Sclerosteosis is a rare genetic bone dysplasia disorder characterised by generalised craniotubular bone modelling. Alongside many clinical appearances marked in sclerosteosis, the auditory system is considerably compromised on several levels during the disease progression. Extensive otolaryngological research on the history of sclerosteosis, the clinical presentation of sclerosteosis, radiographic studies and the gene causing the condition had been documented. No studies had been found describing the audiological profiles, auditory functioning and abnormalities for subjects with sclerosteosis. Thus the object of this study aimed to describe the auditory profile of subjects with sclerosteosis. A cross-sectional descriptive research design and quantitative research approach was followed to investigate the auditory characteristics of subjects with sclerosteosis. Subjects were selected from a database of patients with confirmed diagnoses of sclerosteosis. Ten subjects responded and provided written informed consent. Test procedures included otoscopy, tympanometry, acoustic reflexes, diagnostic pure-tone airand bone-conduction audiometry, speech audiometry, distortion product otoacoustic emissions (DPOAE), auditory brainstem responses (ABR) and computed tomographic (CT) scans. The subjects were assessed with a comprehensive audiological test-battery within a single test session lasting approximately two hours. A CT scan was conducted on a separate occasion shortly after the audiological data were obtained. Normal type A tympanograms were obtained in 50% (n=10/20) of ears. All subjects presented with mixed hearing losses varying from moderate (5%; n=1), severe (55%; n=11) and profound (40%; n=8) degrees across ears. Hearing loss configurations ranged from rising (15%), sloping (35%) and air-conduction thresholds peaking at 2000 Hz (50%). Air bone gaps (ABG) were larger in older subjects, although not statistically significant (p>.05). The CT scans indicated anatomical abnormalities of the external auditory canal, tympanic membrane, middle ear space, ossicles, oval window, round window and the internal auditory canal. The progressive abnormal bone formation in sclerosteosis involved the middle ear, the round and oval windows of the cochlea and internal auditory canal. The progressive abnormal bony overgrowth, which is the hallmark of sclerosteosis, led to functional impairment at various levels in the auditory system. The current findings provided a comprehensive auditory profile for sclerosteosis. Results might be utilised alongside future research findings to direct criteria and audiological indications for surgical and audiological intervention. / Dissertation (MCommunication Pathology)--University of Pretoria, 2013. / gm2014 / Speech-Language Pathology and Audiology / Unrestricted

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Objetivo: Verificar o desempenho de crianças diagnosticadas com deficiência auditiva, de grau leve, condutiva e sensorioneural, no Teste da Habilidade de Atenção Auditiva Sustentada (THAAS) visando constatar se este teste sofre influência da presença de uma deficiência auditiva. Modelo: Estudo do Teste da Habilidade de Atenção Auditiva Sustentada-THAAS em três grupos: grupo 1 (G1) grupo controle, formado por crianças com audição normal; grupo 2 (G2) crianças com deficiência auditiva sensorioneural bilateral de grau leve e grupo 3 (G3) composto por crianças com perda auditiva condutiva bilateral de grau leve. Local: Divisão de Saúde Auditiva ? Hospital de Reabilitação de Aanomalias Craniofaciais ?Universidade de São Paulo (HRAC/USP). Participantes: 90 crianças com idade entre 7 e 11 anos de idade, sendo 30 de cada grupo. Intervenções: Audiometria Tonal Limiar, Imitanciometria e THAAS Resultados: não houve indícios estatisticamente significativos entre sexo e idade, em todos os grupos estudados. Os grupos sensorioneural e condutivo apresentaram desempenho inferior ao grupo controle, em todas as respostas do THAAS. Conclusões: o teste THAAS sofreu influência das perdas auditivas de grau leve, condutivas e sensorioneurais na população estudada, sendo o pior comprometimento para perdas sensorioneurais. / Objective: To verify the performance of children diagnosed with a hearing loss of mild degree, conductive and sensorineural, at Sustained Auditory Attention Ability Test (SAAAT), aiming to observe if this test suffers the influence of the presence of a hearing loss. Model: A study of the Sustained Auditory Attention Ability Test ? SAAAT in three groups: group 1 (G1) control group comprising children with normal hearing, group 2 (G2) children with a bilateral sensorineural hearing loss of a mild degree and group 3 (G3), comprising children with a bilateral conductive hearing loss of a mild degree. Place: Division for Hearing Health ? Craniofacial Anomaly Rehabilitation Hospita, University of São Paulo (HRAC/USP). Participants: 7 to 11 year old children, 30 for each group. Interventions: Pure Tone Audiometry, Immitance Acoustic and SAAAT. Results: There were not any statistically significant indications between sex and age in all studied groups. Sensorineural and conductive groups showed a lower performance related to control group in all answers for SAAAT. Conclusions: SAAAT test suffered the influence of conductive and sensorineural hearing losses of light degree in studied population, the worse compromise occurring for sensorineural losses.

Diss. (sammanfattning) Stockholm : Karol. inst., 2006. / Härtill 4 uppsatser.

Objetivo: Verificar o desempenho de crianças diagnosticadas com deficiência auditiva, de grau leve, condutiva e sensorioneural, no Teste da Habilidade de Atenção Auditiva Sustentada (THAAS) visando constatar se este teste sofre influência da presença de uma deficiência auditiva. Modelo: Estudo do Teste da Habilidade de Atenção Auditiva Sustentada-THAAS em três grupos: grupo 1 (G1) grupo controle, formado por crianças com audição normal; grupo 2 (G2) crianças com deficiência auditiva sensorioneural bilateral de grau leve e grupo 3 (G3) composto por crianças com perda auditiva condutiva bilateral de grau leve. Local: Divisão de Saúde Auditiva ? Hospital de Reabilitação de Aanomalias Craniofaciais ?Universidade de São Paulo (HRAC/USP). Participantes: 90 crianças com idade entre 7 e 11 anos de idade, sendo 30 de cada grupo. Intervenções: Audiometria Tonal Limiar, Imitanciometria e THAAS Resultados: não houve indícios estatisticamente significativos entre sexo e idade, em todos os grupos estudados. Os grupos sensorioneural e condutivo apresentaram desempenho inferior ao grupo controle, em todas as respostas do THAAS. Conclusões: o teste THAAS sofreu influência das perdas auditivas de grau leve, condutivas e sensorioneurais na população estudada, sendo o pior comprometimento para perdas sensorioneurais. / Objective: To verify the performance of children diagnosed with a hearing loss of mild degree, conductive and sensorineural, at Sustained Auditory Attention Ability Test (SAAAT), aiming to observe if this test suffers the influence of the presence of a hearing loss. Model: A study of the Sustained Auditory Attention Ability Test ? SAAAT in three groups: group 1 (G1) control group comprising children with normal hearing, group 2 (G2) children with a bilateral sensorineural hearing loss of a mild degree and group 3 (G3), comprising children with a bilateral conductive hearing loss of a mild degree. Place: Division for Hearing Health ? Craniofacial Anomaly Rehabilitation Hospita, University of São Paulo (HRAC/USP). Participants: 7 to 11 year old children, 30 for each group. Interventions: Pure Tone Audiometry, Immitance Acoustic and SAAAT. Results: There were not any statistically significant indications between sex and age in all studied groups. Sensorineural and conductive groups showed a lower performance related to control group in all answers for SAAAT. Conclusions: SAAAT test suffered the influence of conductive and sensorineural hearing losses of light degree in studied population, the worse compromise occurring for sensorineural losses.

Acromegalia à uma doenÃa endÃcrina rara. Poucos estudos avaliaram sua associaÃÃo com deficiÃncia auditiva (DA) ou distÃrbios de conduÃÃo do impulso neural auditivo.Osresultados sÃo conflitantes. O objetivo à avaliar a transmissÃo auditiva central e perifÃrica em pacientes com acromegalia. AlÃm disso, avaliar a prevalÃncia e caracterÃsticas da DA em pacientes com acromegalia. Foi feito um estudo transversal com 36 pacientes com acromegalia de um ambulatÃrio especializado em Fortaleza-CearÃ. Foram excluÃdos pacientes com otoscopia anormal ou com histÃria familiar de deficiÃncia auditiva, uso de ototÃxicos ou exposiÃÃo a ruÃdo ocupacional. Inicialmente, o grupo foi avaliado por meio de audiometria tonal limiar. Considerou-se deficiÃncia auditiva (DA) quando a mÃdia dos tons puros foi > 25DbNA para baixas freqÃÃncias (250, 500, 1000 e 2000 Hz) ou altas freqÃÃncias (3000, 4000, 6000 e 8000 Hz).O grupo foi dividido em: acromegalia com DA (Acro DA) e sem DA (Acro NDA) e comparados quanto aos nÃveis hormonais e parÃmetros metabÃlicos. Posteriormente, este grupo de pacientes com acromegalia foi dividido em 2 subgrupos: com (n=16) e sem (n=20) diabetes ou intolerÃncia à glicose de jejum (DM/IGJ). Os resultados foram comparados entre os subgrupos e grupo controle (n=20) quantos aos parÃmetros dos potenciais evocados auditivos de tronco encefÃlico (PEATE). Para avaliar a normalidade da distribuiÃÃo foi utilizado o teste de Kolmogorov-Smirnov quando necessÃrio. Utilizou-se os testes qui-quadrado, t de Student e coeficiente de correlaÃÃo de Pearson. O nÃvel de significÃncia estatÃstica escolhido foi p<0.05 (statistical package for social sciences-SPSS 12.0). Dos 36 pacientes estudados, 14 (38,9%) mostraram DA sensorioneural (Acro DA), sendo em 9 casos bilateral e 5 unilateral. Nenhum apresentou DA mista ou condutiva. A prevalÃncia de DM/IGJ foi similar entre os grupos. As frequÃncias de 250, 3000, 4000, 6000 e 8000 Hz foram as mais afetadas e com padrÃo similar em ambos os lados.O grupo com acromegalia apresentou latÃncia da onda I em orelha direita aumentada em comparaÃÃo ao grupo controle (p=0,007), sem diferenÃa quanto aos intervalos interpicos em nenhum dos lados. Esse padrÃo foi o mesmo quando se comparou o subgrupo com DM/IGJ com o controle.TambÃm houve aumento da latÃncia da onda I na orelha direita em relaÃÃo à orelha esquerda no grupo com acromegalia. Em conclusÃo, mostrou-se uma elevada prevalÃncia de DA sensorioneural em pacientes com acromegalia.No entanto, nÃo se observou relaÃÃo dessa deficiÃncia com caracterÃsticas clÃnicas e metabÃlicas da doenÃa.Pacientes com acromegalia associada DM/IGJ mostraram um retardo no impulso neural no nervo auditivo unilateralmente à direita, evidenciando um efeito neuropÃtico nÃo homogÃneo dos distÃrbios do metabolismo dos carboidratos sobre o nervo auditivo. / Acromegaly is a rare endocrine disease. Few studies have evaluated its association with hearing loss (HL) or disturbance in conduction of the auditory neural impulse. The results are conflicting. The aim is to evaluate central and peripheral auditory transmission in acromegalic patients. Besides, to evaluate the prevalence and characteristics of HL in patients with acromegaly. A cross-sectional study was carried out on 36 patients with acromegaly in a specialized ambulatory in Fortaleza-CearÃ. Patients with abnormal otoscopy, family history of hearing loss, use of ototoxic drugs or occupational noise exposure were excluded. Initially, the group was evaluated by pure tone audiometry. HL was considered when pure tone average was > 25 DBHL for low frequencies (250, 500, 1000 e 2000 Hz) or high frequencies (3000, 4000, 6000 e 8000 Hz). The whole group was divided in: acromegaly with HL (Acro HL) and without HL (AcroNHL) and compared in regards to hormonal levels and metabolic parameters. Additionally that group of patients with acromegaly was divided into 2 subgroups: with (n=16) and without (n=20) diabetes mellitus/impaired glucose tolerance (DM/IGT). The results were compared among these subgroups and a control group (n=20) regarding the brainstem auditory evoked potentials (BAEP) parameters.Kolmogorov-Smirnov test was used to evaluate the normality of distribution when necessary. Qui-quadrado, Student t test and Pearson correlation coefficient were carried out. The level of statistical significance chosen was at p<0.05 (statistical package for social sciences-SPSS 12.0). Among 36 patients evaluated, 14 (38,9%) showed sensorioneural HL (Acro HL), being 9 bilateral and 5 unilateral cases. No one had mixed or conductive HL. The prevalence of diabetes/impaired glucose tolerance was similar between the groups. The frequencies 250, 3000, 4000, 6000 and 8000 Hz were the most affected and with a similar pattern in both ears. The group with acromegaly showed a prolonged peak I latency in the right ear in comparison to the control group (p=0,007), with no difference regarding the interpeak intervals in any sides. This pattern was the same when comparing the DM/IGT subgroup to controls. Also, we found a prolonged peak I latency in the right ear in relation to the left ear in the group with acromegaly.In conclusion, a high prevalence of sensorineural HL was shown in patients with acromegaly. Nevertheless, no correlation was observed between this dysfunction and clinical or metabolic characteristics of the disease. Patients with acromegaly associated with DM/IGT showed a delayed neural impulse in the auditory nerve unilaterally in the right side, evidencing a non-homogenous neuropathic effect of the carbohydrate metabolism disturbances on the auditory nerve.

Conductive hearing loss (otitis media) in young children can effect speech and language development. However, little is known about the effects of conductive loss on neural activity in the auditory system. Hypothesis: Conductive hearing loss will change resting activity levels at the inner hair cell synapse, and lead to auditory deprivation of central auditory pathways. A conductive loss was produced by blocking the ear canals in mice. Resting neural activity patterns were quantified in brainstem and midbrain using c-fos immuno-labelling. Experimental subjects were compared to normal hearing controls and subjects with cochlear ablation. Conductive loss subjects showed a trend in reduction in c-fos labelled cells in cochlear nucleus and the central nucleus of inferior colliculus compared to normal controls. Results seen in this study may indicate the influence of conductive hearing loss on the developing auditory brain during early postnatal years when the system is highly plastic.

Conductive hearing loss (otitis media) in young children can effect speech and language development. However, little is known about the effects of conductive loss on neural activity in the auditory system. Hypothesis: Conductive hearing loss will change resting activity levels at the inner hair cell synapse, and lead to auditory deprivation of central auditory pathways. A conductive loss was produced by blocking the ear canals in mice. Resting neural activity patterns were quantified in brainstem and midbrain using c-fos immuno-labelling. Experimental subjects were compared to normal hearing controls and subjects with cochlear ablation. Conductive loss subjects showed a trend in reduction in c-fos labelled cells in cochlear nucleus and the central nucleus of inferior colliculus compared to normal controls. Results seen in this study may indicate the influence of conductive hearing loss on the developing auditory brain during early postnatal years when the system is highly plastic.

Children frequently experience fluctuating conductive hearing loss during and following episodes of otitis media with effusion. With the prevalence of the disease increasing in the non-Aboriginal population in Australia, many children may be at risk of long-term learning and behavioural problems. There are conflicting findings in the research literature regarding the effects of this type of hearing loss. Although it is expected that the insertion of tympanostomy tubes (grommets) will reduce the duration and prevalence of both the disease and the conductive hearing loss, this does not always happen. For some primary school aged children it appears that experience with otitis media with effusion with or without grommet insertion is associated with various education problems including poor academic achievement and inappropriate behaviour. A current concern is whether or not these possible effects would continue to influence the learning and behaviour of children as they continue into their high school years. A review of the literature suggests that multiple factors including interrelationships between experience of otitis media with effusion, family and childcare environments may be involved in explaining why some students do not achieve as expected at school (Roberts et al., 2000). The literature further suggests there may be a cumulative effect in these factors, which introduces both quantitative and qualitative aspects to the discussion. A study was undertaken to identify the impact of otitis media with effusion and its associated sequelae on the learning and behaviour of high school students. Information from parents of high school students in Years 8 and 9 was used to identify students for inclusion in the study and to provide information on their medical and educational history as well as the parents' perceptions of various aspects of the students' learning and behaviour (including social skills). Three groups were formed: a Non-OME/Non-Grommet Group (n = 28), an OME/Grommet Group (n = 17) and an OME/Non-Grommet Group (n = 32). The TORCH, WRAT 3 and the Test of Syntactic Abilities (Screening Test) were administered to the students who also provided their own perceptions of specific aspects of their own learning and behaviour on a survey form. Teachers' perceptions on these areas were also collected. School academic records as well as data from school behavioural records were used. Analysis of the results revealed a range of mild effects. These included poorer academic results particularly for girls with a history of grommets, who also exhibited a lack of confidence in their social skills. An increase in behaviour problems for boys with a history of the disease (with or without grommets) was also found. The study identifies a number of associated teaching and learning issues including noise levels in childcare environments and school classrooms, current teaching and learning methodology and the training of new teachers. It recommends a number of areas for future research including more in-depth questioning of parents regarding their awareness of hearing problems in their children, the use of more fine-grained measures to assess learning outcomes and the identification of possible gender differences associated with the disease and experiences with grommets.

Objective: To evaluate the long-term safety and performance of four different vibroplasty couplers (round window, oval window, CliP and Bell coupler) in combination with an active middle ear implant. Methods: This was a multicentre, prospective, long-term study including 5 German hospitals. Thirty adult subjects suffering from conductive or mixed hearing loss were initially enrolled for the study, 24 of these were included in the final analysis with up to 36 months of postsurgical follow-up data. Bone conduction and air conduction were measured pre- and postoperatively to evaluate safety. Postoperative aided sound field thresholds and Freiburger monosyllable word recognition scores were compared to unaided pre-implantation results to confirm performance. Additional speech tests compared postoperative unaided with aided results. To determine patient satisfaction, an established quality-of-life questionnaire developed for conventional hearing aid usage was administered to all subjects. Results: Mean postoperative bone conduction thresholds remained stable throughout the whole study period. Mean functional gain for all couplers investigated was 38.5 ± 11.4 dB HL (12 months) and 38.8 ± 12.5 dB HL (36 months). Mean word recognition scores at 65 dB SPL increased from 2.9% in the unaided by 64.2% to 67.1% in the aided situation. The mean postoperative speech reception in quiet (or 50% understanding of words in sentences) shows a speech intelligibility improvement at 36 months of 17.8 ± 12.4 dB SPL over the unaided condition. The signal-to-noise ratio (SNR) improved by 5.9 ± 7.2 dB SNR over the unaided condition. High subjective device satisfaction was reflected by the International Inventory for Hearing Aids scored very positively. Conclusion: A significant improvement was seen with all couplers, and audiological performance did not significantly differ between 12 and 36 months after surgery.