclerosis, temporal bone fractures, (presumed) bilateral traumatic cochlear nerve disruption, auto-immune inner ear disease and auditory neuropathy primarily CI are indicated. Traumatic bilateral cochlear nerve disruption is exceptionally unlikely. In cochlear nerve aplasia, testing should be performed prior to meeting indications for ABI. In malformations, ABI is indicated only in severe cochlear hypoplasia or cochlear aplasia. [ABSTRACT FROM AUTHOR]

Abstract In this clinical note we discuss the indications, feasibility, and outcomes of binaural simultaneous cochlear implantation (CI) following bilateral transverse temporal bone (TB) fractures. A 41-year-old male, totally deaf after a bilateral TB fracture, underwent an audiological, electrophysiological, and imaging investigation in order to assess the integrity of the VIIIth cranial nerves. Five months later he received a simultaneous bilateral CI. Speech perception tests were conducted at different time points. A significant advantage by dichotic listening was observed since the beginning of the habilitation program. The patient achieved a 100% word and sentences recognition in quiet at 12 months. His listening skills in noisy conditions were improved by the use of two implants. A CI in TB fractures is feasible if the VIII nerve is intact and the cochlea is spared by the fracture rim. The early timing of the procedure probably contributed to its success by preventing cochlear fibrosis and ossification. Sumario En esta nota clínica discutimos las indicaciones, la factibilidad, y los resultados de la implantación coclear (CI) bilateral simultánea posterior a fracturas transversas bilaterales del hueso temporal (TB). Un varón de 41 años de edad, totalmente sordo después de una fractura bilateral del TB, fue sometido a una investigación audiológica, electrofisiológica y por imágenes, para evaluar la integridad de ambos octavos nervios craneales. Cinco meses después, el sujeto recibido IC bilaterales simultáneos. Se realizaron pruebas de percepción de lenguaje en diferentes momentos. Se observó una ventaja significativa en la audición dicótica desde el inicio del programa de habilitación. El paciente logró un 100% de reconocimiento de palabras y frases en silencio a los 12 meses. Sus habilidades para escuchar mejoraron con el uso de dos implantes. Una IC en fracturas del TB es factible si el VIII par está intacto y la cóclea no está involucrada en el trazo de la fractura. La oportuna realización del procedimiento probablemente contribuyó al éxito al prevenir la fibrosis coclear y la osificación. [ABSTRACT FROM AUTHOR]

Conclusions. We observed 4 cases of paroxysmal positional vertigo (PPV) among 62 cochlear implant (CI) recipients. They occurred in the implanted ear without chronological relation with the surgical procedure or the implant's activation. All of them relapsed within 3 months after an initially successful repositioning maneuver, and finally recovered after the second one. None of the patients showed labyrinthine weakness in the implanted ear. The outbreak of PPV did not affect the patients' speech perception performances. Objectives. To report and discuss the occurrence of PPV after cochlear implantation Patients and methods. Among 32 adult patients who received a Nucleus® CI, 4 suffered from PPV on the basis of Dix-Hallpike's maneuvers. After a Semont's repositioning maneuver, recurrences were similarly addressed. An electro-nystagmography (ENG) recording of caloric irrigation tests was obtained once the symptoms subsided Results. The observed incidence of 12.5% exceeds the figures reported in the literature. No anatomic abnormalities were identified in these patients, nor were any intraoperative or postoperative complications reported. PPV developed 1-12 months after CI surgery in the posterior canal and relapsed within 3 months. ENG showed a normal reflectivity in two patients, while the other two had a significant prevalence of the implanted side. The outcomes of the speech perception tests after CI in all the four patients matched those of their corresponding categories. [ABSTRACT FROM AUTHOR]

Congenital cytomegalovirus (cCMV) is the leading nongenetic cause of sensorineural hearing loss (HL). However, there are no universally accepted approaches to diagnosis, follow-up and treatment. The aim of this study was to evaluate the main characteristics of cCMV-infected children, focusing on their management and long-term hearing outcomes. This retrospective study included all children with cCMV infection who were referred to a third-level referral audiologic center for a 6-year hearing follow-up. The main information collected from the medical records included gestational age, birth weight, trimester of maternal seroconversion, hearing status at birth and after 6 years, hearing fluctuations, treatment with oral valganciclovir (within the first month of life and for 6 months), use of hearing devices, presence of speech-language delay, motor delay, cognitive delay and balance disorders, awareness of cCMV among parents, and parents' engagement in behaviors that could increase the risk of CMV infection during pregnancy. A total of 141 children with cCMV infection (72 males and 69 females; mean gestational age: 37+3 weeks; mean birth weight: 2893 g) were assessed. Overall, 48 children (34.0%) had a diagnosis of speech-language delay, 32 (22.7%) of sensorineural HL (59.4% bilaterally; 50% of profound degree), 18 (12.8%) of motor delay, 16 (11.3%) of balance disorders, and 6 (4.3%) of cognitive delay. Among children with HL, 8 (25.0%) were fitted with hearing aids (5 unilaterally and 3 bilaterally), and 5 (15.6%) had undergone cochlear implantation (1 unilaterally and 4 bilaterally), while a bimodal hearing solution was adopted for 2 (6.3%) patients. Compared to children with asymptomatic cCMV infection, symptomatic children had a higher prevalence of neurological and auditory sequelae (P < 0.01) and bilateral (P = 0.003) and severe-to-profound HL (P = 0.004). Overall, 23 children (16.3%) received oral valganciclovir, and only one of them experienced hearing deterioration. Only 14.9% of mothers and 5% of fathers were aware that cCMV could cause progressive or late-onset HL, and 87.9% of parents (248/282) had engaged in behaviors that increased the risk of CMV infection during pregnancy. This study confirmed the importance of performing a long audiological follow-up in children diagnosed with cCMV infection due to the possible late-onset, progressive and fluctuating nature of HL. Moreover, the study highlighted many current controversies in preventive (poor prenatal education), diagnostic (routine maternal serological screening) and therapeutic (valganciclovir administered to asymptomatic children) approaches to cCMV infection. More efforts should be made to improve prevention strategies and raise awareness of cCMV infection risks among the population. • Congenital CMV is the leading nongenetic cause of sensorineural hearing loss. • Symptomatic children had a higher prevalence of neurological and auditory sequelae. • Only one child out of 23 showed hearing deterioration after treatment with valganciclovir. • Only 5% of fathers were aware that congenital CMV could cause hearing loss. [ABSTRACT FROM AUTHOR]

Abstract: Pontine tegmental cap dysplasia (PTCD) is an exceptionally rare brain stem and cerebellar malformation characterized by ventral pontine hypoplasia, vaulted pontine tegmentum, hypoplasia of the vermis, subtotal absence of middle cerebellar peduncles, lateralized course of the superior cerebellar peduncles, and absence or alteration of the inferior olivary nucleus. The main clinical features are multiple cranial neurophaties and ataxia. Sensorineural hearing loss of varying severity is almost always present. To date, 14 cases of PTCD have been reported in the literature. We present a child with PTCD and profound bilateral sensorineural hearing loss who underwent cochlear implantation. To the best of our knowledge, cochlear implantation in PTCD has not been previously reported. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. At 22 months’ postoperative evaluation, the patient who was placed into speech perception category 0 (no detection of speech) preoperatively progressed to category 3 (beginning word identification). Before implantation, the child had connected speech unintelligible. At the last follow-up, she had connected speech intelligible to a listener who has little experience of a deaf person''s speech. Cochlear implantation allowed this child to improve her quality of life, increasing her self-confidence, independence, and social integration. [Copyright &y& Elsevier]