Prognostic Value of Electrophysiological and MRI Findings for Pediatric Cochlear Implant Outcomes: A Systematic Review.

Purpose: Magnetic resonance imaging (MRI), electric compound action potential (eCAP), and electric auditory brainstem response (eABR) are among the routine assessments performed before and/or after cochlear implantation. The objective of this review was to systematically summarize and critically appraise existing evidence of the prognostic value of eCAP, eABR, and MRI for predicting post-cochlear implant (CI) speech perception outcomes in children, with a particular focus on the lesion site.

Method: The present systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 statement.

Predictors of cochlear implant outcomes in pediatric auditory neuropathy: A matched case-control study.

Objectives: Current evidence supports the benefits of cochlear implants (CIs) in children with hearing loss, including those with auditory neuropathy spectrum disorder (ANSD). However, there is limited evidence regarding factors that hold predictive value for intervention outcomes.

Design: This retrospective case-control study consisted of 66 children with CIs, including 22 with ANSD and 44 with sensorineural hearing loss (SNHL) matched on sex, age, age at CI activation, and the length of follow-up with CIs (1:2 ratio).

An Umbrella Review of Cochlear Implant Outcomes in Children With Auditory Neuropathy.

Purpose: The objective of this overview of systematic reviews (SRs; umbrella review) was to systematically summarize and critically appraise current evidence of cochlear implant (CI) outcomes in children with auditory neuropathy spectrum disorder (ANSD).

Method: This study was guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 statement. The methodological quality and the risk of bias in the included SRs were assessed using A MeaSurement Tool to Assess systematic Reviews 2 checklist and the Risk of Bias in Systematic Reviews tool, respectively.

Analysis of specific risk factors of neurodevelopmental disorder in hearing-impaired infants under ten months of age: "EnTNDre" an opening research stemming from a transdisciplinary partnership.

Introduction: Approximately 20% of children born with severe to profound hearing loss (HL) have an associated disorder that poses a neurodevelopmental risk [1]. The objective of this study is to identify the criteria and profiles of deaf infants at risk of neurodevelopmental disorders (NDD) to provide early intervention.

Methods: Twenty-two infants aged three to ten months with bilateral congenital deafness were included.

[Neonatal hearing screening].

Neonatal hearing screening has been developped in a large number of countries. The rational to build such nationwide programs is robust. The prevalence of hearing impairment of various etiologies is high (1/1,000), diagnosis of hearing impairment in infants is uneasy and is made most of the time after the age of 18 months when treatment is less efficient and, last, appropriate test to screen for hearing impairment are available: Otoacoustic Emission and Auditory Evoked Potential.

Auditory processing disorder in children: the value of a multidisciplinary assessment.

Purpose: Auditory processing disorder (APD) may affect 0.2-5% of the paediatric population. The diagnosis of APD remains difficult because of polymorphic symptoms possibly entangled with other difficulties.

Arterial spin labeling brain MRI study to evaluate the impact of deafness on cerebral perfusion in 79 children before cochlear implantation.

Age at implantation is considered to be a major factor, influencing outcomes after pediatric cochlear implantation. In the absence of acoustic input, it has been proposed that cross-modal reorganization can be detrimental for adaptation to the new electrical input provided by a cochlear implant. Here, through a retrospective study, we aimed to investigate differences in cerebral blood flow (CBF) at rest prior to implantation in children with congenital deafness compared to normally hearing children.

Mutation m.3395A > G in MT-ND1 leads to variable pathologic manifestations.

A non-synonymous mtDNA mutation, m.3395A > G, which changes tyrosine in position 30 to cysteine in p.MT-ND1, was found in several patients with a wide range of clinical phenotypes such as deafness, diabetes and cerebellar syndrome but no Leber's hereditary optic neuropathy.

Intra-cochlear electrode tip fold-over.

Cochlear implantation has been performed safely for over two decades but still has various minor and major complications. We report two cases of an unusual complication of electrode implantation: tip fold-over of the electrode array within the cochlea. Both cases required undergoing explantation and re-implantation.

Pain After Cochlear Implantation: An Unusual Complication?

Objective: To analyze clinical signs and better define the underestimated long-term pain after implantation, to discuss etiological hypothesis, and to propose our department treatment algorithm and results.

Study Design And Setting: Retrospective review of children implanted with complaints of atypical pain in the area of device implantation, not in the immediate postoperative period, more than or equal to 4 on the Visual Analog Pain Scale (VAPS: 0-10) in the ENT pediatric department of Trousseau and Necker Enfants Malades Hospitals between 1998 and 2015.

Patients: All patients had full clinical and electrophysiological checking, and had normal functioning device.

Efficiency of Melatonin as Compared to Pentobarbital for Audiometry Brainstem Response in Children With Associated Disorders.

Purpose: Outpatient pediatric audiometry brainstem response (ABR) uses various techniques (no drug, hydroxyzine, pentobarbital, melatonin). The aim of this study was to evaluate the efficiency of melatonin as compared to pentobarbital in children with associated disorders.

Method: This was a retrospective study that took place in a tertiary care center.

Pediatric cochlear implantation in residual hearing candidates.

Objectives: To propose categories for the various types of residual hearing in children and to review the outcomes of cochlear implantation (CI) in children with these different hearing conditions.

Methods: We identified 53 children with residual hearing who had received a cochlear implant. Five groups were arbitrarily defined based on auditory features: G1, characterized by low-frequency residual hearing (n=5); G2, characterized by severe sensorineural hearing loss (SNHL) and low speech discrimination (n=12); G3, characterized by asymmetric SNHL (n=9); G4, characterized by progressive SNHL (n=15); and G5, characterized by fluctuating SNHL (n=12).

Hearing outcomes in functional surgery for middle ear malformations.

Objectives: To standardize the information for families of children having functional surgery for middle ear malformations, we describe the audiometric results of the subgroup of patients with the most favorable anatomic conditions: viable auditory canal, intact tympanic membrane, mobile stapes, and corresponding to a Jahrsdoerfer score of 8 or higher.

Study Design: Case series, tertiary referral center.

Methods: Charts of patients undergoing functional surgery for congenital middle ear malformations were reviewed for demographic data, preoperative Jahrsdoerfer score, ossicular chain status, type of ossiculoplasty, and audiometric data before and 6 months postsurgery.

Congenital cytomegalovirus is the second most frequent cause of bilateral hearing loss in young French children.

Objective: To estimate the prevalence of congenital cytomegalovirus (cCMV) among causes of bilateral hearing loss in young French children.

Study Design: Children <3 years old with hearing loss were prospectively included at their first visit to a referral center. Cytomegalovirus polymerase chain reaction was performed on dried blood spots from Guthrie cards.

Cochlear implantation and congenital deafness: perceptive and lexical results in 2 genetically pediatric identified population.

Objective: To evaluate the perceptive and linguistic results in 2 predefined genetic population of children with implants.

Study Design: Retrospective cohort study.

Setting: Otolaryngology department of a tertiary referral hospital.

Stapedectomy in children: causes and surgical results in 35 cases.

Objectives: To study children who had undergone stapedectomy at an age younger than 16 years to determine the causes (particularly frequency of congenital anomalies vs otosclerosis) and to analyze the functional results over the short-term, 1-year, and long-term postsurgery time course.

Design: Ten-year retrospective study covering 1998 to 2008.

Setting: Pediatric tertiary care centers.

Screening of SLC26A4, FOXI1 and KCNJ10 genes in unilateral hearing impairment with ipsilateral enlarged vestibular aqueduct.

Objective: To investigate the implication of SLC26A4, FOXI and KCNJ10 genes in unilateral hearing impairment associated with ipsilateral inner ear malformation (Enlargement of the vestibular aqueduct and/or Mondini dysplasia).

Methods: We have gathered 25 patients presenting unilateral hearing impairment and ipsilateral enlarged vestibular aqueduct. For each of the patients, we have analyzed SLC26A4, FOXI1 and KCNJ10 genes sequences.

Temperature-sensitive auditory neuropathy associated with an otoferlin mutation: Deafening fever!

Transient deafness associated with an increase in core body temperature is a rare and puzzling disorder. Temperature-dependent deafness has been previously observed in patients suffering from auditory neuropathy. Auditory neuropathy is a clinical entity of sensorineural deafness characterized by absent auditory brainstem response and normal otoacoustic emissions.

Velopharyngoplasty for noncleft velopharyngeal insufficiency: results in relation to 22q11 microdeletion.

Objective: To evaluate the results of velopharyngoplasty for velopharyngeal insufficiency (VPI) in relation to 22q11 deletion or nonsyndromic VPI.

Design: Retrospective study.

Setting: Academic medical center.

Cochlear implant magnet displacement during magnetic resonance imaging.

Objective: Magnet displacement is a rare but possible complication in patients with cochlear implants. We report 1 case in a young child that occurred during magnetic resonance imaging scanning, despite precautionary measures taken by the surgeon and the radiographer.

Study Design: Retrospective case report.

The presentation and management of laryngeal cleft: a 10-year experience.

Objective: To review the presentation and associated congenital abnormalities of laryngeal cleft and present guidelines for its evaluation and management.

Design: A 10-year retrospective study (1994-2004) with institutional review board approval.

Setting: Two pediatric tertiary care medical centers.