Ergebnisse der mit der Obliteration des runden/ovalen Fensters erreichten Behandlungserfolge bei der einseitigen, akut aufgetretenen, idiopathischen sensorineuralen Hörminderung

ZusammenfassungFormerly explorative tympanotomies including sealing of the round/oval window/s have only been used in treating perilymphatic fistulas. During the last years this operation especially in the middle European countries has also been performed more and more in patients suffering from an ISSNHL. In this chapter the effect of this operation including a simultaneous systematic steroid therapy on hearing levels has been collected from data presented in literature and the results will be presented by means of a systematic review. The survey also includes the results of this operative procedure in 41 patients who have been treated by us.Based on 19 publications dealing with the above mentioned topic only 6 studies (4 of them with information about the hearing loss at the time of admission at the hospital and additionally at a follow-up examination (n = 309)) could be integrated in this study and another 2 in which the hearing gains –regardless of the initial hearing loss- according to the modified „Kanzaki“-criteria (n = 288)) have been evaluated- could be used for estimating the therapeutic effects of tympanoscopy with sealing of the round/oval windosw/s in patients with sudden deafness.The follow-up examination had to exceed a 3 weeks interval up from the time of the operation. It is shown that several methods of evaluation the median hearing loss (PTA4 and PTA5) in pure tone audiograms are suitable to be accepted for the analysis of hearing losses in order to compare the results of the treatment.In each study-group collected from literature and two study-groups of our own patients the hearing loss before therapy was significantly higher than that which had been measured at the follow-up examination.Patients in which tympanoscopy was combined with a systematic application of steroids in a „first-line-mode“ (n = 79) had a hearing loss of 94,1 dB. Their hearing improved up to a hearing level of 70,7 dB at the follow-up examination (hearing gain: 23,4 dB; n = 79). Those who first had got systematic steroids followed by the tympanoscopy („second-line-mode“) had an intitial hearing loss of 105,1 dB and a hearing gain of 38,6 dB. So the final hearing level reached 66,5 dB (n = 197).Median hearing thresholds still rose after detamponation of the external ear canal which was usually done 10 days after the operation up to the follow-up examination.Based on the modified „Kanzaki“-criteria 58,7 % of the patients had a restoration in their hearing exceeding 30 dB. In 18,7 % of the cases the hearing improvement was between 10 and 30 dB. 23,1 % of the patients didn`t profit from tympanoscopy.Indications for deciding to perform an explorative tymapnosocopy which are mentioned in literature are collected and discussed.Explorative tympanotomy including sealing of the round/oval window/s seems to be a very valuable method in treating sudden deafness especially when the hearing loss was severe, profound or even when the patients were totally deaf and the therapeutic procedure had been done sequentially after having performed a systematic steroid application before operation. Further studies have to find out if tympanoscopy used in a „first-line-mode“ can also be a method in which the hearing gain is successful enough that this form of therapy can also be recommended.

Long-term efficacy of vestibular neurotomy in disabling Ménière’s disease and Tumarkin drop attacks

OBJECTIVE When Ménière’s disease (MD) becomes disabling due to the frequency of attacks or the appearance of drop attacks (i.e., Tumarkin otolithic crisis) despite "conservative" medical and surgical treatments, a radical treatment like vestibular neurotomy (VN) is possible. An ideal MD treatment would relieve symptoms immediately and persist after the therapy. The aim of this study was to identify if VN was effective after 10 years of follow-up regarding vertigo and drop attacks, and to collect the immediate complications. METHODS The authors report a retrospective, single-center (i.e., in a single tertiary referral center with otoneurological surgery activity) cohort study conducted from January 2003 to April 2020. All patients with unilateral disabling MD who had received a VN with at least 10 years of follow-up were included. The therapeutic efficacy was defined by complete disappearance of vertigo and drop attacks. The postoperative complications (CSF leak, total deafness, meningitis, death) were determined immediately after the surgery, and the hearing thresholds were determined during the patient follow-up with the pure tone average (PTA). RESULTS A total of 74 patients (of 85 who were eligible), average age 51.9 ± 11.1 years, including 38 men (51.4%), with disabling MD and/or Tumarkin drop attacks (24.3%) received VN, with at least 10 years of follow-up after surgery. After an average follow-up of 12.4 ± 1.7 years (range 10.0–16.3 years), 67 patients (90.5%) no longer presented any vertiginous attacks, and no patient experienced drop attack. The mean variation in early pre- and postoperative PTA was not statistically significant (n = 64, 2.2 ± 10.3 decibels hearing level [range −18 to 29], 95% CI [−0.4 to 4.37]; p = 0.096), and 84.4% of the patients evaluated had unchanged or improved postoperative PTA. Three significant complications were noted, including two surgical revisions for CSF leak. There was no permanent facial paralysis, meningitis, or death. CONCLUSIONS In case of disabling MD (disabling vertigo refractory to conservative vestibular treatments—Tumarkin drop attacks), VN via the retrosigmoid approach must be the prioritized proposal in comparison to intratympanic gentamicin injections, because of the extremely low complication rate and the immediate and long-lasting effect of this treatment on vertigo and falls.

Change in Self-Evaluation of Hearing Status According to Age: A Population-Based Study

Background and Objectives Self-reported hearing levels determined by self-report questionnaires are not always correlated with the audiometric hearing level. It is necessary to ascertain the subjectively perceived normal hearing threshold to determine the need for hearing aids at the appropriate time. The objective is to identify the level of correlation between the self-reported hearing level and the audiometric hearing level and ascertain the subjectively perceived normal hearing threshold for each age group.Subjects and Method This cross-sectional study, using the data from the Korea National Health and Nutrition Examination Survey V, looked at participants with hearing loss who were aged ≥40 years. Pure tone audiometry was conducted bilaterally. Self-reported hearing level was ascertained via survey enquiring about hearing loss. The level of correlation between self-reported hearing level and audiometric hearing level, age-specific patterns of hear-ing self-evaluation and subjectively perceived normal hearing values at which participants complained of hearing loss were main outcomes.Results Mean audiometric thresholds (MATs) increased with the age and degree of self-reported hearing level. The accuracy of self-reported hearing level compared to the audiometric threshold decreased with age both in the better-ear and worse-ear groups. Younger participants tended to overestimate their hearing impairment, while older participants tended to underestimate it. Subjectively perceived normal hearing thresholds increased with age and they were very close to the MATs for each age group.Conclusion Results can be useful for devising different age-group specific pure tone audi-ometry-based diagnostic criteria for self-reported hearing loss, which can be used to determine the need for hearing aid in a timely manner.

Whispered Hearing Analyzer — a device for preventive examinations to detect early hearing loss

Introduction. The problem of hearing loss and deafness, due to the general prevalence of hearing loss in different age groups, is significant not only for medicine, but also for the social sphere of society. Nowadays, just as 40 years ago, preventive medical examinations and initial examinations by an otorhinolaryngologist use methods of testing hearing by means of whispered speech, which has its own characteristics for everyone. This leads to diagnostic errors and does not allow to detect hearing loss in its early stages. Purpose of the study—development of a whispered-speech apparatus for early express diagnosis of hearing loss, and study of the effectiveness of the developed apparatus. Materials and methods. The Department of Hygiene at the Izhevsk State Medical Academy, as part of the program "Umnic-2018" a whispered hearing analyzer — a device for early express diagnosis of hearing loss was developed. The device was tested on the basis of otorhinolaryngologist offices in Izhevsk and Tchaikovsky. The study group included 53 patients aged 19 to 76. As part of the testing of the device, the subjects underwent whispered speech hearing tests using acumetric Voyacek word tables; express diagnostics of the hearing level with the whispered hearing analyzer and tonal threshold audiometry using the ITERA II diagnostic audiometer (Otometrics, Denmark). Results. As a result of the testing of the developed device, we found that the use of the whispered hearing analyzer allows you to reduce the time of examination and increase its diagnostic efficiency compared to conventional methods of hearing level examination at medical check-ups. Discussion. The Whispering Hearing Analyzer is designed to quickly and accurately detect hearing loss during medical examinations. The results obtained with this instrument can only tell you that you have a hearing loss, but it is not possible to make a diagnosis. Conclusion. The problem of disabling hearing loss can be solved through preventive measures, one of which is the developed device for early express diagnosis of hearing loss. The use of this device eliminates the occurrence of diagnostic errors associated with the researcher's speech characteristics, thereby making it possible to detect hearing loss at an early stage.

Cochlear Implantation Outcomes in Children With CDH23 Mutations–Associated Hearing Loss

Objective Mutations in the cadherin 23 gene ( CDH23) have been reported to cause cochlear damage, but few studies have investigated the auditory and speech outcome of patients after cochlear implantation. Here, we describe the genetic, auditory, and postoperative outcomes of patients with CDH23 mutations who received cochlear implants. Study Design Retrospective case review. Setting Tertiary referral center. Methods Targeted deafness-related gene panels were sequenced in Chinese families with profound sensorineural hearing loss. The clinical features of subjects carrying potentially pathogenic CDH23 mutations were analyzed. Results Between 2017 and 2019, we identified 5 children with prelinguistically profound hearing loss at our center who harbored 6 variants of CDH23 that segregated with the disease. Of these, 4 variants were novel (c.2591G>T, c.4785G>C, c.5765A>G, and c.9280_9281insTT). All affected individuals had a loss of outer hair cell function, with an average residual hearing level of 3 to 10 dB SPL. Cochlear implantations were arranged for the patients at 11 to 36 months of age. All children made gains in their hearing, language, and speech performances 14 to 120 months after surgery. Their auditory outcomes improved during follow-up intervals. Conclusion This study revealed that children with congenital cochlear defects caused by CDH23 variants can acquire an acceptable auditory and speech outcome after cochlear implantation. Early genetic detection and prenatal counseling for rare deafness genes such as CDH23 remain a priority for the future.

Hearing loss in Norwegian adults with achondroplasia

Background Achondroplasia is the most common form of disproportionate skeletal dysplasia. The condition is caused by a mutation in the FGFR3 gene, affecting endochondral bone growth, including the craniofacial anatomy. Recurrent otitis media infections, chronic middle ear effusion, and hearing loss are common in children with achondroplasia, but few studies have investigated hearing loss in adults with this condition. Objectives This population-based study investigated the prevalence, severity, and type of hearing loss in Norwegian adults with achondroplasia. Methods We collected data on 45 adults with genetically confirmed achondroplasia: 23 men and 22 women, aged 16–70 years. All participants underwent a comprehensive audiologic assessment, including medical history, pure-tone audiometry, speech audiometry, and impedance audiometry. According to the Global Burden of Disease classification, pure-tone average ≥ 20 decibel hearing level (dB HL) was considered clinically significant hearing loss. Results Insertion of ventilation tubes had been performed in 44% (20/45) of the participants, 49% (22/45) had a history of adenoidectomy, while 20% (9/45) used hearing aids. Hearing loss in at least one ear was found in 53% (24/45) of the participants; in 57% (13/23) of the men and 50% (11/22) of the women. In the youngest age group (age 16–44 years), 50% (14/28) had hearing loss, although predominantly mild (20–34 dB HL). An abnormal tympanometry (Type B or C) was found in 71% (32/45) of the participants. The majority (15/24) had conductive hearing loss, or a combination of conductive and sensorineural hearing loss (8/24). Conclusions Adults with achondroplasia are at increased risk of early hearing loss. Our findings underline the importance of a regular hearing assessment being part of standard care in achondroplasia, including adolescents and young adults. In adult patients diagnosed with hearing loss, an evaluation by an otolaryngologist should be considered, and the need for hearing aids, assistive listening devices, and workplace and educational accommodations should be discussed. Clinical trial registration ClinicalTrials.gov identifier NCT03780153.

The Roles Played by Blood Inflammatory Parameters in Sudden Sensorineural Hearing Loss

Objective: The etiology of sudden sensorineural hearing loss (SSNHL) remains unknown; inflammation may be one cause. We retrospectively studied SSHNL cases in terms of the inflammatory parameters involved. Methods: We reviewed 169 SSNHL cases. The control group contained 132 patients with vocal cord polyps. The C-reactive protein (CRP) level, CRP/serum albumin (Alb) ratio, and neutrophil/lymphocyte ratio (NLR) were compared between the 2 groups. The relationships between these parameters and the time to treatment after SSHNL onset, hearing level, and therapeutic effects were also analyzed. Results: The CRP level, CRP/Alb ratio, and NLR were higher in SSNHL patients than in controls, but only the NLR differed significantly between the 2 groups. We found no significant differences between patients with different hearing levels, those evaluated at various times after SSNHL onset, and those with different outcomes. Conclusion: The NLR is a reliable SSNHL diagnostic marker. The CRP level and CRP/Alb ratio are not useful indicators.

Serum Albumin Levels as a Potential Marker for the Predictive and Prognostic Factor in Sudden Sensorineural Hearing Loss: A Prospective Cohort Study

Objectives: As a common otology emergency, sudden sensorineural hearing loss (SSNHL) has a great impact on quality of life. The diagnosis and treatment of SSNHL remain challenging. This study aims to identify and investigate the association of liver functions with SSNHL.Methods: A total of 135 SSNHL patients and 135 sex- and age-matched controls were prospectively enrolled. The baseline and clinical characteristics, along with liver function levels of participators, were collected. Linear correlation, logistic regression, and receiving operator characteristic curve analysis tests were applied to examine the association between liver function levels and SSNHL.Results: There were no differences in baseline characteristics between SSNHL and control groups. The albumin (ALB) level of the SSNHL group was significantly lower than that in the control group (p < 0.001). The logistic regression showed that the low ALB level may be a predictive factor of SSNHL with an adjusted OR of 0.809 (95% CI, 0.742–0.882, p < 0.001). By comparing the indicators of different prognosis groups, we found that the effective group had a significantly lower hearing impair onset and higher ALB (p = 0.001) and AGR (p = 0.003) levels than the ineffective group. Logistic regression revealed that the hearing level onset (OR, 0.976; 95% CI, 0.956–0.997; p = 0.026) and ALB level (OR, 1.181; 95% CI, 1.071–1.301; p = 0.001) showed strong associations with treatment outcome.Conclusions: Lower ALB levels, within the normal ranges, were associated with the occurrence and unfavorable outcome of SSNHL. However, further research on the underlying mechanisms needs to be conducted.

The correlation between glycosylated haemoglobin levels with pure tone average values in type 2 diabetes mellitus patients

Hearing plays an important role in our daily activities, if it is impaired or lost the quality of life is affected by improper communication. The diabetes mellitus is a chronic metabolic disorder and affects all the systems in the body including auditory system. So assessment of hearing is important in diabetics to know the type, extent of hearing impairment which helps to take treatment decisions and preventive measures. Present study is focused on relationship between glycosylated haemoglobin (HbA1c) levels with pure tone average (PTA) values in type 2 diabetics. Type 2 diabetic patients with (WHI) and without (WoHI) hearing impairment, both the gender was included with age limit between 35-55 years and also ten normal subjects were included as controls. Pure tone average (PTA) is the average of hearing threshold levels at different frequencies; it gives information about an individual’s hearing level in each ear. All the data were expressed as mean ± SE. The mean were analyzed by one way ANOVA. Pearson correlation test was done to see the relationship between right and left ear PTA values in normal subjects, WoHI and WHI groups with HbA1c values. In both the groups pure tone average values are not statistically correlated with HbA1c. The left ear of both WoHI (P=0.977) and WHI (P=0.490) groups shows negative correlation with HbA1c. The HbA1c values are correlated with PTA values in all subjects. The pure tone audiometry is helpful to know the status of hearing sensitivity and type, severity of the hearing impairment in the T2DM patients in relation to HbA1c levels.

Facilitative Language Techniques Used in the Home by Caregivers of Young Children Who Are Deaf or Hard of Hearing

Purpose This research note reports preliminary data from an investigation of facilitative language techniques (FLTs) used in the natural environment by caregivers of children who are deaf or hard of hearing (DHH). The investigation seeks to establish a new method to collect and analyze data on caregiver FLT use in the home. Method This pilot investigation included two children under the age of 36 months with moderate-to-profound sensorineural hearing loss. Both children were consistent users of hearing devices and were pursing oral communication. Data were collected via the Language ENvironment Analysis (LENA) system in the participants' homes. Thirty-six 5-min segments containing the highest adult word count were extracted from each participant's sample. Researchers coded segments for the presence or absence of 10 FLTs within 30-s intervals. Results The collection, coding, and analysis of caregiver FLTs using LENA was a feasible method to investigate caregiver linguistic input in the natural environment. Despite differences in age, sex, and hearing level, the distribution of caregiver FLTs was similar for both participants. Caregivers used high levels of narration, closed-ended questions, and directives throughout the day. Conclusions Results of this investigation provide information about the types of FLTs that are used in the home by caregivers of young children who are DHH. Furthermore, results indicate the feasibility of this method to investigate in-home use of caregiver FLTs.