Patient-reported long-term benefit with an active transcutaneous bone-conduction device.

PURPOSE: To evaluate the long-term benefits in hearing-related quality of life, patient satisfaction and wearing time of patients rehabilitated with an active transcutaneous bone-conduction device. Adverse events and audiological outcomes are reported as secondary outcomes. METHODS: This retrospective, mono-centric cohort analysis involves 16 adults with conductive or mixed hearing loss with a mean device experience of 51.25 months. Patient-reported outcome measures were assessed using the short version of the Speech, Spatial and Qualities of Hearing Scale (SSQ12-B) and the German version of the Audio Processor Satisfaction Questionnaire (APSQ). Audiological outcomes as well as incidence of adverse events were obtained from patients´ charts. RESULTS: The hearing-related quality of life improved significantly within all subscales of the SSQ12-B scoring a mean overall of 2.95 points. Patient satisfaction measured with the APSQ scored 8.8 points on average. Wearing times differed considerably and patients with lower levels of education seemed to use their device longer compared to patients with academic education. Eight minor adverse events were documented, all of which resolved during follow-up. The mean gain in word recognition score at the last follow-up measured at 65 dB was 75.9%, while speech reception threshold was lowered by 35.1 dB. CONCLUSION: Even after several years, patients report significant benefits in hearing-related quality of life and device satisfaction. In combination with a low rate of minor adverse events and significantly improved audiological outcomes, the device is considered as a comfortable and effective option in hearing rehabilitation.

Association of rheumatoid arthritis with hearing loss: a systematic review and meta-analysis.

Rheumatoid arthritis (RA) is a systemic autoimmune disease affecting up to 1% of the worldwide population. RA is associated with multiple extra-articular manifestations (EAMs). Middle ear, cochlea and the auditory nerve are suspected sites of RA activity and hearing loss is a possible novel EAM of RA. Objective was to investigate the association of RA with the different subtypes of hearing loss. This systematic review was performed according to the PRISMA guidelines. A random effects model meta-analysis was conducted and the I2 was used to assess heterogeneity. Twelve studies comprising 20,022 RA patients and 79,244 controls were included in this systematic review. All studies were observational and were rated to a moderate rate of bias. RA patients had nearly fourfold increased odds of sensorineural hearing loss (SNHL) compared with controls (OR 3.42; 95% CI 2.50-4.69; I2 = 13). RA patients also had a significantly increased risk of SNHL (RR 2.28; 95% CI 1.88-2.76; I2 = 0). RA patients did not have increased odds of conductive hearing loss (CHL) and mixed hearing loss (MHL) (OR 1.36; 95% CI 0.52-3.55; I2 = 22); (OR 2.73; 95% CI 0.78-9.58; I2 = 0%). RA is significantly associated with SNHL. RA is not associated with CHL and MHL. Early screening of RA patients with pure tone audiometry should be considered.

Prevalence of potential candidates for electric-acoustic stimulation implant in a hearing-impaired population.

OBJECTIVE: To estimate the prevalence of potential electric-acoustic stimulation (EAS) implant candidates in a hearing-impaired population through a review of auditory examinations. METHODS: In total, 7356 patients underwent audiometric examination in our department between 2011 and 2014. The prevalence of patients meeting the audiometric criteria for EAS and standard cochlear implant (CI) was assessed. RESULTS: The percentage of EAS implant candidates meeting the pure-tone audiometric criteria was 0.71% (n=34) among the hearing-impaired individuals (n=4758) examined in our department, whereas 2.52% (n=120) met the criteria for standard CI. Among the 34 EAS implant candidates, 2 individuals (5.83%) received EAS implant surgery after approval of the EAS device in Japan. CONCLUSIONS: There was a lower prevalence of EAS implant candidates than standard CI candidates. Nevertheless, healthcare professionals should carefully examine the audiograms of patients with high frequency hearing loss with regard to meeting the indication criteria for EAS implant. This will enable patients to gain access to adequate information relating to further examinations and treatment options.

Renal ultrasound abnormalities in children with syndromic and non-syndromic microtia.

OBJECTIVE: Renal abnormalities are commonly considered in the work up of pediatric patients with external ear malformations. However, there is little consensus regarding an appropriate renal screening protocol for patients with microtia. We sought to characterize renal abnormalities detected on ultrasonography in pediatric patients with microtia. METHODS: We conducted a retrospective cohort study of pediatric patients diagnosed with microtia who underwent renal ultrasound from 1991 to 2014 at a single tertiary academic institution. Renal ultrasound reports and medical records were reviewed to assess for renal abnormalities and to determine whether patients required specialist follow-up or interventions. Audiograms and otolaryngology notes were used to determine patterns of hearing loss. The following additional information was recorded from the electronic medical records: patient sex, microtia grade (I-IV), microtia laterality, and known associated syndromes. Characteristics were compared between those who did and did not have renal ultrasound findings using Fisher's exact test. Univariate logistic regression analysis was performed to determine factors associated with renal ultrasound findings. RESULTS: The majority of patients in this cohort were syndromic (n = 51, 64%) with grade III microtia (n = 46, 58%) and conductive hearing loss (n = 58, 72%). Syndromic children with microtia demonstrated a higher crude rate of renal ultrasound abnormalities (22%) than children with isolated microtia (7%). Of these patients, 69% required specialist follow-up. Univariate logistic regression analysis did not identify predictors that were significantly associated with renal ultrasound findings. CONCLUSION: Fairly high rates of abnormalities in syndromic and non-syndromic patients may warrant screening renal ultrasound in all patients with microtia, especially given the high percentage of findings requiring renal follow-up. A prospective study to formally evaluate screening efficacy is needed.

Características clínico-epidemiológicas de pacientes ancianos con Hipoacusia atendidos en el Hospital Calixto García / Clinical and epidemiological characteristics of elderly patients with hearing loss treated in Calixto García University Hospital

Introducción: El envejecimiento en Cuba constituye el principal problema demográfico y la Hipoacusia es una de las condiciones crónicas más frecuentes en los adultos mayores. Objetivo: Describir las características clínico-epidemiológicas de los pacientes de la tercera edad con Hipoacusia atendidos en el Hospital Universitario General Calixto García en el período de septiembre de 2015 hasta septiembre de 2016. Material y Método: Se realizó un estudio descriptivo, transversal en el Hospital Universitario General Calixto García durante el período de septiembre de 2015 hasta septiembre de 2016. La población (N) estuvo constituida por 387 pacientes de la tercera edad, quienes asistieron a consulta en el período estudiado. Resultados: Predominó la Hipoacusia en los pacientes con edades entre 65 y 79 años (67,2 por ciento) y el sexo masculino fue el más afectado con esta discapacidad (69,5 por ciento). La Hipoacusia en el anciano se asoció con frecuencia a otras afecciones crónicas como las enfermedades cardiovasculares. La Presbiacusia o Hipoacusia asociada a la edad fue la causa más frecuente de sordera. Conclusiones: La Hipoacusia resultó ser una discapacidad frecuente en los pacientes masculinos de la tercera edad. La misma estuvo asociada a enfermedades crónicas no trasmisibles. Las perdidas auditivas neurosensoriales y mixtas, de moderada y severa intensidad, fueron las más frecuentemente encontradas en la audiometría tonal(AU)

Introduction: Aging constitutes the main demographic problem in Cuba, and hearing loss is one of the most frequent chronic conditions in older adults. Objective: To describe the clinical and epidemiological characteristics of elderly patients with hearing loss, treated in Calixto Gracía University Hospital during the period from September 2015 to September 2016. Material and Method: A descriptive cross-sectional study was conducted in Calixto Garcia University Hospital during the period from September 2015 to September 2016. The population (N) was composed of 387 elderly patients, who went to the doctor´s office during the period under study. Results: Hearing loss predominated in patients aged from 65 to 79 years (67,2 percent), and the male sex was the most affected by this disability (69,5 percent). Hearing loss in the elderly was frequently associated with other chronic conditions such as cardiovascular diseases. Age-related hearing loss (Presbycusis) or hearing loss associated with age was the most frequent cause of deafness. Conclusions: Hearing loss was a frequent disability in male elderly patients. It was associated with non-communicable chronic diseases. Sensorineural and mixed hearing losses of moderate and severe intensity were the most frequent ones found in the tonal audiometry(AU)

Otologic and Rhinologic Manifestations of Eosinophilic Granulomatosis with Polyangiitis.

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic autoimmune disease that manifests as asthma, recurrent sinusitis and peripheral eosinophilia. In this study, we investigated the clinical features of the ear and nasal manifestations of EGPA in comparison with those of granulomatosis with polyangiitis (GPA). MATERIALS AND METHODS: Twenty-one patients diagnosed with EGPA were studied. The frequency of otologic manifestations, the degree of hearing loss and the frequency of nasal symptoms were assessed. The onset of ear symptoms, sinusitis and asthma in patients with EGPA were also examined. RESULTS: Eleven patients (52.4%) with EGPA demonstrated otologic symptoms. The EGPA patients commonly presented mild-to-moderate mixed or sensorineural hearing loss. The pattern of hearing loss was mainly flat, and all but 1 patient achieved complete remission from their hearing impairments. Eighteen patients (85.7%) with EGPA demonstrated nasal symptoms. Patients with EGPA showed a significantly higher incidence of nasal polyps than did those with GPA. The median Lund and Mackey scoring system score was 13.7 for patients with EGPA, and ethmoid sinus shadows were more severe than those of the maxillary sinus. Most ear symptoms associated with EGPA were observed after definitive diagnosis, although sinusitis and asthma tended to manifest themselves before diagnosis. There were significant differences between the onset of ear symptoms and those of asthma and sinusitis. CONCLUSION: As over 80% of patients with EGPA had nasal symptoms and over half had ear symptoms, otolaryngologists should be aware of this disease. Recognition of the characteristic ear and nasal symptoms are thought to be particularly important to obtain an early diagnosis of EGPA.

[Epidemiological and demographic analysis of indications for Baha® surgery in Poland--multicenter study]. / Analiza epidemiologiczna i demograficzna wskazan do implantacji systemu Baha® w Polsce--badanie wieloosrodkowe.

AIM OF THE STUDY: To collect and analyze epidemiological and demographical data of patients qualified for and implanted with the Baha® system in Poland. MATERIAL AND METHOD: 17 out of 28 otolaryngology departments performing Baha® implantation in Poland participated in the study. Up to date there were 286 patients registered in database. Data were obtained from the preoperative questionnaires including information such as age and gender, indications for the implantation, previous hearing aid use as well as data regarding the surgical technique. RESULTS: The most frequent indication for the Baha® system was bilateral mixed hearing loss (51%), followed by SSD (18%), bilateral conductive hearing loss (17%), unilateral mixed (8%) and conductive (6%) hearing loss. In 11% of subjects hearing impairment was congenital versus 89% cases of acquired. The mean age was 44 years with the slight prevalence of women (52%). 63% of patients did not have previous experience with hearing aids. The most frequent surgical technique was classic dermatome single stage procedure performed under general anesthesia (65%). CONCLUSIONS: Data gather in this multicentre research serve as a valuable source of information on qualifications for the Baha®, shows demographic spectrum of adult recipients in Poland. It also presents the preferences of surgical procedures. Outcomes of the study may constitute a reference for each centre participating in this research as well as for new centers starting the Baha® procedure.

Otological diagnoses and probable age-related auditory neuropathy in "younger" and "older" elderly persons.

OBJECTIVE: Audiological data from a population based epidemiological investigation were studied on elderly persons. Specific diagnoses of otological and audiological disorders, which can result in hearing loss, were searched for. DESIGN: A retrospective register study. STUDY SAMPLE: Three age cohorts, 474 70- and 75-year olds ("younger"), and 252 85-year olds ("older"), were studied. Clinical pure tone and speech audiometry was used. Data from medical files were included. RESULTS: Conductive hearing loss was diagnosed in 6.1% of the "younger" elderly persons, and in 10.3% of the "older" ones. Specific diagnoses (chronic otitis media and otosclerosis) were established in about half of the cases. Sensorineural hearing loss, other than age-related hearing loss and noise induced hearing loss, was diagnosed in 3.4 % and 5.2% respectively. Severely impaired speech recognition, possibly reflecting age-related auditory neuropathy, was found in 0.4% in the "younger" group, and in 10% in the "older" group. Bilateral functional deafness was present in 3.2% of the 85-year-old persons, but was not present in the 70-75-year group. CONCLUSION: The incidence of probable age-related auditory neuropathy increases considerably from 70-75 to 85 years. There are marked differences between "younger" and "older" elderly persons regarding hearing loss that severely affects oral communication.

[Hearing loss and airway problems in children with mucopolysaccharidoses]. / Hipoacusia y problemas de vía aérea en niños con mucopolisacaridosis.

INTRODUCTION: Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that causes the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, hearing loss and airway obstruction in the ENT area. OBJECTIVE: Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. METHODS: A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007-2010. RESULTS: Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed mild bilateral sensorineural hearing loss; another case was diagnosed as mixed hearing loss. The remaining auditory pattern was moderate bilateral conductive hearing loss. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. CONCLUSIONS: Children with MPS are at increased risk for developing sensorineural hearing loss. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management.

[Major sickle cell anaemia and hypoacusia: about 112 cases in Yopougon, Côte d'Ivoire]. / Drépanocytose majeure et hypoacousie: à propos de 112 cas relevés à Yopougon, Côte d'Ivoire.

The objective of this study is to evaluate the impact of major sickle cell anaemia on the auditory function of the patients. It was a prospective survey conducted in the university hospital of Yopougon (Republic of Côte d'Ivoire). The auditory function of 112 major sickle cell anaemia patients 5- to 40-five-years-old, was compared with 112 healthy controls. 17% (19/112) of major sickle cell anaemia patients presented hearing loss from 30 dB to 65 dB versus 4% of controls. The average age of deaf patients was 26-years-old (7 years to 40-years-old). The sex-ratio was 0,73. The SC form with 47% (9/19) of deafness was the form the most frequently found followed by form SS, 37% (7/19) and form S beta+ thalassaemia 16% (3/19). The form S beta 0 thalassaemia did not record deafness patients. However the form having shown the highest rate of attack was the form S beta+ thalassaemia with 25% (3/12) followed by form SC 21% (9/43) and form SS 14% (7/51) of deafness. Deafness was generally light 73, 7% of the cases and often bilateral (52.6% of the cases). The audiometric loss affected both extremes of the hearing range, but was more significant in lower tones (58% of cases). Higher tones represented 5% of the cases. It was often sensorineural hearing loss 58% of the cases. Mixed hearing loss represented 42% of the cases.

Pure-tone threshold description of an elderly French screened population.

OBJECTIVES: To describe the distribution of pure-tone hearing thresholds of a Caucasian population living in the south of France aged 70 years and older. To establish age- and sex-adjusted normative hearing thresholds based on results of subjects free of noise and ototoxic drug exposure and to compare them with hearing thresholds of exposed (E) subjects. DESIGN: Cross-sectional analysis of a longitudinal epidemiologic cohort study. SETTING: Montpellier suburb, south of France. PARTICIPANTS: A total of 778 subjects 70 years old and older were examined. Noise exposure, ototoxic medication use, and medical history were collected. Hearing thresholds were obtained via pure-tone audiometry. After excluding patients with ear-related disease, 659 subjects were further analyzed (270 men and 389 women). Noise or ototoxic medication exposure was found in 364 subjects (E subjects), whereas 295 had no exposure (nonexposed [NE] subjects). METHODS: Median pure-tone thresholds, lower deviation, and upper deviation were calculated for the NE subjects with a statistical method similar to the ISO 7029 norm and were compared with thresholds of E subjects. RESULTS: Hearing thresholds, especially in high frequencies, increased with age more for women than for men. Median thresholds of E subjects were significantly higher than those for the NE sample in men. CONCLUSION: Age- and sex-adjusted hearing thresholds could well be useful in the study of the impact of environmental and genetic factors on hearing loss in the elderly. The next step would be to quantify the impact of noise, ototoxic drug exposure, and genetics using these age- and sex-adjusted thresholds.

Fully implantable hearing device as a new treatment of conductive hearing loss in Franceschetti syndrome.

The objective is to investigate a new therapeutic option for treatment of conductive hearing loss in children with Franceschetti syndrome. A Carina fully implantable device with the MET V transducer for conductive applications was implanted in a 14-year-old male suffering from bilateral conductive hearing loss (mean PTA loss: 70 dB on the right ear and 64 dB on the left ear) secondary to Franceschetti (a.k.a Treacher Collins) syndrome with bilateral agenesis of external ear canal and ossicular malformation. Postoperative unaided PTA was unchanged by the surgical procedure. When the implant was activated, mean PTA was 29 dB improved on the implanted ear. The capacity of the Carina MET ossicular stimulator to provide appropriate gain relative to the degree of hearing loss indicates that the device may be a viable treatment for children with agenesis of external auditory canal and severe malformation of the middle ear. However, these initial results need to be furthered by: (1) additional studies including a greater number of patients to confirm these preliminary results and (2) a long-term follow-up must be carried out to detect possible long-term cochlear adverse effects, in particular on the basilar membrane.