Persistent Hearing Loss Research Articles

A New Biomarker on Bone Resorption in Chronic Otitis Media: Osteoprotegerin and NLRP3 Inflammasome Gene Polymorphisms.

Chronic Otitis Media (COM) is a chronic inflammation of the middle ear and mastoid with persistent membrane perforation and hearing loss. Osteoprotegerin (OPG) and NOD like receptor protein 3 (NLRP3) play an important role in bone metabolism. The aim of the study was to investigate the role of OPG and NLRP3 gene polymorphism on ossicular chain resorption in COM. Fourty COM patients and 20 healhty control group were included in the study. While 20 patients underwent ossiculoplasty, 20 patients underwent type 1 tympanoplasty. DNA was isolated from peripheral blood using the DNA kit. OPG gene c.226A > C (p.Thr76Pro) and NLRP3 gene c.592G > A (p. Val198Met) polymorphisms were genotyped using melting curve analysis technique. NLRP3 gene polymorphism were determined in 6 of 20 patients (30%) in ossiculoplasty group, 4 of 20 patients (20%) in type 1 tympanoplasty group and 3 of 20 patients (15%) in control group. OPG gene polymorphism were determined in 5 of 20 patients (25%) in ossiculoplasty group, 3 of 20 patients (15%) in type 1 tympanoplasty group and 1 of 20 patients (5%) in control group, respectively. There was no statistically significant difference between groups regarding to results. Although the difference was not significant NLRP3 and OPG gene polymorphisms were higher in the ossiculoplasty group. Since NLRP3 and OPG gene polymorphisms were determined to be higher numerically in the ossiculoplasty group, OPG and NLRP3 gene regulation system may have an effect on ossicular chain destruction in COM.

Results of Active Middle Ear Implantation in Patients With Mixed Hearing Loss After Middle Ear Surgery: A Prospective Multicenter Study (the ROMEO Study).

ObjectivesThis study was conducted to evaluate the user satisfaction, efficacy, and safety of round window (RW) vibroplasty using the Vibrant Soundbridge (VSB) in patients with persistent mixed hearing loss after mastoidectomy.Methods The study included 27 patients (mean age, 58.7 years; age range, 28–76 years; 11 men and 16 women) with mixed hearing loss after mastoidectomy from 15 tertiary referral centers in Korea. The VSB was implanted at the RW. The Korean translation of the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire and the Korean version of the International Outcome Inventory for Hearing Aids (K-IOI-HA) questionnaire were used to evaluate user satisfaction as the primary outcome. The secondary outcome measures were audiological test results and complication rates.ResultsThe mean scores for ease of communication (61.3% to 29.7% to 30.2%), reverberation (62.1% to 43.1% to 37.4%), and background noise (63.3% to 37.7% to 34.3%) subscales of the APHAB questionnaire significantly decreased after VSB surgery. The mean K-IOI-HA scores at 3 and 6 months after surgery were significantly higher than the mean preoperative score (18.6 to 27.2 to 28.1). The postoperative VSB-aided thresholds were significantly lower than the preoperative unaided and hearing aid (HA)-aided thresholds. There was no significant difference between preoperative unaided, preoperative HA-aided, and postoperative VSB-aided maximum phonetically balanced word-recognition scores. None of the 27 patients experienced a change in postoperative bone conduction pure tone average. One patient developed temporary facial palsy and two developed surgical wound infections.ConclusionRW vibroplasty resulted in improved satisfaction and audiological test results in patients with mixed hearing loss after mastoidectomy, and the complication rate was tolerable.

Otitis media outcomes of a combined 10-valent pneumococcal Haemophilus influenzae protein D conjugate vaccine and 13-valent pneumococcal conjugate vaccine schedule at 1-2-4-6\u2009months: PREVIX_COMBO, a 3-arm randomised controlled trial

BackgroundAboriginal children living in Australian remote communities are at high risk of early and persistent otitis media, hearing loss, and social disadvantage. Streptococcus pneumoniae and non-typeable Haemophilus influenzae (NTHi) are the primary pathogens. We compared otitis media outcomes in infants randomised to either a combination of Synflorix™ (PHiD-CV10, with protein D of NTHi) and Prevenar13™ (PCV13, with 3, 6A, and 19A), with recommended schedules for each vaccine alone. We previously reported superior broader overall immunogenicity of the combination schedule at 7 months, and early superiority of PHiD-CV10 compared to PCV13 at 4 months.MethodsIn an open-label superiority trial, we randomised (1:1:1) Aboriginal infants at 28 to 38 days of age, to either Prevenar13™ (P) at 2–4-6 months (_PPP), Synflorix™ (S) at 2–4-6 months (_SSS), or Synflorix™ at 1–2-4 months plus Prevenar13™ at 6 months (SSSP). Ears were assessed using tympanometry at 1 and 2 months, combined with otoscopy at 4, 6, and 7 months. A worst ear diagnosis was made for each child visit according to a severity hierarchy of normal, otitis media with effusion (OME), acute otitis media without perforation (AOMwoP), AOM with perforation (AOMwiP), and chronic suppurative otitis media (CSOM).ResultsBetween September 2011 and September 2017, 425 infants were allocated to _PPP(143), _SSS(141) or SSSP(141). Ear assessments were successful in 96% scheduled visits. At 7 months prevalence of any OM was 91, 86, and 90% in the _PPP, _SSS, and SSSP groups, respectively. There were no significant differences in prevalence of any form of otitis media between vaccine groups at any age. Combined group prevalence of any OM was 43, 57, 82, 87, and 89% at 1, 2, 4, 6, and 7 months of age, respectively. Of 388 infants with ear assessments at 4, 6 and 7 months, 277 (71.4%) had OM that met criteria for specialist referral; rAOM, pOME, or CSOM.ConclusionsDespite superior broader overall immunogenicity of the combination schedule at 7 months, and early superiority of PHiD-CV10 compared to PCV13 at 4 months, there were no significant differences in prevalence of otitis media nor healthy ears throughout the first months of life.Trial registrationACTRN12610000544077 registered 06/07/2010 and ClinicalTrials.govNCT01174849 registered 04/08/2010.

Tinitus (Acúfeno) en la población adulta nicaragüense

El tinnitus (acúfeno) es descrito por el paciente como “zumbido” o “silbido” “pitido” dentro del oído, los cuales se producen sin que exista fuente sonora externa que lo origine, su prevalencia real es difícil de precisar sobre todo en nuestro medio ya que la mayoría de la población que acude al hospital acude por otras causas otológicas y audiológicas. El objetivo de esta investigación consistió en describir el perfil epidemiológico y clínico del tinnitus en la población nicaragüense de 25 a 65 años de edad atendidos en la consulta externa por el servicio de otorrinolaringología en el Hospital Antonio Lenín Fonseca durante el periodo comprendido entre el 1 de enero y el 31 de diciembre de 2018. Para ello se realizó un estudio observacional, descriptivo, de corte transversal. Se revisaron 312 expedientes clínicos de pacientes diagnosticados con patologías del oído de los cuales 15 tenían diagnóstico de tinnitus asociada o no a otras patologías otológicas; Se empleó una técnica de muestreo probabilística sistemática. La información fue recolectada haciendo uso de una ficha de recolección de datos, la cual fue validada mediante una prueba piloto, para ello se revisaron 35 expedientes clínicos. El procesamiento estadístico de las variables: edad, sexo, tratamiento farmacológico y pruebas diagnósticas, se llevó a cabo haciendo uso del programa informático IBM-SPSS® versión 25.0 para Windows 7 de 32 bits, utilizando un análisis descriptivo, expresado en frecuencia y porcentajes. Se obtuvieron los siguientes resultados, el 4.8% de los pacientes con patologías del oído presentó tinnitus; el sexo femenino se vio afectado en el 3.2%, la edad mayormente afectada fue la comprendida entre 51 – 59 años, los pacientes con diagnóstico único de tinnitus no recibieron ningún fármaco, fueron diagnosticados mediante otoscopía y audiometría. El tinnitus persistente representó el 4.8 % de los hallazgos clínicos presentes en la segunda reevaluación del paciente diagnosticados con patologías del oído y un 3.8 % estaba relacionado con hipoacusia persistente. Concluimos que el tinnitus prevaleció en el 4.8% del grupo poblacional estudiado y supuso el 0.4% de los diagnósticos en la población que acude a consulta por afectación otológica.

Tuberculous otitis media: rare cause of recurrent facial nerve palsy

<p class="abstract">Tuberculous otitis media (TOM) is a rare form of tuberculosis, due to tubercular otitis media, thick caseous material and granulation tissue gets deposited in middle ear cleft causing bone erosion, sequestration, ossicular destruction, heading loss which can be conductive or mixed and rarely facial paralysis. In this case report we discuss tubercular otitis media as a cause of recurrent facial nerve paralysis. 54 year old male patient with no known previous comorbidities presented to ENT OPD on 12 October 19 with complaints of scanty, mucopurulent foul smelling occasionally blood stained continuous otorrhoea (R)×02 years, painless progressive and persistent hearing loss ×1 year and recurrent (R) sided facial nerve palsy, 03 episodes in last 2 years which were spontaneous and sudden in onset, showed gradual recovery with oral medication. Tubercular otitis media is not a very common diagnosis with which an otolaryngologist comes across. It generally presents with otorrhea and hearing loss which mimics more commoner ear disease. Typical findings of multiple tympanic membrane perforations are not necessarily seen. There can be involvement of facial nerve and presence of refractory otorrhoea with atypical features may be considered indicative of tubercular pathology. A clinician should have high index of suspicion whenever there are signs which outlies symptoms in form of severity. This case highlights the diagnostic challenge and high index of clinical suspicion required for diagnosis. Combination of appropriate diagnostic modality along with good clinical vigilance will aid in early diagnosis and start of early medication which reduces chances of any subsequent complications.</p><p class="abstract"> </p>

Reconstruction of the outer attic wall by cortical bone and bone cement.

To asses using cortical bone and bone pate with and without glass ionomer bone cement (GIBC) for reconstructing the outer attic wall (OAW) defect during cholesteatoma surgery without mastoid cavity obliteration. This is a prospective case series of 25 patients who underwent primary surgery for cholesteatoma with presence of OAW defect that was reconstructed by cortical bone graft and bone pate, further fixation of the cortical bone graft in place was done by GIBC in 18 patients. There was significant improvement of persistent otorrhea and hearing loss after surgery (P<0.001). Recurrence of cholesteatoma was found in 2 patients (8%), residual TM perforation was found in one patient (4%). Reconstruction of OAW by cortical bone and bone pate is an effective surgical option to decrease the incidence of recurrence in cholesteatoma surgery. Glass ionomer bone cement can be added safely to fix the cortical bone graft in the OAW defect.

The results of hearing impairment diagnostics in schoolchildren of 1-11 forms

Objectives - to analyze the results of the screening of hearing impairment (audiological screening) in schoolchildren of grades 1-11, and to identify the need to expand the stages of audiological screening. Material and methods. The study included 216 pupils from the 1st up to the 11th forms. During the study, in all the children, the following procedures were carried out: otoscopy, acoustic impedancemetry, registration of delayed evoked otoacoustic emission, assessment of speech intelligibility, questioning the parents about the presence and frequency of ENT diseases and signs of hearing loss. Results. The positive results in the registration of delayed evoked otoacoustic emission correlated with the number of episodes of ENT diseases. In most cases, when detecting instrumental deviations from the normal state of the auditory analyzer, the parents noted the child's difficulties in learning and communication, but did not seek medical help. Conclusion. The hearing impairment screening allows for the timely detection of the initial manifestations of hearing impairment of any etiology in children of different age groups. It is advisable to conduct screening examination of hearing impairment in pupils of 1-11 forms, in order to prevent the persistent hearing loss. In case of the detection of the initial signs of the auditory system pathology, the timely rehabilitation of such patients is recommended.

Endoscopic Management of Malleus Head Fixation in Revision Stapes Surgery.

Malleus ankylosis is an uncommon finding during stapes surgery, which can result in functional failure if undetected during primary surgery. Its management can be difficult and different approaches and solutions have been described to date, most of which require interruption of the ossicular chain. We report the management of such condition through a transcanal endoscopic technique, with preservation of ossicular chain integrity. The procedure was carried out in a patient affected by otosclerosis with persistent hearing loss after primary stapes surgery. Surgical treatment of malleus head fixation through a totally endoscopic transcanal approach during revision stapes surgery. Pure tone audiometry, speech audiometry. The patient experienced a complete closure of the air-bone gap. No postoperative vertigo or sensorineural hearing loss were observed. The present report describes the transcanal endoscopic management of malleus ankylosis during revision stapes surgery, which has not been previously reported in the literature. Pearls related to this approach and advantages in comparison to previously described approaches of the literature are discussed as well.

Мутации в гене GJB2 у детей с двусторонней тугоухостью в Кыргызстане

The purpose of this work was to identify and study the prevalence of mutations in the GJB2 gene encoding the connexin 26 protein in the Kyrgyz Republic. Hearing loss is currently the most widespread disease. This paper presents a study of 89 patients with persistent bilateral sensorineural hearing loss and deafness of unknown etiology. All patients were divided into two groups. One group included patients with an unburdened family history, the second group included patients with a burdened family history. When clarifying the etiology of the disease, we can assume further dynamics of the hearing thresholds, as well as select the necessary tactics for managing such patients for early rehabilitation. As a result of molecular genetic research, mutations in the GJB2 gene were detected in 19 patients (21,3%). The 35delG mutation was found in a homozygous state in 5 children from parents of Russian and Tatar origin. In 4 families, parents were in an assorted marriage. Among 62 Kyrgyz, mutations in the GJB2 gene were detected in 9 cases, which accounted for 14,5% of cases. The 35delG mutation among the Kyrgyz was found only in the compound heterozygous state with the 235delC mutation in 3 children and with the –23 + 1G&gt; A mutation in one child.

Unmask the silent hearing loss: a study of audiological profile in children with chronic adenotonsillitis

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Chronic adenotonsillitis results in obstruction of eustachian tube (ET) due to edema, by mechanical obstruction and the upper respiratory tract infection disrupts the mucocilliary action of ET which can lead to otitis media with effusion (OME). This when unresolved can result in persistent hearing loss and this might cause subsequent delay in development of language, social behaviour, learning difficulties which will affect the academic performance. Young children as are unable to voice their hearing loss and sometimes due to inattentiveness of parents to child’s hearing disorder; this might be neglected. The condition remains masked for a long time and hence need to be unmasked for its appropriate management.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; It is cross sectional study involving cases of chronic adenotonsillitis from November 2016 to May 2018. After enrolling the patients who met the inclusion criteria, their demographic details, examination findings including the findings of x-ray nasopharynx, pure tone audiometry (PTA) and tympanometry were noted for analysis. &lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; Total 100 patients were studied in which history of hearing loss was seen in 23% and parents’ suspicions was in 22% of cases whereas hearing loss was demonstrated in 51% of cases using PTA ranging from 16-70 db in hearing level. Analyses of tympanogram revealed 43% to have either type B or type C tympanogram which are suggestive of OME.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; Audiological screening for children diagnosed with chronic adenotonsillitis needs to be made mandatory to detect OME, the silent hearing loss as parents as well as children will miss out on it.&lt;/p&gt;

Outcomes of unilateral idiopathic sudden sensorineural hearing loss: Two decades of experience.

Objectives(a) Determine the demographic and medical risk factors for patients who presented with unilateral idiopathic sudden sensorineural hearing loss (ISSNHL); (b) identify treatments that patients underwent; (c) evaluate the adequacy of follow‐up and compliance with long‐term hearing rehabilitation.MethodsRetrospective review of patients who presented with unilateral ISSNHL between January 1998 and December 2017 at a tertiary care academic medical center.ResultsTwo hundred‐four patients met inclusion criteria. Of these, 129 (63.2%) did not undergo treatment at an outside hospital prior to our evaluation. In this subgroup, the average pretreatment pure tone average (PTA) was 61.9 ± 2.5 dB (dB). The most common treatment was oral steroids and was recommended in 76 patients (59.9%). Patients also underwent intratympanic (IT) steroid injections (7.2%) or oral steroids followed by salvage IT injections (19.4%). Mean follow‐up duration was 17.9 (±29.2) months, and posttreatment PTA (45.6 ± 2.6 dB) was significantly better than baseline (P < .001). In this cohort, hearing amplification was infrequently recommended. Less than 20% of patients reported active hearing amplification use at their most recent visit. At follow‐up, 90 patients (69.8%) reported subjective improvement in hearing after treatment. Only 55 patients (42.6%) showed improvement in PTA compared to their pretreatment audiograms.ConclusionMany patients with ISSNHL experienced audiometric improvement after treatments, but most had persistent hearing loss. The duration of follow‐up was short. Most patients did not use long‐term hearing amplification. Future studies are needed to identify factors that contribute to reduced follow‐up and low compliance with hearing amplification use in ISSNHL.Level of Evidence2c.

Methadone associated long term hearing loss and nephrotoxicity; a case report and literature review

BackgroundMethadone is a long-acting opioid receptor agonist. Reported adverse effects of methadone include constipation, respiratory depression, dizziness, nausea, vomiting, itching, sweating, rhabdomyolysis, QT prolongation, and orthostatic hypotension. Hearing loss has been rarely reported following methadone use, and when reported, long term follow-up is rare.Herein we report a case of methadone poisoning with rhabdomyolysis, acute kidney injury, and persistent hearing loss documented by a 2 year follow up.Case presentationThe patient was a 34 years old male who presented with a reduced level of consciousness and acute hearing loss after suicidal ingestion of 40 mg of methadone while experiencing family-related stresses. He had no prior history of methadone use, abuse, or addiction.Initial laboratory testing was significant for a serum creatinine concentration of 4.1 mg/dl, a mixed metabolic and respiratory acidosis, thrombocytopenia, abnormal hepatic transaminases, and coagulation tests. The patient then developed severe rhabdomyolysis. Also, audiometry showed a bilateral sensorineural hearing loss. The patient required hemodialysis for 11 days while his metabolic abnormalities gradually resolved. However, his hearing loss was persistent, as demonstrated by 2 years of follow up.ConclusionOur patient simultaneously had kidney failure, rhabdomyolysis, and permanent hearing loss following methadone poisoning. Although rare, ototoxicity and permanent hearing loss may happen in cases of methadone poisoning. While opioid-induced hearing loss is uncommon, methadone toxicity should be taken into account for any previously healthy patient presenting with acute hearing loss with or without rhabdomyolysis.

Persistent Hearing Loss among World Trade Center Health Registry Residents, Passersby and Area Workers, 2006-2007.

Background: Prior studies have found that rescue and recovery workers exposed to the 9/11 World Trade Center (WTC) disaster have evidence of increased persistent hearing and other ear-related problems. The potential association between WTC disaster exposures and post-9/11 persistent self-reported hearing problems or loss among non-rescue and recovery survivors has not been well studied. Methods: We used responses to the World Trade Center Health Registry (Registry) enrollment survey (2003–2004) and first follow-up survey (2006–2007) to model the association between exposure to the dust cloud and persistent hearing loss (n = 22,741). Results: The prevalence of post-9/11 persistent hearing loss among survivors was 2.2%. The adjusted odds ratio (aOR) of hearing loss for those who were in the dust cloud and unable to hear was 3.0 (95% CI: 2.2, 4.0). Survivors with persistent sinus problems, headaches, PTSD and chronic disease histories had an increased prevalence of reported hearing problems compared to those without symptoms or chronic problems. Conclusions: In a longitudinal study, we observed an association between WTC-related exposures and post-9/11 self-reported hearing loss among disaster survivors.

Endoscopic endonasal transpterygoid transnasopharyngeal management of petroclival chondrosarcomas without medial extension.

Chondrosarcomas of the skull base are malignant tumors for which surgery is the primary therapeutic option. Gross-total resection has been demonstrated to improve survival in patients with these tumors. Chondrosarcomas arising from the petroclival synchondrosis harbor particularly unique anatomical considerations that have long been a barrier to achieving such a resection. Endoscopic endonasal transpterygoid approaches have been recently used to gain improved access to such lesions; however, these approaches have classically relied on a medial to lateral transclival trajectory, which provides limited exposure for complete resection of lateral disease. In this paper the authors describe an endoscopic endonasal transpterygoid transnasopharyngeal approach that provides comprehensive access to the petroclival region through dissection of the eustachian tube with resection of the cartilaginous torus tubarius. Of note, the authors have previously demonstrated the superior outcomes and validity of this approach relative to other cranial base techniques for petroclival chondrosarcomas. Surgical outcomes in 5 cases of chondrosarcoma without medial extension are detailed. Gross-total resection was achieved in 4 of 5 patients. Postoperative complications included transient palatal numbness in all patients and eustachian tube dysfunction due to the approach. With tympanostomy tube placement, no patient had persistent hearing loss. Overall, this approach appears to be a safe and effective technique for resection of petroclival chondrosarcomas.

Revision Stapes Surgery in a Tertiary Referral Center: Surgical and Audiometric Outcomes.

Objectives:To evaluate pure-tone audiometric results in otosclerosis patients undergoing revision stapes surgery following previous middle ear interventions.Methods:A retrospective cohort study was performed in a tertiary referral center. Consecutive otosclerosis patients who underwent revision stapes surgeries, following previous middle ear interventions, for persistent conductive hearing loss, recurrent conductive hearing loss, or vertigo and had available postoperative pure-tone audiometry were included. Mean pre- and postoperative air conduction, bone conduction, and air-bone gap averaged over 0.5, 1, 2, and 3 kHz were obtained. Audiometric outcomes were obtained at 7 weeks postoperatively.Results:In total, 63 consecutive otosclerosis patients who underwent 66 revision stapes surgeries were included. Air-bone gap improved significantly with a mean gain of 19 dB (95% CI, 15-22). Air conduction improved significantly with a mean gain of 18 dB (95% CI, 14-23). Bone conduction did not change significantly, with a mean deterioration of 0 dB (95% CI, –2 to 1). Air-bone gap closure to 10 dB or less was achieved in 38% of cases and to 20 dB or less in 80% of cases. Indication for surgery, previous type of procedure, primary cause of failure, and current surgical technique were not significantly associated with air-bone gap closure to 10 dB or less. Indication for surgery and primary cause of failure were associated with one another.Conclusions:Compared to the available literature, a slightly larger gain in air conduction and air-bone gap was achieved in our study. Air-bone gap closure to 10 dB or less was achieved less often in our study.

Noise induced hearing loss (NIHL) in wasit corporation textile industries

Background: Noise Induced Hearing Loss (NIHL) is used to symbolize accumulative and persistent hearing loss due to progressive exposure to high levels of noise for months or year. Design: A cross-sectional study. Setting: Al-Karama Teaching Hospital and Wasit Co. Textile Industries. Aims and objective: To specify frequency of hearing loss among workers in the knitting industry, to registration the level of the noise in the weaving department and to specify the realization about the impact of noise on the hearing. Materials and methods: From June to August 2011, the level of noise in the weaving department has been recorded. 200 workers had been sent to our hospital, interviewed according to already prepared questioner and audio logically assessed. Results and discussion: The mean level of noise at 12measurement points was 87-96dB. Which was greater than the allowable noise level for continuous 8 hours working per day? It has been established that 113 workers had NIHL, 29% of hearing loss (the greater percentage) was in the mild category (26-40dB) at high frequency (4-6KHz) group and the minimal NIHL notch within 2 kHz and 4KHz were 6% and 22% from the workers respectively. The hearing difficulty was not the most common complaints 29.4%, while ear discomfort was 55.4% and tinnitus was 43%, because NIHL usually include the high frequencies at first hence the worker can promote the NIHL and he didn't complain from loss of hearing. No one used the personal noise protective devices (PPD) and only 44 workers (24%) (P-value≤ 0.1) who considers that the noise is a hazard on health. Conclusion: Wasit Co. Textile Industries Noise measurement is greater than the allowable noise scale for 8 hours. NIHL can be developed many years before worker will complain from hearing loss.

Annual hearing screening in girls with Turner Syndrome: Results from the first three years in Glasgow

BackgroundHearing loss is prevalent in girls with Turner Syndrome (TS). A number of cross-sectional studies have confirmed that conductive hearing loss due to middle ear disease and permanent, progressive sensorineural hearing loss are both very common and often unrecognised in TS. Hearing screening has been suggested by many authors and is recommended in some recent national audiology guidelines (every 3–5 years in an international consensus statement, every 1–2 years in the USA and every year in the UK). The effectiveness of such regular hearing screening has not been assessed before. Study aims and methodsIn January 2016 we began a programme of annual hearing screening for all girls attending the West of Scotland TS clinic. We have collected data on age, karyotype, ear and hearing symptoms, otoscopy findings, audiometric test results and subsequent outcomes for the first three years of our programme. Our aim is to assess whether this screening programme is an effective use of resources, with a worthwhile rate of detecting new otological problems and without an excessive additional workload for the audiology department. ResultsTwenty-six girls participated in the screening programme and 8 of these had no hearing issues or ear abnormalities at any time over the 3 years. Two girls had cholesteatoma, 4 had tympanic membrane retractions and/or perforations, and 9 had OME (of which 6 were transient and 3 persistent, with 2 of these requiring treatment). One cholesteatoma, one perforation and one persistent OME were already known about but all other diagnoses were new as a result of the screening programme. Five girls have persistent conductive hearing loss (plus another 6 who had a transient episode of conductive loss due to middle ear fluid) and 6 have some degree of sensorineural hearing loss (3 purely sensorineural, 3 mixed). At the time that a hearing loss was diagnosed, the parents had concerns about the hearing in 4 cases and no concerns in 12. Six girls have been fitted with hearing aids since we started the screening programme, each for different indications (sensorineural hearing loss in two cases, and one case each of persistent OME, post cholesteatoma surgery, tympanic membrane perforation and unilateral tympanic membrane retraction respectively). Two girls have had ear surgery as a result of referral from the programme (one for ventilation tubes, one for cholesteatoma). ConclusionsThe screening programme has picked up ear disease in 69% of girls with TS with only a small additional workload for the audiology department. The ongoing detection of new problems in the second and third years of the programme attests to the value of continued regular hearing screening for girls with TS.

Актуальные вопросы мастоидопластики при реконструктиивных операциях на среднем ухе

Surgical treatment of patients suffering from chronic suppurative otitis media is an important issue of otiatry. The high frequency of unsatisfactory results of surgical treatment of patients with chronic suppurative otitis media, the lack of optimal (generally accepted) approach to their surgical treatment, the persistent hearing loss after sanitizing operations determines the relevance of this problem: the need to identify the causes of unsatisfactory results, develop an algorithm and new methods of surgical treatment. ENT physicians generally believe that the obliteration of the mastoid cavity, applied after modified radical mastoidectomy, is sufficient to prevent such problems as otorrhea, infection, the proliferation of granulation tissue and hearing loss. Obliteration can be performed using various methods and materials, including autologous bone chips and dust, hydroxyapatite, cartilage, and periosteal-pericranial flaps. Obliteration of the mastoid cavity was proposed to facilitate healing and epithelialization and can be performed during the initial procedure of the canal plasty or during the secondary delayed procedure. Obliteration reduces the volume of the mastoid process cavity, thereby reducing the size of the lumen, which is necessary for effective control of the disease. Reduction of depth and the total volume of the mastoid cavity also provides the elimination of the areas with permanent accumulation of moisture, theoretically reducing the frequency of the necessary cleaning.

Ossicular Erosion from a Posterior Pars Tensa Retraction Cholesteatoma

A 14-year old female with Down syndrome presented with a 3-year history of recurrent purulent left otorrhea. The discharge had become more frequently blood-tinged. Otologic examination revealed a stenotic ear canal with polypoid granulation obstructing the view of the tympanic membrane. High resolution computerized tomographic (HRCT) imaging of the temporal bone was performed to assess the status of the middle ear and mastoid. Particular attention was given to assess for bony erosion associated with cholesteatoma formation.&#x0D; Comparison of the scutum on coronal view (Figure 1) did not show a marked visual difference in the sharpness of the scutum edge. As erosion of the scutum edge is the hallmark radiological finding in the diagnosis of a pars flaccida or attic retraction-based acquired cholesteatoma, a confident radiologic diagnosis of cholesteatoma via this pathophysiologic mechanism could not be given.&#x0D; Figure 1.Coronal HRCT images of the scutum. The left scutum (white arrow) does not appear to be blunted when compared to the right scutum, which is known to be intact. The left middle ear space is entirely filled with a soft tissue lesion, whilst there is only soft tissue in the right epitympanum lateral and superior to the ossicles.&#x0D; &#x0D; Meticulous examination of the ossicles on axial view (Figure 2) showed a subtle thinning of the short process of the incus by sharply marginated, seemingly expansile soft tissue medially located between the incus and the tympanic segment of the facial nerve.&#x0D; Exploratory surgery via an initial external end-aural approach revealed the presence of a posterior pars tensa retraction cholesteatoma underneath the granulation polyp. The cholesteatoma had extended medial to the ossicular chain, causing erosion of the long process and medial portion of the short process of the incus, as well as the entire stapes superstructure. Definitive surgery consisting of a canal-wall down mastoidectomy via a post-auricular approach, cartilage graft tympanoplasty without ossicular chain reconstruction, meatoplasty and partial mastoid obliteration was performed.&#x0D; This case is particularly instructive with regards to two issues: otologic disease in Down syndrome and radiological evidence of erosive middle ear disease. An increased incidence of otologic conditions in Down syndrome is well established in the medical literature. These include external auditory canal stenosis, ossicular chain abnormalities and otitis media with effusion (OME).1,2 Cholesteatoma as a sequelae of undiagnosed or untreated OME has to be suspected in children with Down syndrome, especially in those with recurrent otorrhea and persistent hearing loss. Unfortunately, the identification of a cholesteatoma may be difficult due to stenosis of the external auditory canal or a sub-optimal otologic examination due to behavioral problems in children with Down syndrome.2 These factors were both present in this particular case, as the cholesteatoma remained undiagnosed for several years despite regular consultations with an otolaryngologist.&#x0D; Radiologic evaluation with high-resolution computerized tomographic (HRCT) imaging is extremely important in these situations. As described by Barath et al., the “typical findings associated with cholesteatoma include a sharply marginated expansile soft-tissue lesion, retraction of the tympanic membrane, scutum blunting, and erosion of the tympanic tegmen and ossicles. Holotympanic absence of bony changes is suggestive of otitis media without cholesteatoma formation, whereas presence of bony erosions (along with clinical suspicion) indicates cholesteatoma.”3 In this particular case, the presence of soft tissue within the epitympanum and antrum accompanied by the subtle evidence of ossicular erosion were crucial in the decision to advise and perform surgery. Although it may be argued that a high clinical suspicion based on the suggestive otological history in a child with Down syndrome may be enough to warrant surgical exploration, it cannot be disputed that the radiological findings help in advising patients pre-operatively about the indications for and expected outcomes of surgical management. In this case, it also impacted on the surgical approach – a transmeatal procedure appropriate for a limited middle ear exploration was initially performed, with conversion to a standard post-auricular approach appropriate for more extensive mastoid surgery once the presence and extent of the cholesteatomatous disease was confirmed intra-operatively.&#x0D;

Neurobrucellosis Presenting as Acute Psychosis

A 45-year-old female without any past or family history of psychiatric illness presented to the emergency department with complaints of abnormal behavior, irrelevant talking, restlessness, episodic crying, and decreased sleep of 2-day duration. On detailed interview, the attendants gave a history of an intermittent headache of 6-month duration and hearing impairment of 4-month duration. On investigation, her cerebrospinal fluid was reactive, and brucella titers were positive. She received appropriate treatment for 6 months and a short course of antipsychotics. Her symptoms settled, but she had persistent hearing loss. Psychosis as well as hearing loss is a very rare presentation of brucellosis. The case highlights the importance of considering neurobrucellosis as a differential diagnosis in patients with any unexplained neuropsychiatric symptoms such as acute psychosis or hearing loss.