Cochlear Implantation in Inner Ear Malformations: Considerations Related to Surgical Complications and Communication Skills.

INTRODUCTION: There are particular challenges in the implantation of malformed cochleae, such as in cases of facial nerve anomalies, cerebrospinal fluid (CSF) leaks, erroneous electrode insertion, or facial stimulation, and the outcomes may differ depending on the severity of the malformation. The aim of this study was to assess the impact of inner ear malformations (IEMs) on surgical complications and outcomes of cochlear implantation. METHODS: In order to assess the impact of IEMs on cochlear implant (CI) outcomes, 2 groups of patients with similar epidemiological parameters were selected from among 863 patients. Both the study group (patients with an IEM) and control group (patients with a normal inner ear) included 25 patients who received a CI and completed at least 1 year of follow-up. Auditory performance, receptive and expressive language skills, and production and use of speech were evaluated preoperatively and at least 1 year after implantation. Types of surgical complications and rates of revision surgeries were determined in each group. RESULTS: In the study group, the most common malformation was an isolated enlarged vestibular aqueduct (EVA) (44.8%). Overall, the patients with IEMs showed significant improvement in auditory-verbal skills. In general, the patients who had normal cochleae scored significantly better compared to patients with IEMs (p < 0.05). The complication rate was significantly lower in the control group compared to the study group (p = 0.001), but the rate of revision surgeries did not differ significantly (p = 0.637). CONCLUSION: It is possible to improve communication skills with CIs in patients with IEMs despite the variations in postoperative performances. Patients with EVA, incomplete partition type 2, and cochlear hypoplasia type 2 were the best performers in terms of auditory-verbal skills. Patients with IEMs scored poorly compared to patients with normal cochleae. CSF leak (gusher or oozing) was the most common complication during surgery, which is highly likely in cases of incomplete partition type 3.

Methods and preliminary outcomes of pediatric auditory brainstem implantation.

OBJECTIVE: The objective was to provide information about methods used and preliminary outcomes for pediatric ABI (auditory brainstem implant). STUDY DESIGN: An analysis of outcome was performed in children who received an ABI. METHODS: Twelve children received a MED-EL ABI system. Progress in audition and language was monitored through parental reports, questionnaires, profiles, and closed-set tests. RESULTS: The median number of active electrodes was 9 of 12. Seven of 12 users consistently respond to sound, and 5 of 12 do not. Highest performers can recognize words in small sets and have begun to use some words. CONCLUSION: Auditory brainstem implants appear to be beneficial for some pediatric patients who cannot benefit from traditional cochlear implant surgery. Benefits in the short-term can be recognition of environmental sounds, recognition of some words and very commonly used phrases, and the beginning use of words. Although some of our ABI users demonstrate no response to sound, they do want to wear their sound processors all waking hours. The cause of lack of response may be related to the second intervention, which might have led to displacement of the electrode array, or presence of additional handicaps or syndromes. However, the results are less than optimal. The relatively short postoperative follow-up duration is a considered weakness of this study.

The Effect of An Indirect Voice Therapy Approach on the Voice of Children With Vocal Fold Nodules: A Prospective Cohort Study.

PURPOSE: To investigate the effect of a combination indirect voice therapy approach on the management of vocal nodules in the pediatric population. METHODS: A prospective cohort study with thirty children (24 males and 6 females; mean age 8.8 ± 2.2) with a diagnosis of vocal fold nodule (VFN) was conducted. All participants were included in an 8-week voice therapy program consisting of vocal hygiene education and parental rule-setting. Videolaryngoscopic examination, auditory-perceptual evaluation, and acoustic voice analysis were performed immediately before and after the intervention. RESULTS: The comparison of videolaryngoscopic findings before and after the therapy revealed that the size of the nodules decreased significantly (P < 0.001). Findings from the laryngeal examination showed that the vast majority of children 73.3% (n = 22) had no VFN anymore after the intervention. The auditory-perceptual evaluation showed that overall severity of dysphonia decreased significantly [from 50 (40-70) to 38 (30-45), P < 0.001]. Jitter local (P = 0.031) and Jitter local abs (P = 0.043) parameters statistically differed after the therapy. There were not any statistically significant differences for mean F0, shimmer local (%), shimmer local (dB), and mean HNR. CONCLUSIONS: The voice therapy method in this study has a clear benefit on the voice of children with VFN and can be used in clinical practice. Specifically, clinicians should consider the parents as a core component of voice therapies and pay attention to vocal hygiene education for optimal management of VFN related childhood dysphonia.

Management of children with auditory neuropathy spectrum disorder (ANSD).

INTRODUCTION: ANSD is a challenging problem. OBJECTIVE: To present our experience on management of the children with ANSD with respect to clinical data. METHODS: This retrospective study included all children younger than 16 years of age who applied to the department between 2005 and 2013 (with the exception of newborn hearing screening NHS referrals). The data were derived from pure tone, OAEs and ABR tests, and further medical risk factors of the subjects were evaluated. RESULTS: ANSD was recognized in 74 ears of 40 children (B/U: 34/6) among 1952 children with SNHL (2.04%) detected among 9520 applicants to the department (0.42%). The clinical tests revealed that hearing loss greater than 15dB was present in both ears of 38 cases. The degree of hearing loss was profound in 48% children, severe in 12% children, moderate in 28% children, mild in 10% children and normal in 5% children. ABRs were absent/abnormal in 37/3 ears and CMs were detected in all. Acoustic reflexes were absent in all ears. Rehabilitation was managed by CI and hearing aids in 15 and 23 cases, respectively. FM system was given to two cases displaying normal hearing but poor speech discrimination in noisy environments. CONCLUSION: ANSD is a relatively challenging problem for the audiology departments because of its various clinical features and difficulties in management. Our patients with ANSD most commonly displayed profound hearing loss. The number of overlooked cases may be minimized by performing ABR and OAE in every case referred with the suspicion of hearing loss.

Audiologic evaluations of children with mucopolysaccharidosis.

INTRODUCTION: Mucopolysaccharidosis is a hereditary lysosomal storage disease, which develops due to a deficiency in the enzymes that play a role in the metabolism of glycosaminoglycans (GAG). The incidence of mucopolysaccharidosis is 1/25,000, with autosomal recessive inheritance (except for MPS II). Mucopolysaccharidosis occurs in seven different types, each with a different congenital deficiency of lysosomal enzymes. In mucopolysaccharidosis patients, even though progression of clinical findings is not prominent, the disease advances and causes death at early ages. Facial dysmorphism, growth retardation, mental retardation, and skeletal or joint dysplasia are the most frequently found symptoms in these patients. OBJECTIVE: The purpose of our study is to present the types of hearing loss types and tympanometric findings of patients with mucopolysaccharidosis referred to our clinic with suspicion of hearing loss. METHODS: After otorhinolaryngological examination, 9 patients with different types of mucopolysaccharidosis, underwent to immittance and audiometric evaluations, performed according to their physical and mental abilities, and ages, in order to determine their hearing thresholds. RESULTS: The audiometric findings of the 9 patients followed with mucopolysaccharidosis were reported separately for each case. CONCLUSION: Based on the high frequency of hearing loss in mucopolysaccharidosis patients, early and detailed audiological evaluations are highly desirable. Therefore, regular and systematic multidisciplinary evaluations are very important.

Delayed prelingual cochlear implantation in childhood and puberty.

OBJECTIVE: To evaluate the results of delayed cochlear impantion performed in childhood and puberty in the light of speech language pathology assessments. METHODS: Totally 49 children with prelingual profound hearing loss were included in the study. All children received a cochlear implant between the ages of 5 and 19 years (Group 1 aged between 5 and 9 years, group 2 aged between 10 and 14 years, group 3 aged between and 15 and 19 years). The MAIS, MUSS and PLS-4 scores of children were evaluated one month before, and one year and two years after the operation. The descriptive statistics included several independent variables; age of implantation; gender; trade name of the implant; preoperative duration of hearing aid use; preoperative special education, family support and additional handicap. These variables were categorical variables. We used repeated measures analysis of variance to test improvements in MAIS, MUSS and PLS scores, and whether this improvement depend on the independent variables. In addition, we also tested the interaction between time and the independent variables. RESULTS: The preoperative MAIS, MUSS and language scores were significantly higher in older children compared to younger children (p<0.01). After two years these scores were similar between the all age groups (p>0.4). There was a significant age and time interaction (p=0.005). That is, improvement continued in all age groups in a parallel way and group 2 reached to the level of group 1 after two years. However, group 3 almost reached to a plateau level after two years. The family support was associated with the MAIS, MUSS and language scores of the patients (p=0.01), and there was a family support-time interaction (p<0.0001). In group 1 and 2, the way of communication shifted from total communication (lip reading, sign language, auditory) to auditory-verbal communication in a significant number of the patients (p<0.01). However, that change in the way of communication was not statistically significant in group 3 (p>0.05). CONCLUSION: The decision of delayed cochlear implantation in children can be made in the light of following parameters. A good family support is most important. The patients must be wearing hearing aids regularly since early childhood, and preferably use the auditory verbal communication. Evaluation of the patient with MAIS, MUSS and PLS is important to understand the level of receptive and expressive communication level.

Cochlear Implantation in Extraordinary Cases.

BACKGROUND: Although cochlear implantation has been almost a standard otological procedure worldwide, it may still create a dilemma for the surgeon in some unusual instances such as Seckel syndrome, aural atresia and posterior fossa arachnoid cyst. CASE REPORT: Three extraordinary cases of cochlear implantation were reported. The first case was a case of Seckel syndrome with a cardiac pacemaker due to complete atrioventricular block. The second case had posterior fossa arachnoid cyst that had retrosigmoid cyst removal and cochlear implantation simultaneously. The last case had cochlear implantation in the ear with congenital aural atresia. All cases could be implanted successfully with full electrode insertion and good audiological outcome. Delayed facial paralysis that occurred in the patient with arachnoid cyst resolved spontaneously. CONCLUSION: This study addressed the efficiency of cochlear implantation in cases of Seckel syndrome, complete atrioventricular block managed with cardiac pacemaker, congenital aural atresia and posterior fossa arachnoid cyst. In addition, the retrosigmoid approach and cochlear implantation can be performed simultaneously.

Management of children with auditory neuropathy spectrum disorder (ANSD) / Tratamento de crianças com espectro da neuropatia auditiva (ENA)

ABSTRACT INTRODUCTION: ANSD is a challenging problem. OBJECTIVE: To present our experience on management of the children with ANSD with respect to clinical data. METHODS: This retrospective study included all children younger than 16 years of age who applied to the department between 2005 and 2013 (with the exception of newborn hearing screening NHS referrals). The data were derived from pure tone, OAEs and ABR tests, and further medical risk factors of the subjects were evaluated. RESULTS: ANSD was recognized in 74 ears of 40 children (B/U: 34/6) among 1952 children with SNHL (2.04%) detected among 9520 applicants to the department (0.42%). The clinical tests revealed that hearing loss greater than 15 dB was present in both ears of 38 cases. The degree of hearing loss was profound in 48% children, severe in 12% children, moderate in 28% children, mild in 10% children and normal in 5% children. ABRs were absent/abnormal in 37/3 ears and CMs were detected in all. Acoustic reflexes were absent in all ears. Rehabilitation was managed by CI and hearing aids in 15 and 23 cases, respectively. FM system was given to two cases displaying normal hearing but poor speech discrimination in noisy environments. CONCLUSION: ANSD is a relatively challenging problem for the audiology departments because of its various clinical features and difficulties in management. Our patients with ANSD most commonly displayed profound hearing loss. The number of overlooked cases may be minimized by performing ABR and OAE in every case referred with the suspicion of hearing loss.

Resumo Introdução: Espectro da neuropatia auditiva ainda é uma condição clínica desafiadora. Objetivo: Apresentar nossa experiência no tratamento de crianças com espectro da neuropatia auditiva em relação aos dados clínicos. Método: Este estudo retrospectivo incluiu crianças menores de 16 anos de idade que deram entrada no departamento entre 2005 e 2013 (com exceção de encaminhamentos para triagem auditiva neonatal). Foram avaliados os dados obtidos a partir dos exames de audiometria tonal, emissões otoacústicas (EOA), potencial evocado auditivo de tronco encefálico (ABR) e outros fatores de risco. Resultados: Das 1.952 crianças com perda auditiva neurossensorial (2,04%) detectadas dentre os 9.520 candidatos que deram entrada no departamento (0,42%), espectro da neuropatia auditiva foi reconhecida em 74 orelhas de 40 crianças (B/U: 34/6). Os testes clínicos revelaram que uma perda auditiva superior a 15 dB estava presente em ambas as orelhas em 38 casos. O grau de perda auditiva das crianças era profundo em 48%, grave em 12%, moderado em 28%, leve em 10%, e normal em 5%. ABR estava ausente/anormal em 37/3 orelhas e microfonia coclear foi detectado em todas as crianças. Reflexos acústicos estavam ausentes em todas as orelhas. A reabilitação foi tratada com implante coclear e aparelhos auditivos em 15 e 23 casos, respectivamente. Um sistema FM foi utilizado em dois casos que apresentavam audição normal, mas discriminação deficiente da fala em ambientes ruidosos. Conclusão: Espectro da neuropatia auditiva é um problema desafiador para os departamentos de audiologia, devido às suas várias características clínicas e dificuldades no tratamento. Em nossos pacientes a perda auditiva profunda foi a mais frequente. O número de casos negligenciados pode ser diminuído com a realização dos exames ABR e EOA em todos os casos encaminhados com suspeita de perda auditiva.

Association of GJB2 gene mutation with cochlear implant performance in genetic non-syndromic hearing loss.

OBJECTIVE: To analyze the association of GJB2 gene mutations with cochlear implant performance in children. METHODS: Sixty-five consecutive children who underwent cochlear implantation due to congenital profound senseurineural hearing between 2006 and 2008 were included in the study. In children, GJB2 gene mutation analysis was performed. Their auditory performance was assessed using MAIS, MUSS and LittlEARS tests. RESULTS: Twenty-two of sixty-five patients GJB2 mutations, and 35delG was the most frequent mutation. No significant difference was found between the auditory performance of mutation positive and negative children after one year follow up (p>0.05). CONCLUSION: GJB2 gene mutations do not impact on the outcome of cochlear implantation.

Audiologic evaluations of children with mucopolysaccharidosis / Avaliação audiológica em crianças com mucopolissacaridose

ABSTRACT INTRODUCTION: Mucopolysaccharidosis is a hereditary lysosomal storage disease, which develops due to a deficiency in the enzymes that play a role in the metabolism of glycosaminoglycans (GAG). The incidence of mucopolysaccharidosis is 1/25,000, with autosomal recessive inheritance (except for MPS II). Mucopolysaccharidosis occurs in seven different types, each with a different congenital deficiency of lysosomal enzymes. In mucopolysaccharidosis patients, even though progression of clinical findings is not prominent, the disease advances and causes death at early ages. Facial dysmorphism, growth retardation, mental retardation, and skeletal or joint dysplasia are the most frequently found symptoms in these patients. OBJECTIVE: The purpose of our study is to present the types of hearing loss types and tympanometric findings of patients with mucopolysaccharidosis referred to our clinic with suspicion of hearing loss. METHODS: After otorhinolaryngological examination, 9 patients with different types of mucopolysaccharidosis, underwent to immittance and audiometric evaluations, performed according to their physical and mental abilities, and ages, in order to determine their hearing thresholds. RESULTS: The audiometric findings of the 9 patients followed with mucopolysaccharidosis were reported separately for each case. CONCLUSION: Based on the high frequency of hearing loss in mucopolysaccharidosis patients, early and detailed audiological evaluations are highly desirable. Therefore, regular and systematic multidisciplinary evaluations are very important.

Resumo Introdução: Mucopolissacaridose (MPS) é uma doença hereditária de depósito lisossômico, decorrente da deficiência das enzimas que influenciam o metabolismo dos glicosaminoglicanos (GAGs). A incidência de MPS é de 1/25.000 habitantes, resultante de herança autossômica recessiva (exceto no caso de MPS II). MPS se apresenta na forma de sete tipos diferentes e, em cada tipo, ocorre uma deficiência congênita distinta de enzimas lisossômicas. Embora em pacientes com MPS os achados clínicos não sejam geralmente observados, a doença progride em seu curso natural e costuma levar a óbito pacientes muito jovens. Dismorfismo facial, retardo de crescimento, retardo mental e displasia esquelética ou articular são os sinais e sintomas mais frequentemente observados nesses pacientes. Objetivo: A finalidade do presente estudo foi apresentar os tipos de perda auditiva e os achados timpanométricos de pacientes com MPS encaminhados para nossa clínica com suspeita de perda auditiva. Método: Em seguida ao exame otorrinolaringológico, nove pacientes com diferentes tipos de MPS, foram submetidos a avaliações imitanciométricas e testes audiométricos de acordo com sua faixa etária eficiência física e mental, no intuito de identificar seus limiares auditivos. Resultados: Os achados audiométricos dos nove pacientes acompanhados por MPS foram descritos separadamente para cada caso. Conclusão: Baseado na alta frequência de perda auditiva em pacientes com MPS, avaliação audiológica precoce e detalhada é altamente desejável. Para tanto, é importante que sejam realizados avaliações multidisciplinares periódicas e sistemáticas.

Distortion product otoacoustic emission findings in Behçet's disease and rheumatoid arthritis.

BACKGROUND AND OBJECTIVE: This study aimed to assess cochlear functions in Behçet's disease and rheumatoid arthritis (RA) using otoacoustic emission testing (OAE), which objectively assesses outer hair cells in the cochlea. METHODS: Patients with Behçet's disease (n = 16) and RA (n = 11) as well as 20 controls were tested using pure tone audiometry and transiently evoked (TEOAEs) and distortion product OAEs (DPOAEs). RESULTS: Pure tone results in the Behçet's group were not significantly different from controls (p > 0.05). Pure tone results in the RA group were significantly different than in controls at 250, 500 and 6,000 Hz (p < 0.05). TEOAEs could be obtained in all participants. DPOAE amplitudes in the RA group and controls were similar (p > 0.05). DPOAE amplitudes were significantly higher in the Behçet's group than in controls at 1 and 2 kHz (p < 0.05). CONCLUSION: A subtle deterioration in hearing can be seen at low and high frequencies in RA, which is not related to outer hair cell dysfunction as detected by DPOAE testing. In the patients with Behçet's disease who have hearing thresholds within the normal limits, outer hair cell functions seem spared with an increased activity in the apical regions of the cochlea.