Objective: To evaluate the benefits of a contralateral hearing aid on expressive and receptive la... more Objective: To evaluate the benefits of a contralateral hearing aid on expressive and receptive language development and auditory perception performance over a 36-month post-surgical period in children implanted on a single side with a cochlear device for bilateral pre-lingual profound sensorineural hearing loss. Methods: Seventy-five patients with idiopathic profound sensorineural hearing loss were included. The cases were divided into two groups: cochlear implant users (50 patients, "CI group") and cochlear implant plus hearing aid users (25 patients, "CI+HA group"). Language development and auditory performance were compared in the two groups during the first 3 years following cochlear implant surgery. The Preschool Language Scale-4 was used to assess language development and the LittlEars® Auditory Survey, Meaningful Auditory Integration Scale and Meaningful Use of Speech Scale were employed to assess auditory perception performance. Results: Language development in the CI+HA group was superior to that in the CI group at 6 months post-surgery, in terms of receptive and expressive language development; auditory perception performance was also superior in the CI+HA group, compared to the CI group. Conclusion: The use of a contralateral hearing aid in cochlear implanted children with prelingual sensorineural hearing loss positively contributes to language development and auditory perception performance.
Introduction: ANSD is a challenging problem. Objective: To present our experience on management o... more 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.
ORL; journal for oto-rhino-laryngology and its related specialties, 2007
This study aimed to assess cochlear functions in Behçet's disease and rheumatoid arthritis (R... more 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. 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). 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). 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 detec...
Otoacoustic emission (OAE) testing enables us to identify the cochlear component of a hearing dis... more Otoacoustic emission (OAE) testing enables us to identify the cochlear component of a hearing disorder and to monitor objectively minute changes in cochlear status undetectable by other audiological methods. Contralateral sound-induced suppression is mediated by medial superior olivary complex efferents which induce hyperpolarization counteracting the amplifying effects of outer hair cell (OHC) activity. The aim of this study was to assess functions of cochlea and its efferents in migraine using OAE testing and contralateral suppression of transiently evoked OAEs (TEOAE). Fifty-three migraineurs (106 ears) and 41 healthy subjects (82 ears) were included and pure tone audiometry (PTA), speech discrimination scores (SDS), distortion product OAE (DPOAE), TEOAE and contralateral suppression of TEOAEs were tested. PTA and SDS of migraineurs and controls were not different ( P > 0.05). DPOAEs were tested between 1 and 6 kHz and a significant difference was detected only at 5 kHz freque...
Objective. (1) To analyze reported speech perception outcomes in patients with inner ear malforma... more Objective. (1) To analyze reported speech perception outcomes in patients with inner ear malformations who undergo cochlear implantation, (2) to review the surgical complications and findings, and (3) to compare the 2 classification systems of Jackler and Sennaroglu. Data Sources. PubMed, Scopus (including Embase), Medline, and CINAHL Plus. Review Methods. Fifty-nine articles were included that contained speech perception and/or intraoperative data. Cases were differentiated depending on whether the Jackler or Sennaroglu malformation classification was used. A metaanalysis of proportions examined incidences of complete insertion, gusher, and facial nerve aberrancy. For speech perception data, weighted means and standard deviations were calculated for all malformations for short-, medium-, and long-term follow-up. Speech tests were grouped into 3 categoriesclosed-set words, open-set words, and open-set sentencesand then compared through a comparison-of-means t test. Results. Complete insertion was seen in 81.8% of all inner ear malformations (95% CI: 72.6-89.5); gusher was reported in 39.1% of cases (95% CI: 30.3-48.2); and facial nerve anomalies were encountered in 34.4% (95% CI: 20.1-50.3). Significant improvements in average performance were seen for closed-and open-set tests across all malformation types at 12 months postoperatively. Conclusions. Cochlear implantation outcomes are favorable for those with inner ear malformations from a surgical and speech outcome standpoint. Accurate classification of anatomic malformations, as well as standardization of postimplantation speech outcomes, is necessary to improve understanding of the impact of implantation in this difficult patient population.
Objective: The objective was to provide information about methods used and preliminary outcomes f... more 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 so...
ORL; journal for oto-rhino-laryngology and its related specialties, 2007
This study aimed to assess cochlear functions in Behçet's disease and rheumatoid arthritis (R... more 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. 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). 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). 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 detec...
ORL; journal for oto-rhino-laryngology and its related specialties, 2007
This study aimed to assess cochlear functions in Behçet's disease and rheumatoid arthritis (R... more 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. 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). 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). 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 detec...
Mucopolysaccharidosis is a hereditary lysosomal storage disease, which develops due to a deficien... more 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. 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. 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. The audiometric findings of the 9 patients followed with mucopolysaccharidosis were reported separately for each case. 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.
Background: Although cochlear implantation has been almost a standard otological procedure worldw... more 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.
International Journal of Pediatric Otorhinolaryngology, 2015
To evaluate the results of delayed cochlear impantion performed in childhood and puberty in the l... more To evaluate the results of delayed cochlear impantion performed in childhood and puberty in the light of speech language pathology assessments. 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. The preoperative MAIS, MUSS and language scores were significantly higher in older children compared to younger children (p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.01). After two years these scores were similar between the all age groups (p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;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&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;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&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.01). However, that change in the way of communication was not statistically significant in group 3 (p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;0.05). 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.
ANSD is a challenging problem. To present our experience on management of the children with ANSD ... more ANSD is a challenging problem. To present our experience on management of the children with ANSD with respect to clinical data. 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. 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 ...
International Journal of Pediatric Otorhinolaryngology, 2011
To analyze the association of GJB2 gene mutations with cochlear implant performance in children. ... more To analyze the association of GJB2 gene mutations with cochlear implant performance in children. 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. 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&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;0.05). GJB2 gene mutations do not impact on the outcome of cochlear implantation.
Objective: To evaluate the benefits of a contralateral hearing aid on expressive and receptive la... more Objective: To evaluate the benefits of a contralateral hearing aid on expressive and receptive language development and auditory perception performance over a 36-month post-surgical period in children implanted on a single side with a cochlear device for bilateral pre-lingual profound sensorineural hearing loss. Methods: Seventy-five patients with idiopathic profound sensorineural hearing loss were included. The cases were divided into two groups: cochlear implant users (50 patients, "CI group") and cochlear implant plus hearing aid users (25 patients, "CI+HA group"). Language development and auditory performance were compared in the two groups during the first 3 years following cochlear implant surgery. The Preschool Language Scale-4 was used to assess language development and the LittlEars® Auditory Survey, Meaningful Auditory Integration Scale and Meaningful Use of Speech Scale were employed to assess auditory perception performance. Results: Language development in the CI+HA group was superior to that in the CI group at 6 months post-surgery, in terms of receptive and expressive language development; auditory perception performance was also superior in the CI+HA group, compared to the CI group. Conclusion: The use of a contralateral hearing aid in cochlear implanted children with prelingual sensorineural hearing loss positively contributes to language development and auditory perception performance.
Introduction: ANSD is a challenging problem. Objective: To present our experience on management o... more 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.
ORL; journal for oto-rhino-laryngology and its related specialties, 2007
This study aimed to assess cochlear functions in Behçet's disease and rheumatoid arthritis (R... more 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. 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). 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). 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 detec...
Otoacoustic emission (OAE) testing enables us to identify the cochlear component of a hearing dis... more Otoacoustic emission (OAE) testing enables us to identify the cochlear component of a hearing disorder and to monitor objectively minute changes in cochlear status undetectable by other audiological methods. Contralateral sound-induced suppression is mediated by medial superior olivary complex efferents which induce hyperpolarization counteracting the amplifying effects of outer hair cell (OHC) activity. The aim of this study was to assess functions of cochlea and its efferents in migraine using OAE testing and contralateral suppression of transiently evoked OAEs (TEOAE). Fifty-three migraineurs (106 ears) and 41 healthy subjects (82 ears) were included and pure tone audiometry (PTA), speech discrimination scores (SDS), distortion product OAE (DPOAE), TEOAE and contralateral suppression of TEOAEs were tested. PTA and SDS of migraineurs and controls were not different ( P > 0.05). DPOAEs were tested between 1 and 6 kHz and a significant difference was detected only at 5 kHz freque...
Objective. (1) To analyze reported speech perception outcomes in patients with inner ear malforma... more Objective. (1) To analyze reported speech perception outcomes in patients with inner ear malformations who undergo cochlear implantation, (2) to review the surgical complications and findings, and (3) to compare the 2 classification systems of Jackler and Sennaroglu. Data Sources. PubMed, Scopus (including Embase), Medline, and CINAHL Plus. Review Methods. Fifty-nine articles were included that contained speech perception and/or intraoperative data. Cases were differentiated depending on whether the Jackler or Sennaroglu malformation classification was used. A metaanalysis of proportions examined incidences of complete insertion, gusher, and facial nerve aberrancy. For speech perception data, weighted means and standard deviations were calculated for all malformations for short-, medium-, and long-term follow-up. Speech tests were grouped into 3 categoriesclosed-set words, open-set words, and open-set sentencesand then compared through a comparison-of-means t test. Results. Complete insertion was seen in 81.8% of all inner ear malformations (95% CI: 72.6-89.5); gusher was reported in 39.1% of cases (95% CI: 30.3-48.2); and facial nerve anomalies were encountered in 34.4% (95% CI: 20.1-50.3). Significant improvements in average performance were seen for closed-and open-set tests across all malformation types at 12 months postoperatively. Conclusions. Cochlear implantation outcomes are favorable for those with inner ear malformations from a surgical and speech outcome standpoint. Accurate classification of anatomic malformations, as well as standardization of postimplantation speech outcomes, is necessary to improve understanding of the impact of implantation in this difficult patient population.
Objective: The objective was to provide information about methods used and preliminary outcomes f... more 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 so...
ORL; journal for oto-rhino-laryngology and its related specialties, 2007
This study aimed to assess cochlear functions in Behçet's disease and rheumatoid arthritis (R... more 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. 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). 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). 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 detec...
ORL; journal for oto-rhino-laryngology and its related specialties, 2007
This study aimed to assess cochlear functions in Behçet's disease and rheumatoid arthritis (R... more 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. 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). 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). 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 detec...
Mucopolysaccharidosis is a hereditary lysosomal storage disease, which develops due to a deficien... more 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. 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. 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. The audiometric findings of the 9 patients followed with mucopolysaccharidosis were reported separately for each case. 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.
Background: Although cochlear implantation has been almost a standard otological procedure worldw... more 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.
International Journal of Pediatric Otorhinolaryngology, 2015
To evaluate the results of delayed cochlear impantion performed in childhood and puberty in the l... more To evaluate the results of delayed cochlear impantion performed in childhood and puberty in the light of speech language pathology assessments. 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. The preoperative MAIS, MUSS and language scores were significantly higher in older children compared to younger children (p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.01). After two years these scores were similar between the all age groups (p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;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&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;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&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.01). However, that change in the way of communication was not statistically significant in group 3 (p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;0.05). 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.
ANSD is a challenging problem. To present our experience on management of the children with ANSD ... more ANSD is a challenging problem. To present our experience on management of the children with ANSD with respect to clinical data. 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. 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 ...
International Journal of Pediatric Otorhinolaryngology, 2011
To analyze the association of GJB2 gene mutations with cochlear implant performance in children. ... more To analyze the association of GJB2 gene mutations with cochlear implant performance in children. 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. 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&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;0.05). GJB2 gene mutations do not impact on the outcome of cochlear implantation.
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