Bilateral Mixed Hearing Loss Research Articles

Outcome Evaluation of BAHA Attract System Implantations.

Bone Anchored Hearing Aids (BAHAs) are currently well-utilized for treatment of hearing-impaired patients with unilateral or bilateral mixed and conductive hearing loss as well as patients with single sided deafness. The objective of this study is to evaluate outcome of BAHA Attract system in terms of surgical, functional,audiological and cosmetic results This is a prospective observational study of 15 consecutive patients of single sided deafness (SSD) who qualified for and underwent BAHA Attract surgery at a tertiary care hospital over a duration of two years. Parameters analysed were: (1) Surgery and wound healing, (2) Post-operative functional results (Bern Benefit for Single sided deafness questionnaire (BBSSQ)), (3) audiological results (free field speech in noise audiometry in two situations: with signal from implanted side and from contralateral side), (4) Cosmetic results (introduction of novel Three Points Scoring System (TPSS). Clinico-radiological planning of surgical site for implant is also described.Mean surgical time was 39 minutes and healing was uneventful in 73 % of patients. All patients reported improvement in all ten hearing situations with maximum improvement for sound localization. Significant improvement of speech in noise was seen especially when signal was presented from implanted side. All patient had full score in TPSS for cosmesis except one patient. This was the same patient in whom clinico-radiologically planned site was discarded intraoperatively due to overpneumatised temporal bone.BAHA Attract system is safeand effective solution for single sided deafness with acceptable cosmesis.

Bone-Anchored Hearing Aid Effects on Vestibular Function: A Preliminary Report.

Cochlear receptors are sensitive to vibratory stimuli. Based on this sensibility, bone-anchored hearing aids have been introduced to correct unilateral or bilateral conductive or mixed hearing loss and unilateral deafness. The vestibular system is also sensitive to the vibratory stimulus and this type of response is used in clinics to test its functionality. Being aware of this double separated sensibility, we wondered whether bone vibration, which activates the acoustic receptors of patients with bone conduction aids, can also influence the functionality of the vestibular system. To this end, we recruited 12 patients with a bone-anchored hearing aid and evaluated their vestibular function with and without an activated vibratory acoustic device. Our results show that the vibratory stimulus delivered by the bone conduction aid also reaches and stimulates the vestibular receptors; this stimulation is evidenced by the appearance or modification of some nystagmus findings during bedside vestibular testing. Despite this, none of these patients complained of dizziness or vertigo during prosthesis use. Nystagmus that appeared or changed during acoustic vibratory stimulation through the prosthesis was almost all predominantly horizontal, unidirectional with respect to gaze or body position, inhibited by fixation, and most often consistent with vestibular function tests indicating peripheral vestibular damage. The findings of sound-evoked nystagmus seem to indicate peripheral rather than central vestibular activation. The occurrence of some predominantly horizontal and high-frequency induced nystagmus seems to attribute the response mainly to the utricle and lateral semicircular canal.

Functional outcomes for speech-in-noise intelligibility of NAL-NL2 and DSL v.5 prescriptive fitting rules in hearing aid users.

The primary aim of this research study is to assess whether differences exist in the application of the NAL-NL2 and DSL v.5 prescription formulas in terms of speech-in-noise intelligibility. Data from 43 patients, were retrospectively evaluated and analyzed. Inclusion criteria were patients with bilateral conductive, sensorineural, or mixed hearing loss, already using hearing aids for at least 1year, and aged 18years or older. Patients were categorized into two groups based on the prescriptive method employed by the hearing aid: NAL-NL2 or DSL v.5. Pure tone audiometry, speech audiometry, free field pure tone and speech audiometry withthehearing aid, and Matrix sentence test were performed. The Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire was used to assess the personal audiological benefit provided bythe hearing aid. No statistically significant differences were found comparing the free-field pure tone average (FF PTA) and the free-field Word Recognition Score (FF WRS). Comparing the Speech Reception Threshold (SRT) parameter of patients with NAL-NL2 vs DSL v.5, no statistically significant difference was found, thus highlighting a condition of comparability between the two prescription methods in terms of speech-in-noise intelligibility. Comparing the results of the APHAB questionnaire, no statistically significant differences were evident for all subscales and overall benefit. When conducting a comparison between male and female patients using the NAL-NL2 method, no differences were observed in SRT values, however, the APHAB questionnaire revealed a difference in the AV subscale score for the same subjects. Our analysis revealed no statistically significant differences in speech-in-noise intelligibility, as measured by the SRT values from the Matrix Sentence Test, when comparing the two prescriptive methods. This compelling result reinforces the notion that, functionally, both methods are comparably effective in enhancing speech intelligibility in real-world, noisy environments. However, it is crucial to underscore that the absence of differences does not diminish the importance of considering individual patient needs and preferences in the selection of a prescriptive method.

Comparison between Baha® 5 and the new Baha 6 Max sound processors: results among Baha attract users

ABSTRACT The Cochlear™ Baha® 6 Max is a high-power sound processor (SP) with a fitting range up to 55 dB HL for treating patients with conductive, mixed, or single-sided sensorineural hearing loss. Our objective was to compare the audiological performance and the subjective experience of the Baha 6 Max SP among experienced adult users of the Baha 4 or Baha 5 SP on the Baha Attract system. Fifteen adults (≥18 years) with bilateral mixed (n = 7) or bilateral conductive (n = 8) hearing loss were recruited as part of the pilot study. After 2 weeks of trial period with the new SP, the performance of Baha 5 and Baha 6 Max SP was tested in a single-blind study. Pure tone audiometry, speech audiometry, and signal-to-noise ratio were determined with both SPs and in the unaided condition. The results of the subgroups with different types of hearing loss were analyzed. Subjective experience and quality of life questionnaires were also completed and compared between the SPs and the unaided hearing condition. Aided thresholds with the Baha 6 Max SP were significantly better on 250 and 8000 Hz than with the Baha 5 SP. The Baha 6 Max SP also provided better suprathreshold test results and signal-to-noise ratio, which the patients’ subjective assessments in both subgroups corroborated. Based on our results, the data shows that the Baha 6 Max SP is an optimal solution for patients using the Baha Attract system.

Audiometric evaluation and diagnosis of conductive hearing loss

Definitive diagnosis of conductive hearing loss requires a comprehensive audiological evaluation by a skilled audiologist. Threshold testing is performed with both air and bone conduction stimuli and frequently necessitates masking strategies. The fundamental aspects of bone conduction audiometry will be reviewed. Patients with bilateral conductive or mixed hearing loss need advanced strategies, such as the Rainville estimation method to acquire ear specific thresholds.1 One hallmark of conductive hearing loss is preserved word recognition due to intact cochlear function. Immittance testing is another important component to comprehensive audiometry and can provide important information about the site of the conductive lesion. This article will review the fundamental aspects of the audiologic assessment of conductive hearing loss and touch on areas of recent research advances, including the assessment of high frequency conductive hearing loss using specialized bone conduction hardware.

The Merits of Bilateral Application of Middle Ear Implants in Patients With Bilateral Conductive and/or Mixed Hearing Loss.

This study investigated sound localization abilities in patients with bilateral conductive and/or mixed hearing loss (BCHL) when listening with either one or two middle ear implants (MEIs). Sound localization was measured by asking patients to point as quickly and accurately as possible with a head-mounted LED in the perceived sound direction. Loudspeakers, positioned around the listener within a range of +73°/-73° in the horizontal plane, were not visible to the patients. Broadband (500 Hz-20 kHz) noise bursts (150 ms), roved over a 20-dB range in 10 dB steps was presented. MEIs stimulate the ipsilateral cochlea only and therefore the localization response was not affected by crosstalk. Sound localization was better with bilateral MEIs compared with the unilateral left and unilateral right conditions. Good sound localization performance was found in the bilaterally aided hearing condition in four patients. In two patients, localization abilities equaled normal hearing performance. Interestingly, in the unaided condition, when both devices were turned off, subjects could still localize the stimuli presented at the highest sound level. Comparison with data of patients implanted bilaterally with bone-conduction devices, demonstrated that localization abilities with MEIs were superior. The measurements demonstrate that patients with BCHL, using remnant binaural cues in the unaided condition, are able to process binaural cues when listening with bilateral MEIs. We conclude that implantation with two MEIs, each stimulating only the ipsilateral cochlea, without crosstalk to the contralateral cochlea, can result in good sound localization abilities, and that this topic needs further investigation.

Development of a smartphone-based Turkish digits in noise test

BackgroundPure-tone audiometry is the traditional gold standard for assessment and screening of hearing impairment, but it requires the use of calibrated devices and soundproof booth. Mobile apps can offer a good alternative to traditional methods in limited circumstances and in some emergency situations, especially when traditional methods are not available. The aim of the study was to develop an easily accessible mobile-based Turkish Digit in Noise Test screening test for the assessment of hearing.MethodsThe digits were read by a male speaker whose native language is Turkish. A mobile application was developed in accordance with the Android operating system. Twenty-five individuals with normal hearing, 25 individuals with bilateral mild sensorineural hearing loss, 25 with bilateral mild mixed hearing loss, and 25 with bilateral mild conductive hearing loss total of 100 participated in the study. All participants were between the ages of 18–60. The Turkish Digit in Noise Test mobile application was applied to the participants and the test scores were compared among the types of hearing loss.ResultsThe Turkish Digit in Noise Test scores of participants with bilateral mild sensorineural hearing loss (19.4 ± 5.39) and mixed hearing loss (22.96 ± 4.52) were lower than those of participants with normal hearing (39.68 ± 6.82) (p < .01). In addition, the results of participants with bilateral mild sensorineural hearing loss (19.4 ± 5.39) and mixed hearing loss (22.96 ± 4.52) were lower than those of participants with conductive hearing loss (36.88 ± 6.31) (p < .01).ConclusionAn easy and accessible Turkish Digit in Noise Test mobile application has been developed using which one can assess his/her hearing.

Comparison of Active Bone Conduction Hearing Implant Systems in Unilateral and Bilateral Conductive or Mixed Hearing Loss.

To assess and compare binaural benefits and subjective satisfaction of active bone conduction implant (BCI) in patients with bilateral conductive or mixed hearing loss fitted with bilateral BCI and patients with monaural conductive hearing loss fitted with monaural BCI. ITA Matrix test was performed both on patients affected by bilateral conductive or mixed hearing loss fitted with monaural bone conduction hearing implant (Bonebridge, Med-El) before and after implantation of contralateral bone conduction hearing implant and on patients with monaural conductive or mixed hearing loss before and after implantation of monaural BCI. The Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire was administered to both groups of subjects and the results were compared with each other. Patients of group 1 reported a difference of 4.66 dB in the summation setting compared to 0.79 dB of group 2 (p < 0.05). In the squelch setting, group 1 showed a difference of 2.42 dB compared to 1.53 dB of group 2 (p = 0.85). In the head shadow setting, patients of group 1 reported a difference of 7.5 dB, compared to 4.61 dB of group 2 (p = 0.34). As for the APHAB questionnaire, group 1 reported a mean global score difference of 11.10% while group 2 showed a difference of -4.00%. Bilateral BCI in patients affected by bilateral conductive or mixed hearing loss might show more advantages in terms of sound localisation, speech perception in noise and subjective satisfaction if compared to unilateral BCI fitting in patients affected by unilateral conductive hearing impairment. This may be explained by the different individual transcranial attenuation of each subject, which might lead to different outcomes in terms of binaural hearing achievement. On the other hand, patients with unilateral conductive or mixed hearing loss fitted with monaural BCI achieved good results in terms of binaural hearing and for this reason, there is no absolute contraindication to implantation in those patients.

Long-term Outcome of Sound Localization with Baha® Attract System.

Spatial hearing is a critical feature in daily life. However, there is quite a range in hearing loss patients regarding the effect of bone conduction device on localization performance. To analyze the localization performance in patients with bilateral conductive or mixed hearing loss fitted with one Baha® Attract system. This prospective study included 12 patients who had followed up for more than one year. The parameters analyzed included (1) audiological results: sound field threshold, speech discrimination scores (SDSs), and sound localization test, and (2) functional results: scores for the Speech, Spatial and Qualities of Hearing Scale (SSQ) and the Chinese translation of the Spatial Hearing Questionnaire (C-SHQ). The audiological assessments showed a reduction of 28.5 dB in the mean sound field thresholds and improvements of 61.7% in the SDSs for disyllabic words. The root mean square error improved slightly with the Baha® Attract system. Patients showed promising results in the functional questionnaire assessments, with significant improvements in the SSQ and C-SHQ scores. Although most patients were not able to localize sound accurately after surgery, the change in the scores of the SSQ and C-SHQ indicated that the Baha® Attract system could improve spatial hearing.

Mucopolysaccharidosis type 4 (Morquio syndrome): A case report

Abstract Introduction Mucopolysaccharidosis (MPS) IVA (Morquio syndrome) is a rare lysosomal storage disease, that is caused by a deficiency in the enzyme N-acetylgalactosamine-6-sulfatase (GALNS) due to an autosomal recessive mutation in the GALNS gene. Herein, we present a MPS IVA case without regular follow-up, whose enzyme replacement was planned to start at age 30 for the first time, so as to attract attention to this rare disease. Clinical Case A 30-year-old female patient diagnosed with MPS IVA, that was evaluated due to corneal clouding referred to the Marmara University endocrinology outpatient clinic for enzyme replacement. On admission, she complains of impaired vision, hearing loss, hypoglycemic symptoms and numbness of hands and feet. While she was normal for about 2 years after birth, she was noticed for delays in crawling and walking from 2–3 year of age. However, she had definitive diagnosis of MPS-IVA at the age of 9 years, with low enzymatic analysis and genetic mutation in the GALNS gene located on chromosome 16q24.3. She operated on for bilateral glaucoma and cerebellar mass when she was 17. Her parents were third degree consanguineous, and she had one healthy younger brother. On physical examination, blood pressure: 99/67 mmHg, pulse: 72/min, temperature: 36°C, SpO2:99. She was in a wheelchair. Her height was 92 cm, weight was 30 kg, BMI: 25.6 kg/m2. Disproportionate short stature, coarse facial features, hypertelorism, genu valgum, pectus carinatum, kyphoscoliosis, were detected. Her neuromotor development was normal. Laboratory findings were unremarkable except for vitamin D hypovitaminosis. Radiographic findings were dysostosis multiplex, include abnormally shaped vertebral bodies with anterior beaking, posterior scalloping, platyspondyly and dens hypoplasia, thoracolumbar kyphosis, short, broad metacarpals, acetabular dysplasia, genu valgum, pectus carinatum, paddle-shaped ribs and short, and thick clavicles (Fig 1). Bone densitometry results were in osteoporotic range BMD at the femoral neck was 0.491 g/cm2, Z score was -2.8 and at the lumbar spine was 0.707 g/cm2, Z score was -3.6. She continued the 6-min walk test with the help of a walker and knee pad for 2.37 minutes and walked 3.4 meters. Echocardiography demonstrated that ejection fraction was 65%, normal left ventricular systolic function, no ventricular segmental wall motion defect, aortic 3 cusps, mild to moderate aortic valvular regurgitation, mild mitral valvular regurgitation. Pectus carinatum was detected on thorax CT imaging, whereas a restrictive disease pattern was not observed in PFT and DLCO. Bilateral severe mixed hearing loss was detected. Recombinant human GALNS (elosulfase alfa), 2.0 mg/kg/week, was planned to administer IV in 4 hr. Conclusion Because of the progressive nature of MPS- IVA, early diagnosis and early initiation of enzyme replacement therapy are critical to prevent cumulative systemic manifestations of the patient, especially the skeletal system.

How I do it: Cochlear Osia 2 System surgery placement

BackgroundThe Cochlear™ Osia® 2 System is an active transcutaneous bone-anchored hearing implant with a newly developed piezoelectric transducer that is fixed to a titanium implant (BI300).MethodsIt uses digital piezoelectric stimulation to bypass non-functional areas of the natural hearing system and send sound directly to the cochlea. This device is designed to meet the needs of patients with unilateral and bilateral conductive or mixed hearing loss and single-sided deafness.ConclusionWe show step by step how to place the new active transcutaneous bone conduction implant, Cochlear™ Osia® 2 System, which utilizes a piezoelectric actuator anchored to the mastoid bone through an osseointegrated screw.

A Case of a &lt;i&gt;Novel Noggin (NOG)&lt;/i&gt; Gene Mutation in Congenital Hearing Loss

Congenital stapedial fixation is a type of conductive hearing loss in which impairment of the sound-conduction mechanism is caused by congenital or acquired fixation of the stapes. It is genetically heterogeneous, but it has been recently reported to be also caused by mutations in the &lt;i&gt;Noggin (NOG)&lt;/i&gt; gene. The authors have experienced a 6-year old boy with bilateral mixed hearing loss, who has a family history of hearing loss. No stapedial reflex was observed, and temporal bone CT showed no abnormality in the middle ear, as well as in the ossicles. Genetic study revealed novel &lt;i&gt;NOG&lt;/i&gt; gene mutations, which have never been reported before as a relevant gene mutation for congenital hearing loss related with stapedial fixation. Under the possible diagnosis of congenital stapedial fixation with mixed hearing loss caused by &lt;i&gt;NOG&lt;/i&gt; gene mutations, the patient has started to wear bilateral hearing aids and is being followed up for possible surgical therapy. Here, we report this case of congenital mixed hearing loss caused by novel &lt;i&gt;NOG&lt;/i&gt; gene mutations with a review of the literatures.

Speech in Noise With Bilateral Active Bone Conduction Implant for Conductive and Mixed Hearing Loss.

To evaluate speech in noise results and subjective benefit in bilateral active bone conduction implant (ABCI) for bilateral mixed hearing loss. Prospective, comparative. Ear, Nose and Throat Unit, Department of Surgical Sciences, University of Turin. Seven patients with conductive/mixed hearing loss. Patients underwent simultaneous or sequential bilateral surgery for ABCI. The speech intelligibility in noise was assessed with the Ita Matrix test in summation, squelch and head shadow settings. First, the tests were performed with one device activated in the ear with lower speech recognition score, then with both devices. Patients filled in an Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire to investigate the hearing aid benefit. When bilateral devices were activated, an improvement of signal-to-noise ratio was observed in all settings. The difference between bilateral and unilateral stimulation is 4.66 dB ( p = 0.016) in the summation, 2.24 dB ( p = 0.047) in the squelch, 7.50 dB ( p = 0.016) in the head shadow setting.Looking at the APHAB global score (GS), patients report lower mean scores, hence less difficulties, when using two devices (GS, 21.9%; standard deviation (SD), 8.28) rather than one (GS, 33.0%; SD, 10.24) ( p = 0.018). In symmetric mixed bilateral hearing loss, rehabilitation with an ABCI fitted bilaterally shows audiologic advantages in speech perception in noise, not only thanks to the summation effect and by reducing head shadow but also by improving the binaural unmasking based on the squelch effect. Audiometric outcomes are confirmed by the GSs obtained in the APHAB questionnaire.

Surdité dans l’artérite à cellules géantes : à propos d’une observation

IntroductionHearing loss is a rare manifestation in giant cell arteritis. The different types of deafness are possible with a predominance of sensorineural deafness. Case reportWe report a 75-year-old woman who presented with typical manifestations of giant cell arteritis associated concomitantly with the occurrence of bilateral mixed hearing loss confirmed on the audiogram. Corticosteroids allowed a rapidly favorable clinical and biological outcome. The follow-up audiogram at 3 months was markedly improved and showed a decrease in sensorineural hearing loss and disappearance of conductive hearing loss. ConclusionAny rapid onset deafness in an inflammatory context in the elderly should lead to a search for giant cell arteritis. The diagnosis can be difficult in the absence of other typical manifestations, especially since the biopsy of the temporal artery most often comes back negative. Corticosteroids are usually effective.

The Baha® Attract System Implantations Significantly Improve the Quality of Life of Hearing-Impaired Patients in Long-Term Observations.

The aim of this study was to assess the impact of the Baha® Attract system implantation on the quality of life of hearing-impaired patients, who were qualified for surgery due to various audiological indications. A total of 96 patients implanted with the Baha® Attract system were asked to fill in the set of questionnaires: the Glasgow Benefit Inventory, the Abbreviated Profile of Hearing-Aid Benefit, and the BAHA Aesthetic, Hygiene, and Use. Totally 79 patients responded and were then analyzed. Patients were divided into 4 groups: A: with bilateral mixed or conductive hearing loss, B: with single-sided deafness, C: with unilateral mixed or conductive hearing loss, and D: others. There was a significant improvement in quality of life measured by the Glasgow Benefit Inventory in all the analyzed groups, with a mean total score of 29.4 points (P < .001). Similarly, the evaluation by the Abbreviated Profile of Hearing Aid Benefit questionnaire showed a significant improvement in terms of the global score in all the analyzed groups, with a mean gain of 38.6% (P < .001). There were no differences between the groups. More than 90% of patients found the Baha® Attract system easy to place on their heads and maintain good hygiene. Of all the implant users, 81% were satisfied with the final aesthetic effect. The implantation of the Baha® Attract system significantly improves the quality of life of hearing-impaired patients in all subjec- tive scales used. The system is effective for all audiological indications when strictly adhered to. The majority of patients are very satisfied with the aesthetic, hygienic, and utility aspects of the device.

Postoperative Benefit of Bone Anchored Hearing Systems: Behavioral Performance and Self-Reported Outcomes.

Introduction Bone anchored hearing solutions are a well-known option for patients with a conductive, mixed conductive-sensorineural hearing loss and those with single-sided deafness. Objective The aim of the present study was to evaluate the Ponto bone-anchored hearing system in terms of behavioral performance and self-reported outcomes, by comparing unaided and aided performance (softband and abutment), as well as aided performance with the sound processor on softband (preoperatively) versus abutment (postoperatively). Methods Fourteen adult bone-anchored candidates, with either a bilateral ( n = 12) or unilateral ( n = 2) conductive or mixed hearing loss, participated in the present study. Sound-field hearing thresholds were evaluated unaided and aided (softband and abutment). A speech-in-noise test was also performed unaided and aided for two spatial configurations (S0N90; implanted side; S0N90; nonimplanted side). The Glasgow Health Status Inventory and the Speech, Spatial and Quality of sound questionnaires were administered pre- and postsurgery to compare quality of life and perceived unaided and aided performance. Skin reaction (Holgers scores) was evaluated at 15 days, 6 weeks, and 10 weeks after surgery. Results Significant improvements postoperatively relative to unaided were obtained for sound-field thresholds at all tested frequencies. Additionally, sound-field thresholds were significantly improved with the sound processor on abutment relative to the softband at frequencies > 1 kHz. Improved performance postoperatively relative to unaided was also obtained in the speech-in-noise test and in self-reported outcomes. Conclusions Improvements in behavioral performance and self-reported outcomes were obtained with the sound processor mounted on abutment.

Piezoelectric bone conduction hearing implant Osia® - audiological and quality of life benefits.

&lt;b&gt;Introduction:&lt;/b&gt; Nowadays, there are many options to treat hearing-impaired patients: tympanoplastic surgery, hearing aids and a wide range of implantable devices.&lt;/br&gt;&lt;/br&gt; &lt;b&gt;Aim:&lt;/b&gt; The aim of this study is to present the mid-term audiological and quality of life benefits after the implantation of Osia®, an active piezoelectric bone conduction hearing implant. &lt;/br&gt;&lt;/br&gt; &lt;b&gt;Material and methods:&lt;/b&gt; The state of the tissues in the implanted area, as well as audiological and quality of life results were analyzed at six, nine and twelve months after implantation in a group of four adult patients with bilateral mixed hearing loss (1 after bilateral canal-wall-down mastoidectomy, 2 with chronic simple otitis media and after myringoplasty in the opposite ear, 1 with bilateral otosclerosis and after stapedotomy in the opposite ear). &lt;/br&gt;&lt;/br&gt; &lt;b&gt;Results:&lt;/b&gt; No postoperative complications were found in any of the cases. One year after surgery the mean audiological gain in FF PTA4 (pure tone average for 0.5, 1, 2, and 4 kHz) was 52.2 ± 3.5 dB in comparison to the unaided situation, the mean speech understanding with Osia® in quiet was 90 ± 8.2% for 50 dB SPL, 98.8 ± 2.5% for 65 dB SPL and 100 ± 0% for 80 dB SPL, and the mean speech understanding with Osia® in noise was 37.5% ± 23.6 for 50 dB SPL, 93.8 ± 4.8% for 65 dB SPL and 98.8 ± 2.5% for 80 dB SPL. There was also an evident improvement in the quality of hearing as well as in the quality of life, measured by APHAB (Abbreviated Profile of Hearing Aid Benefit) and SSQ (Speech, Spatial and Qualities of Hearing Scale). &lt;/br&gt;&lt;/br&gt; &lt;b&gt;Conclusions:&lt;/b&gt; The Osia® is an effective treatment option for patients with bilateral mixed hearing loss. The mid-term audiological and quality of life results are excellent, but further observations including bigger groups of patients and a longer follow- -up are required.

Prevalence And Pattern Of Hearing Loss At A State Tertiary Health Facility In Damaturu, North-eastern Nigeria

Background: Hearing loss being a hidden disability attracts less sympathy from the public as well as governmental and non governmental organizations.Objective: The Objective of the study was to determine the prevalence and pattern of hearing loss among patients seen at the out patient unit of Ear, Nose and Throat clinic of the Yobe State University Teaching Hospital, Damaturu, Yobe State, Nigeria. Patients and Methods: This was a retrospective study of all registered patients with hearing impairment seen at the department of Ear, Nose and Throat, Head and Neck Surgery of the Yobe State University Teaching Hospital, Damaturu from 1st January, 2018 to 31st December, 2018. Case files were retrieved and reviewed. Data was extracted and entered into a Proforma. Extracted data include socio-demographic information, mode of presentation, examination findings, diagnosis, investigations done, treatment offered and outcome. The generated data was analyzed using SPSS version 20. Results: Seventy four, out of the total 1268 patients seen at the department of Ear, Nose and Throat clinic within the period under review, had hearing loss making a hospital prevalence of hearing loss of 5.8%. Median age of the participants was 20 years (range 2months-80years). Middle ear infections accounted for majority of the aetiologic factors and most patients presented with bilateral, moderate mixed hearing loss. Most patients had ear dressing to achieve dryness while 6 had tympano-mastoidectomy. Conclusions: The prevalence of hearing loss is low, with most patients presenting with ear discharge. The pattern found in the majority of the patients is bilateral moderate mixed hearing loss

Etiology of early hearing loss in Brazilian children

IntroductionHearing loss etiology depends on the population studied as well as on the ethnicity and the socio-economic condition of the analyzed region. Etiological diagnosis contributes to the improvement of preventive measures and to the early identification of this deficiency. ObjectiveTo identify the etiological factors of hearing loss and its prevalence in a tertiary hospital in southern Brazil, to verify the frequency of mutations in GJB2 and GJB6 genes, and to correlate the degree of hearing loss with the etiological factors of deafness. MethodsThis prevalence study involved 140 children with bilateral sensorineural or mixed hearing loss. Medical history, physical examination, audiometry, and evoked auditory brainstem response were conducted. Imaging and genetic examinations were also performed. ResultsEtiologies and their prevalence were as follows: (a) indeterminate causes, 31.4%; (b) conditions related to neonatal period, 22.1%; (c) genetic, 22.1%; (d) auditory neuropathy, 10%; (e) other factors (cortical malformation, intracranial hemorrhage, and internal ear malformations), 7.9% and (f) congenital infections, 6.4%. Within the genetic cases, ten homozygous and seven heterozygotes of the 35delG mutation were identified, besides two cases of rare variants of GJB2: p.Try172* and p.Arg184Pro. One case with homozygosis of del(GJB6-D13S1830) was found. Regarding severity of hearing loss, in 78.6% of the cases the degree of hearing loss was profound and there were no significant differences when comparing between etiologies. ConclusionThe number of indeterminate etiologies is still high and congenital CMV infection may be a possible cause of undiagnosed etiology for hearing loss. The predominance of etiologies related to neonatal conditions and infectious causes are characteristic of developing countries. The most prevalent mutation was 35delG, the main GJB2 gene, probably because of the European influence in the genotype of our population.

An experience of managing bilateral mixed hearing loss due to postoperative retroauricular fistula with cartilage conduction hearing aids

An experience of managing bilateral mixed hearing loss due to postoperative retroauricular fistula with cartilage conduction hearing aids