I. Rouillon

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). The main audiometric features and outcomes of the groups were analyzed. Results: The mean age at implantation was 10.15 years (range, 2.5-21 years). The mean preoperative score for the discrimination of open-set words was 48%; this score increased to 74% at 12 months and 81% at 24 months after the CI procedure (G1 to G5, respectively: 79/62/77%, 50/81/88%, 59/75/86%, 35/74/67%, and 39/69/80%). Children who were implanted after 10 years of age did not improve as much as those who were implanted at a younger age (open-set word list speech perception [OSW] score at 12 months: 62% vs 83%; P = .0009). Shorter delays before surgery were predictive of better performance (P = .003). Inner ear malformation and SLC26A4 mutations were not predictive of the outcome. Conclusions: CIs provide better results compared with hearing aids in children with residual hearing. Factors that may impact the benefits of CIs in patients with residual hearing are age, delay in performing the CI procedure, which ear is implanted, and initial underestimation of the patient’s hearing difficulties.

Newborn hearing screening: Prevalence and medical and paramedical treatment of bilateral hearing loss in a neonatal series in the Île-de-France region of France

OBJECTIVESnWe report results for newborn hearing screening in a cohort of children born in the Île-de-France region of France, as part of a national screening program set up by the French national health insurance agency.nnnMATERIALS AND METHODSnA prospective study was performed on neonates undergoing hearing screening by automated auditory brainstem response at 35 dB in maternity departments between 2005 and 2011. In case of positive findings, a further check was performed; if this was also positive in one or both ears, the child was referred to the diagnostic center.nnnRESULTSnThe study recruited 27,885 births; 96% of neonates were tested. Retest was positive in 0.84% of cases. Bilateral hearing loss was diagnosed in 0.63% of infants. Fifty-nine percent of these had ≥ 1 risk factor. Hearing normalized by end of follow-up in 25% of cases. Hearing loss was moderate in 59% of hearing-impaired children, severe in 12% and profound in 29%. Mean age at hearing aid fitting ranged from 4 months in profound hearing loss to 11.4 months in moderate hearing loss. In children receiving a cochlear implant, mean age at implantation was 14 months.nnnCONCLUSIONnNewborn hearing screening is now public policy. It is effective in terms of exhaustiveness, age at diagnosis and early management. Caution is appropriate in the treatment of moderate hearing loss. In case of moderate hearing loss associated with otitis media serosa, transtympanic aerators should be suggested as of the age of 6 months to enable hearing threshold measurement. Hearing aid fitting can then be considered around 9 months of age if hearing has not improved.

How to perform ABR in young children.

The diagnosis of hearing loss, especially in the context of newborn hearing screening, is mostly based on auditory brainstem response (ABR). According to the official CCAM nomenclature, ABR consists of recording early auditory evoked potentials to detect thresholds, study conduction times and measure amplitudes (corresponding to codes CDQP006 when performed without general anesthesia, and CDQP014 when performed with general anesthesia). ABR must be rigorously performed and interpreted, always in combination with a complete ENT examination and behavioral audiometry as soon as possible. In order to obtain good quality recordings, ABR must be performed with the infant totally immobile, during a nap. Several protocols can be used according to the childs age in order to obtain good quality sleep. ABR contribute to a precise hearing diagnosis, allowing early management by the first months of life.

Long term results after cochlear implantation: Schooling and social insertion of teenagers and young adults.

INTRODUCTIONnTo analyse the long-term impact of cochlear implantation (CI) in deaf patients on perception and language, and on schooling and social insertion.nnnMETHODnA total of 131 profoundly deaf patients that had unilateral CI, aged at follow up 16 to 26 years old and with 5 years minimum of follow up were included for the study. 84 of them had profound congenital deafness (Gc) and 47 had progressive deafness (Gp). In Gc, the mean age at CI was 5 years (3-16), the mean FU was 15 years. In Gp, the mean age at CI was 9 years (3-17 years), the mean FU was 11 years. The perceptive scores (open set sentences (OSS), word in open set sentences (WSS)), the intelligibility rate (SIR), the lexical scores (EVIP) were analysed and compared to the Schooling Status (SS) & Social Insertion (SSSI) (University/Working/Handicapped environment) and Classified as (Mainstream or Specialized). Both groups were compared.nnnRESULTSnThe mean results for Gc and Gp respectively were for the OSS score: 67.1% and 80.7% (P=0.009) and the SIR: 4.5 and 4.8 (P=0.049). EVIP scores were: in Gc, 56% of patients had normal or≥+1xa0SD; in Gp 72% of patients had normal or≥+1xa0SD. The SSSI: 63% in Gc and 83% in Gp were in Mainstream Schooling. Low level of EVIP scores were linked to Specialized environment in both groups (P=0.01, P=0.04).nnnDISCUSSIONnLong-term results show that differences have to be expected whether implanted children had congenital or progressive deafness. In both groups, strong correlations remain between perceptive results, lexical scores and the SSSI.nnnCONCLUSIONnOn the long term the lexical level remains a relevant tool to assess the evolution of implantees. For those who do not reach a near to normal lexical level, impact on the schooling and the social insertion has to be expected.

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

PURPOSEnOutpatient 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.nnnMETHODnThis was a retrospective study that took place in a tertiary care center. Eighty-three children (34 girls and 49 boys) had performed ABR under pentobarbital (GPent) or melatonin (GMel) between 2013 and 2014 and were included. All children had associated neurological or behavioral disorders or had failed a previous ABR using another technique. Success rate, defined as completed binaural investigation, delay, and duration of sleep (minutes), as well as side effects, were compared between GPent and GMel.nnnRESULTSnThere were 56 patients in GMel and 27 in GPent, with a mean age at test of 3 years and 10 months (1-13 years) and 4 years and 1 month (1-14.5 years), respectively. Success rate was 76.8% and 88.8%, respectively (p > .05), sleep duration was 23 and 153 min (p < .0001), and mean delay was 35 and 54 min. No side effects have been reported.nnnCONCLUSIONSnMelatonin is a drug widely used, particularly for electroencephalogram in children. Sleep duration allowed a success rate that was comparable to pentobarbital. Melatonin seems to be an efficient alternative to pentobarbital for pediatric ABR.

Childhood psychogenic hearing loss: Identification and diagnosis

Psychogenic hearing loss, formerly known as functional or non-organic hearing loss, is a classic cause of consultation in infantile audiology. Risk factors include female gender, and age 8 or 12 years. Onset is relatively sudden, without impact on schooling or voice quality. Audiometric signs comprise non-superimposable audiometric thresholds (variable audiometric results), bilaterality, flat mean audiometric curve, and discrepancy between pure-tone and speech audiometry. The child needs reassuring during audiometric examination: attention-diversion techniques may be effective. Objective audiometry allows positive diagnosis, followed by rehabilitation and psychological care.

How I do it Cochlear implantation and magnet removal: A silicone mold to maintain the transmitter coil

G. was diagnosed with bilateral profound deafness and had homozygous mutation of connexine 26. He received a left cochlear implant (CI24 M Cochlear) in 2001 at 4 years old. Because of severe and constant pain on the left side, he was explanted in March 2008 and was right side implanted in September 2008 (CI24RE Cochlear). In November 2010, right sided-headaches and pain developed around the implant location, associated with fainting spells that would increase when the transmitter coil was touching the inner magnet. The implant could no longer be used. Skin and soft tissue were normal, cerebral CT scan was normal as well. The auditory tests were good. No etiology was found in cutaneous allergy tests, blood tests, and vertebral column radiographies studies. Psychological support did not improved symptoms. As a matter of fact, all attempts to bring the magnet at a short distance from the device raised again the pain. Because of the boy’s desire to wear his implant again, we decided to surgically take the magnet out. After surgery, pain and headaches completely resolved and the boy wore his implant again. With the retainer disk proposed by Cochlear (Fig. 1), his transmitter coil fell all the time. As the boy had a very short haircut, the idea was to maintain the transmitter coil on the skull with a larger sticking surface. We decided to use a new mold. Using the

Pediatric cochlear reimplantation: Decision-tree efficacy

OBJECTIVESnThe context leading to pediatric cochlear reimplantation (CreI) can be complex. The objectives of this study were to define initial CreI indications, analyze final diagnosis and draw up a decision-tree.nnnMETHODSnA retrospective study included patients undergoing CreI between 2005 and 2015. Demographic characteristics, CreI circumstances and technical reports were collected. Circumstances indicating CreI were classified in 3 groups: performance decrement, suspected device failure, or medical. After CreI, final diagnoses were classified in 2 groups: confirmed failure (DFail) or medical (DMed).nnnRESULTSn69 out of 734 cochlear implantation surgeries were for CreI (8%). Manufacturers reports were available in 64 cases (93%). Two principal causes were found: trauma and infection. Initial indications were: performance decrement: 27%; device failure: 56%; and medical: 17%. Final diagnoses were: DFail: 72%; and DMed: 28%. Initial indication and final diagnosis were similar in 86% of cases. The majority of the 14% initial indication errors belonged to the performance decrement group. Traumatic causes correlated with risk of initial indication error (P=0.039).nnnCONCLUSIONnApart from spontaneous device failure, the two causes of CreI were infection and trauma. Using the present decision algorithm, half of the complex cases were resolved after CreI.

Sequential bilateral cochlear implants in children and adolescents: Outcomes and prognostic factors

OBJECTIVESnCochlear implants (CI) have enabled an increasing proportion of deaf children to develop oral communication. Despite the well-known benefits of bilateral implantation, many of these children and teenagers have only a unilateral implant. The aim of this study was to evaluate the benefits of sequential bilateral CI and the influence of relevant factors on outcome.nnnMATERIAL AND METHODSnA single-center retrospective study included 109 children and adolescents who received a second sequential CI between 2008 and 2016. Subjects were evaluated before sequential implantation and subsequently at 3, 12 and 24 months, on Speech Intelligibility Rating and speech perception tests: Categories of Auditory Performance, word and sentence recognition in silence and in noise. The influence of inter-implant interval and performance with the first CI were analyzed.nnnRESULTSnIn the majority of patients, sequential CI provided significant improvement in speech and intelligibility perception. These benefits were seen not only for short but also for long inter-implant intervals. Some subjects with poor performance with their first implant showed significant progression after sequential bilateral implantation.nnnCONCLUSIONnIn view of the benefits of sequential bilateral CI, we suggest that a second CI should be proposed to all unilaterally implanted children and adolescents, regardless of inter-implant interval and initial performance with the first CI. Further studies need to be conducted to identify prognostic factors for success in sequential contralateral implantation.

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. The frequent use of fine and pre-curved electrodes particularly with the use of an insertion tool necessitates routine postoperative radiological evaluation of the electrode array. Our cases demonstrate the benefit of systematic imaging including the possible use of the Cone Beam CT intraoperatively.