Audiologic findings in patients with hypophosphatemic rickets/osteomalacia

This paper is a review of the connection between sensorineural hearing loss and rickets.

Equivalent noise level generated by drilling onto the ossicular chain as measured by laser Doppler vibrometry: A temporal bone study

Background: Inadvertent drilling on the ossicular chain is one of the causes of sensorineural hearing loss (HL) that may follow tympanomastoid surgery. A high-frequency HL is most frequently observed. It is speculated that the HL is a result of vibration of the ossicular chain resembling acoustic noise trauma. It is generally considered that using a large cutting burr is more likely to cause damage than a small diamond burr. Aim: The aim was to investigate the equivalent noise level and its frequency characteristics generated by drilling onto the short process of the incus in fresh human temporal bones. Methods and Materials: Five fresh cadaveric temporal bones were used. Stapes displacement was measured using laser Doppler vibrometry during short drilling episodes. Diamond. and cutting burrs of different diameters were used. The effect of the drilling on stapes footplate displacement was compared with that generated by an acoustic signal. The equivalent noise level (dB sound pressure level equivalent [SPL eq]) was thus calculated. Results: The equivalent noise levels generated ranged from 93 to 125 dB SPL eq. For a 1-mm cutting burr, the highest equivalent noise level was 108 dB SPL eq, whereas a 2.3-mm cutting burr produced a maximal level of 125 dB SPL eq. Diamond burrs generated less noise than their cutting counterparts, with a 2.3-mm diamond burr producing a highest equivalent noise level of 102, dB SPL eq. The energy of the noise increased at the higher end of the frequency spectrum, with a 2.3-mm cutting burr producing a noise level of 105 dB SPL eq at 1 kHz and 125 dB SPL eq at 8 kHz. In contrast, the same sized diamond burr produced 96 dB SPL eq at 1 kHz and 99 dB at 8 kHz. Conclusion:This study suggests that drilling on the ossicular chain can produce vibratory force that is analogous with noise levels known to produce acoustic trauma. For the same type of burr, the larger the diameter, the greater the vibratory force, and for the same size of burr, the cutting burr creates more vibratory force than the diamond burr. The cutting burr produces greater high-frequency than lower-frequency vibratory energy.

The effects of mass loading the ossicles with a floating mass transducer on middle ear transfer function

Hypothesis: The aim of this study was to measure the mass loading effect of an active middle-ear implant (the Vibrant Soundbridge) in cadaver temporal bones. Background: Implantable middle ear hearing devices such as Vibrant Soundbridge have been used as an alternative to conventional hearing aids for the rehabilitation of sensorineural hearing loss. Other than the obvious disadvantage of requiring implantation middle ear surgery, it also applies a direct weight on the ossicular chain which, in turn, may have an impact on residual hearing. Previous studies have shown that applying a mass directly on the ossicular chain has a damping effect on its response to sound. However, little has been done to investigate the magnitude and the frequency characteristics of the mass loading effect in devices such as the Vibrant Soundbridge. Methods: Five fresh cadaver temporal bones were used. The stapes displacement was measured using laser Doppler vibrometry before and after the placement of a Vibrant Sound-bridge floating mass transducer. The effects of mass and attachment site were compared with the unloaded response. Measurements were obtained at frequencies between 0.1 and 10 kHz and at acoustic input levels of 100 dB sound pressure level. Each temporal bone acted as its own control. Results: Placement of the floating mass transducer caused a reduction of the stapes displacement. There were variations between the bones. The change of the stapes displacement varied from 0 dB to 28 dB. The effect was more prominent at frequencies above 1,000 Hz. Placing the floating mass transducer close to the incudostapedial joint reduced the mass loading effect. Conclusion: The floating mass transducer produces a measurable reduction of the stapes displacement in the temporal bone model. The effect is more prominent at high frequencies.

The search of a genetic basis for noise-induced hearing loss (NIHL)

Background and aim: Knowledge about the genetic factors responsible for noise-induced hearing loss (NIHL) is still limited. This study investigated whether genetic factors are associated or not to susceptibility to NIHL. Subjects and methods: The family history and genotypes were studied for candidate genes in 107 individuals with NIHL, 44 with other causes of hearing impairment and 104 controls. Mutations frequently found among deaf individuals were investigated (35delG, 167delT in GJB2, Delta(GJB6- D13S1830), Delta(GJB6- D13S1854) in GJB6 and A1555G in MT-RNR1 genes); allelic and genotypic frequencies were also determined at the SNP rs877098 in DFNB1, of deletions of GSTM1 and GSTT1 and sequence variants in both MTRNR1 and MTTS1 genes, as well as mitochondrial haplogroups. Results: When those with NIHL were compared with the control group, a significant increase was detected in the number of relatives affected by hearing impairment, of the genotype corresponding to the presence of both GSTM1 and GSTT1 enzymes and of cases with mitochondrial haplogroup L1. Conclusion: The findings suggest effects of familial history of hearing loss, of GSTT1 and GSTM1 enzymes and of mitochondrial haplogroup L1 on the risk of NIHL. This study also described novel sequence variants of MTRNR1 and MTTS1 genes.

Role of the Mitochondrial Mutations, m. 827A > G and the Novel m. 7462C > T, in the Origin of Hearing Loss

Samples from 30 deaf probands exhibiting features suggestive of syndromic mitochondrial deafness or from families with maternal transmission of deafness were selected for investigation of mutations in the mitochondrial genes MT-RNR1 and MT-TS1. Patients with mutation m. 1555A>G had been previously excluded from this sample. In the MT-RNR1 gene, five probands presented the m. 827A>G sequence variant, of uncertain pathogenicity. This change was also detected in 66 subjects of an unaffected control sample of 306 Brazilian individuals from various ethnic backgrounds. Given its high frequency, we consider it unlikely to have a pathogenic role on hereditary deafness. As to the MT-TS1 gene, one proband presented the previously known pathogenic m. 7472insC mutation and three probands presented a novel variant, m. 7462C>T, which was absent from the same control sample of 306 individuals. Because of its absence in control samples and association with a family history of hearing impairment, we suggest it might be a novel pathogenic mutation.

Análise das manifestações auditivas em individuos com hipertensão arterial sistêmica

Objective: To determine the prevalence of auditory manifestations in individuals with hypertension and analyze the association between hearing loss, systemic hypertension and quality of life in hypertensive patients. Method: This was a prospective, observational, case-control study, carried out from June 2010 to December 2013 at the University Hospital Onofre Lopes, in Natal, Brazil, which involved 120 patients of both sexes were analyzed with a diagnosis of hypertension and 120 patients without a diagnosis of hypertension. The audiological function was assessed by tonal and vocal audiometry. The quality of life was defines by the MINICHAL BRASIL questionnaire. Results: The prevalence of hearing loss was high in both groups (82.5 % and 75.8 %, in hypertension group and control, respectively, p=0.003). The sensorineural was the most common type of hearing loss (48.5 %) in hypertension group while conductive hearing loss was predominant (61.5 %) in the control group. There were no difference in the intensity of hearing loss between the groups (p=0,21). The main hearing complaint was hearing loss (51 %), followed by ear pain (14 %). There was worse quality of life in hypertensive individuals with hearing loss (p= 0.0001). Conclusion: Hypertensive individuals showed higher prevalence of auditory events, including hearing loss, sensorineural hearing loss is predominant . Hearing loss is associated with worse quality of life of hypertensive individuals even when these pressure values are within normal limits

Percepção de fala dos deficientes auditivos usando aparelho de amplificação com algoritmo de redução de ruído

Frequentemente, os indivíduos com perda auditiva têm dificuldade de entender a fala no ambiente ruidoso. OBJETIVO: O objetivo deste estudo foi avaliar clinicamente o desempenho dos indivíduos adultos com deficiência auditiva neurossensorial, com relação à percepção da fala, utilizando o aparelho de amplificação sonora individual digital com o algoritmo de redução de ruído denominado Speech Sensitive Processing, ativado e desativado na presença de um ruído. MATERIAL E MÉTODO: Este estudo de casos foi realizado em 32 indivíduos com deficiência auditiva neurossensorial de graus leve, moderado ou leve a moderado. Foi realizada a avaliação por meio de um teste de percepção de fala, onde se pesquisou o reconhecimento de sentenças na presença de um ruído, para obter a relação sinal/ruído, utilizando o aparelho auditivo digital. RESULTADOS: O algoritmo pôde proporcionar benefício para a maioria dos indivíduos deficientes auditivos, na pesquisa da relação sinal/ruído e os resultados apontaram diferença estatisticamente significante na condição em que o algoritmo encontrava-se ativado, comparado quando o algoritmo não se encontrava ativado. CONCLUSÃO: O uso do algoritmo de redução de ruído deve ser pensado como alternativa clínica, pois observamos a eficácia desse sistema na redução do ruído, melhorando a percepção da fala.

Achados audiológicos e eletrofisiológicos de indivíduos com a síndrome G/BBB

A síndrome G/BBB é uma condição rara, caracterizada por hipertelorismo, fissura de lábio e palato e hipospádia. Não foram encontrados trabalhos sobre a audição em indivíduos com esta síndrome. OBJETIVO: Investigar a função auditiva em pacientes com síndrome G/BBB quanto à ocorrência ou não de perda auditiva e a condução nervosa auditiva periférica e central. MATERIAL E MÉTODO: Catorze pacientes de 7 a 34 anos, do gênero masculino, com a síndrome G/BBB, foram avaliados por meio de otoscopia, audiometria, timpanometria e potenciais evocados auditivos de tronco encefálico (PEATE). Forma de Estudo: Estudo de série clínico prospectivo. RESULTADOS: Limiares audiométricos normais em 12 (66,7%) pacientes da amostra e alterados em dois (33,3%), sendo um com perda condutiva e um neurossensorial. Quanto ao PEATE, foram encontrados: latências absolutas da onda I normais em todos os pacientes, aumento das latências absolutas da onda III e V em dois e seis pacientes respectivamente; latências interpicos I-III, III-V e I-V aumentadas em quatro, três e oito pacientes, respectivamente. CONCLUSÃO: Perdas auditivas periféricas podem ocorrer na síndrome G/BBB. Há evidências de comprometimento das vias auditivas centrais em nível do tronco encefálico. Estudos com exames de imagem são necessários para maior clareza dos achados clínicos.

Auditory evaluation in patients with type 1 diabetes

Objectives: We performed a prospective clinical study of the cochleovestibular symptoms and the risk cofactors and characteristics of hearing loss in patients with type 1 diabetes.Methods: Group I consisted of 40 patients with type I diabetes, and group 2 consisted of 20 control subjects without diabetes. All participants answered a questionnaire, and their medical records were reviewed. They also were submitted to otorhinolaryngological examinations and to auditory tests (pure tone audiometry and acoustic immitance and auditory brain stem response [ABR] tests).Results: Dyslipidemia, hypertension, retinopathy, and diabetic neuropathy were not frequent in the patients of group 1, but incipient nephropathy was present in 47.5% of them. The most frequent cochleovestibular symptoms were tinnitus and hearing loss. Sensorineural hearing loss was found in 4 patients of group I and was predominantly bilateral, symmetric, and affecting the high frequencies, coexisting with normal vocal discrimination. These patients had a longer time from diabetes diagnosis and had poor glycemia control. A delay of ABR interpeak latency I-III was observed in 11.25% of the group I ears. All patients of group 2 presented normal audiograms and ABR tests.Conclusions: In group 1, the most frequent cochleovestibular symptoms were tinnitus and hearing loss. The sensorineural hearing loss was mild, symmetric, and predominantly high-frequency. A delay of ABR interpeak latencies was detected in the patients of group I who had normal audiometric thresholds.

Hypoacousis prevalence in Kaiowá and Guarani indigenous children

OBJETIVOS: identificar a prevalência de hipoacusia em crianças indígenas Kaiowá e Guarani. MÉTODOS: estudo transversal, com uma amostra de 126 crianças indígenas de zero a 59 meses da Terra Indígena de Caarapó, em Mato Grosso do Sul, Brasil. As crianças foram submetidas ao exame das emissões otoacústicas evocadas transitórias, que serviu como triagem auditiva. O reteste foi realizado nas crianças que apresentaram resultado alterado na triagem auditiva. Os casos que, no reteste, permaneceram alterados foram encaminhados para o exame da imitanciometria. RESULTADOS: na triagem auditiva, foram identificadas 25 (23,6%) crianças com resultado alterado; dessas, 17 apresentaram resultado normal no reteste e 6 permaneceram com resultado alterado, sendo encaminhadas para imitanciometria. A prevalência de hipoacusia identificada ao final do estudo foi de 5,6%, sendo 3 (2,8%) do tipo condutiva e 3 (2,8%) do tipo neurossensorial. Estas últimas foram encaminhadas à avaliação otorrinolaringológica complementar para confirmação diagnóstica. As alterações auditivas identificadas neste estudo não apresentaram diferenças significantes quanto ao sexo e grupo etário. CONCLUSÕES: a prevalência de alteração auditiva encontrada nesta população alerta para a necessidade de implantação de programas de saúde auditiva e sua articulação com outras ações desenvolvidas na atenção à saúde infantil dos Kaiowá e Guarani.

Avaliação audiológica em pacientes portadores de hipotireoidismo

Surdez no hipotireoidismo já foi descrita por diversos autores, porém a fisiopatologia é incerta. OBJETIVOS: Realizar avaliações audiológicas de pacientes com hipotireoidismo adquirido. MATERIAL E MÉTODOS: Grupos de estudo: hipotireoidismo (GH, n-30) e controle (GC, n-30). Parâmetros estudados: gênero, tempo do hipotireoidismo, co-morbidades, sintomas cócleo-vestibulares, exames bioquímicos e hormonais, limiares audiométricos, PEATE e EOAT. RESULTADOS: Todos os participantes eram mulheres; em GH 70% dos pacientes apresentavam tireoidite de Hashimoto e 60% tinham diagnóstico de hipotireoidismo há mais de 5 anos. Depressão e hipertensão foram as principais co-morbidades do grupo GH. Todos os pacientes de GH tinham valores elevados de TSH e 50% deles apresentavam diminuição de T4 livre. Surdez neurossensorial foi detectada em 22 orelhas de GH e em 7 de GC. PEATE mostrou-se normal nos participantes do GC e alterado em 10 orelhas do GH, havendo predomínio do aumento de L-V. EOAT estiveram ausentes em 12 orelhas de GH e em 4 de GC. CONCLUSÕES: Os pacientes com hipotireoidismo apresentaram mais sintomas cócleo-vestibulares, limiares audiométricos mais elevados, prolongamento das latências absolutas de L-V no PEATE e ausência ou redução as amplitudes das emissões otoacústicas. Tais alterações não estavam associadas aos níveis de TSH e T4 livre.

Processamento auditivo na perda auditiva unilateral: relato de caso

A perda auditiva unilateral representa grande risco para o atraso acadêmico, a comunicação, o desenvolvimento social e também para o processamento auditivo. Assim, o objetivo deste estudo foi avaliar as habilidades auditivas de localização, fechamento, figura-fundo, resolução temporal e ordenação temporal simples de um sujeito do gênero masculino, 17 anos de idade, com diagnóstico de perda auditiva sensorioneural unilateral de grau profundo, de causa idiopática, sem outros comprometimentos. O processo de avaliação constituiu da aplicação de um questionário, da realização da avaliação audiológica clínica convencional (audiometria tonal, logoaudiometria e imitanciometria) e de testes de processamento auditivo monóticos (SSI ipsilateral, Teste de fala filtrada) e dióticos (Localização sonora, Memória auditiva para sons verbais, Memória auditiva para sons não verbais, AFT-R). Apenas o teste de Localização sonora apresentou resultados alterados. Não foram relatadas queixas relacionadas à habilidade de localização sonora, atenção, discriminação e compreensão auditiva. No caso em estudo, a perda auditiva sensorioneural unilateral de grau profundo não pareceu restringir o desenvolvimento das habilidades do processamento auditivo avaliadas, exceto no que se refere à localização da fonte sonora.

Identificação de zonas mortas da cóclea por meio do teste TEN

O audiograma ou o relato do paciente não é suficiente para determinar a presença ou não de zonas mortas na cóclea (ZMC), nem identificar sua extensão. OBJETIVO: Investigar, utilizando o teste TEN, ZMC de indivíduos com perda auditiva neurossensorial (PANS). CEDALVI/ HRAC-USP-Bauru, de agosto de 2003 a fevereiro de 2004. TIPO DE ESTUDO: Estudo de coorte contemporânea com corte transversal. Casuística e Métodos: O TEN foi aplicado nos grupos G1 (5 mulheres com limiares tonais aéreos dentro do padrão de normalidade); G2 (4 mulheres e 5 homens com PANS moderada plana); G3 (19 mulheres e 24 homens com PANS com o grau variando entre leve a profundo). RESULTADOS: Para G1, o valor de TEN para eliminar o tom de teste foi, em média, próximo ao limiar absoluto para todas as freqüências. Não foi observada ZMC em nenhuma das orelhas testadas do G2. Para as 76 orelhas do G3, 6 não apresentaram indício de ZM. CONCLUSÕES: O TEN é efetivo para indicar ZMC em indivíduos com PANS descendente. Há evidência de diferença na detecção do tom puro na presença de ruído entre indivíduos com PANS em altas freqüências e com PANS plana, pois se observou diferença significativa entre o limiar mascarado e absoluto apenas para PANS descendentes e não para as planas.

Manifestações otorrinolaringológicas em crianças com AIDS

Introduction: The study of otolaryngologic manifestations in children HIV + can lead to early diagnosis of AIDS, allowing specific treatment, responsible by reduced morbidity and mortality. Objectives: Detect the otolaryngologic manifestations in children with AIDS and alert to the importance of the early diagnosis. Study design: Clinical prospective. Material and method: We evaluated 22 children with AIDS assisted at Faculdade de Medicina de Botucatu (São Paulo, Brasil). The medical records were reviewed and the children were submitted to otolaryngologic and hearing acuity exams. Results: We evaluated 12 boys and 10 girls, whose ages ranged from 8 months to 12 years. In B and C clinical classification were included 18 children who were using anti-retroviral medicaments. Physical examination mainly indicated cervical lymphadenopathy (18 cases), paleness of the nasal mucous membrane with abundant mucous secretion over the nasal epithelium (15 cases) and retraction of tympanic membranes (seven cases). The main otolaryngologic diagnoses were: rhinosinusitis (16 cases), oral candidiasis (13 cases), inadequate eustachian tube function (seven cases) and recurrent tonsillar infections (six cases). Conductive hearing loss were detected in 4 children. No child presented sensorineural hearing loss. Conclusions: The main otolaryngologic manifestations presented by the children with HIV virus were rhinosinusitis, oral candidiasis, inadequate eustachian tube function and recurrent tonsillar infections. The allergic aspect of the nasal mucous membrane and the cervical lymphadenopathy were frequent signs and could alert the otolaryngologyst to AIDS during the exam.

Seqüência de moebius: Manifestações clínicas e avaliação auditiva

Introduction: Children with Moebius syndrome may present paralysis of cranial nerves III, IV, V, VI, VII, VIII, IX, X and XII, compromising motor and sensorial functions. Hands and feet defects (syndactyly, equinovarus and arthrogryposis) are frequently associated. These manifestations can be attributed to the use of misoprostol during pregnancy to induce abortion. Study design: Clinical prospective. Aim: To evaluate the main clinical manifestations, hearing acuity and possible etiologic factors in children with Moebius syndrome. Material and method: The children were submitted to clinical, otolaryngological and hearing acuity assessment. Hearing acuity was evaluated through behavioral tests, pure tone audiometry, tympanometry and auditory brainstem response (ABR). We investigated possible etiologic factors. Results: Three boys and two girls were evaluated. The main manifestations were: facial paralysis, paralysis of masseter muscle, defects in dental occlusion, retraction of tympanic membrane, equinovarus, oblique palpebral fissure and tongue atrophy. Conductive hearing loss was detected in three children and sensorineural hearing loss in one child. The use of misoprostol during pregnancy was reported by four mothers. Conclusions: The children with Moebius syndrome evaluated in the present study manifested palsies of various cranial nerves, especially V, VII and XII nerves, responsible for motor and sensorial alterations. Inadequate eustachian tube function associated to conductive hearing loss was frequent. The use of misoprostol during pregnancy was reported by the mothers and it was considered a possible etiologic factor.