Update on congenital stapes footplate fixation and juvenile otosclerosis.

Juvenile otosclerosis and congenital stapes footplate fixation. A systematic review and meta-analysis of surgical outcomes and management.

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Active middle ear implants, such as the Vibrant Soundbridge, are used as an important part in the rehabilitation of sensorineural, conductive hearing, or mixed hearing loss. The attachment of the Vibrant Soundbridge at the round window and the usage of the Vibroplasty couplers strongly expanded the application of the Vibrant Soundbridge.The Vibrant Soundbridge is developed for patients who have an intolerance to hearing aids and a moderate to profound sensorineural hearing loss. The VSB also provides an optimal solution for patients with failed middle ear reconstructions or patients with atresia. To capture the improvement with the VSB Implant with different hearing losses a literature analysis was conducted. The functional gain was analyzed for 107 patients with conductive hearing loss and for 214 patients with sensorineural hearing loss out of 14 studies.Patients with conductive and mixed hearing loss resulted in a functional gain from 30 to 58 dB with the VSB. Patients with a pure sensorineural hearing loss showed a functional gain of 23-30 dB. The VSB bone conduction threshold shift was analyzed for all studies conducted in the years between 2000 and 2009. In 11 of the 16 studies there was no significant (p=0.05) change found. In 5 studies, the pre- to post-surgical bone conduction threshold shift was less than 10 dB. None of these studies measured a threshold shift of more than 10 dB.The flexible attachment at either the long process of the incus with sensorineural hearing loss, with an conductive hearing loss at the round window or the use of Vibroplasty couplers at the oval window, head of the stapes or round window makes the VSB an extremely versatile instrument. If patients can't wear conventional hearing aids, had failed middle ear reconstructions or atresia the VSB presents, due to the significant hearing improvement in any type of hearing loss, an ideal solution.

We describe a dominant Japanese patient with progressive conductive hearing loss who was diagnosed with NOG-related symphalangism spectrum disorder (NOG-SSD), a spectrum of congenital stapes fixation syndromes caused by NOG mutations. Based on the clinical features, including proximal symphalangism, conductive hearing loss, hyper­opia, and short, broad middle, and distal phalanges of the thumbs, his family was diagnosed with stapes ankylosis with broad thumbs and toes syndrome (SABTT). Genetic analysis revealed a heterozygous substitution in the NOG gene, c.645C>A, p.C215* in affected family individuals. He had normal hearing on auditory brainstem response (ABR) testing at ages 9 months and 1 and 2 years. He was followed up to evaluate the hearing level because of his family history of hearing loss caused by SABTT. Follow-up pure tone average testing revealed the development of progressive conductive hearing loss. Stapes surgery was performed, and his post-operative hearing threshold improved to normal in both ears. According to hearing test results, the stapes ankylosis in our SABTT patient seemed to be incomplete at birth and progressive in early childhood. The ABR results in our patient indicated the possibility that newborn hearing screening may not detect conductive hearing loss in patients with NOG-SSD. Hence, children with a family history and/or known congenital joint abnormality should undergo periodic hearing tests due to possible progressive hearing loss. Because of high success rates of stapes surgeries in cases of SABTT, early surgical interventions would help minimise the negative effect of hearing loss during school age. Identification of the nature of conductive hearing loss due to progressive stapes ankylosis allows for better genetic counselling and proper intervention in NOG-SSD patients.

Background: Pediatric otosclerosis is characterized by progressive conductive hearing loss with a relatively low incidence, compared to adults. The treatment approaches range from conservative options, such as hearing aids, to surgical managements including stapedectomy and stapedotomy. Aim: To compare hearing outcomes (air-bone gap<10 dB) after stapedectomy vs. stapedotomy in patients with juvenile otosclerosis. Methods: We conducted a systematic search in Google scholar, PubMed, and Scopus. Studies reporting the outcomes of stapedectomy and/or stapedotomy and those specifically defining the mixed data from data of each procedure for the patients under the age of 18 years old with juvenile sclerosis were included. On the other hand, post-operative air-bone gap was extracted. There was no time limitation for search of studies. Results: After evaluating all studies, post-operative air-bone gap below 10dB ranged from 66% to 91% of cases in stapedectomy group and from 66% to 92% in stapedotomy group. Conclusion:Based on the reviewed studies, we found similar success rates in hearing outcome of the patients with juvenile otosclerosis following stapedotomy and stapedectomy.

Otosclerosis in childhood and adolescence or juvenile otosclerosis is a rare disorder resulting in conductive hearing loss. A 9-year-old boy presented to our clinic, suffering from moderate hearing loss. According...

A otospongiose é uma osteodistrofia do osso temporal caracterizada pela reabsorção e neoformação óssea desordenada. Clinicamente, caracteriza-se por disacusia condutiva, neurossensorial, e/ou mista, progressiva e zumbidos. O início dos sintomas ocorre entre 30 e 40 anos de idade sendo rara sua manifestação na infância. Descrevemos o caso de um paciente de 11 anos de idade, com quadro de hipoacusia unilateral progressiva há 5 anos. O exame otorrinolaringológico revelou mancha rubra de Schwartze em orelha esquerda. A audiometria, imitanciometria e a tomografia computadorizada demonstraram características sugestivas de otospongiose. Realizamos uma revisão dos aspectos clínicos, diagnósticos e da conduta terapêutica da otospongiose na infância.

SummaryOtospongiosis is an osteodystrophy of the temporal bone, characterized by disordered neoformation and deposition of bone, characterized by the presence of a progressive conductive, sensorineural or mixed hearing loss and tinnitus. Typically, otospongiosis presents as a slowly progressive conductive hearing loss in the third to fourth decade of life. Uncommonly children and adolescents may also have conductive or sensorineural hearing loss caused by otosclerosis. We describe a case of an 11-year-old patient, with progressive unilateral conductive hearing loss for 5 years. The otoscopic examination revealed a positive Schwartz's sign in the left ear. Audiometry, impedanciometry and CT scan showed characteristics that suggested otospongiosis. We reviewed clinical aspects, diagnosis and the therapeutic approach for otospongiosis in children.

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On the basis of strong research, universal newborn screening should be performed before age 1 month with repeat or follow-up testing for those who do not pass performed before age 3 months and intervention started before age 6 months. On the basis of strong research and consensus statement, tympanostomy tubes should be considered for individuals with bilateral persistent middle ear effusion for 3 months or greater and a documented conductive hearing loss. On the basis of consensus statement, all children with suspected hearing loss should have an age appropriate hearing test. On the basis of strong research, the most common form of congenital hearing loss is genetic. Most of this is nonsyndromic hearing loss.

Three aspects of brainstem response audiometry were investigated in the present study. (1) The brainstem response threshold was compared with the pure-tone audiogram in 40 patients with conductive hearing loss. The brainstem response threshold has a one-to-one relationship with the mean of the pure-tone thresholds at 2 and 4 kHz. The correlation coefficient in this comparison is 0.84 and the standard error of the estimate is 8.3 dB. Taking into account corresponding results in cochlear hearing loss [Drift et al.: Audiology 26: 1-10, 1987] it is concluded that the brainstem response threshold provides a good estimate of the amount of peripheral hearing loss, independent of the type of hearing loss. (2) It was shown [Drift et al.: Audiology 27: 260-270, 1988] that different types of peripheral hearing loss can be distinguished reliably with brainstem response audiometry. Parameters relevant for this distinction were the horizontal shift of the latency-level curve (1(L) curve), that of its derivative and the response threshold. In the clinical situation measurement of the response threshold is not always possible due to restlessness of the patient. To simulate this situation we randomly truncated the lower parts of the 1(L) curves of quiet patients. The test group consisted of 22 adult normally hearing subjects, 79 patients with cochlear hearing loss, 40 with conductive hearing loss and 22 with mixed hearing loss. Linear discriminant analysis was applied to the horizontal shift of the 1(L) curve and of its derivative. The brainstem diagnosis 'normal hearing' correctly excludes a conductive hearing loss in 98% of the cases and the brainstem diagnosis 'cochlear hearing loss' does so in 79%. The brainstem diagnosis 'conductive hearing loss' correctly predicts a conductive component of hearing loss in 94% of the cases and the brainstem diagnosis 'mixed hearing loss' does so in 90%. The distinction between cochlear hearing loss and normal hearing is not reliable, neither is the distinction between conductive and mixed hearing loss. (3) The amount of the conductive component of hearing loss can be estimated by the horizontal shift of the 1(L) curve. Statistical comparison with the mean of the air-bone gaps at 2 and 4 kHz gave a correlation coefficient of 0.77, a standard error of the estimate of 9.7 dB, and a slope of the regression line of 0.93. An overestimation of about 7 dB has to be taken into account in case of mixed hearing loss.

Characterization of hearing loss in pediatric patients with osteogenesis imperfecta

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Psoriatic Involvement of the Ear

The International Outcome Inventory for Hearing Aids (IOI-HA) is a seven-item hearing-specific questionnaire. It was developed with the purpose of evaluating the efficacy of hearing aid rehabilitation. Few psychometric properties have been presented for a Swedish translation of the IOI-HA. Furthermore, previous studies have examined the IOI-HA in mainly sensorineural hearing losses, and we do not know how the type of hearing loss affects the outcome. To evaluate the hearing aid fitting outcome measured in a clinical setting using a Swedish translation of the International Outcome Inventory for Hearing Aids (IOI-HA), to determine the psychometric properties of the translation, and to examine how a number of demographic variables such as type of hearing loss affect the outcome. A descriptive and correlational study in a retrospective sample. Two hundred and twenty-four (107 females and 117 males; ages 27-94 yr with an average of 66.1 yr) first-time hearing aid users. Mostly digital hearing aids (97.8%) were fitted monaurally (60%) or binaurally (40%) between 2007 and 2009. The subjects were mailed the IOI-HA questionnaire six months after their final appointment, and the completed questionnaire was returned by mail to the clinic. The psychometric properties were evaluated and compared to previous studies using the IOI-HA. The associations between the outcome scores and a number of demographic variables (age, gender, degree of hearing loss, type of hearing loss, number of hearing aids, and type of hearing aids) were examined. Based on the pure tone audiograms, the subjects were divided into three groups; those with conductive hearing losses, sensorineural hearing losses, and mixed hearing losses. For these groups, the differences in outcome measured as IOI-HA were examined. The psychometric properties of the present translation of the IOI-HA showed resemblance in many aspects to previous reports. Furthermore, the type of hearing loss seems to affect the IOI-HA outcome. Hearing loss increases with increasing age, and hearing aid use increases with increasing degree of hearing loss. Subjects with sensorineural hearing losses show significantly poorer scores on items concerning introspective aspects of the outcome in comparison to subjects with mixed hearing losses and subjects with conductive hearing losses. Monaurally fitted subjects tend to report lower scores on average, but monaural or binaural hearing aid fitting do not significantly affect the subjective outcome. The psychometric properties of the present Swedish translation of the IOI-HA show resemblance in many aspects to previous reports, but the differences observed could be due to differences in the study populations. Overall, the demographic variables examined could not be used as predictors for the hearing aid fitting outcome, and more reliable predictors need to be identified.