Garabedian En

Medical and surgical complications in pediatric cochlear implantation.

OBJECTIVES To report complications of cochlear implantation (CI) in children and to analyze risk factors. DESIGN Retrospective study from January 1, 1990, through April 30, 2008, with a mean follow-up of 5.5 years (range, 1 month to 17 years). SETTING Tertiary academic center. PATIENTS Four hundred thirty-four patients younger than 16 years. Mean age at CI was 4.7 (range, 0.6-16.0) years. Forty-one children (9.4%) underwent CI when younger than 24 months. Forty-three (9.9%) had inner ear malformations. MAIN OUTCOME MEASURES Complications after CI, classified into early (0-8 days) or delayed (>8 days) and major or minor. Spontaneous failures of internal devices were excluded. Correlation to age at CI, local trauma, and inner ear malformations were analyzed using the chi(2) test. RESULTS Forty-three patients (9.9%) experienced complications. Delayed complications occurred in 28 patients (65.1%), with a mean delay of 2.2 (range, 0.1-8.4) years. Twenty-four patients (5.5%) had major complications, consisting of severe cutaneous infections (15 patients), magnet displacement (3), meningitis (2), cholesteatoma (2), cerebrospinal fluid leak (1), and electrode misplacement (1). Nineteen (4.4%) had minor complications, consisting of vertigo (9 patients), soft-tissue infection (5), persistent otitis media (4), and facial palsy (1). Complications led to reimplantation in 13 of the 43 patients (30.2%). Trauma to the mastoid area (14 patients) and inner ear malformations (51) were highly correlated with major delayed complications (P < .001) and early minor complications (P < .001), respectively. Young age at CI was not correlated with any type of complication. CONCLUSIONS Complications of CI in children are common, with trauma as a major factor. Inner ear malformations should prompt specific preventive management. Cochlear implantation in young children did not appear to be a risk factor in this study.

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. Setting This case is presented by the ENT Department of Armand Trousseau Paediatrics Hospital, Paris, France. Patient An 8-year-old child, implanted with a Freedom Contour Nucleus cochlear implant, was referred for a 1.5-T cerebral magnetic resonance imaging because of a progressive neurologic disorder. Conclusion This rare complication underlines the importance of risk information and preventive measures required, even in case of compatible devices, for performing a magnetic resonance imaging examination in patients wearing a cochlear implant with removable magnet.

Usher syndrome and cochlear implantation.

Objective To evaluate the symptoms leading to diagnosis and the quality of rehabilitation after cochlear implantation in Usher syndrome. Study Design Retrospective cohort study. Setting ENT department of a tertiary referral hospital. Patients Among 210 patients given an implantation in the Ear, Nose, and Throat department, 185 were congenitally deaf and 13 had Usher syndrome (7.0%). Five had a family history of Usher, and eight were sporadic cases. Eleven cases were Usher type I, one was Usher type III, and one was not classified. The age at implantation ranged from 18 months to 44 years (mean, 6 years 1 month). The mean follow-up was 52 months (range, 9 months to 9 years). Main Outcome Measures All patients had audiophonological and clinical examination, computed tomography scan of the temporal bones, ophthalmologic examination with fundoscopy, and an electroretinogram. Cerebral magnetic resonance imaging and vestibular examination were performed in 9 of 13 and 10 of 13 cases, respectively. Logopedic outcome measured preimplant and postimplant closed- and open-set word recognition and oral expression at follow-up. Results The most frequent initial sign of Usher syndrome was delayed walking, with a mean age of 20 months. Among the 172 other congenitally deaf children with implants, when deafness was not associated with other neurologic disorders, the mean age at walking was 14 months (p < 0.001). The fundoscopy was always abnormal after the age of 5 years, and the electroretinogram was abnormal in all cases. Vestibular function was abnormal in all but one case (nonclassified). The computed tomography scan and the magnetic resonance imaging were always normal. Logopedic results with cochlear implants showed good perception skills in all but one case. The best perceptive results were obtained in children implanted before the age of 9 years. Oral language had significantly progressed in 9 of 13 at follow-up. There was no relation between the visual acuity and the logopedic results. Conclusion The earliest clinical sign associated with deafness evoking Usher syndrome is late walking. The electroretinogram is the only reliable examination to enable the diagnosis. When severe profound deafness is associated with late walking, the electroretinogram should be systematically proposed. Logopedic results are linked to precocity of implantation, and early Ushers diagnosis contributes to optimize speech therapy.

A Case of Respiratory Depression in a Child With Ultrarapid CYP2D6 Metabolism After Tramadol

We discuss a case of severe respiratory depression in a child, with ultrarapid CYP2D6 genotype and obstructive sleep apnea syndrome, after taking tramadol for pain relief related to a day-case tonsillectomy.

Audiophonological results after cochlear implantation in 40 congenitally deaf patients: preliminary results

The aim of this study is to evaluate the prognostic factors of audiophonological results in cochlear implant in congenitally deaf patients. Between 1991 and 1996. 40 congenitally deaf children underwent cochlear implantation in our department, at an average age of 7 years (median: 5 years). The results of speech therapy were evaluated with a mean follow-up of 2 years and were classified according to four criteria: perception of sound, speech perception, speech production and the level of oral language. For each criterion, a score was established ranging from zero to four. These scores were weighted according to age such that the results before and after implantation only reflected the changes related to the implantation. The prognostic factors for good results were: a good level of oral communication before implantation, residual hearing, progressive deafness and implantation at a young age. On the other hand, poor prognostic factors were: the presence of behavioral disorders and poor communication skills prior to implantation. Overall, the major prognostic factor for a good outcome appeared to be the preoperative level of oral language, even if this was rudimentary.

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. RESULTS The analysis of SLC26A4 revealed only eight heterozygous SLC26A4 sequence variants, three of them being novel (p.Met147Ile, p.Asn538Asn and p.Leu627Arg). None of the patients carried a second mutation on the other allele. Moreover, the SLC26A4 locus was excluded by segregation analysis in two families. No mutations were present in FOXI1 and KCNJ10 genes. CONCLUSIONS Together, these data suggest that SLC26A4, FOXI1 and KCNJ10 are not major determinants in unilateral deafness and enlarged vestibular aqueduct compared with their implication in Pendred syndrome and non-syndromic bilateral enlarged vestibular aqueduct.

Craniofacial dermoids: an embryological theory unifying nasal dermoid sinus cysts.

Objective The nasal dermoid sinus cyst (NDSC) is an uncommon congenital lesion presenting as a large panel of midline craniofacial anomalies. The objective of this study was to review and reanalyze embryological hypotheses concerning NDSCs and to propose an embryological theory unifying the various anatomical characteristics of these lesions. The first case of frontal localization of a NDSC extending within the diploetic bone in a 9-month-old boy, presenting as a median frontal fistula with recurrent frontal swelling, 6 months after a mild frontal trauma is presented. Results Complete surgical removal was performed, and there was no evidence of either persistent or recurrent disease 2 years after his surgery. The embryological and anatomical origins of NDSCs are reviewed. This article reexamines and discusses major embryological theories on NDSC pathogenesis and proposes to refute the “prenasal space” theory of Grünwald and rehabilitate a forgotten embryological hypothesis, which unifies the main various clinical presentations of NDSCs.

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. PATIENTS Eleven of 45 patients with 22q11 microdeletion (group 1) and 9 patients without 22q11 microdeletion (group 2) with noncleft VPI (hypoplastic velum or hypodynamic velopharynx and deep pharynx) underwent velopharyngoplasty (midline pharyngeal flap with superior pedicle). Exclusion criteria included cleft palate, submucous cleft palate, all syndromic cases, and all associated malformations (except those related to 22q11 microdeletion in patients with DiGeorge syndrome). MAIN OUTCOME MEASURES Speech assessment before surgery using the Borel-Maisonny scale and at 9 months and 24 months after surgery. Velopharyngeal insufficiency was classified as normal, inconsistent, mild, moderate, and severe. RESULTS Before surgery, in group 1, 3 patients had mild and 8 had severe VPI, and in group 2, 1 had mild and 8 had severe VPI. Postoperative outcomes at 9 months showed that in group 1, 2 patients had excellent results (normal and inconsistent) and 9 had mild VPI, while in group 2, 6 patients had excellent results and 3 had mild VPI (P = .03). Postoperative outcomes at 24 months showed that in group 1, 10 patients had excellent results and 1 had mild VPI, while in group 2, 8 patients had excellent results and 1 had mild VPI. CONCLUSIONS Surgical treatment of noncleft VPI by pharyngoplasty was efficient in 10 of the 11 patients (91%) in the 22q11 group and in 8 of the 9 patients (89%) in the nonsyndromic group. Postoperative remission took longer for patients with the 22q11 microdeletion than for the control group. However, long-term results following surgical treatment were equally good in the 2 groups.

Mycophenolate mofetil as a treatment of steroid dependent Cogan's syndrome in childhood.

Cogans syndrome is a rare chronic inflammatory disorder which typically associates an ophthalmological and vestibulocochlear involvement, with a risk of systemic symptoms in 50-70% of cases. Autoimmune origin has been consolidated by the recent discoveries of Lunardi as a result of the dysregulation of the response of B and T lymphocytes. We report here a pediatric case of Cogans syndrome, with long term follow-up and complete vestibulocochlear and ophthalmologic examination. During the acute phase, early steroids treatment (prednisone 1mg/kg per day) was effective on ocular lesions and hearing loss. The patient required high steroid doses to maintain remission, suggesting the necessity for steroid sparing immunosuppressive agents. We introduced mycophenolate mofetil (MMF), an immunosuppressive agent mainly used in solid organ transplantation. After steroid withdrawal the patient remained in complete remission on MMF. This is the first report of MMF treatment in Cogans syndrome. MMF produces a targeted inhibition of the proliferation of B and T lymphocytes, particularly interesting in Cogans syndrome. Low long term toxicity and overall good tolerance make MMF a new treatment option in steroid dependent Cogans syndrome.

Head and neck infantile myofibromatosis : a report of three cases

Infantile myofibromatosis (IM) is a proliferative disorder characterized by the development of single or multiple nodular lesions arising from cutaneous, subcutaneous, muscular, bone or visceral structures. This proliferation may occur at any anatomical site, but in 30% of the cases it involves the head and neck. We report here three cases of head and neck IM occurring in young children and presenting as solitary lesions. The clinical heterogeneity and the misleading histopathological appearances can make the diagnosis difficult. The treatment is surgical but the low rate of recurrence and the possibility of spontaneous tumoral regression may lead to conservative surgery or therapeutic abstention.