Remissão total do zumbido pediátrico: relato dos resultados de uma abordagem não medicamentosa da disfunção tubária / Total remission of pediatric tinnitus: reporting the results of a non-drug approach to tubal dysfunction / Remisión total del acufeno pediátrico: informe de los resultados de un enfoque no farmacológico a la disfunción tubárica

Introdução: Zumbido é um sintoma crescente na população pediátrica e sua multifatoriedade etiológica demanda amplas investigações para utilizar-se adequadas intervenções. Objetivos: relatar os efeitos de uma abordagem não medicamentosa em um caso de remissão total do zumbido pediátrico oriundo da disfunção tubária. Método: Este artigo apresenta um indivíduo com sete anos, do sexo feminino e com queixa de zumbido crônico associado a disfunção tubária, considerado escasso na literatura. Além disso, este estudo descreve as avaliações médicas (neurológica e otorrinolaringológica), a avaliação audiológica e psicoacústica do zumbido, o processo diagnóstico e a intervenção fonoaudiológica realizada. Utilizou-se uma abordagem não medicamentosa que contemplou exercícios miofuncionais orofaciais, Manobra de Valsalva e limpeza nasal. Resultados: Após um mês de intervenção, com a prática diária dos exercícios, o indivíduo estudado referiu ausência da percepção do sintoma e das demais queixas auditivas. Este resultado também foi constatado nas avaliações audiológicas. Conclusão: A abordagem não medicamentosa da disfunção tubária demonstrou bons resultados frente ao manejo do zumbido crônico, para o presente caso. (AU)

Introduction: Tinnitus is a growing symptom in the pediatric population and its etiological multifactorial demands extensive investigations to use appropriate interventions. Objective: report the effects of a non-medicated approach of a case of total remission of pediatric tinnitus from tube dysfunction. Methods: This article presents a seven-year-old female patient with a complaint of chronic tinnitus associated with tubal dysfunction, considered rare in the literature. Besides that, this study describes the medical evaluations (neurological and otorhinolaryngological), the audiological and psychoacoustic evaluations of the tinnitus, the diagnostic process and the speech-language intervention performed. It was used a non-medication approach that included orofacial myofunctional exercises, Valsalva maneuver and nasal cleaning. Results: After a month of intervention, with the daily practice of exercises, the studied subject reported the absence of perception of the symptom and other auditory complaints. This result was also verified in the audiological evaluations. Conclusion: The non-medication approach to tubal dysfunction has shown good results in relation to the management of chronic tinnitus for the present case. (AU)

Introducción: Acufeno es un síntoma creciente en la población pediátrica y su multifatoriedad etiológica demanda amplias investigaciones para utilizar adecuadas intervenciones. Objetivos: Informar los efectos de un enfoque no farmacológico de un caso de remisión total del acufeno pediátrico oriundo de la disfunción tubárica. Metodos: Este artículo presenta un sujeto con siete años, del sexo femenino y con queja de acufeno crónico asociado a disfunción tubárica, considerado escaso en la literatura. Además, este estudio describe las evaluaciones médicas (neurológica y otorrinolaringológica), la evaluación audiológica y psicoacústica del zumbido, el proceso diagnóstico y la intervención fonoaudiológica realizada. Se utilizó un abordaje no medicamentoso que contempló ejercicios miofuncionales orofaciales, Maniobra de Valsalva y limpieza nasal. Resultados: Después de un mes de intervención, con la práctica diaria de los ejercicios, el sujeto estudiado refirió ausencia de la percepción del síntoma y de las demás quejas auditivas. Este resultado también se constató en las evaluaciones audiológicas. Conclusión: El abordaje no medicamentoso de la disfunción tubárica demostró buenos resultados frente al manejo del acufeno crónico, para el presente caso. (AU)

[Imaging evaluation of cerebrospinal fluid otorrhea associated with inner ear malformation in children].

Objective: To explore the imaging evaluation of cerebrospinal fluid (CSF) otorrhea associated with inner ear malformation (IEM) in children. Methods: The clinical data of 28 children with CSF otorrhea associated with IEM confirmed by surgical exploration in Beijing Children's Hospital, from Nov, 2016 to Jan, 2021, were analyzed retrospectively,including 16 boys and 12 girls, aged from 8-month to 15-year and 8-month old, with a median age of 4-year old. The shapes of stapes were observed during the exploration surgery, and the imaging features of temporal bone high resolution CT(HRCT) and inner ear MRI pre- and post-operation were analyzed. Results: In 28 children with CSF otorrhea, 89.3%(25/28) had stapes footplates defect during exploration. Preoperative CT showed indirect signs such as IEM, tympanic membrane bulging, soft tissue in the tympanum and mastoid cavity. IEM included four kinds: incomplete partition type I (IP-Ⅰ), common cavity (CC), incomplete partition type Ⅱ (IP-Ⅱ), and cochlear aplasia (CA); 100%(28/28) presented with vestibule dilation; 85.7%(24/28) with a defect in the lamina cribrosa of the internal auditory canal. The direct diagnostic sign of CSF otorrrhea could be seen in 73.9%(17/23) pre-operative MRI: two T2-weighted hyperintense signals between vestibule and middle ear cavity were connected by slightly lower or mixed intense T2-weighted signals, and obvious in the coronal-plane; 100%(23/23) hyperintense T2-weighted signals in the tympanum connected with those in the Eustachian tube.In post-operative CT, the soft tissues in the tympanum and mastoid cavity decreased or disappeared as early as one week. In post-operative MRI, the hyperintense T2-weighted signals of tympanum and mastoid decreased or disappeared in 3 days to 1 month,soft tissues tamponade with moderate intense T2-weighted signal were seen in the vestibule in 1-4 months. Conclusions: IP-Ⅰ, CC, IP-Ⅱ and CA with dilated vestibule can lead to CSF otorrhea. Combined with special medical history, T2-weighted signal of inner ear MRI can provide diagnostic basie for most children with IEM and CSF otorrhea.HRCT and MRI of inner ear can also be used to evaluate the effect of surgery.

Patulous Eustachian tube and palatine defect in a Dachshund with chronic unilateral otitis externa and otitis media.

BACKGROUND: Patulous Eustachian tube (pET) is a rare dysfunction of the Eustachian tube described in humans. It is characterized by failure of the ET to close, resulting in unrestricted passage of air, sound and material between the nasopharynx and the middle ear. OBJECTIVE: To report a case of pET associated with otitis in a dog. ANIMAL: A 6-year old-female spayed Dachshund dog. METHODS AND MATERIALS: Otoscopic examination, cytological evaluation, culture and susceptibility, computerized tomography (CT), video-otoscopic flushing and surgery. RESULTS: Left ear otoscopic examination revealed erythema, purulent frothy discharge, ceruminous gland hyperplasia, stenosis and a partial tear of the tympanum. Cytological evaluation from the left external canal showed neutrophils, macrophages, rods and cocci. Aerobic culture showed predominantly multidrug-resistant Pseudomonas aeruginosa. The CT findings of the left ear included chronic changes in the external canal, marked lysis of the tympanic bulla and marked dilation of the ET. During video-otoscope flushing, saline drained through the mouth. Bilateral incomplete hypoplasia of the soft palate was noted. Total ear canal ablation and bulla osteotomy with ET dissection were curative. Histopathological findings were compatible with chronic otitis externa (OE) and media. CONCLUSION AND CLINICAL IMPORTANCE: To the best of the authors' knowledge, this is the first case of pET described in animals. The ET dysfunction and palatine defect were likely the cause of the otitis in this dog. Clinicians should investigate pET in animals with signs of OE characterized by frothy liquid and food fragments in the ear canal in addition to sneezing after drinking water.

The prolactin inducible protein/gross cystic disease fluid protein-15 deficient mice develop anomalies in lymphoid organs.

The Prolactin Inducible Protein (PIP) is a 15 kDa protein secreted by normal apocrine glands, including salivary, lacrimal and sweat glands. PIP levels are normally low in the mammary glands of healthy individuals, but high levels have been observed in pathological conditions of the breast such as benign breast cystic disease and breast cancer. While the function of PIP is not well elucidated, accumulating evidence strongly point to a role in both innate and adaptive immunity. Using PIP deficient mice (Pip-/- mice) our laboratory demonstrated that loss of PIP function led to impaired T helper type 1 response and cell mediated immunity. In the present study we provide additional supporting evidence showing abnormal lymphocytic distribution in primary and secondary lymphoid organs of Pip-/- mice. Significant morphological changes in the Eustachian tube, an immune-protected site where PIP is normally found, were also associated with the absence of PIP. Collectively, these results further support an immuno-regulatory role for PIP and have implications for a spectrum of immune-related illnesses including otitis media and hearing loss as well as breast cancer.

Paper patching for patulous eustachian tube.

BACKGROUND: Paper patching, a method that places cigarette paper over the most mobile quadrants of the tympanic membrane, is one of the treatment options for patulous eustachian tube (PET). AIMS/OBJECTIVES: The objective of this study was to compare the outcomes of two different treatment strategies for PET. MATERIAL AND METHODS: Twenty-three patients underwent paper patching of the tympanic membrane and 16 patients were treated with nasal saline irrigation with or without ipratropium bromide nasal spray. Medical records were reviewed for resolution of PET symptoms as categorical variables (complete remission, partial remission, or no improvement) with a minimum follow-up of 3 months. RESULTS: Immediately after undergoing paper patching, 20 of the 23 patients (87.0%) reported complete remission (CR). The percentage of CR after paper patching was 82.6% at 1 month and 65.2% at 3 months. A greater percentage of patients reported CR of aural symptoms in the paper patching group than in the nasal irrigation group at both 1 and 3 months after treatment (p < .05). CONCLUSIONS AND SIGNIFICANCE: Repetitive paper patching resolves aural discomfort in most PET patients for at least 3 months and can be considered as a first-line treatment option for PET in the outpatient setting.

Otologic disorders in Turner syndrome.

INTRODUCTION: Patients with Turner syndrome (TS) have craniofacial malformations, such as Eustachian tube hypoplasia and dysfunction and velar dysfunction, which foster acute otitis media. The aim of this study was to inventory pediatric otologic disorders in patients with TS at their first ENT consultation in our center. PATIENTS AND METHODS: We reviewed the ENT consultation data of pediatric TS patients followed in our center between 2005 and 2015: otoscopy, hearing threshold, and history of acute otitis media or ENT surgery. Data were compared according to karyotype: X monosomy (45,X), mosaic (45,X/46,XX), isochromosome (46,Xi [Xq]), X ring chromosome X (XrX), with Y material, and "other". RESULTS: Ninety patients, with mean age 11.9years (±4.8years) at first ENT consultation, were included: 29% showed tympanic abnormality on otoscopy, 21% had hearing loss, 24% had history of recurrent acute otitis media; 18% had undergone adenoidectomy, 24% T-tube insertion, and 5.6% tympanoplasty. No particular karyotype was associated with higher risk of hearing loss or acute otitis media. CONCLUSION: Patients with TS showed high prevalence of pediatric otologic disorders; they therefore require close and prolonged ENT follow-up.

Eustachian tube communicating with sphenoid sinus: report of a novel anatomical variant.

Reports of congenital anomalies of the Eustachian Tube (ET) are scarce, and often associated with chromosomal abnormalities. We report a unique case of a completely bony left Eustachian tube which communicated with the sphenoid sinus. This report details these findings and discusses the potential embryological basis and implications of such an unusual anatomy, in the context of a comprehensive literature review.

Acoustic Transmission Characteristics of a Eustachian Tube Volitionally Opened in Two Living Subjects.

OBJECTIVE: To assess the acoustic transmission characteristics of the Eustachian tube (ET) in living subjects in verified patent and closed ET states to facilitate the detection and quantification of ET function using acoustic measures such as sonotubometry. PATIENTS: The two subjects in this study had no history of ear disease nor previous ear surgery and were capable of volitionally opening and closing their ET. INTERVENTIONS: Tympanometry and otologic examinations were used to confirm ET patent and closed states by observing tympanic membrane movement with respiration and by acoustic immitance measurements during forced respiration. A series of 500-ms long chirps containing frequencies from 100 Hz to 10 kHz were introduced into the nasal cavity during both ET states and recorded by microphones in both the contralateral naris and external auditory canal. MAIN OUTCOME MEASURES: Acoustic energy transmission through the ET across the 0.1 to 10 kHz frequency range in the closed state versus the patent state. RESULTS: An increase in acoustic energy transmission occurs across the frequencies of 1 to 4 kHz between the closed and patent ET states, particularly in frequencies below 2.5 kHz. CONCLUSIONS: Results support sonotubometry as a potential diagnostic tool for ET dysfunction. Acoustic differences between the ET states manifest as a general increase in transmitted signal amplitude. Characterizing the acoustic properties in the verified patent and closed ET states allows investigators to more reliably interpret sonotubometric tests of ET function.

Avaliação auditiva periférica em crianças com síndrome de Down / Peripheral hearing evaluation in children with Down syndrome

Objetivo Caracterizar o sistema auditivo periférico de indivíduos com síndrome de Down, por meio da audiometria convencional e de altas frequências. Métodos Estudo do tipo transversal e observacional. Participaram 15 indivíduos com síndrome de Down, de ambos os gêneros, entre 7 e 15 anos de idade. Foram realizados os seguintes procedimentos: Meatoscopia, Timpanometria com pesquisa do reflexo acústico ipsilateral e contralateral, Audiometria Tonal, Audiometria Vocal e Audiometria de Altas Frequências. Resultados Houve predomínio de perda auditiva condutiva de grau leve, em uma ou ambas as orelhas. As médias dos limiares auditivos para a audiometria convencional ficaram abaixo de 20 dBNA e, para a audiometria de altas frequências, ficaram entre 20 e 40 dBNA. O coeficiente de correlação de Pearson revelou correlação moderada positiva, entre os limiares de 9 a 14 kHz e a idade. Conclusão De forma geral, não foram observadas diferenças significativas, quando comparadas as orelhas direita e esquerda de indivíduos com síndrome de Down, na audiometria tonal, imitanciometria e logoaudiometria. A maioria das crianças apresentou alteração de orelha média e perda auditiva condutiva. A audiometria de altas frequências sugere o início de prejuízo da função coclear, que pode estar associado às otites médias frequentes e/ou à degeneração coclear precoce. .

Purpose This study sought to characterize the peripheral auditory system of individuals with Down syndrome (DS) using conventional and high-frequency audiometry. Methods We performed a cross-sectional and observational study. Fifteen individuals with DS, who were of both genders and between 7 and 15 years of age, participated in this study. The following procedures were performed: otoscopy, tympanometry with ipsilateral and contralateral acoustic reflex, pure-tone audiometry, vocal audiometry and high-frequency audiometry. Results There was a predominance of mild conductive hearing loss in one or both ears. The mean hearing thresholds for conventional audiometry were below 20 dB HL and between 20 and 40 dB HL for high-frequency audiometry. The Pearson correlation coefficient indicated a moderate positive correlation between the 9-14 kHz thresholds and age. Conclusion Overall, no significant differences were observed when comparing the right and left ears of individuals with DS, in regards to pure-tone audiometry, immittance testing and speech audiometry. Most children showed middle ear abnormalities and conductive hearing loss. Moreover, high-frequency audiometry suggested the onset of impaired cochlear function, which may be associated with frequent otitis media episodes and/or early cochlear degeneration. .

Eustachian tube duplication: a unique anomaly.

Very few eustachian tube anomalies have been published in the literature and have consisted of descriptions of diverticula, hypoplasia/aplasia, fistula, or aberrant associated musculature. We present a girl with a novel anomaly consisting of a eustachian tube duplication that originates in the nasopharynx and exits posterior to a microtic and atretic ear. We review the literature on eustachian tube anomalies and also consider the derivation of this anomaly.

A novel, computed tomography guided, trans-cutaneous approach to treat refractory autophony in a patient with a patulous eustachian tube.

OBJECTIVE: We report a novel, computed tomography guided treatment for autophony associated with a patulous eustachian tube. METHODS: Case report and literature review of the management of patulous eustachian tube. RESULTS: A 36-year-old woman presented with disabling autophony. Otoscopic examination revealed a poorly mobile right tympanic membrane. High resolution temporal bone computed tomography excluded superior semicircular canal dehiscence. The patient was diagnosed with patulous eustachian tube but failed to achieve therapeutic benefit from conventional endoscopic placement of a silicone elastomer suspension implant adjacent to the eustachian tube. Subsequently, she underwent further silicone elastomer suspension implant placement via a trans-cutaneous, computed tomography guided approach. The patient achieved prompt symptomatic relief and remained symptom-free at six-month follow up. CONCLUSION: This is the first published description of treatment of patulous eustachian tube using a computed tomography guided, trans-cutaneous approach. It serves to highlight to otolaryngologists the fact that computed tomography guided treatment of patulous eustachian tube may control disabling symptoms in patients who have failed to respond to convention endoscopic management.

Technical feasibility of endoscopic eustachian tube catheter placement: a cadaveric analysis.

BACKGROUND: Patulous eustachian tube (pET) can result in transmission of sound from the pharynx to the middle ear (ME) via an abnormally patent ET. The objective of this study was to evaluate the technical feasibility of a reversible transnasal procedure for pET using an occluded silastic catheter to close the ET. METHODS: Ten sides were evaluated in five cadaver heads. Size 14, 16, and 18G catheters were occluded with bone wax to create a semirigid solid tube. They were placed transnasally, under endoscopic guidance through the ET orifice to span the entire ET length. Proper placement in the ME was confirmed by tympanotomy. Each attempt was graded on a four-point scale based on ease of placement: 3+, 2+, 1+, and 0. RESULTS: The 16G was the easiest to place with the best fit and was confirmed in the ME in eight cases. The 14G catheter was next easiest to place and was observed in the ME in seven cases. It generally had an extremely tight fit and propensity to impart mucosal trauma. The 18G was the most difficult to place and was observed in the ME in six cases. The catheter was noted to displace easily because of a loose fit. Tympanic membrane or ossicular injury was not noted in any trial. CONCLUSION: These cadaveric data suggest that a semirigid catheter provides the technical capability to reversibly occlude the ET via the transnasal endoscopic route. Further confirmation is necessary in human studies to determine its effectiveness for management of pET.

A rare case of accessory maxilla and bilateral Tessier no. 7 clefts, a 10-year follow-up.

A Tessier no. 7 cleft is a lateral facial cleft which originates from the oral cavity and extends towards the tragus, involving both soft-tissue and skeletal components. A male patient presenting with both maxillary jaw duplication and bilateral Tessier no. 7 clefts, which has been reported only twice in the literature, is described. Bilateral facial clefts, macrostomia and chondro-cutaneous remnants were noted, which were repaired and resected. With further growth, facial asymmetry and asymmetric facial nerve dysfunction became apparent. Radiographic examination showed an accessory maxillary jaw and a flattened and hypoplastic right coronoid process. A maxillary alveolar cleft was also present between the left second bicuspid and the second permanent molar. This case may represent an under-recognized phenotype with an unusual combination of maxillary jaw duplication, macrostomia, Tessier no. 7 clefts, and chondro-cutaneous remnants. A long-term follow-up of these patients is recommended as they often develop craniofacial deformities later in life.

Endoscopic ligation of the patulous eustachian tube as treatment for autophony.

OBJECTIVES/HYPOTHESIS: Patulous eustachian tube remains a challenging management problem in otolaryngology. The autophony experienced by this patient population can be severe, and as yet no reliable surgical method exists to reduce or eliminate this annoying symptom. Our objective was to develop a novel endoscopic technique to assist these patients. STUDY DESIGN: Longitudinal case series. METHODS: A prospective longitudinal study was conducted of a series of consecutive patients undergoing surgery for patulous eustachian tube. Under transnasal endoscopic guidance, using a combination of fat plugging, endoluminal cauterization, and suture ligation, 14 ears underwent surgical treatment during a 4-year period. The main outcome measure assessed was the level of autophony present after surgery as compared to baseline. Audiometric outcomes, surgical time, and complications were also recorded. RESULTS: There was sustained satisfactory subjective improvement in the autophony in 12 of the 14 ears (85.7%) at primary surgery, with nine of these 12 ears (75%) demonstrating full autophony cessation. Wilcoxon signed rank test showed a significant improvement in autophony in the study population compared to baseline (z = 3.16, P < .001). The mean operating length of time per ear over the full duration of our series was approximately 45 minutes. Postoperative audiometry showed that surgery had no impact on hearing. CONCLUSIONS: Multilayer endoscopic ligation of the patulous eustachian tube resulted in a high autophony improvement rate. The procedure is technically straightforward, and results were sustained at 6-month follow-up.

Pathological features in the LmnaDhe/+ mutant mouse provide a novel model of human otitis media and laminopathies.

Genetic predisposition is recognized as an important pathogenetic factor in otitis media (OM) and associated diseases. Mutant Lmna mice heterozygous for the disheveled hair and ears allele (Lmna(Dhe/+)) exhibit early-onset, profound hearing deficits and other pathological features mimicking human laminopathy associated with the LMNA mutation. We assessed the effects of the Lmna(Dhe/+) mutation on development of OM and pathological abnormalities characteristic of laminopathy. Malformation and abnormal positioning of the eustachian tube, accompanied by OM, were observed in all of the Lmna(Dhe/+) mice (100% penetrance) as early as postnatal day P12. Scanning electronic microscopy revealed ultrastructural damage to the cilia in middle ears that exhibited OM. Hearing assessment revealed significant hearing loss, paralleling that in human OM. Expression of NF-κB, TNF-α, and TGF-ß, which correlated with inflammation and/or bony development, was up-regulated in the ears or in the peritoneal macrophages of Lmna(Dhe/+) mice. Rugous, disintegrative, and enlarged nuclear morphology of peritoneal macrophages and hyperphosphatemia were found in Lmna(Dhe/+) mutant mice. Taken together, these features resemble the pathology of human laminopathies, possibly revealing some profound pathology, beyond OM, associated with the mutation. The Lmna(Dhe/+) mutant mouse provides a novel model of human OM and laminopathy.